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 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
 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
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
 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.
 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
 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%
 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
 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%
 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
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
Contd.Contd.
 promotes regression of left ventricular mass in younger
children.
 Improvement in the degree of atrioventricular valve
regurgitation.
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) 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%
Proper Patient
Sellection!!!!!!
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.
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.
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.
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
 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]
 Complications:
 Early complications: pleural effusion, pericardial
effusion, sternal wound infection, arrhythmia,
stroke……
 Late complications: atrial arrhythmia, ventricular
dysfunction, protein-losing enteropathy,
thromboembolic events
 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
 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!
 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]
 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
Single ventricle dr. prashant medanta

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Single ventricle dr. prashant medanta

  • 1.
  • 2.  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
  • 3.  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
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  • 5. 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
  • 6.  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.
  • 7.  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
  • 8.  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%
  • 9.  In patients with excessive pulmonary blood flow with systemic obstruction-  DKS procedure (Norwood + BT shunt)
  • 10.  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%
  • 11.  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
  • 12. 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
  • 13. Contd.Contd.  promotes regression of left ventricular mass in younger children.  Improvement in the degree of atrioventricular valve regurgitation.
  • 14. Intra-atrial baffle / Lateral tunnel method Extra-cardiac conduit method
  • 15. To drain all systemic veins directly into PA Preserve the Functioning single ventricle for systemic circulation
  • 16.  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%
  • 18. 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.
  • 19. 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.
  • 20. 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.
  • 21. 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
  • 22.  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]
  • 23.  Complications:  Early complications: pleural effusion, pericardial effusion, sternal wound infection, arrhythmia, stroke……  Late complications: atrial arrhythmia, ventricular dysfunction, protein-losing enteropathy, thromboembolic events
  • 24.  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
  • 25.  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!
  • 26.  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]
  • 27.  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

Editor's Notes

  1. Tricuspid atresia 是一切的濫觴