Hostname: page-component-586b7cd67f-2brh9 Total loading time: 0 Render date: 2024-11-23T00:52:07.375Z Has data issue: false hasContentIssue false
  • English
  • Français

Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle

Published online by Cambridge University Press:  23 October 2009

Nicodème Sinzobahamvya ,
Claudia Arenz ,
Julia Reckers ,
Joachim Photiadis ,
Peter Murin ,
Ehrenfried Schindler ,
Viktor Hraska  and
Boulos Asfour
Nicodème Sinzobahamvya*
Affiliation:
Department of Paediatric Thoracic and Cardiovascular Surgery
Claudia Arenz
Affiliation:
Department of Paediatric Thoracic and Cardiovascular Surgery
Julia Reckers
Affiliation:
Department of Cardiac Intensive care
Joachim Photiadis
Affiliation:
Department of Paediatric Thoracic and Cardiovascular Surgery
Peter Murin
Affiliation:
Department of Paediatric Thoracic and Cardiovascular Surgery
Ehrenfried Schindler
Affiliation:
Department of Anaesthesiology and Critical Care Medicine; Congenital Cardiac Center (“Deutsches Kinderherzzentrum”), Sankt Augustin, Germany
Viktor Hraska
Affiliation:
Department of Paediatric Thoracic and Cardiovascular Surgery
Boulos Asfour
Affiliation:
Department of Paediatric Thoracic and Cardiovascular Surgery
*
Correspondence to: Nicodème Sinzobahamvya, M.D., Deutsches Kinderherzzentrum, Asklepios Klinik, Arnold-Janssen-Strasse, 29 53757 Sankt Augustin, Germany. Phone: 49 2241 249601; Fax: 49 2241 249602; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Totally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.

Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.

Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.

Keywords

Univentricular heartspulmonary hypertensionright isomerismAristotle complexity score
Type
Original Articles
Information
Cardiology in the Young , Volume 19 , Issue 6 , December 2009 , pp. 594 - 600
Copyright
Copyright © Cambridge University Press 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Delisle, G, Ando, M, Calder, AL, et al. Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 1976; 91: 99–122.Google Scholar
2.Anderson, RH, Macartney, FJ. Pulmonary venous abnormalities. In: Robert H Anderson et al (ed.) Paediatric Cardiology. Churchill Livingstone, London, 2002, pp 867–899.Google Scholar
3.O’Brien, SM, Jacobs, JP, Clarke, DR, et al. Accuracy of the Aristotle basic complexity score for classifying the mortality and morbidity potential of congenital heart surgery operations. Ann Thorac Surg 2007; 84: 2027–2037.Google Scholar
4.Heinemann, MK, Hanley, FL, Van Praagh, S, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. Ann Thorac Surg 1994; 57: 88–91.CrossRefGoogle Scholar
5.Gaynor, JW, Collins, MH, Rychik, J, Gaughan, JP, Spray, TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999; 117: 506–514.Google Scholar
6.Sinzobahamvya, N, Arenz, C, Brecher, AM, Urban, AE. Atrial isomerism: a surgical experience. Cardiovasc Surg 1999; 7: 436–442.Google Scholar
7.Lodge, AJ, Rychik, J, Nicolson, SC, Ittenbach, RF, Spray, TL, Gaynor, JW. Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg 2004; 78: 1688–1695.Google Scholar
8.Caldarone, CA, Najm, HK, Kadletz, M, et al. Surgical management of total anomalous pulmonary venous drainage: Impact of coexisting cardiac anomalies. Ann Thorac Surg 1998; 66: 1521–1526.Google Scholar
9.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.Google Scholar
10.Chowdhury, UK, Airan, B, Sharma, R, et al. Surgical considerations of univentricular heart with total anomalous pulmonary connection. Indian Heart J 2000; 52: 192–197.Google Scholar
11.Scheewe, J, Becker, K, Böning, A, Fischer, G, Kramer, HH, Cremer, J. Mid-term outcome of infants with functional single ventricle (SV) and total anomalous pulmonary venous connection (TAPVC). Thorac Cardiovasc Surg 2004; 52, DOI:10.1055/s-2004-816627.Google Scholar
12.Sachdev, MS, Jena, PK, Kurup, RP, Varghese, R, Kumar, RS, Coelho, R. Outcome of single ventricle and total anomalous pulmonary connection. Asian Cardiovasc Thorac Ann 2006; 14: 367–370.CrossRefGoogle Scholar
13.Sadiq, M, Stümper, O, De Giovanni, JV, et al. Management and outcome of infants and children with right atrial isomerism. Heart 1996; 75: 314–319.Google Scholar
14.Hashmi, A, Abu-Sulaiman, R, McCrindle, BW, Smallhorn, JF, Williams, WG, Freedom, RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31: 1120–1126.Google Scholar
15.Yun, TJ, Al-Radi, OO, Adatia, I, et al. Contemporary management of right atrial isomerism: effect of evolving therapeutic strategies. J Thorac Cardiovasc Surg 2006; 131: 1108–1113.Google Scholar
16.Rose, V, Izukawa, T, Moës, CA. Syndromes of asplenia and polysplenia: a review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840–852.Google Scholar
17.Lacour-Gayet, F, Clarke, D, Jacobs, J, et al. . The Aristotle Committee. The Aristotle score: a complexity-adjusted method to evaluate surgical results. Eur J Cardiothorac Surg 2004; 25: 911–924.Google Scholar
18.Jhang, WK, Chang, YJ, Park, CS, Oh, YM, Kim, YH, Yun, TJ. Hybrid palliation for right atrial isomerism associated with obstructive total anomalous pulmonary venous drainage. Interact Cardiovasc Thorac Surg 2008; 7: 282–284.Google Scholar