Management and results of tapvc

1. TAPVC - MANAGEMENT

2. TYPES

6. DIAGNOSIS

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
✦ Vertical Vein - Normal Cardiac Size
✦ ASD - Cardiomegaly
✦ White out / Ground Glass Opacity lung field
★ DDX
• Meconium Aspiration Syndrome
• Hyaline Membrane Disease
✦ Enlarged PA Segment

11. SC-TAPVC - SNOWMAN

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 and Investigation 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 and RV 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
• Always Diagnostic
• 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

20. SC-TAPVC CATHETER ANGIO

21. IC-TAPVC CATHTER ANGIO

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

23. SC- TAPVC CTA

24. CARDIAC TAPVC
• CTA
✦ Common chamber opening into
CS
✦ Small LA
✦ Dilated RA
✦ ASD

25. IC-TAPVC CTA

26. DECISION MAKING

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

31. MEDICAL TREATMENT

32. MEDICAL THERAPY
• Only supportive 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

33. CATHETER BASED INTERVENTION

34. BAS
• Short term palliation
• 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/ STENTING VV
• Occasionally performed to relieve obstruction
• Indication
✦ Sick neonates presenting with obstructed TAPVC
• Technically challenging

38. SURGICAL TREATMENT

39. PRINCIPLES
• Principle
✦ On CPB 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

40. CLOSED APPROACH
William H Muller

42. SC - TAPVC - POSTERIOR
APPROACHPaul Ebert

46. SC-TAPVC RIGHT BIATRIAL APPROACH
Shumacker and King

48. RIGHT SIDED APPROACH - LA
Kirklin

51. TRANS RA TRANS LA
Cooley

54. SUPERIOR APPROACH
Tucker

56. CS-TAPVC -CS CUT BACK
Malm

58. CS-TAPVC
Van Prragh

60. IC- TAPVC - ORIENTATION OF COMMON CHAMBER

63. SUTURELESS TECHNIQUE
Lacour Gayet

66. CONTROVERSIES

67. PRIMARY SUTURELESS VS CONVENTIONAL REPAIR

68. PVO AND REOPERATIONPostOpPVOReopPVO

69. OVERALL AND EARLY MORTALITYOverallMortalityEarlyMortality

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
Not ligated vertical vein - 10
Median Follow up - 4.7 years
Spontaneous closure - 5
Required Ligation - 4

75. Circulation. 2010;122:2718-2726

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
✦ Popoff for 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

78. POST OPERATIVE MANAGEMENT

79. PHYSIOLOGY
• Preoperative
✦ Small LA
✦ Underfilled LV
✦ RA/RV Volume Overload
✦ Increased Qp
• Postoperative
✦ Offloading of Right Heart
✦ Volume loading in not compliant LV

80. ANTICIPATED PROBLEMS
• Non Compliant 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 surges is 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
✦ Adequate sedation 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 Fluid Bolus
• 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

86. RESULTS

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 of Death
• Low Cardiac Output ( 70%)
• Sepsis (30%)

90. RISK FACTORS FOR EARLY 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
<2 Kg
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
• Most surviving 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

101. REOPERATION

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

104. Circulation. 2010;122:2718-2726

108. CTA
• Narrowing in PV 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

118. THANK YOU