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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: n.sinzobahamvya@asklepios.com
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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

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