Faculty Collaboration Database

Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle. Cardiol Young 2009 Dec;19(6):594-600

Date

10/24/2009

Pubmed ID

19849875

DOI

10.1017/S1047951109991296

Scopus ID

2-s2.0-76349114725 (requires institutional sign-in at Scopus site)   26 Citations

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.

Author List

Sinzobahamvya N, Arenz C, Reckers J, Photiadis J, Murin P, Schindler E, Hraska V, Asfour B

Author

Viktor Hraska MD Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Anastomosis, Surgical
Cardiac Surgical Procedures
Female
Heart Defects, Congenital
Humans
Infant
Infant, Newborn
Male
Palliative Care
Postoperative Complications
Pulmonary Veins
Retrospective Studies
Survival Rate
Treatment Outcome