Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension: A case report and Mini Review


Vol 7 | Issue 3 | September-December 2021 | Page: 10-16 | Vishnu Datt, Rachna Wadhwa, Manish Kumar, Varun Sharma, Ripon Chaudhary, Sanjula Virmani

DOI: 10.13107/jaccr.2021.v07i03.185

Author: Vishnu Datt [1], Rachna Wadhwa [1], Manish Kumar [1], Varun Sharma [1], Ripon Chaudhary [1], Sanjula Virmani [1]

[1] Department of Anaesthesiology and Intensive Care, GIPMER, G.B. Pant Hospital (University of Delhi) New Delhi, India.
Address of Correspondence
Dr. Rachna Wadhwa,
GIPMER, G.B. Pant Hospital (University of Delhi) New Delhi, India.

Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension: A case report and Mini Review


The total anomalous pulmonary venous communication [TAPVC] is a rare cyanotic congenital cardiac defect accounting for 1.5-3% of the congenital heart disease, in which pulmonary venous [PV] blood drains directly into the right side of the heart or into the systemic veins. Neonates with obstructive TAPVC may present with cyanosis, metabolic acidosis, respiratory failure, and shock. A subset of patients with unobstructed TAPVC may remain symptoms free and attain adulthood, or present with pulmonary congestion, pulmonary arterial hypertension [PAH]. The anesthetic management of either obstructed TAPVC or unobstructed with PAH can be quite challenging. The described patient is a 23-year male who presented with self – limiting single episode of chest pain, palpitations and dyspnea, diagnosed as supracardiac unobstructed TAPVC with ostium secundum atrial septal defect [OS – ASD] and PAH, who underwent successful intracardiac repair under cardiopulmonary bypass [CPB]. The protocol for the cardiac surgery during the COVID-19 pandemic for perioperative considerations and triage recommendations was strictly followed to reduce the risk of exposure to patients and healthcare workers. The objective of this case report and review is to recognize the spectrum of various clinical presentations in TAPVC, and to describe the diagnosis and perioperative management of TAPVC.
Key Words: Adult Supra cardiac TAPVC, unobstructed, PAH, cardiopulmonary bypass, corrective surgery, balanced general anesthesia


1. Herlong JR, Jaggers JJ, Ungerleider RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies. Ann Thorac Surg. 2000;69(suppl): S56–S69.
2. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008; 153:807.
3. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890.
4. Ross FJ, Joffe D, Latham GJ. Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection. Semin Cardiothorac Vasc Anesth. 2017;21(2):138-144
5. Hines MH, Hammon JW. Anatomy of total anomalous pulmonary venous connection. Oper Tech Thorac Cardiovasc Surg. 2001; 6:2-7.
6. Seale AN, Uemura H, Webber SA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010; 122:2718-2726
7. Domadia S, Ram Kumar S, Smith JKV, Pruetz JD. Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction. Pediatric Cardiology. 2018; 39:1346–1354.
8. Files MD, Morra B. Total Anomalous Pulmonary Venous Connection: Preoperative Anatomy, Physiology, Imaging, and Interventional Management of Postoperative Pulmonary Venous Obstruction. Semin Cardiothorac Vasc Anesth. 2017; 21(2):123-131
9. Padalino MA, Cavalli G, De Franceschi M, et al. Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience. J Card Surg. 2014; 29:678-685.
10. Hancock Friesen CL, Zurakowski D, Thiagarajan RR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg. 2005; 79:596-606
11.Munsi AS, Hussain M, Rima R, Biswas R, Sayeed A. Clinical profile of patients with Total Anomalous Pulmonary Venous Return and their short-term outcome in pediatric cardiac centre at Dhaka Shishu Hospital Bangla J of Med Scie.2015 JULY Vol. 14 No. 03:270-273
12.Haworth SG. Total anomalous pulmonary venous return. Prenatal damage to pulmonary vascular bed and extrapulmonary veins. Br Heart J. 1982; 48:513-524.
13. Kyser JP, Bengur AR, Siwik ES. Preoperative palliation of newborn obstructed total anomalous pulmonary venous connection by endovascular stent placement. Cathter Cardiovasc Intev. 2006; 67(3):473-6
14. Konstantinov IE. Primary sutureless repair of total anomalous pulmonary venous connection: the value of intrapleural hilar reapproximation. Journal of Thoracic and Cardiovascular Surgery.2006; 132(3):729–730
15. Kong D, Pan C, Dong L, Jin H, Shu X. Rare variant of mixed total anomalous pulmonary venous drainage (TAPVD) in a 55-year-old patient. Eur Heart J Cardiovasc Imaging. 2015; 16:227
16. Fred M. Wu, Sitaram M. Emani, Michael J. Landzberg, and Anne Marie Valente, Rare Case of Undiagnosed Supracardiac Total Anomalous Pulmonary Venous Return in an Adult. Circulation.2014; 130, 1205-1207
17. Qasim A .Supracardiac Total Anomalous Pulmonary Venous Return. Pediatric echocardiography: online instructions n interpretive echocardiography for improved detection of congenital heart disease in clinical settings
18. Gökmen Akkaya 1 · Çağatay Bilen 2 · Osman Nuri Tuncer 3 · Mehmet Fatih Ayık 3 · Yüksel Atay. Evaluation of Surgical Treatment in Patients with Total Anomalous Pulmonary Venous Connection. EJCM 2018;6(3):107-112
19. S. Adil Husain, MD, Elaine Maldonado, MD, Debbie Rasch, MD, Joel Michalek, MD, Richard Taylor, MD et al. Total Anomalous Pulmonary Venous Connection: Factors Associated with Mortality and Recurrent Pulmonary Venous Obstruction. Ann Thorac Surg 2012; 94:825–32
20.Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Williams WG, et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation. 2007;115(12):1591-1598
21. Wu CL, Kan CD, Wu JM, Wang JN. Risk Factors of Total Anomalous Pulmonary Venous Connection Surgery. Pediatrics & Health Research 2016;1(3):1-6
22. White BR, Ho DY, Faerber JA , Katcoff H, Glatz AC , Mascio CE et al.Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction. Ann Thorac Surg . 2019 ;108(1):122-129.
23. Cheng JW, Tonelli AR, Patterson G, and Krasushki RA et al, Pharmacologic Management of Perioperative Pulmonary Hypertension. J Cardiovasc Pharmacol. 2014; 63(4): 375–384
24. Abbas AE, Fortuin FD, Schiller NB, Appleton CP, Moreno CA et al. A simple method for noninvasive estimation of pulmonary vascular resistance. Journal of the American College of Cardiology 2003. 41 1021–1027
25. Patel V, Jimenez E, Cornwell L, Tran T, Paniagua D et al. Cardiac Surgery During the Coronavirus Disease 2019 Pandemic: Perioperative Considerations and Triage Recommendations. J Am Heart Assoc. 2020;9: e017042
26. Datt V, Khurana P, Aggarwal S, Mishra S , Sujith CN, and Virmani S. Perioperative Management of a Patient with Double Orifice Mitral Valve with Supramitral Ring with Subaortic Membrane with Ventricular Septal Defect and Severe Pulmonary Hypertension: Report of a Rare Case. Ann Card Anaesth. 2019; 22(2): 215–220
27. Kaestner M, Schranz D, Warnecke G, Apitz C, Hansmann G et al. Pulmonary hypertension in the intensive care unit. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. European Paediatric Pulmonary Vascular Disease. Network endorsed by ISHLT and DGPK. Heart. 2016;102: ii57–ii66.
28.Taylor MB, Laussen PC. Fundamentals of management of acute postoperative pulmonary hypertension. Pediatr Crit Care Med. 2010;11: S27–S29.
29. Mubeen M, Singh AK, Agarwal SK, Pillai J, Kapoor S, et al. Mitral valve replacement in severe pulmonary arterial hypertension. Asian Cardiovasc Thorac Ann. 2008; 16:37– 42.
30. Coutinho GF, Antunes MJ. Surgery for mitral stenosis in patients with pulmonary hypertension: How far can we go?. J Thorac Cardiovasc Surg 2016;152:302-3
31. Myers PO, Tissot C, Beghetti M. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease – Do we have the good tools to predict success? Circ J 2014;78:4-11
32. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D34–41 ]
33. Ziyaeifard M, Alizadehasl A, Massoumi G. Modified Ultrafiltration During Cardiopulmonary Bypass and Postoperative Course of Pediatric Cardiac Surgery. Res Cardiovasc Med. 2014; 3(2): e17830.
34.Onoe M, Oku H, Kitayama H, Matsumoto T, Kaneda T. Modified ultrafiltration may improve postoperative pulmonary function in children with a ventricular septal defect. Surg Today. 2001;31(7):586–90
35. Condliffe R, Kiely DG. Critical care management of pulmonary hypertension. BJA Education 2017; 17 (7): 228–234
36. Thunberg CA, Morozowich ST, Ramakrishna H. Inhaled therapy for the management of perioperative pulmonary hypertension. Ann Card Anaesth. 2015 ; 18(3): 394–402
37. Sarkar MS, Desai PM. Pulmonary Hypertension and Cardiac Anesthesia: Anesthesiologist’s perspective. Ann card anesth, 2018; 21(2): 116-122
38. Burnes ML, Boettcher BT, Woehlck HJ, Zundel MT, Iqbal Z et al. Hydroxocobalamin as a Rescue Treatment for Refractory Vasoplegic Syndrome After Prolonged Cardiopulmonary Bypass. J Cardiothorac Vasc Anesth.2017;31(3): 1012-1014
39. Abman SH, Hansmann G, Archer SL, et al. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation. 2015; 132( 21): 2037– 2099
40.Denault A, Deschamps A, Tardif JC, Lambert J, Perrault L. Pulmonary hypertension in cardiac surgery. Curr Cardiol Rev. 2010; 6:1–4.
41. Kameny RJ, Fineman J, Adatia I. Perioperative Management of Pediatric Pulmonary Hypertension. Adv Pulmonary Hypertens. 2016; 15:87–91
42. Gorter TM, Verschuuren EAM, Veldhuisen DJ, Elke S Hoendermis ES et al. Right ventricular recovery after bilateral lung transplantation for pulmonary arterial hypertension. Interact Cardiovasc Thorac Surg. 2017;24(6):890-897

How to Cite this Article: Datt V, Wadhwa R, Kumar M, Sharma V, Chaudhary R, Virmani S | Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension- Case Report and Mini Review | Journal of Anaesthesia and Critical Care Case Reports | September-December 2021; 7(2): 10-16.

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