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The univentricular repair indications, procedures, outcomes and controversies
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The univentricular repair indications, procedures, outcomes and controversies

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  • Heterotaxy syndromes refer to disorders of lateralization whereby the arrangement of abdominal and thoracic viscera differ from normal and mirror-image of normal. hypoplastic left heart syndrome alone, the most common form of univentricular heart, a crude median incidence of 2.3 cases per 10 000 live births was derived when data was pooled from 36 studies. Tricuspid atresia, the second most common subtype of univentricular heart, is thought to occur less than once for every 10 000 live births10 and was present in 2.9% and 1.4% of congenital heart disease autopsy and clinical series, respectively.10 DILV comprises 1% of all congenital heart malformations.
  • As a rule of thumb, values _85% and _75% signify increased and decreased pulmonary blood flow, respectively. Univentricular Heart Paul Khairy, MD, PhD et al.Circulation.2007 In the largest series of unoperated patients (n_83), Moodie et al reported that 70% with wellformed single left ventricles died before age 16, with an annual attrition rate of 4.8%.57 The natural history is even bleaker for patients with univentricular hearts of right ventricular morphology, with 50% survival 4 years after diagnosis. 57 The most common causes of mortality were arrhythmias, congestive heart failure, and sudden unexplained death. Thus, despite the overall grim prognosis in unoperated patients, some adults with DILV, transposition of the great arteries, and well-balanced circulations may survive into their seventh decade with acceptable functional capacity and preserved ventricular function.
  • Allocate and preserve the single ventricle to pump blood to a high pressure systemic circulation. With this in mind, the systemic venous blood volume should go directly to the PA so that we don’t overload the single ventricle. For this venous return to work without the aid of a strong pump or ventricle, we have to preserve a low resistance pulmonary circulation. So, eventually we will end up with a free flowing systemic venous return to the PA without going to the ventricle, and have a good functioning ventricle to circulate blood to the systemic arteries. This is the Fontan circulation.
  • The recommendation is to have a staged operation especially if the patient presents at a very young age. The type of palliation would depend on the physiology and the age of the patient . General objectives of initial surgical palliation are to provide unobstructed systemic outflow, unobstructed systemic and pulmonary venous return, and controlled pulmonary blood flow . In patients with severe pulmonary obstruction or atresia, this is currently accomplished by an aortopulmonary shunt, such as a modified Blalock-Taussig shunt, or bidirectional cavopulmonary anastomosis (Glenn shunt).
  • In the presence of pulmonary overcirculation or unrestricted pulmonary blood flow, the neonate should have a procedure to somehow restrict this PBF because the high PBF can steal blood from the systemic circulation, flood the lungs and cause congestion and later pulmonary hypertension. We simply place a band around the pulmonary artery via a sternotomy approach, rarely a thoracotomy.
  • The fact that this shunt could induce mild pulmonary vascular disease, ventricular dysfunction, or pulmonary artery distortion was not the first preoccupation of the surgeon, as it is now. The success of a shunt is currently evaluated by an acceptable relief of cyanosis without a significant volume overload, by its induction of pulmonary growth without an impairing effect on the pulmonary vasculature, even not during growth after Fontan repair, and by allowing the patient to reach an age at which a Fontan circuit can be safely created.
  • Fontan Operation After 3 Decades What We Have Learned Welton M. Gersony, MDCirculation 2008 Because there is no ventricular contraction to pump blood through the lungs, elevated pulmonary artery pressure is an absolute contraindication for the Fontan procedure. Therefore, because of the normal high pulmonary vascular resistance in newborns, it is not possible to create a Fontan circulation at birth.
  • For children with aortic arch obstruction and univentricular cardiac anatomy, surgical management is most commonly accomplished by an initial Norwood operation, with either systemic arterial or ventricular source of pulmonary blood flow, which is a parallel arrangement. That is followed some months later by conversion to a superior cavopulmonary source of pulmonary blood flow, resulting in the desired series circulation
  • It is of utmost imporrtance to prepare thses babies prior to a NW/Sano/hybrid. Again, the aim is to balance the pulmonary and systemic circulation in such a way that the pulmonary blood flow will not severely steal from the systemic perfusion. We are able to do this mostly by maintaining patency of the duct with prostaglandin, and maintaining a reasonable PAP.
  • For children with aortic arch obstruction and univentricular cardiac anatomy, surgical management is most commonly accomplished by an initial Norwood operation, with either systemic arterial or ventricular source of pulmonary blood flow, which is a parallel arrangement. That is followed some months later by conversion to a superior cavopulmonary source of pulmonary blood flow, resulting in the desired series circulation. Norwood stage 1 procedure, performed within the first 2 weeks of life, are to provide unobstructed pulmonary venous return, permanent systemic outflow from the right ventricle, and temporary pulmonary blood supply to allow the pulmonary vasculature to develop and mature. The main pulmonary artery is divided, the proximal portion is anastomosed to the ascending aorta, the aortic arch is repaired and augmented, and pulmonary blood flow is maintained via a modified Blalock-Taussig shunt
  • For children with aortic arch obstruction and univentricular cardiac anatomy, surgical management is most commonly accomplished by an initial Norwood operation, with either systemic arterial or ventricular source of pulmonary blood flow, which is a parallel arrangement. That is followed some months later by conversion to a superior cavopulmonary source of pulmonary blood flow, resulting in the desired series circulation
  • Does the shunt type determine mid-term outcome after Norwood operation? † Joachim Photiadis a , Nicodème Sinzobahamvya a , Christoph Haun b , Martin Schneider c , Peter Zartner c , Ehrenfried Schindler d , Boulos Asfour a and Viktor Hraška a + Author Affiliations a Department of Paediatric Cardio-Thoracic Surgery, German Paediatric Heart Centre (‘Deutsches Kinderherzzentrum’), Asklepios Clinic, Sankt Augustin, Germany b Department of Cardiac Intensive Care, German Paediatric Heart Centre (‘Deutsches Kinderherzzentrum’), Asklepios Clinic, Sankt Augustin, Germany Accepted November 15, 2011. This study reviews 109 neonates undergoing the Norwood procedure in the same interval between October 2002 and December 2009. The Sano (38) or BT shunt (71) was assigned according to the surgeon's preference. Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation (13/34, P  = 0.004), and ACS ≥20 (15/39, P  = 0.001), but not shunt type (8/37, P  = 0.95). Increased number of shunt-related interventions before the Glenn procedure were noted with Sano (32.4 versus 6.5%, P  = 0.002). Increased number of shunt-related interventions before the Glenn procedure were noted with Sano (32.4 versus 6.5%, P  = 0.002). CONCLUSIONS Preoperative risk factors, regardless of shunt type, influence midterm survival after the Norwood procedure with an excellent outcome in low-risk patients, while high-risk cases still incur a significant mortality. Sano shunt interventions occurred with increased numbers. Although, Sano shunt may be the only feasible option in some instances, given the possible negative effects of ventriculotomy on right ventricle function, the widespread use of Sano shunt should be reconsidered.
  • Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart George Ballard, Shane Tibby, Owen Miller, Thomas Krasemann, Eric Rosenthal, David Anderson, Conal Austin, Shakeel Qureshi, and John Simpson* European Journal of Echocardiography (2010) 11, 870–874 The hybrid procedure has been developed, which maintains systemic blood flow by stenting of the arterial duct and limits pulmonary blood flow by the application of bilateral branch pulmonary artery bands.11,18 This provides an opportunity to relieve critical left heart obstruction, while leaving options open with regard to the final type of repair. . Our preferred route for patients with aortic stenosis with a borderline left ventricle is a hybrid operation, leaving the atrial septum intact, unless it is critically restrictive, whereas some institutions may prefer a Norwood operation, leaving the atrial septum intact. Conclusions Our data, although retrospective, are encouraging that the use of the hybrid procedure may lead to achieving a biventricular circulation in a higher proportion of patients than was thought possible previously and we use a serial application of the aortic discriminant score to assist the decision-making progress.
  • Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart George Ballard, Shane Tibby, Owen Miller, Thomas Krasemann, Eric Rosenthal, David Anderson, Conal Austin, Shakeel Qureshi, and John Simpson* European Journal of Echocardiography (2010) 11, 870–874 The hybrid procedure has been developed, which maintains systemic blood flow by stenting of the arterial duct and limits pulmonary blood flow by the application of bilateral branch pulmonary artery bands.11,18 This provides an opportunity to relieve critical left heart obstruction, while leaving options open with regard to the final type of repair. . Our preferred route for patients with aortic stenosis with a borderline left ventricle is a hybrid operation, leaving the atrial septum intact, unless it is critically restrictive, whereas some institutions may prefer a Norwood operation, leaving the atrial septum intact. Conclusions Our data, although retrospective, are encouraging that the use of the hybrid procedure may lead to achieving a biventricular circulation in a higher proportion of patients than was thought possible previously and we use a serial application of the aortic discriminant score to assist the decision-making progress.
  • Bidirectional Glenn and Antegrade Pulmonary Blood Flow: Temporary or Definitive Palliation? Davide F. Calvaruso, MD, Antonio Rubino, MD, Salvatore Ocello, MD, Nicoletta Salviato, MD, Diego Guardì, MD, David F. Petruccelli, MD, Adriano Cipriani, MD, Khalil Fattouch, MD, PhD, Salvatore Agati, MD, Carmelo Mignosa, MD, Lucio Zannini, MD, and Carlo F. Marcelletti, MD Department of Pediatric Cardiac Surgery “Marta e Milagros,” Azienda di Rilievo Nazionale e di Alta Specializzazione, Ospedale Civico, Palermo; Department of Cardiac Surgery, University of Palermo, Palermo; Department of Pediatric Cardiac Surgery, Ospedale S. Vincenzo, Taormina (ME); and Department of Pediatric Cardiac Surgery, Istituto Gaslini, Ospedale Pediatrico, Istituto di Ricovero e Cura a Carattere Scientifico, Genova, Italy Ann Thorac Surg 2008;85:1389 –96)
  • The second stage palliative procedure consists of a bidirectional Glenn shunt or hemi-Fontan procedure and is usually undertaken as soon as the pulmonary arteries have grown sufficiently to allow a low PVR, usually between 2–6 months After surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not perfused by some unidentified factor produced by the liver.2
  • Decreased chance of pulmo hypertension and good oxyegn saturatio with less return flow to the heart by getting rid of the BTS as soon as possible. Also you will have to prepare the heart for a totally separate pulmonary and systemic circulations, hence, this stage. Single-Stage Versus Two-Stage Modified Fontan Procedure Cardiovascular & Thoracic Unit Ramathibodi Hospital, Mahidol University Bangkok, Thailand
  • The second stage palliative procedure consists of a bidirectional Glenn shunt or hemi-Fontan procedure and is usually undertaken as soon as the pulmonary arteries have grown sufficiently to allow a low PVR, usually between 2–6 months After surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not perfused by some unidentified factor produced by the liver.2
  • The second stage palliative procedure consists of a bidirectional Glenn shunt or hemi-Fontan procedure and is usually undertaken as soon as the pulmonary arteries have grown sufficiently to allow a low PVR, usually between 2–6 months After surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not perfused by some unidentified factor produced by the liver.2
  • The second stage palliative procedure consists of a bidirectional Glenn shunt or hemi-Fontan procedure and is usually undertaken as soon as the pulmonary arteries have grown sufficiently to allow a low PVR, usually between 2–6 months After surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not perfused by some unidentified factor produced by the liver.2
  • The second stage palliative procedure consists of a bidirectional Glenn shunt or hemi-Fontan procedure and is usually undertaken as soon as the pulmonary arteries have grown sufficiently to allow a low PVR, usually between 2–6 months After surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not perfused by some unidentified factor produced by the liver.2
  • The second stage palliative procedure consists of a bidirectional Glenn shunt or hemi-Fontan procedure and is usually undertaken as soon as the pulmonary arteries have grown sufficiently to allow a low PVR, usually between 2–6 months After surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not perfused by some unidentified factor produced by the liver.2
  • .A hemi-Fontan procedure consists of an end-to-side anastomosis between the SVC, which is divided from the right atrium, and the right pulmonary artery. The right pulmonary artery is not divided, resulting in blood flow from the SVC into the right and left pulmonary arteries. Children who undergo a hemi-Fontan procedure may remain cyanotic because blood from the IVC is not directed to the lungs (3). The hemi-Fontan procedure also includes a second component, in which the cardiac end of the divided SVC is attached to the main pulmonary artery or the under surface of the right pulmonary artery. The open end of the SVC is either oversewn or occluded with a polytetrafluoroethylene patch, a surgical approach that allows total cavopulmonary Fontan circulation to be completed at a later time. To complete Fontan circulation, the lower stump of the SVC is connected to the IVC with a conduit
  • .A hemi-Fontan procedure consists of an end-to-side anastomosis between the SVC, which is divided from the right atrium, and the right pulmonary artery. The right pulmonary artery is not divided, resulting in blood flow from the SVC into the right and left pulmonary arteries. Children who undergo a hemi-Fontan procedure may remain cyanotic because blood from the IVC is not directed to the lungs (3). The hemi-Fontan procedure also includes a second component, in which the cardiac end of the divided SVC is attached to the main pulmonary artery or the under surface of the right pulmonary artery. The open end of the SVC is either oversewn or occluded with a polytetrafluoroethylene patch, a surgical approach that allows total cavopulmonary Fontan circulation to be completed at a later time. To complete Fontan circulation, the lower stump of the SVC is connected to the IVC with a conduit
  • HemiFontan: The superior vena cava–right atrial orifice was closed within the right atrium by using a circular patch of 0.6 mm Gore-Tex (W. L. Gore &Assoc, Flagstaff, AZ) polytetrafluoroethylene (PTFE). If a left superior vena cava was present, it was routinely anastomosed to the left pulmonary artery.
  • The hemi- Fontan creates a large cavopulmonary connection that facilitates augmentation of the branch pulmonary arteries if needed and simplifies completion of the inferior vena cava to pulmonary artery pathway at the time of the completion Fontan. Based on computational fluid dynamic modeling, the hemi-Fontan procedure followed by the LCT Fontan minimizes energy losses and optimizes the distribution of inferior vena cava flow into both lungs Lateral Tunnel Fontan in the Current Era: Is It Still a Good Option? John W. Brown, MD, Mark Ruzmetov, MD, PhD, Benjamin W. Deschner, BS, Mark D. Rodefeld, MD, and Mark W. Turrentine, MD Section of Cardiothoracic Surgery, Indiana University School of Medicine, Indianapolis, Indiana Ann ThoracSurg 2010;89:556-563
  • Because there is no ventricular contraction to pump blood through the lungs, elevated pulmonary artery pressure is an absolute contraindication for the Fontan procedure. Therefore, because of the normal high pulmonary vascular resistance in newborns, it is not possible to create a Fontan circulation at birth.
  • The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure Tyler B. Fredenburg, MD, Tiffanie R. Johnson, MD and Mervyn D. Cohen, MBChB Ann Thorac Surg 2005;80:665-72 the original Fontan procedure, in which the SVC was connected to the right pulmonary artery (RPA) and the right atrium to the pulmonary artery. LPA left pulmonary artery. modified Fontan procedure, with the connection of the right atrium to the pulmonary artery. LPA left pulmonary artery, RPA right pulmonary artery. Because these connections subjected the right atrium into dilatation, the atrium would fail. Prof. de leval recognized this and he studied the flow dynamics of the fontan circulation. He then introduced the lateral tunnel modification in 1988. In 1990, the extracardiac modification and fenestration also surfaced.
  • shows the intraatrial method of creating cavopulmonary Fontan circulation, in which an intraatrial conduit connects the IVC to the right pulmonary artery (RPA). AA ascending aorta, LPA left pulmonary artery. the extraatrial method of creating cavopulmonary Fontan circulation, in which an extraatrial conduit connects the IVC to the right pulmonary artery (RPA). AA ascending aorta, LPA left pulmonary artery. In the intraatrial tunnel method, the conduit is constructed with both the lateral wall of the right atrium and prosthetic material (Fig 7). The inferior aspect of the tunnel is anastomosed to the IVC and the superior aspect is anastomosed to the pulmonary arteries. A benefit of using this circuit is that the conduit enlarges as the child grows; thus, it may be used in children as young as 1 year old. However, use of an internal conduit may lead to atrial arrhythmia (3). The extracardiac conduit method usually is performed only in patients older than 3 years. In this method, a polytetrafluoroethylene tube graft is placed between the transected IVC and the pulmonary artery, bypassing the right atrium (Fig 8). The entire atrium is left with low pressure, which leads to less atrial distention, arrhythmia, and thrombosis. However, this conduit cannot enlarge as the patient grows, and the procedure should be performed only in patients who are large enough to accept a graft of adequate size to allow adult IVC blood flow (3). The fenestration functions as a “pop-off” valve (a right-to-left shunt) to prevent rapid volume overload to the lungs, limit caval pressure, increase preload to the systemic ventricle, and increase cardiac output; however, cyanosis may result from the right-to-left shunt.
  • the LTF involves placement of an intra-atrial Gore-Tex or pericardial baffle. Although excellent midterm and long-term results of the LTF have been documented, potential risks includes atrial distension, postoperative arrhythmias, and the need for cardiopulmonary bypass with ischemic arrest or ventricular fibrillation to construct the intra-atrial baffle[2, 8, 9]. The ECF, introduced in 1990 [7], has several potential advantages, including the preservation of normal atrial pressure for the entire atrium, the absence of extensive intraatrial suture lines, possible avoidance of cardiopulmonary bypass (assuming no intracardiac repairs are needed), and the feasibility of early or late fenestration without the use of the extracorporeal bypass. However, this procedure has potential disadvantages related to using the extracardiac prosthetic tube, including the lack of growth potential, the delay of completing the Fontan to an older patient age, potential conduit stenosis secondary to intimal peel, and an increased risk of thromboembolism with the need for urgent reoperation.
  • Contemporary Fontan Operation: Association Between Early Outcome and Type of Cavopulmonary Connection Robert D. Stewart, MD, MPHa,*, Sara K. Pasquali, MD, MHSb,c, Jeffrey P. Jacobs, MDd, Daniel K. Benjamin, PhDc, James Jaggers, MDe, Julie Cheng, MAa, Constantine Mavroudis, MDa, Marshall L. Jacobs, MDa,* aPediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, Ohio b Division of Cardiology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina d Congenital Heart Institute of Florida, All Children's Hospital, and Children's Hospital of Tampa, St. Petersburg and Tampa, Florida e Department of Surgery, University of Colorado School of Medicine, Denver, Colorado Accepted for publication January 3, 2012. 2,747 patients
  • Pulmonary Artery Size and Late Functional Outcome After Fontan Operation Jae Suk Baek, MD, Eun Jung Bae, MD, PhD, Gi Beom Kim, MD, Woong-Han Kim, MD, PhD, Jeong Ryul Lee, MD, PhD, Yong Jin Kim, MD, PhD, Eun-Ah Park, MD, Whal Lee, MD, PhD, and Chung Il Noh, MD, PhD Departments of Pediatrics, and Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital; and Department of Radiology, Seoul National University Hospital, Seoul, South Korea (Ann Thorac Surg 2011;91:1240 –7) The sizes of both left and right pulmonary arteries were assessed at the hilum by cardiac CT, and the PA index (PAI) was calculated as described by Nakata and colleagues [3]. We divided our patients into three groups according to the PAI: hypoplastic PA (group I, PAI 180 mm2/m2), relatively small PA (group II, 180 mm2/m2 PAI 250 mm2/m2), and good PA (group III, PAI 250 mm2/m2).. 120 Fontan patients. ). Their mean (SD) age was 19.3 5.6 years (range, 9.2 to 37.2 years), and the mean (SD) age at their initial Fontan procedure was 3.9 3.1 years (range, 0.8 to 17.6 years). The mean (SD) elapsed time since the initial Fontan operation was 12.8 3.7 years (range, 5.1 to 20.4 years). The atriopulmonary connection was performed in 35 patients, lateral tunnel Fontan operation in 65 patients, and extracardiac Fontan operation in 20 patients.Their mean age was 19.3 5.6 years. impedance increased abruptly only when PAI was less than or equal to 100 mm2/m2.
  • Insights After 40 Years of the Fontan Operation Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’? Riad B.M. Hosein a, Andrew J.B. Clarkea, Simon P. McGuirka, Massimo Grisellia, Oliver Stumperb, Joseph V. De Giovannib, David J. Barrona and William J. Brawna,* + Author Affiliations aDepartment of Cardiac Surgery, Birmingham Children’s J ThoracCardiovascSurg 2012;143:904-909
  • Insights After 40 Years of the Fontan Operation World Journal for Pediatric and Congenital Heart Surgery 2010. B) Factors influencing catch-up growth Masamichi Ono, MD,a Dietmar Boethig, MD,b Heidi Goerler, MD,a Melanie Lange, MS,a Mechthild Westhoff-Bleck, MD,c and Thomas Breymann, MDa J Thorac Cardiovasc Surg 2007;134:1199-206 it is the volume-unloading operation (BDG shunt or single-stage Fontan operation) and not completion of the Fontan operation that results in the improved hemodynamic status and permits improved growth and suggest that a staged approach to completion of the Fontan operation is important because BDG shunting can be performed in infancy.
  • Insights After 40 Years of the Fontan Operation World Journal for Pediatric and Congenital Heart Surgery 2010. B) Factors influencing catch-up growth Masamichi Ono, MD,a Dietmar Boethig, MD,b Heidi Goerler, MD,a Melanie Lange, MS,a Mechthild Westhoff-Bleck, MD,c and Thomas Breymann, MDa J Thorac Cardiovasc Surg 2007;134:1199-206 it is the volume-unloading operation (BDG shunt or single-stage Fontan operation) and not completion of the Fontan operation that results in the improved hemodynamic status and permits improved growth and suggest that a staged approach to completion of the Fontan operation is important because BDG shunting can be performed in infancy.
  • Center and University of Utah, SaltLake City, Utah; cDepartment of Pediatrics, Children’s Hospital Boston, Boston, Massachusetts; dNew England Research Institutes, Watertown, Massachusetts; eNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland; fDepartment of Pediatrics, Medical University of South Carolina, Charleston, South Carolina; gDepartment of Pediatrics, Duke University Medical Center, Durham, North Carolina; hDepartment of Pediatrics, University of Toronto and Hospital for Sick Children, Toronto, Canada; and iDepartment of Pediatrics, Columbia University Medical Center, New York, New York B) The Fontan Procedure Our Odyssey Continues* Carl Lewis Backer, MD, FACC Chicago, Illinois .jJ. Am. Coll. Cardiol. 2008;52;114-116 In many respects, considering the single ventricle physiology of these patients, they are doing surprisingly well. The Pediatric Heart Network multicenter study of 546 Fontan patients concluded, calthough these patients do remarkably well, they do not keep up with age-matched control subjects in exercise ability.
  • Contemporary Outcomes After the Fontan Procedure A Pediatric Heart Network Multicenter Study Page A. W. Anderson, MD,* Lynn A. Sleeper, SCD,† Lynn Mahony, MD,‡ Steven D. Colan, MD J Am CollCardiol 2008;52:85–98 Durham, North Carolina; Watertown and Boston, Massachusetts; Dallas, Texas; Charleston, South Carolina; Children who have undergone a Fontan procedure for palliation of a functional single ventricle are at risk for medical complications. A total of 1,078 subjects from 7 centers in the U.S. and Canada were screened; 644 were study eligible, and 546 were enrolled. Functional Health Status includes physical and psychosocial status , exercise tolerance,
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA Protein losing enteropathy is characterized by excessive loss of proteins from serum into the intestinal lumen, possibly due to impedance to drainage of the thoracic duct by high superior caval venous pressure together with mesenteric vascular inflammation.
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA
  • The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure Tyler B. Fredenburg, MD, Tiffanie R. Johnson, MD and Mervyn D. Cohen, MBChB Ann Thorac Surg 2005;80:665-72 Surgical ligation of the thoracic duct may cure plastic bronchitis by decreasing intrathoracic lymphatic pressure and flow
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure Tyler B. Fredenburg, MD, Tiffanie R. Johnson, MD and Mervyn D. Cohen, MBChB Ann Thorac Surg 2005;80:66 Right to left shunting via fenestration and drainage of coronary sinus Left to right shunting may occur through aorta-pulmonary collaterals or incomplete occlusion of previous artificial shunts.
  • THE FONTAN CIRCULATION Marc Gewillig Heart 2005;The ventricle thus evolves from being volume overloaded and overstretched, to overgrown and (severely) underloaded. It should therefore not be a surprise that the deprived ventricle in a Fontan circuit shows systolic and diastolic dysfunction. The ventricle may now enter a vicious cycle whereby the low preload results in remodelling, reduced compliance, poor ventricular filling, and eventually continuously declining cardiac output. This phenomenon of progressive ‘‘disuse hypofunction’’ occurs at a chronic preload of less than 70% of the ‘‘due’’ preload.9 Our knowledge of low flow conditions, chronic congestion, and limited ventricular preload is, however, still very limited.
  • The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure Tyler B. Fredenburg, MD, Tiffanie R. Johnson, MD and Mervyn D. Cohen, MBChB Ann Thorac Surg 2005;80:665-72 Total bypass of the right side of the heart and completion of the total cavopulmonary circuit result in a marked reduction of preload to the systemic ventricle. Chronic preload depletion perpetuates systolic and diastolic dysfunction of the ventricle, resulting in impaired compliance, poor ventricular filling, and eventually low cardiac output. Tyler B. Fredenburg, Mervyn D. Cohen, MBChB Ann Thorac Surg 2005 Surgical revision should be considered in patients with failing Fontan circulations; experienced centers report combined perioperative cardiac transplantation and mortality rates of 2.4% to 6.7%. Univentricular Heart Paul Khairy,
  • The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure Tyler B. Fredenburg, MD, Tiffanie R. Johnson, MD and Mervyn D. Cohen, MBChB Ann Thorac Surg 2005;80:665-72
  • Palliation by BCPS with APBF should be achieved early in life. Do we need fenestration when performing two-staged total cavopulmonary connection using an extracardiac conduit? Yorikazu Harada*, Shunji Uchita, Takahiko Sakamoto, Mitsuhiro Kimura, Kentaro Umezu,Kiyohiro Takigiku, Satoshi Yasukouchi Department of Cardiovascular Surgery and Cardiology, Nagano Children’s Hospital, 3100 Toyoshina, Azumino, Nagano 399-8288, Japan Interactive CardioVascular and Thoracic Surgery 9 (2009) 50–55
  • Variable 1-Stage 2-Stage p -value Deaths 4/15 (26.6%) 0/13 (0%) 0.044 . 2-stage procedure for 13 high-risk patients and younger children (< 4 years old). The age range at operation was 4–21 years in the 1-stage group, and 3–11 years in the 2-stage group. Body weights ranged from 11 to 54 kg in the 1-stage group and 13 to 31 kg in the 2-stage group. Bangkok, Thailand followed up on a regular basis (median duration, 60 months) . We believe in the advantages of fenestration for adaptation during the initial stage of Fontan circulation, and routinely perform a 5-mm fenestration in all patients at least 7 years old. It was found that none needed closure of the fenestration, and systemic oxygen saturation was more than 95% in all cases. We believe the prior bidirectional Glenn shunt procedure will provide favorable outcomes, particularly reduced cyanosis, preserved myocardial function due to decreased volume overload in the univentricular heart, and avoidance of atrioventricular valve regurgitation. 2 had central venous pressure > 30 mm Hg, and 2 had pulmonary edema from signifi cant
  • Intermediate-term outcome following the fontan operation: a survival, functional and risk-factor analysis ☆ Nelson Alphonso*, Max Baghai, PremSundar, Robert Tulloh, Conal Austin and David Anderson Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas Hospitals NHS Trust, St Thomas Street, London SE1 9RT, UK ↵ ☆ Presented at the joint 17th Annual Meeting of the European Associationfor Cardio-thoracic Surgery and the 11th Annual Meeting of the EuropeanSociety of Thoracic Surgeons, Vienna, Austria, October 12-15, 2003. Accepted June 28, 2005. Over a median follow up of 54 months (1–133), Univariate analysis identified older age at operation (≫4 years) ( P =0.04), higher postoperative pulmonary artery pressure at 24h ( P =0.012), arrhythmia postoperatively ( P =0.03) or during follow-up ( P =0.01) and the requirement for anticoagulation during follow-up ( P =0.03) as significant predictors of poorer outcome. Patients who had an intermediate BCPS ( P =0.002) or Norwood Stage 1 ( P =0.05) had a better outcome.
  • The intensive care of infants with hypoplastic left heart syndrome U Theilen, L Shekerdemian . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Arch Dis Child Fetal Neonatal Ed 2005Prenatal diagnosis has allowed for more careful planning of delivery and immediate postnatal care of infants with HLAlthough the reported impact of prenatal diagnosis on overall survival is variable, infants diagnosed prenatally are certainly less likely to experience the sequelae of pulmonary overcirculation or acidosis. HS.
  • Midterm to Long-Term Outcome of Total Cavopulmonary Connection in High-Risk Adult Candidates Yasuhiro Fujii, MD, Shunji Sano, MDAnn Thorac Surg 2009 The TCPC procedure carries a greater risk for the adult patient than for children because the adult functional ventricle usually presents with complications caused by long-term chronic hypoxia, ventricular volume overload, and increased venous pressure, such as arrhythmia, protein-losing enteropathy (PLE), pleural effusion, ventricular dysfunction, and limited exercise capacity. 1 early death from cardiac tamponade due to bleeding after removal of the temporary pacing wire 1 late death due to heart failure. The patient with a late death was a 31-year-old man who was diagnosed as having doubleoutlet right ventricle, common atrioventicular valve canal, pulmonary stenosis, and asplenia, and had previously undergone a right original Blalock-Taussig shunt. The patient had six risk factors: age, asplenia, pulmonary vascular resistance (3.8 wood units), NYHA class III, low ejection fraction (35%), and arrhythmia (paroxysmal atrial fibrillation). The patient was referred to our institute for a TCPC completion . Although we selected a staged operation because of his low cardiac function, his arterial blood oxygen pressure was less than 40 mm Hg after the BDG procedure, and his exercise tolerance did not improve. Hence, we performed a fenestrated extracardiac TCPC without aortic cross-clamp 2 months after the BDG procedure. In the early postoperative period, although the paroxysmal atrial fibrillation was diminished and the ventricular ejection fraction was improved to 50%, his NYHA status remained class III in the early postoperative period. Hence, synchronized biventricular pacing was applied because he had morphologically bilateral ventricles, however, it was not effective to improve his symptoms. Although he tolerated the operation well and was discharged from the hospital, he died of heart failure 9 months after the TCPC.
  • In conclusion, TCPC could be offered to high-risk adult Fontan candidates, with an acceptable mortality rate. Both the TCPC completion and the TCPC conversion improved the patients’ arrhythmia and NYHA functional class. Most of the adult TCPC candidates tolerated the TCPC during the early postoperative period. However, the accumulation of risk factors may influence their late mortality. To improve the late clinical outcome for this patient population, early TCPC completion or conversion is therefore required before complications occur.
  • Impact of Age at Fontan Completion on Postoperative Hemodynamics and Long-Term Aerobic Exercise Capacity in Patients With Dominant Left Ventricle Shuichi Shiraishi, MD, ToshikatsuYagihara, MD, Koji Kagisaki, MD, IkuoHagino, MD, Hideo Ohuchi, MD, Junjiro Kobayashi, MD, and Soichiro Kitamura, MD Departments of Cardiovascular Surgery and Pediatrics, National Cardiovascular Center, Osaka, Japan
  • The Fontan Procedure Our Odyssey Continues* Carl Lewis Backer, MD, FACC Chicago, Illinois .jJ. Am. Coll. Cardiol. 2008;52;114-116
  • The Fontan Procedure Our Odyssey Continues* Carl Lewis Backer, MD, FACC Chicago, Illinois .jJ. Am. Coll. Cardiol. 2008;52;114-116
  • Fenestration in the Fontan circulation, first described in 1990 by Bridges et al. w1x, allows for decrease in venous pressure in the circuit and augmentation of cardiac output, which is believed to be advantageous especially soon after the operation.
  • The Fontan Procedure Our Odyssey Continues* Carl Lewis Backer, MD, FACC Chicago, Illinois .jJ. Am. Coll. Cardiol. 2008;52;114-116 a finding “at odds” with the commonly held belief that improving arterial O2 saturation will improve exercise tolerance.
  • Current approaches to treatment of patients with single-ventricle physiology do not adversely affect.
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA Although women can successfully complete pregnancy with a Fontan circulation, the associated physiological haemodynamic changes have a significant influence on their abnormal heart function. Even by 20–28 weeks gestation, myocardial oxygen consumption and heart rate normally increase by 20% and stroke volume by 40%, the latter related to a physiological increase in blood volume. This fluid retention may result in atrial distension,
  • Heart 2006;92:1290-1294 doi:10.1136/hrt.2005.085407 Pregnancy and delivery in women after Fontan palliation W Drenthen, P G Pieper, J W Roos-Hesselink, W A van Lottum, A A Voors, 1Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands B) Univentricular Heart Paul Khairy, MD, PhD. Circulation 2007
  • Heart 2006;92:1290-1294 doi:10.1136/hrt.2005.085407 Pregnancy and delivery in women after Fontan palliation W Drenthen, P G Pieper, J W Roos-Hesselink, W A van Lottum, A A Voors, 1Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands B) Univentricular Heart Paul Khairy, MD, PhD. Circulation 2007
  • The Fontan operation remains a palliative procedure, but for patients born with a single ventricle it is a dramatic improvement and a true milestone in the history of pediatric cardiac surgery. Modifications of the Fontan operation,2 staging with a bidirectional cavopulmonary shunt (ie, the bidirectional Glenn [BDG] shunt),3 and creation of fenestration in the Fontan pathway4 have reduced the mortality and morbidity associated with this operation.
  • We have our work cut out for us. Personally, having experienced the perioperative situations in the more affluent societies of the west, I can say that we can be at par with our international colleagues in the treatment of physiologically single ventricles if only we can help them whilst they are good risk candidates. Right now we can already see a clear picture of the current trends in managing these patients.
  • We happily accept the challenge of looking after them.
  • We have taken small and big steps in looking after these patients especially those with left-dominat ventricles.
  • We are learning the ropes
  • And slowly but surely we have been getting good short term results
  • We have taken the plunge with out-stretched arms
  • And we survived and so did our patients.
  • Fontan Operation After 3 Decades What We Have Learned Welton M. Gersony, MDCirculation 2008 Fontan’s visionary operation and its modifications over the ensuing decades have re-established nonturbulent flow and substantially reduced cyanosis for patients with severe hypoplasia of one ventricle. This procedure was subsequently known as Fontan anastomosis, and it has been applied to repair several congenital cardiac malformations. Both the lateral tunnel and extracardiac conduit modificationsof the Fontan procedure are the current standards for right heart bypass. .Creation of Fontan circulation is palliative by nature. To accomplish physiologic correction of blood flow, Fontan and colleagues initially used a Glenn shunt, in which the right pulmonary artery was connected to the superior vena cava (SVC) and the SVC–atrial junction was ligated. A valved conduit connection between the right atrium or right atrial appendage and the left pulmonary artery was then created with an aortic homograft.
  • Fontan Operation After 3 Decades What We Have Learned Welton M. Gersony, MDCirculation 2008 Fontan’s visionary operation and its modifications over the ensuing decades have re-established nonturbulent flow and substantially reduced cyanosis for patients with severe hypoplasia of one ventricle. This procedure was subsequently known as Fontan anastomosis, and it has been applied to repair several congenital cardiac malformations. Both the lateral tunnel and extracardiac conduit modificationsof the Fontan procedure are the current standards for right heart bypass. .Creation of Fontan circulation is palliative by nature. To accomplish physiologic correction of blood flow, Fontan and colleagues initially used a Glenn shunt, in which the right pulmonary artery was connected to the superior vena cava (SVC) and the SVC–atrial junction was ligated. A valved conduit connection between the right atrium or right atrial appendage and the left pulmonary artery was then created with an aortic homograft.
  • Cardiac Magnetic Resonance Versus Routine Cardiac Catheterization Before Bidirectional Glenn Anastomosis in Infants With Functional Single Ventricle A Prospective Randomized Trial David W. Brown, MD; Kimberlee Gauvreau, ScD; Andrew J. Powell, MD; Peter Lang, MD; Steven D. Colan, MD; Pedro J. del Nido, MD; Kirsten C. Odegard, MD; Tal Geva, MD Circulation . 2007;116:2718-2725.) Routine preoperative catheterization is standard practice in patients with single-ventricle physiology before bidirectional Glenn anastomosis. The main goals of the cardiac cath. procedure include assessment of anatomic and hemodynamic suitability for surgery and catheterbased interventions such as balloon dilation of aortic coarctation. However, cardiac catheterization is associated with morbidity, especially in the high-risk group of infants with singleventricle physiology,3–7 and exposure to ionizing radiation in childhood has been linked to increased risk of subsequent cancer. Patients with pulmonary vein stenosis, pulmonary hypertension, severe ventricular dysfunction, severe atrioventricular valvar regurgitation, known large aortopulmonary or venous collateral vessels, or coarctation of the aorta (defined as _50% narrowing of the aortic isthmus relative to the descending aorta or _3 m/s peak Doppler flow velocity) were excluded because these conditions are generally considered to warrant catheterization for hemodynamic assessment or transcatheter intervention. Although echocardiography provides sufficient diagnostic imaging in many patients with singleventricle physiology,9,10 the results of this trial illustrate the additional utility of CMR, especially in imaging extracardiac anatomy in patients with suboptimal acoustic windows or other technical limitations. Another implication of our study is that in the absence of evidence of pulmonary hypertension, routine measurement of pulmonary vascular resistance is not necessary before bidirectional Glenn operation. Indeed, the range of resistance values found in the catheterization group was within acceptable limits, and patients in the CMR group did not fare differently after bidirectional Glenn operation as a result of the absence of these data.
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA
  • Contemporary Outcomes After the Fontan Procedure A Pediatric Heart Network Multicenter Study Page A. W. Anderson, MD,* Lynn A. Sleeper, SCD,† Lynn Mahony, MD,‡ Steven D. Colan, MD J Am CollCardiol 2008;52:85–98 Durham, North Carolina; Watertown and Boston, Massachusetts; Dallas, Texas; Charleston, South Carolina; Children who have undergone a Fontan procedure for palliation of a functional single ventricle are at risk for medical complications. A total of 1,078 subjects from 7 centers in the U.S. and Canada were screened; 644 were study eligible, and 546 were enrolled. Functional Health Status includes physical and psychosocial status , exercise tolerance, although these patients do remarkably well, they do not keep up with age-matched control subjects in exercise ability. Smaller EDV, as compared with normal subjects, might be a reflection of aerobic deconditioning and might contribute to the blunted ability to increase stroke volume with exercise . Our finding of abnormal diastolic function in 72% of children who had undergone a Fontan has not been previously reported and is concerning. These indexes are dependent on cardiac loading conditions and are unable to distinguish between enhanced chamber compliance and impaired relaxation
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA
  • The Fontan circulation SandeepNayak MBBS MD FRCA PD Booker MBBS MD FRCA The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure Tyler B. Fredenburg, MD, Tiffanie R. Johnson, MD and Mervyn D. Cohen, MBChB Ann Thorac Surg 2005;80:66 Right to left shunting via fenestration and drainage of coronary sinus Left to right shunting may occur through aorta-pulmonary collaterals or incomplete occlusion of previous artificial shunts.
  • The Fontan circulation: who controls cardiac output? Marc Gewilliga,*, Stephen C. Brownb, BenedicteEyskensa, Ruth Heyinga, Javier Ganamea, Werner Budtsa, Andre La Gerchea, Matthias Gorenfloa aPaediatric and Congenital Cardiology, University Hospitals Leuven, Belgium bPaediatric Cardiology, University of the Free State, South Africa Received 11 August 2009; received in revised form 12 November 2009; accepted 17 November 2009 2010 Published by European Association for Cardio-Thoracic Surgery. However, such drugs will fail to achieve a comparable increase of output in a Fontan circulation with limited preload: the ventricle will squeeze harder but not much more! . No matter how good the pump, it can only pump out what comes in, both at rest and during exercise. Transpulmonary flow is determined by the transpulmonary gradient and transpulmonary resistance. PVR appears to be the major determinant of cardiac output in postoperative Fontan patients.
  • Is morbidity influenced by staging in the fontan palliation? A singlecenter review. Department of Congenital Cardiac Surgery, University Hospital Gent, Belgium. Contemporary Outcomes After the Fontan Procedure A Pediatric Heart Network Multicenter Study Page A. W. Anderson, MD,* Lynn A. Sleeper, SCD,† Lynn Mahony, MD,‡ Steven D. Colan, MD J Am CollCardiol 2008;52:85–98 Durham, North Carolina; Watertown and Boston, Massachusetts; Dallas, Texas; Charleston, South Carolina; Children who have undergone a Fontan procedure for palliation of a functional single ventricle are at risk for medical complications. A total of 1,078 subjects from 7 centers in the U.S. and Canada were screened; 644 were study eligible, and 546 were enrolled. Functional Health Status includes physical and psychosocial status , exercise tolerance,
  • Persistent Antegrade Pulmonary Blood Flow Post-Glenn Does Not Alter Early Post-Fontan Outcomes in Single-Ventricle Patients Robert G. Gray, MD, Karen Altmann, MD, Ralph S. Mosca, MD, AshwinPrakash, MD, Ismee A. Williams, MD, MS, Jan M. Quaegebeur, MD, PhD, and Jonathan M. Chen, MD Columbia University College of Physicians and Surgeons, and Joan and Sanford Weill Medical College of Cornell University, New York, New York Ann ThoracSurg 2007;84:888 –93 BDG Physiologic advantages include a reduction in ventricular volume load with improved mechanical efficiency, improved arterial oxygen saturation, and prevention of pulmonary hypertension. Our practice is to base this decision in part upon the pre-BDG PA pressure, the size of the native PAs, and any technical difficulty anticipated for its removal at the time of eventual Fontan. In situations in which the resistance is believed to be low and the PA pressure is 20 mm Hg or more, we routinely eliminate antegrade flow. disadvantage of subjecting the upper body and brain to high venous pressure, which in turn can lead to a low transcranial pressure gradient and neurologic damage. And the superior vena cava syndrome came mostly when you had a pulsatile pressure in the superior vena cava, let’s say, of 28, 30 over 15. And then if you just release the azygos vein, everything came out pretty well. expect 150% to 200% of the volume load on the ventricle. So I think we should keep in mind that we are overloading these ventricles. Of course, if they are not candidate for Fontan, this is the compromise.
  • The bidirectional Glenn operation: A risk factor analysis for morbidity and mortality Brian E. Kogon, MD,a Courtney Plattner, BA,a Traci Leong, PhD,b Janet Simsic, MD,c Paul M. Kirshbom, MD,a and Kirk R. Kanter, MDa
  • Medium-Term Outcomes of Kawashima and Completion Fontan Palliation in Single-Ventricle Heart Disease With Heterotaxy and Interrupted Inferior Vena Cava Shelby Kutty, MD, Michele A. Frommelt, MD, David A. Danford, MD, and James S. Tweddell, MD University of Nebraska/Creighton University Joint Division of Pediatric Cardiology, Children’s Hospital and Medical Center, Omaha, Nebraska; and Divisions of Cardiology and Cardiothoracic Surgery, Departments of Pediatrics and Surgery, Medical College of Wisconsin, and Herma Heart Center, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin Ann ThoracSurg 2010;90:1609-1613 Extracardiac or lateral tunnel connection are the most common methods for completion Fontan after Kawashima, other techniques such as placement of an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC [8] or connection of the hepatic venous flow into the azygous system have been reported with acceptable results [10].
  • Medium-Term Outcomes of Kawashima and Completion Fontan Palliation in Single-Ventricle Heart Disease With Heterotaxy and Interrupted Inferior Vena Cava Shelby Kutty, MD, Michele A. Frommelt, MD, David A. Danford, MD, and James S. Tweddell, MD University of Nebraska/Creighton University Joint Division of Pediatric Cardiology, Children’s Hospital and Medical Center, Omaha, Nebraska; and Divisions of Cardiology and Cardiothoracic Surgery, Departments of Pediatrics and Surgery, Medical College of Wisconsin, and Herma Heart Center, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin Ann ThoracSurg 2010;90:1609-1613 Extracardiac or lateral tunnel connection are the most common methods for completion Fontan after Kawashima, other techniques such as placement of an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC [8] or connection of the hepatic venous flow into the azygous system have been reported with acceptable results [10].

The univentricular repair indications, procedures, outcomes and controversies The univentricular repair indications, procedures, outcomes and controversies Presentation Transcript

  • Karyn P. Luna, M.D.
  •  Updates on the indications, timing and outcome of univentricular repair. Present surgical options. Discuss the controversies regarding the modifications of the Fontan operation.
  •  A heart that lacks two well-developed ventricles: Hypoplastic left heart syndrome Hypoplastic right heart
  •  Tricuspid atresia+/- pulmonary atresia with intact ventricular septum, double inlet left ventricle, double outlet right ventricle, unbalanced atrioventricular septal defects and hypoplastic left heart syndrome.
  •  Generally, the risk to siblings and offspring of affected individuals is 2% to 5% (19% -33% of siblings HLHS) . Clinical manifestations and initial management hinge on the presence or absence of pulmonary and systemic outflow obstruction. Natural history Left ventricle dominant= 70% died before age 16 Right ventricle dominant =50% died before 4 years Fontan operation: 20-year survival rate and freedom from heart transplantation of 80%-82%. Paul Khairy, MD, PhD et al.Circulation. 2008 Staged repair allows progressive adaptation of the heart and lungs and reduces the overall perioperative morbidity and mortality. SandeepNayak,PD Booker MBBS MD FRCA
  •  Create unobstructed systemic outflow, unobstructed systemic and pulmonary venous return Provide balanced pulmonary and systemic circulations. Totally separate the systemic and pulmonary venous return and provides pulmonary blood flow without a ventricular pumping chamber. Effective strategies to preserve pulmonary, ventricular and valvular function. Paul Khairy, MD, PhD et al.Circulation.2007
  •  Balanced circulation: No early intervention The aim of the initial palliation is to provide complete relief of any systemic obstruction, if it exists, and provide pulmonary blood flow just sufficient to allow adequate oxygen delivery to tissues and pulmonary arterial growth. Procedures: Pulmonary Artery banding Modified Blalock-Taussig shunt Modified Norwood 1/Sano repair Hybrid procedure
  • Setting:Pulmonary overcirculation Pulmonary artery banding : Pulmonary blood flow must be minimized to ensure that PVR is kept low and the ventricle does not have an excessive volume load.
  • Setting: Hypoxemia Modified Blalock-Taussig shunt PDA stenting
  • Advantages of mBTS: Improve oxygen saturation Facilitate growth of pulmonary arteriesDisadvantages of mBTS: Ventricular volume overload Increase in pulmonary arterial pressure Distortion of pulmonary arteries
  • The long-term effects of marked single-ventricle preload and inefficient oxygenation via an arterial shunt rarely allowed survival beyond the second or third decade of life. Welton M. Gersony, MD Circulation 2008
  • Setting: HLHS Modified Norwood 1 or Sano repair Hybrid procedure First 2 weeks of life Permanent systemic outflow utilising the right ventricle Temporary pulmonary blood supply to allow the pulmonary vasculature to develop and mature. Up to 20-25% mortality rate
  • U Theilen, L Shekerdemian. Arch Dis Child Fetal Neonatal Ed 2005
  •  Modified Norwood 1 The main pulmonary artery(MPA) is divided Proximal MPA is anastomosed to the ascending aorta Aortic arch is repaired Augmented pulmonary blood flow is maintained via a mBTS
  •  Sano repair: Similar to NW 1 but with RV-PA conduit instead of mBTS. Theoretical advantage of avoidance of aorto-pulmonary runoff, resulting in higher coronary and systemic perfusion pressures and reducing the incidence of ventricular.
  •  Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation and ACS ≥20, but not shunt type. Increased number of shunt-related interventions before the Glenn procedure were noted with Sano. CONCLUSIONS: Preoperative risk factors, regardless of shunt type, influence midterm survival after the Norwood procedure with an excellent outcome in low-risk patients, while high-risk cases still incur a significant mortality. Given the possible negative effects of ventriculotomy on right ventricle function, the widespread use of Sano shunt should be reconsidered. Does the shunt type determine mid-term outcome after Norwood operation? Joachim Photiadisa, et al November 15, 2011.
  • Setting: HLHS The Hybrid Procedure Interventional ductal stenting & surgical bilateral pulmonary artery banding An alternative to the NW in HLHS for high-risk patients This approach has been extended to borderline left heart structures. David Anderson, Conal Austin, Shakeel Qureshi et al. European Journal of Echocardiography (2010)
  •  Hybrid vs NW: Hybrid has lower 1st stage mortality but higher interstage mortality. Possible causes are closing PFO, short stent, myocardial ischemia. = the 2nd stage is a more difficult operation. Pizarro et al. Eur J CTS. 2008Overall, no difference in outcome at 6 months. Pizarro et al Thorac CVS 2010
  •  The advantage of the BDG/HemiFontan over the Blalock-Taussig shunt is to allow an increase of effective pulmonary blood flow without an increase in total pulmonary blood flow and cardiac work. Davide F. Calvaruso, et al Ann Thorac Surg 2008 An interstage mortality of 10–15% Most interstage deaths are sudden and apparently unpredictable. U Theilen, L Shekerdemian. Arch Dis Child Fetal Neonatal Ed 2005
  • Setting: Cyanosis or pulmonary overcirculation or balanced CavoPulmonary Connection Bidirectional Glenn Shunt or Hemi-Fontan operation : Duration of 15 months because after surgery, the patient is at risk of developing intrapulmonary arteriovenous shunts SandeepNayak, PD Booker MBBS MD FRCA
  • Benefits of Cavopulmonaryconnection: Decreased cyanosis Decreased intracardiac volume overloadO MonteinNgodngamthaweesuk et al Asian CardiovascThorac Ann 2007
  • HEMIFONTAN OPERATION
  • HEMIFONTAN OPERATION
  • Procedures Bidirectional Glenn Hemi-FontanCardiopulmonary Bypass +/- +SVC-PA connection + +Transection of SVC + -Repair of TV & PA +/- +
  • Procedures Bidirectional Glenn Hemi-FontanEarly Mortality rate 5%-10% SameIdeal type of Fontan Extracardiac Lateral TunnelNoncompetitive Fontan + +flow patternPost-Fontan Mortality rate 21% 2%-5%
  • Operative mortality rate of BDG/HemiFontan is 5%-7%Freedom from failure at 10 years was 99.2% for theFontan group and 67.4% for the BDG patients. Jenkins KJ.Eur J Cardiothorac Surg 2010
  •  Modified Fontan operation: Total cavopulmonary connection (TCPC) 18 months – 4 yrs. old (or 6-18 months post-Glenn or hemi- fontan)
  • ORIGINAL FONTAN MODIFIED FONTAN
  • LATERAL TUNNEL FONTAN EXTRACARDIAC FONTAN
  • LATERAL TUNNEL EXTRACARDIAC Intra- atrial baffle from IVC  A tube graft from the IVC to SVC to RPA outside the heart.
  • LATERAL TUNNEL EXTRACARDIAC
  • Lateral Tunnel ExtracardiacAge/Weight >/= 2 years old >/= 5 years old (13 kg)CPB + +/-Growth potential + -Exercise tolerance Similar SimilarArrhythmia Similar SimilarThrombosis Low risk High riskEffusions Less prolonged ProlongedEarly mortality 2%-5% Similar20-yr. survival 82% Similar
  •  STS Congenital Heart Surgery Database: 2,747 Fontan operations (2000 to 2009) in 68 centers A right-dominant ventricle 45%. Extracardiac conduit Fontan vs lateral tunnel)was performed in 63%; in all, 65% were fenestrated. In multivariable analysis the extracardiac conduit Fontan was associated with significantly higher Fontan takedown/revision and Fontan failure and longer postoperative hospital stay. Conclusions: The lateral atrial tunnel Fontan may be associated with superior early outcomes. Constantine Mavroudis, Marshall L. Jacobs, et al January 3, 2012.
  •  Age above 4 years Normal ventricular function Adequate pulmonary artery size No distortion of pulmonary arteries from prior shunt surgery Low pulmonary artery pressure (below 15 mmHg) Low pulmonary vascular resistance Normal systemic venous drainage No atrioventricular valve leak Normal heart rhythm No right atrial enlargement
  •  Negative prognostic factors for both early and late outcome: Ventricular function Pulmonary artery pressure William J. Brawn.Circulation. 2001
  •  Three groups of PA sizes using the PA index (PAI) Hypoplastic PA (<180 mm2/m2)=impedance increase especially at 100mm2/m2 Relatively small PA (180-250 mm2/m2) Good PA (250mm2/m2) Jae Suk Baek et al. Ann Thorac Surg 2011
  • ACC/AHA 2008 GUIDELINES GUCH
  •  Recent 15-year survival reports post-Fontan: 85% was recently reported by the Boston Children’s Hospital , USA 82 ± 3% recently reported by Birmingham Children’s Hospital, UK Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Long-term viability after the creation of a Fontan circulatory arrangement is most dependent on maintenance of sinus rhythm, the behavior of the pulmonary vasculature, and the performance characteristics of the ventricle Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010 Riad B.M. Hoseina, William J. Brawn J ThoracCardiovascSurg 2012
  •  Norwood 1 operation (n=9):100% early mortality rate Glenn shunts (n=128): 5.4% mortality rate HemiFontan (n=11): 100% early midterm survival rate BTS to Fontan operation (n=6) : 17% Glenn to Fontan operation (n=9): 25% Modified Fontan operation (n=36): 5.5% mortality rate
  •  A 15 year follow-up on somatic growth after Fontan operation: Body weight and BMI have significantly improved by 1 year after the operation. Height has significantly improved by 2 years postoperatively. Patients with BDG shunts showed significantly better weight and BMI at the time of the Fontan operation compared with those without prior BDG shunts . Fenestration is beneficial suggesting that mild arterial desaturation is well tolerated and permits catch-up growth. The pace of catch-up growth after the Fontan operation is much slower than that after biventricular repair. Masamichi Ono et al. J Thorac Cardiovasc Surg 2007
  •  Oxygen delivery index, at least through their preadolescent years, was normal or near normal to reach a relatively normal height. Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010
  •  Physical and Psychosocial Summary scores of the Child Health Questionnaire Parent Form were within the normal range for over 80% of patients. Carl Lewis Backer.J. Am. Coll. Cardiol. 2008 Greater differences between parent and child-completed scores for the domains of physical functioning and impact of physical limitations. Children may “adapt” better to their situation and rate the impact of the illness to be better. Recommendation: Clinicians should consider both parent and patient viewpoints when counseling patients. Parent- Versus Child-Reported Functional Health Status After the Fontan Procedure Linda M. Lambert et al. Pediatrics2009
  •  Finding of apparently impaired systolic function in the RV subgroup relative to the LV and mixed subgroups is consistent with the general opinion that the structure of the RV is suboptimal for a systemic ventricle. The tricuspid valve is more likely to fail as a systemic AV valve. Page A. W. Anderson, MD, et al. J Am CollCardiol 2008
  •  Arrhythmia occur in 10-40% of patients regardless of the type of Fontan. Cardiac rhythm is important in this circulation: Loss of atrio-ventricular synchronisation will cause an increase of the pulmonary venous atrial pressure and/or a diminished ventricular preload, Both of which are known to have negative effects on a Fontan circuit. Marc Gewilliga et al. European Association for Cardio-Thoracic Surgery 2010. Ventricular tachycardia was detected 15 years post surgery, especially in older patients with older age at Fontan operation. Yuki Nakamura, MD .J Thorac Cardiovasc Surg 2010
  •  Lymphatic circulation may be affected by high venous pressure and impaired thoracic duct drainage. Increased pulmonary lymphatic pressure may result in interstitial pulmonary edema, lymphedema &/or protein losing enteropathy. Protein losing enteropathy is characterized by excessive loss of proteins from serum into the intestinal lumen with mesenteric vascular inflammation. Manifestations include oedema, immunodeficiency, ascites, malabsorption of fat, hypercoagulopathy, hypocalcaemia, and hypomagnesaemia. SandeepNayak , PD Booker MBBS MD FRCA
  •  During a 10-yr follow-up, PLE incidence is about 13%. Poor prognosis (60% 5-yr and 20% 10-yr survival after diagnosis). Treatment: Diet low in salt and high in calories, protein content, and medium chain triglycerides. Diuretics, corticosteroids, heparin, and octreotide (a somatostatin analogue) Fontan fenestration; Fontan takedown or revision; or cardiac transplantation. SandeepNayak , PD Booker MBBS MD FRCA
  •  Plastic bronchitis : less than 1%–2% of patients. Noninflammatory mucinous casts that form in the tracheobronchial tree and obstruct the airway. Related to the increased enteric loss of alpha-1-antitrypsin Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010 Clinical manifestations are dyspnea, cough, wheezing, and expectoration of casts, which may cause asphyxia, cardiac arrest, or death. Medical management is difficult; often require repeat bronchoscopy to remove the thick casts. Treatment: Fontan fenestration; Fontan takedown or revision or transplantation. Tyler B. Fredenburg, Mervyn D. Cohen, MBChB .Ann Thorac Surg 2005
  •  Circulating ‘‘hepatic factors,’’ which are responsible for either stimulating a repressor of pulmonary arteriovenous malformations (PAVMs) or repressing an activator of PAVMs. Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010
  •  The ventricle evolves from being volume overloaded and overstretched, to overgrown and (severely)underloaded. The deprived ventricle in a Fontan circuit shows systolic and diastolic dysfunction. The ventricle may now enter a vicious cycle whereby the low preload results in remodelling, reduced compliance, poor ventricular filling, and eventually continuously declining cardiac output. This phenomenon of progressive ‘‘disuse hypofunction’’ occurs at a chronic preload of less than 70% of the ‘‘due’’ preload. Marc Gewillig. Heart 2005
  • Freedom from failure at 10 years was 99.2% for theFontan group and 67.4% for the BDG patients. Jenkins KJ.Eur J Cardiothorac Surg 2010The mean age at the time of failure symptoms 17.2 ± 6.3years.The indications for transplantation was protein-losingenteropathy, arrhythmia with ventricular dysfunction ,and heart failure Paolo Ferrazzi. Ann Thorac Surg 2001
  •  Staged vs. Primary Fontan operation (or total cavopulmonary connection) Antenatal diagnosis Age Fenestrated vs. non-fenestrated Fontan Arrhythmia Anticoagulation Cognitive, psychosocial and somatic development Pregnancy
  •  SVC and IVC to PA connections originally were performed at the same time, which in many patients resulted in a marked increase in blood flow to the lungs, pulmonary lymphatic congestion, and prolonged problems due to pleural effusions. Currently, surgeons create total cavopulmonary Fontan circulation in at least two stages to allow the patients body to adapt to the different hemodynamic states and reduce overall surgical morbidity and mortality. Tyler B. Fredenburg,Mervyn D. Cohen, MBChB. Ann Thorac Surg 2005
  •  Better outcome for staged TCPC because of the gradual ventricular unloading and remodelling. An excessive acute volume load reduction gives rise to a fatal afterload mismatch. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn/ hemi-Fontan procedure. Doty . Int J Cardiol,2006 Advantages of staging towards Fontan operation are almost universally accepted. Yorikazu Harada et al. Interactive CardioVascular and Thoracic Surgery 2009
  •  One-stage modified Fontan in low-risk patients (n=15): at least 4 years for the lateral tunnel, and older than 6 years for the extracardiac conduit; McGoon ratio > 1.8 or PA index > 200, low PA pressure (< 15 mm Hg), and no AVVR. 2-stage procedure for high-risk patients (n=13). Result: 4 hospital deaths in the 1-stage group (operative mortality, 26.6%) No death occurred in the 2-stage group ( p < 0.05). Three of the 4 deaths were due to elevated CVP, despite preoperative cardiac catheterization demonstrating mean PA pressure < 20 mm Hg. O MonteinNgodngamthaweesuk et al. Asian CardiovascThorac Ann 2007
  •  The 1-stage modified Fontan procedure may put patients at higher surgical risk due to poor cardiovascular adaptation. O MonteinNgodngamthaweesuk et al. Asian CardiovascThorac Ann 2007 An intermediate Glenn shunt and lower postoperative pulmonary artery pressure as significant predictors of better outcome. Nelson Alphonso, David Anderson Presented at the joint 17th Annual Meeting of the European Associationfor Cardio-thoracic Surgery and the 11th Annual Meeting of the EuropeanSociety of Thoracic Surgeons, Vienna, Austria, October 12-15, 2003.
  •  The reported impact of prenatal diagnosis on overall survival is variable Infants diagnosed prenatally are certainly less likely to experience the sequelae of pulmonary overcirculation or acidosis. Prenatal diagnosis is associated with improved neurological outcome and with a lower incidence of preoperative acidosis and ventricular dysfunction, and the need for less inotropic support. U Theilen, L Shekerdemian. Arch Dis Child Fetal Neonatal Ed 2005
  • < 7 yrs old > 7 yrs oldOperative mortality Similar 5.4%Ventricular dysfunction Late EarlyArrythmia Similar SimilarArteriovenous Rare More frequentmalformation Carlo Pace Napoleone Eur JCardiothorac Surg 2010
  •  All patients see a progressive decline in their exercise capacity, but the decrease in exercise capacity was accelerated if the Fontan surgery was performed in adolescents and adults rather than in children. In a serial angiographic study comparing Fontan surgerybefore and after the age of 3 years, it was demonstrated that the cardiac index was preserved if Fontan surgery took place before 3 years of age, and progressively deteriorated if performed at a later age. Yves d’Udekem, Eur J Cardiothorac Surg 2011
  •  Fifteen patients underwent TCPC completion: extracardiac conduit (n = 13), lateral tunnel (n =1), and direct anastomosis of the inferior vena cava and pulmonary trunk (n = 1) The mean age at operation was 27 +/- 9 years (range, 16-52). The mean follow-up period was 57 +/- 45 months (0 to 154). All patients had at least 2 risk factors (2 to 8). Mean pulmonary artery pressure of 20 mm Hg or higher is their only contraindication to TCPC . Mean PVR of 4.0 wood units, pulmonary arterial index of 150, and ejection fraction of 40% are usually acceptable. Midterm to Long-Term Outcome of TCPC in High-Risk Adult Candidates Shunji Sano, MD, et al . Ann Thorac Surg 2009
  •  Overall mortality rate was 13.3%. Conclusion: To improve quality of life and long-term prognosis, TCPC completion should be performed as soon as possible, and TCPC conversion should be performed at an early stage of the morbidities. : TCPC could be offered to high-risk adult Fontan candidates, with an acceptable mortality rate. Midterm to Long-Term Outcome of TCPC in High-Risk Adult Candidates Shunji Sano, MD, et al . Ann Thorac Surg 2009
  •  Long-standing cyanosis and volume overload to the ventricle are believed to cause progressive ventricular fibrosis by inducing histopathologic changes, which must result in both systolic and diastolic dysfunction. The ventricular EF and cardiac indexes were significantly higher at 5 and 10 years after the Fontan procedure had been completed in younger patients. Earlier elimination of hypoxia allows better growth of respiratory and skeletal muscles, which would subsequently increase the cardiac index and exercise capacity, especially at 5 and 10 years after the Fontan procedure. Shuichi Shiraishi et al
  •  Only 13% to 16% of patients who underwent Fontan at 3 years of age had moderate to severe AV valve regurgitation, versus 23% to 26% who underwent Fontan at 3 years of age (p 0.01) due to prolonged volume overloading in the patients who were older at Fontan. Conclusion: Support current trends toward primary Fontan at an early age. Carl Lewis Backer, MD, FACC . J. Am. Coll. Cardiol. 2008;52;114-116
  • ESC GUIDELINES ON GUCH 201O
  • Indications: Small pulmonary arteries Borderline CVP 15 mmHg Poor ventricular function (EF <50%) Moderate-to-severe AVVR Adult patients HLHS Advantages: Improve cardiac output Decrease venous pressure (lessen effusions and Protein-losing enteropathy) Disadvantages: Desaturation Paradoxical embolization Strategy of closure: Transcatheter device John W. Brown et al Ann ThoracSurg2010
  •  Indications for fenestration closure Persistent O2 sat’n of <90% Passed the occlusion test: Does not experience a fall in blood pressure or mixed venous saturation , No rise in right atrial pressure >20 mmHg Jenkins KJ.Eur J Cardiothorac Surg 2010
  •  After closure of Fontan fenestration: No change in peak exercise capacity as demonstrated by percent of predicted VO2, percent predicted ventilatory anaerobic threshold, heart rate, or O2 pulse. This is despite a 12% increase in O2 saturation at peak exercise. Finding demonstrates that exercise tolerance in the Fontan patient is limited by peak blood flow in the pulmonary vascular bed, which is passively perfused by systemic venous pressure. Carl Lewis Backer, MD, FACC. J. Am. Coll. Cardiol. 2008;52;114-116
  •  All patients with clinical thromboembolic events were taking warfarin at the time of the event. Warfarin (INR 1.5 - 2.0.) Aspirin: daily antiplatelet therapy for most Fontan patients Welton M. Gersony, MD Circulation 2008 Warfarin + Aspirin in higher risk: RA-PA connection, external conduit, sluggish venous circulation, or low cardiac output, history of thromboembolism. Yves d’Udekem .Eur J Cardiothorac Surg 2007
  •  Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population. Jonas RA, et al.Ann Thorac Surg 2011
  •  By 20–28 weeks gestation, myocardial oxygen consumption and heart rate normally increase by 20% and stroke volume by 40% The physiological increase in blood volume result in atrial distension. Left uterine displacement help avoid sudden hypotension Although women can successfully complete pregnancy with a Fontan circulation, the associated physiological haemodynamic changes have a significant influence on their abnormal heart function. SandeepNayak , PD Booker MBBS MD FRCA
  •  Women can successfully complete pregnancy after adequate Fontan palliation without important long- term sequelae, although it is often complicated by clinically significant (non-)cardiac events. I Subfertility or infertility and menstrual disorders were common. W Drenthen et al. Heart 2006 In the absence of Eisenmenger physiology, pregnancy in the context of cyanotic heart disease has been associated with 30% incidence of maternal cardiovascular complications and prematurity. Paul Khairy, MD, PhD. Circulation 2007
  •  Pregnancy is contraindicated in patients with severely reduced pulmonary blood flow or with severe pulmonary vascular disease (Eisenmenger syndrome) or if ventricular function is poor. Cyanosis poses a significant risk to the foetus, with a live birth unlikely (12%) if oxygen saturation is <85%. ESC Guidelines on GUCH 201o. European Heart Journal
  •  Early staged operation: mBTs for diminutive right-sided structures Norwood operation for diminutive left-sided structures Pulmonary artery band for pulmonary overcirculation No procedure in early infancy for balanced circulations. BDG/HemiFontan at 6 months of age Total cavopulmonary connection at >2 yrs old At least on aspirin ACE inhibitor for signs of ventricular dysfunction Aggressive repair of AV valve regurgitation Fenestration of Fontan circuit for high-risk candidates Counselling and close follow-up.
  •  The average 80% twenty-year survival rate of Fontan operation is based on a mix of patients who had their procedures done before the advent of and after the TCPC, hence, the outcome of current surgical trends could be better. The Univentricular repair, however, remains a challenge to the entire perioperative team.
  •  In 1971, Fontan and Baudet described an atriopulmonary anastomosis as definitive palliation for tricuspid atresia. Mortality rates with the classic atrial pulmonary connection approximated 15% to 25%. It was originally thought that the right atrium, a pulsatile chamber, would improve pulmonary blood flow. However, in a series of experiments by de Leval et al , it was discovered that the right atrium dilated and then lost contractile function, which resulted in turbulence and energy loss and actually decreased pulmonary blood flow Right Atrium with Classic Fontan Circulation Patients with atriopulmonary Fontan circulation are predisposed to development of complications. The right atrium is exposed to elevated systemic and right atrial pressure, which leads to right atrial dilatation and hypertrophy (Fig 11). Dilatation may be severe, and it may lead to complications such as arrhythmia and swirling of blood in the enlarged atrium, which causes stasis and results in poor blood flow to the lungs. Dilatation also may be a predisposing factor for clot formation.Secondary dilatation of the coronary sinus also is seen. Total cavopulmonary connections have the advantage of saving energy compared with the original RA-PA connections,eliminating less effective blood flow via a large, turbulent atrium that sends blood directly to the pulmonary artery. Welton M. Gersony, MD Circulation 2008
  • In 1988, de Leval and colleagues proposed total cavopulmonary connection by a lateral tunnel as an alternative to the Fontan-type operation with hemodynamic advantagesand reduced atrial complications.1990 when Marcelletti and colleagues described total cavopulmonary connection using anextracardiac conduit from the inferior vena cava to the PA. This has beneficial hemodynamiceffects on systemic venous flow, while the entire atrium is left at low pressure with minimalatrial sutures.
  •  CXR: assess lung disease ECG: rhythm Echocardiography: systemic valvular status, ventricular function, central pulmonary arterial anatomy ,PAP, SVC and IVC anatomy MRI: pulmonary arterial anatomy and flows, collateral circulation, other data to confirm echocardiographic questions Cardiac cath: pulmonary arterial anatomy, PVR
  •  Although echocardiography is widely used in patients with single-ventricle physiology, its ability to comprehensively image all the relevant anatomy, particularly the thoracic vasculature, may be limited in some patients. Four patients in the catheterization group (10%) had findings that were not appreciated at the initial procedure, including a large systemic venovenous collateral vessel, a diffusely small left pulmonary artery requiring intraoperative arterioplasty, a RV aneurysm at a shunt insertion site, and an abscess around a Blalock-Taussig shunt. No new intraoperative or postop. findings in the CMR group. In the absence of evidence of pulmonary hypertension, routine measurement of PVR is not necessary before BDG. Pedro J. del Nido, MD et alCirculation. 2007
  •  Age The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle. Veldtman et al. Heart 2001
  • All patients with a Fontan circulation have anabnormal cardiorespiratory response to exercise. They have a blunted heart rate response, and Limited ability to increase stroke volume withexercise, due to impaired ventricular function anddifficulty in increasing ventricular preload. SandeepNayak, PD Booker MBBS MD FRCA
  •  Functional health status: Over 80% of subjects scored in the normal range on the CHQ. Parents perceived their children as having lower physical and psychosocial functional status. The lower Physical Summary scores are similar to those for children who have undergone thoracic organ transplantation or cardioverter defibrillator implantation Exercise performance. Maximal exercise performance was lower than normal and worse in older subjects due to: absence of a subpulmonary pumping chamber, abnormal endothelial cell function, increased PVR and SVR, decreased muscle mass, & deconditioning Page A. W. Anderson, MD, et al. J Am CollCardiol 2008
  •  Augment Preload: Increase systemic venous return: Low intrathoracic pressure (low respiratory rates, short inspiratory times, low PEEP, and tidal volumes of 5–6 ml kg, normocarbia, and a low PVR). Normovolemia Decrease afterload: Vasodilators Hyperventilation tends to impair pulmonary blood flow, despite the induced respiratory alkalosis, because of the increased mean intrathoracic pressure. SandeepNayak,PD Booker MBBS MD FRCA
  •  Arrhythmia incidence is similar between EC & LT due to: Right atrial dilatation Sinus node dysfunction High risk to develop arrhythmia: ventricular dysfunction, bilateral superior venae cavae, and heterotaxy syndrome Management: Surgical Maze operation Catheter ablation
  •  Due to the absence of pulsatile blood flow and underfilling of the pulmonary vascular bed, patients with Fontan circulation are at increased risk for formation of pulmonary arteriovenous malformations. Tyler B. Fredenburg, MD, Mervyn D. Cohen, MBChB . Ann Thorac Surg 2005 Effect of residual shunts: volume overload on the ventricle and may induce an irreversible increase in PVR secondary to high regional pulmonary blood flow. Sources of shunts: Fenestration, drainage of coronary sinus aorta-pulmonary collaterals & incomplete occlusion of previous artificial shunts. SandeepNayak , PD Booker MBBS MD FRCA
  •  Typically, cardiac output in a Fontan circulation at rest is decreased to 70% (range 50–80%) of normal for body surface area. Preload to the ventricle is determined by transpulmonary flow (PVR) and a fenestration if present. Since the 1990s when excessive volume overloading and acute unloading have been avoided, ventricular dysfunction has become a less important risk factor. A decrease of afterload without preload reserve will not result in increase of output, but may cause hypotension. Excessive afterload on the other hand may be detrimental,especially in the systemic RV. Marc Gewilliga, European Association for Cardio-Thoracic Surgery. 2010.
  •  Decreasing age, as well as intervals, in staged Fontan palliation have beneficial influence on major complications and outcome. K Francois, M Tamim, T Bove, PediatrCardiol, July 1, 2005. Subjects who were older at time of Fontan had worse AV valve function and decreased likelihood of being in sinus rhythm. Poorer valve function and a decrease in sinus rhythm might be related to a longer period of volume overloading . Recommendation: To complete the Fontan at an earlier age. Page A. W. Anderson, MD, et al. J Am CollCardiol 2008
  •  Indications to leave a pulmonary antegrade flow: Low pre-BDG PA pressure Borderline sizes of the native Pas Definitive palliation for high risk patients for Fontan. Davide F. Calvaruso, et al Ann Thorac Surg 2008 Those without APBF did not enjoy the same degree of PA growth as those with APBF. These differences did not correlate with appreciable differences in clinical outcome after BDG or Fontan.
  •  Advantages : Hepatic flow & pulsatile hemodynamics are maintained that may decrease the tendency for collaterals/AVMs Preserves the ability to catheterize the pulmonary arteries from the femoral veins. Disadvantages : Excessive pulmonary flow Elevated pressures resulting in persistent effusions Brian E. Kogon et al. The incidence of clinical superior vena caval syndrome did not differ between groups with or without antegrade flow. Robert G. Gray, et al. Ann ThoracSurg 2007
  •  Kawashima procedure results in a ‘near complete’ Fontan circulation The Spo2 early after post- Kawashima Fontan was not as high as after control Fontan in most cases, but often improved throughout the postoperative follow-up. Shelby Kutty MD, James S. Tweddell, MD Ann ThoracSurg 2010
  •  Early redirection of hepatic flow after the Kawashima procedure may actually decrease or result in resolution of PAVMs. The resolution of hypoxemia after incorporation of the hepatic veins into the cavopulmonary circuit is likely due to a combination of PAVM resolution and elimination of hepatic venoatrial right-to-left shunting. Extracardiac or lateral tunnel connection are the most common methods for completion Fontan Comparative survival rates after Fontan procedure in heterotaxy and nonheterotaxy patients have been similar. Shelby Kutty MD, James S. Tweddell, MD Ann ThoracSurg 2010