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 Single Ventricle: congenital cardiac malformations that
lack two completely well developed ventricles, and in
which func...
 Classifications of Single ventricle:
 Hearts with common inlet atrioventricular connection [Double-
inlet RV/ Double-in...
1.Inadequate pulmonary circulation (or ductal dependent)
2.Excessive pulmonary circulation without systemic obstruction
3....
 Medical management: (temporary palliation)
 Prostaglandin E1
 Restoraration of normal acid base status
 Maintain End ...
 In patients with Pulmonary
outflow obstruction:
pulmonary atresia/stenosis
 Consistently SaO2 <75~80%
 Systemic to pul...
 In patients with excessive pulmonary blood flow:
 SaO2> 85% strongly suggest excessive pulmonary blood flow
 Volume ov...
 In patients with excessive pulmonary blood flow with
systemic obstruction-
 DKS procedure (Norwood + BT shunt)
 One of the following conditions:
 Mean PA pressure up to 20mmHg
 2 PVR≦ < 4 Wood units but reactive to vasodilators
 ...
 Bidirectional cavopulmonary shunt as a staging
Maneuver, usually combined with repair of associated lesion
leading to po...
COMMENTSCOMMENTS
 End to side connection between the cranial end of SVC & right
pulmonary artery
 Ligation of of azygous...
Contd.Contd.
 promotes regression of left ventricular mass in younger
children.
 Improvement in the degree of atrioventr...
Intra-atrial baffle /
Lateral tunnel method Extra-cardiac conduit method
To drain all systemic veins
directly into PA
Preserve the Functioning single ventricle for
systemic circulation
 Goal: BEFORE proceeding to the Fontan circulation,
appropriately regulating pulmonary blood flow since newborn
period
(1...
Proper Patient
Sellection!!!!!!
Graham and Johns pointed out that theGraham and Johns pointed out that the
following issues or criteria were not includedf...
NECESSARY CRITERIANECESSARY CRITERIA
1. Undistorted pulmonary artery anatomy;
2. Low pulmonary vascular resistance;
3. Low...
METHODMETHOD
 The lateral tunnel technique involves placement of a
baffle along the lateral aspect of the right atrium,
w...
Post operative managementPost operative management
 Minimize PVR
 Monitor systemic & pulmonary pressure & indicators of ...
 Early mortality: 7.7%
 Late mortality:
 Survival 93% at 5years, 91% at 10 years
 Most common causes of death
 Thromb...
 Complications:
 Early complications: pleural effusion, pericardial
effusion, sternal wound infection, arrhythmia,
strok...
 Atrial arrhythmia
 Major risk factor for morbidity and functional
decline after the Fontan procedure
 Incidence: 10~40...
 Protein-losing enteropathy
 Very poor prognosis!!! : 5-yr survival 59%, 10-yr
survival <20%
 Incidence: 3.8%~10%
 Lym...
 Ventricular dysfunction/heart dailure: 8.3%
 Preload reduced to 50~70% of normal for BSA
 Ventricle: from volume overl...
 Right atriomegaly and hepatic dysfunction
 Dilatation of the coronary sinus
 Pulmonary arteriovenous malformations
 M...
Single ventricle dr. prashant medanta
Single ventricle dr. prashant medanta
Single ventricle dr. prashant medanta
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Single ventricle dr. prashant medanta

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single ventricle surgical management by dr. prashant agrawal medanta the medicity

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Single ventricle dr. prashant medanta

  1. 1.  Single Ventricle: congenital cardiac malformations that lack two completely well developed ventricles, and in which functionally there is only a single ventricular chamber that supports both pulmonary and systemic circulations
  2. 2.  Classifications of Single ventricle:  Hearts with common inlet atrioventricular connection [Double- inlet RV/ Double-inlet LV]  Hearts with absence of one atrioventricular connection [Tricuspid atresia / Mitral atresia]  Hearts with common atrioventricular valve and only one well- developed ventricle [unbalanced common atrioventricular canal defect]  Hearts with only one fully developed ventricle  Hypoplastic left heart syndrome  Double-outlet right ventricle and a ventricular septal defect remote from semilunar valves  Other rare forms of univentricular hearts
  3. 3. 1.Inadequate pulmonary circulation (or ductal dependent) 2.Excessive pulmonary circulation without systemic obstruction 3. Excessive pulmonary circulation with systemic obstruction 4. Balanced pulmonary flow
  4. 4.  Medical management: (temporary palliation)  Prostaglandin E1  Restoraration of normal acid base status  Maintain End organ perfusion & function  Aim of surgical intervention: to improve the natural history by balancing blood flow between the pulmonary and systemic circulations and ultimately separating these two circulations.
  5. 5.  In patients with Pulmonary outflow obstruction: pulmonary atresia/stenosis  Consistently SaO2 <75~80%  Systemic to pulmonary arterial shunt: modified B-T shunt: graft from subclavian A to PA  To improve SaO2
  6. 6.  In patients with excessive pulmonary blood flow:  SaO2> 85% strongly suggest excessive pulmonary blood flow  Volume overload to the single ventricle: cause CHF  Pressure overload to the pulmonary arteriole tree: cause pulmonary vascular disease  Tx: PA banding!  Exclude obstruction of systemic outflow!  SaO2 80~85%
  7. 7.  In patients with excessive pulmonary blood flow with systemic obstruction-  DKS procedure (Norwood + BT shunt)
  8. 8.  One of the following conditions:  Mean PA pressure up to 20mmHg  2 PVR≦ < 4 Wood units but reactive to vasodilators  Surgically repairable PA hypoplasia or discrete stenosis present  McGoon ratio is 2.0 and repairable  LVED volume is 2SD above the mean[compared with normal structural heart]  Target SpO2: 78~85%
  9. 9.  Bidirectional cavopulmonary shunt as a staging Maneuver, usually combined with repair of associated lesion leading to poor outcome of Fontan’s operation, including pulmonary arterial stenosis, atrioventricular valve regurgitation, and systemic outflow tract obstruction.  To reduce the volume load on single ventricle & maintain a viable Spo2.  So to preserve ventricular function for the subsequent Fontan
  10. 10. COMMENTSCOMMENTS  End to side connection between the cranial end of SVC & right pulmonary artery  Ligation of of azygous  If b/l SVC, both should be connected to respected Pas  The cavopulmonary shunt increases effective pulmonary blood flow without volume-loading the ventricle.  Systemic venous collateralization may worsen hypoxemia by reducing the effective pulmonary blood flow  There are concerns about the growth of the pulmonary arteries .  Pulmonary arteriovenous fistulae may be a universal consequence of the bidirectional cavopulmonary shunt
  11. 11. Contd.Contd.  promotes regression of left ventricular mass in younger children.  Improvement in the degree of atrioventricular valve regurgitation.
  12. 12. Intra-atrial baffle / Lateral tunnel method Extra-cardiac conduit method
  13. 13. To drain all systemic veins directly into PA Preserve the Functioning single ventricle for systemic circulation
  14. 14.  Goal: BEFORE proceeding to the Fontan circulation, appropriately regulating pulmonary blood flow since newborn period (1) Improve clinical symptoms: cyanosis, CHF (2) Provide optimal pulmonary artery architecture and low pulmonary vascular resistance (3) Preserve systolic and diastolic ventricular function (4) Preserve atrio-ventricular valve function (5) Relieve systemic ventricular outflow tract obstruction (6) Provide anatomic setup for a definitive Fontan repair  Ideal saturation: SpO2 80%
  15. 15. Proper Patient Sellection!!!!!!
  16. 16. Graham and Johns pointed out that theGraham and Johns pointed out that the following issues or criteria were not includedfollowing issues or criteria were not included  Diastolic dysfunction,  Ventricular hypertrophy,  Systemic outflow tract obstruction,  Right ventricular type of single ventricle,  Extensive systemic aortopulmonary collaterals.
  17. 17. NECESSARY CRITERIANECESSARY CRITERIA 1. Undistorted pulmonary artery anatomy; 2. Low pulmonary vascular resistance; 3. Low ventricular end diastolic pressure; 4. Absence of obstruction to systemic outflow; and 5. Preservation of systemic atrioventricular valve function.
  18. 18. METHODMETHOD  The lateral tunnel technique involves placement of a baffle along the lateral aspect of the right atrium, which conveys IVC blood to SVC orifice.  4 mm fenestration is made in the medial aspect of baffle to prevent the systemic venous pressure risisng to intolerable limit.  Large ASD is created to prevent any restriction of flow between atria.  Allows to preserve systemic cardiac output at the expense of some reduction in arterial saturation.  Lower operative mortality &p/o pleural effusion.
  19. 19. Post operative managementPost operative management  Minimize PVR  Monitor systemic & pulmonary pressure & indicators of good cardiac output as strength of pulse, urine output, BP, CRT.  Systemic venous hypertension lead to reflex arterial constriction lead to increased afterload may impair cardiac output- milrinone, nitroprusside  Arrhythmias- atrial pacing lowers atrial filling pressure & augments cardiac output  Pleural effusion & ascites- complete drainage
  20. 20.  Early mortality: 7.7%  Late mortality:  Survival 93% at 5years, 91% at 10 years  Most common causes of death  Thromboembolism: intra-cardiac thrombus, lack of Aspirin / Warfarin  Heart failure: morphological RV, high RA pressure, protein-losing enteropathy  Sudden death: cardiac arrhythmias [within fist 5 years after Fontan surgery]
  21. 21.  Complications:  Early complications: pleural effusion, pericardial effusion, sternal wound infection, arrhythmia, stroke……  Late complications: atrial arrhythmia, ventricular dysfunction, protein-losing enteropathy, thromboembolic events
  22. 22.  Atrial arrhythmia  Major risk factor for morbidity and functional decline after the Fontan procedure  Incidence: 10~40%  Most common: Sinus node dysfunction!! [sinus node injury while REDO or disturbance of its blood supply]  Cause: RA dilation, RA incision  Tx: anti-coagulant if refractory
  23. 23.  Protein-losing enteropathy  Very poor prognosis!!! : 5-yr survival 59%, 10-yr survival <20%  Incidence: 3.8%~10%  Lymphatic drainage dysfunction  Tx: anticongestive therapy, steroid, heparin, atrial fenestration, heart transplantation!
  24. 24.  Ventricular dysfunction/heart dailure: 8.3%  Preload reduced to 50~70% of normal for BSA  Ventricle: from volume overload and overstretched to severely undeloaded →”Disuse hypofunction”: remodelling, reduced compliance, poor ventricular filling, continuous declining cardiac output  Tx: heart transplantation! [Late take down or fenestration before heart transplantation]
  25. 25.  Right atriomegaly and hepatic dysfunction  Dilatation of the coronary sinus  Pulmonary arteriovenous malformations  Myocardial dysfunction and failure  Ventricular outflow obstruction  Obstruction of pulmonary veins  Recanalization of ligated main pulmonary trunk  Systemic venous collateralisation  Bronchitis  Pancreatitis  WPW syndrome

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