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Management of tapvc
This document discusses the diagnosis and management of total anomalous pulmonary venous connection (TAPVC). It covers the types of TAPVC, diagnostic tools including ECG, CXR, echocardiography and catheterization, and surgical and interventional treatment options. The key points are: 1. TAPVC is diagnosed using imaging modalities like echocardiography, CT, and catheterization to identify the anomalous pulmonary vein drainage. 2. Surgical repair is the definitive treatment and involves anastomosis of the pulmonary veins to the left atrium. Factors like age, type of TAPVC, and presence of obstruction determine timing of surgery. 3. Post-operative management focuses on stabil
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Management of tapvc
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- 7. DIAGNOSIS • Clinical Presentation •ECG • Imaging ✦ CXR ✦ Echocardiography ✦ Contrast CT ✦ Catheter Angiography
- 8. ECG • Obstructed TAPVC ✦Right ventricular hypertrophy ★ qR complex or only R wavein right chest leads ✦ Right atrial enlargement is infrequent. ★ Only seen in cardiac TAPVC with obstruction at ASD level • Non-obstructed TAPVC ✦ Resembles that of an OS-ASD ✦ Right axis deviation ✦ Right atrial and ventricular enlargement ✦ Incomplete right bundle branch block pattern.
- 9. CXR • Non Obstructed ✦Cardiomegaly ✦ RV Apex ✦ RA and RV Enlargement ✦ Enlarged PA Segment ✦ Pulmonary Plethora
- 10. CXR • Obstruction ✦ VerticalVein - Normal Cardiac Size ✦ ASD - Cardiomegaly ✦ White out / Ground Glass Opacity lung field ★ DDX • Meconium Aspiration Syndrome • Hyaline Membrane Disease ✦ Enlarged PA Segment
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- 12. OBSTRUCTED TAPVC • CXR ✦Plethoric Lung Fields / Ground Glass appearance ✦ Absence of cardiomegaly ✦ Indistinct cardiac silhouette • DDX ✦ Meconium Aspiration ✦ Hyaline Membrane Disease
- 13. ECHOCARDIOGRAPHY • Diagnostic andInvestigation of Choice • Important Observation ✦ PV not draining into LA ✦ Type of TAPVC ★ Cardiac - Dilated CS ★ Supracardiac - Dilated innominate vein and SVC ★ Infracardiac - Dilated IVC ✦ Insertion of Vertical Vein ✦ Narrowing of Vertical Vein ✦ Adequacy of ASD
- 14. ✦ RA andRV Dilatation ✦ TR ✦ PAH ✦ RV Function ✦ Small LA ✦ LV size and Function ✦ PV Narrowing ✦ Size ✦ Doppler - Velocity > 2m/s ✦ Coexisting Congenital Defect ✦ VSD ✦ PDA
- 17. CARDIAC CATHERISATION • AlwaysDiagnostic • Indication ✦ Measure PVRI / Reversibility in older children ✦ Diagnosis not clear on Echocardiography ✦ Intervention - BAS, PV dilatation, Vertical Vein Dilatation • PA/PV Angiogram ✦ PA injection with venous follow through preferred over direct PV injection ✦ Anatomy of TAPVC ✦ Type ✦ Drainage ✦ Site of Obstruction
- 18. CARDIAC CATHERISATION • Oximetry ✦Similar saturation in all chamber • Drawbacks ✦ Delays management ✦ Aggravate pulmonary edema ✦ Contrast related
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- 22. CROSS SECTIONAL IMAGING •CT and MRI • Indication ✦ Complex pulmonary venous drainage pattern, not clearly defined on Echo ✦ Heterotaxy syndromes with TAPVC ✦ Post operative Pulmonary vein Stenosis • Drawbacks ✦ Contrast related ✦ Requires sedation
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- 24. CARDIAC TAPVC • CTA ✦Common chamber opening into CS ✦ Small LA ✦ Dilated RA ✦ ASD
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- 27. FACTORS TO CONSIDER •Age of Patient • Weight of the patient • Type of TAPVC • Obstructed or Non Obstructed • Clinical Status of Patient ✦ Ventilator ✦ Inotropes ✦ Acidosis • LV and RV function • PAH • Presence of PVOD
- 28. INDICATION FOR SURGERY •Diagnosis of TAPVC
- 29. TIMING OF SURGERY •Immediate Emergency Surgery ✦ Sick Neonate ✦ Obstructed TAPVC • Urgent ✦ Presentation before 6 months • As soon as feasible ✦ Presentation between 6 months to 1 year • >1 year age ✦ PVRI < 8 U.m2 ✦ PVRI >8 U.m2, but reversible
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- 32. MEDICAL THERAPY • Onlysupportive in managing TAPVC • Intended to stabilise the patient before surgery • Goals ✦ Correct acidosis and hypotension ✦ Fluid management ✦ Mechanical ventilation • Prostaglandin ✦ Helpful in keeping ductus venosus open in infra-diaphragmatic TAPVC ✦ Other forms - Increases right to left shunting and systemic desaturation
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- 34. BAS • Short termpalliation • Indication ✦ Sick neonate with TAPVC ✦ Obstruction at ASD level • Goal ✦ Improve mixing ✦ Improve cardiac output ✦ Relief systemic and pulmonary congestion • Technically challenging ✦ Small left atrium ✦ Echocardiographic guidance is often helpful • Success of a BAS ✦ Drop in pulmonary artery pressure ✦ Increase in systemic pressure ✦ Drop in systemic saturation
- 35. BALLON DILATION/ STENTINGVV • Occasionally performed to relieve obstruction • Indication ✦ Sick neonates presenting with obstructed TAPVC • Technically challenging
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- 39. PRINCIPLES • Principle ✦ OnCPB or Circulatory arrest ✦ Identify 4 PV, common chamber and dissect and loop vertical vein ✦ Achieve wide tension free non obstructing anastomosis between common chamber and LA ✦ Avoiding purse stringing of suture line ✦ Divert all pulmonary venous blood to LA ✦ Close intra-atrial communication ★ Patch Material • Pericardium • Dacron ( Polyethylene terephthalate ) ✦ Ligate vertical vein close to insertion into systemic vein
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- 42. SC - TAPVC- POSTERIOR APPROACHPaul Ebert
- 46. SC-TAPVC RIGHT BIATRIALAPPROACH Shumacker and King
- 48. RIGHT SIDED APPROACH- LA Kirklin
- 51. TRANS RA TRANSLA Cooley
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- 56. CS-TAPVC -CS CUTBACK Malm
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- 60. IC- TAPVC -ORIENTATION OF COMMON CHAMBER
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- 67. PRIMARY SUTURELESS VSCONVENTIONAL REPAIR
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- 69. OVERALL AND EARLYMORTALITYOverallMortalityEarlyMortality
- 70. VERTICAL VEIN LIGATION •Proponents of Ligation ✦ Large left to right shunt with RV volume overload ✦ Requires additional procedure for closure ✦ No survival benefit ✦ Ligating the vertical vein close to its insertion into the systemic vein, the vertical vein adds to the reservoir of the LA • Proponents of Leaving the vertical vein open ✦ Serves as a pop off for the non compliant left heart ✦ Spontaneously closes over time ✦ Less turbulent immediate postoperative course, especially in obstructed TAPVC
- 74. Surviours - 12 Notligated vertical vein - 10 Median Follow up - 4.7 years Spontaneous closure - 5 Required Ligation - 4
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- 76. LA ENLARGMENT • Proponents ✦Increase in reservoir capacity ✦ Better LV Filling and Cardiac Output ✦ Decreased incidence of Pulmonary venous hypertension and reactive PAH • Oponents ✦ Incorporation of common venous sinus enlarges LA adequately ✦ Patch material is non contractile • Literature ✦ No significant difference in survival with LA enlargement ★ Katz NM, Kirklin JW, Pacifico AD. Concepts and practices in surgery for total anomalous pulmonary venous connection. Ann Thorac Sure 1977
- 77. PFO • Benefits ✦ Popofffor RV in patients with severe PAH ✦ Maintain cardio output at the cost of desaturation • Drawbacks ✦ Risk of paradoxical embolism ✦ Significant right to left requiring additional procedure for closure
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- 79. PHYSIOLOGY • Preoperative ✦ SmallLA ✦ Underfilled LV ✦ RA/RV Volume Overload ✦ Increased Qp • Postoperative ✦ Offloading of Right Heart ✦ Volume loading in not compliant LV
- 80. ANTICIPATED PROBLEMS • NonCompliant LV ✦ > Acute Dilatation > Septal Dysfunction > LV Dysfunction > Increase in LA Pressure • Non Compliant LA > Surges in LA pressure > ✦ PVH > PAH Crisis ✦ Pulmonary Hemorrhage > Stiff Lungs • Post Op PAH > RV Dysfunction > LCOS • LV Dysfunction > LCOS
- 81. COMPLICATIONS OF SURGERY •Bleeding • LV diastolic dysfunction (Low Compliance) • RV dysfunction • Pulmonary hypertension and PAH crisis • Gradient across anastomosis • Pulmonary vein stenosis
- 82. STRATEGIES • Avoid surgesis LA pressure ✦ Fluid Restriction ✦ Avoid Fluid Bolus • Inodilators ✦ Dobutamine/Milrinone • Afterload Reduction - Vasodilators ✦ NTG/SNP • Avoid Triggers of PAH • Refractory PAH ✦ Echo - R/o Anastamotic stenosis/ Pulmonary vein stenosis ✦ NO Ventilator ✦ Mechanical Support
- 83. ISSUES • LV dysfunction ✦Inodilators ✦ Monitor LA pressure ✦ Positive pressure ventilation ✦ Prevent fluid overloading ( Low Threshold for PD) ✦ ECMO
- 84. ISSUES • PAH ✦ Adequatesedation in the first 24-48 hours ✦ Avoid ★ Hypercardbia ★ Hypoxia ★ Acidosis ✦ Adequate lung recruitment ✦ Pulmonary dilatation ★ SNP, Milrinone ✦ Avoid triggers of PAH ★ Suctioning ✦ Refractory Cases - NO Ventilation
- 85. PEARLS • Avoid FluidBolus • Accept low BP if no Lactic Acidosis • Avoiding Weaning when LA pressure high • Low threshold for Peritoneal dialysis • Avoid triggers of PAH • Elective CPAP after extubation helps with LV dysfunction
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- 87. MORTALITY • Early Mortality ✦STS - 10-30% ✦ Individual Centers - 2-20% • Late Mortality ✦ Very few late mortality ✦ Survival depends on early survival
- 88. MODES OF DEATH •Cardiac Failure • PAH Crisis • Sepsis • Pathophyiological correlates to mortality ✦ Preoperative cardiopulmonary instability ★ Acidosis ★ Ventilator Requirement ✦ Post op PAH Crisis ★ Preop Lung Injury ★ CPB related ★ Postop Acidosis ✦ Progression of Pulmonary Vein Stenosis
- 89. • Cause ofDeath • Low Cardiac Output ( 70%) • Sepsis (30%)
- 90. RISK FACTORS FOREARLY DEATH • Low birth weight • Infracardiac TAPVC • Mixed TAPVC • Obstructed TAPVC • Poor preoperative physiological state • Postop Pulmonary Venous Obstruction • Increased PVRI • Single ventricle physiology
- 92. Over all mortality <2Kg Mixed vs Others Postop PVO Reoperation PVO Reoperation Mixed TAPVC
- 94. HEMODYNAMIC OUTCOME • RV- Size decreases to normal • PA - Pressure normalises • LA ✦ Size increases to normal ✦ Compliance below normal • LV ✦ Volume increases to normal ✦ Systolic function improves to normal • Rhythm ✦ Ectopic pacemaker activity due to damage of internodal preferential pathway
- 95. FUNCTIONAL STATUS • Mostsurviving patients are NYHA 1 ary and musculoskeletal exercise performance after repair for total anomalous pulmonary venous connection during infancy. J Thorac Cardiovasc Surg 2007;133:1533-9.
- 99. AIIMS RESULT • Patients- 248 (168 boys, 80 girls) • Age - 1 day to 24 years (median 8 months) ; 145 infants • Weight - 2 to 52 kg (median 5 kg). 70% were less than the 50th percentile of predicted weight for age and sex. • Type ✦ Supracardiac - 134 (54%) ✦ Cardiac - 80 (32.2%) ✦ Infracardiac - 9 (3.6%) ✦ Mixed - 25 (10.1%) • Obstructed TAPVC - 50 (20.2%) • Mod-Sev PAH - 76 patients (30.2%) Choudhary SK, Bhan A, Sharma R, et al. Total anomalous pulmonary venous connection: surgical experience in Indians. Indian Heart J. 20
- 100. • Emergency surgery- 45 patients (18.1%) • Circulatory Arrest - 114 • Early Mortality - 45 (19.1%) ✦ Pulmonary arterial hypertensive crisis - 19 ✦ Low cardiac output syndrome - 17 • Risk Factors ✦ Age < 1 year (OR 2.16; 95% CI: 1.22-3.82, p=0.008) ✦ Severe pulmonary arterial hypertension (AR 5.86; 95% CI: 2-17, p=0.001), ✦ Emergency surgery (OR 3.65; 95% CI: 1.59-8.38, p=0.002) • Late Mortality - 4 • Follow-up ranged from 1 to 180 months (median 48 months). • Actuarial survival at 12 years - 92.6% +/- 2.8%. Choudhary SK, Bhan A, Sharma R, et al. Total anomalous pulmonary venous connection: surgical experience in Indians. Indian Heart J. 20
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- 102. ANASTAMOTIC STENOSIS • Incidence- 10% • Occurs early postoperative period ✦ Later anastomosis grows and child grows • Factors which decrease risk ✦ Open anastomosis ✦ Wide anastomosis ✦ Absorbable monofilament sutures ✦ No difference between continuous and interrupted suture technique • Reoperation ✦ Risk of restenosis ✦ Poor outcome
- 103. PULMONARY VEIN STENOSIS •Incidence - 5- 15% • Usually occurs within the first 6 months • Pathophysiology ✦ Diffuse fibrotic thickening of PV ✦ Localised narrowing at PV-LA junction ✦ May or may not be associate with anastomotic stenosis • Presentation ✦ Progressive Dyspnea • Investigation ✦ Echocardiography ★ PV Flow velocity > 2m/s ✦ Cross Sectional Imaging
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- 108. CTA • Narrowing inPV just proximal to insertion into common chamber/LA
- 109. PULMONARY VEIN STENOSIS •Treatment ✦ Steroids or Chemotherapy to minimise fibrosis ✦ Balloon dilatation/stenting ✦ Ostial endarterectomy of intimal hyperplasia IRevising the common pulmonary vein to left atrium anastomosis with or without patch enlargement ✦ Sutureless repair
- 115. • Developed PVO- 71/406 (17.5%) ✦ Cardiac - 9/67 (13%) ✦ Supracardiac -25/205 (12%) ✦ Infracardiac - 25/110 (23%) ✦ Mixed - 11/37 (30%) • Median time to diagnosis from surgery - 49days ( 0 - 5.9 yrs) ✦ Diagnosed within 6 months of surgery - 59/71 (83%) ✦ Echo - 30% , Angiography - 54% and CT/MRI - 16% • Intervention ✦ 60 patients required intervention ✦ 56 received intervention ★ 50% required more than 1 intervention
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