Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect

Marien Lenoir; Margaux Pontailler; Régis Gaudin; Sébastien Gerelli; Daniel Tamisier; Damien Bonnet; Bari Murtuza; Pascal R Vouhé; Olivier Raisky

doi:10.1093/ejcts/ezx194

Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect

Eur J Cardiothorac Surg. 2017 Sep 1;52(3):590-598. doi: 10.1093/ejcts/ezx194.

Authors

Marien Lenoir¹, Margaux Pontailler¹, Régis Gaudin¹, Sébastien Gerelli¹, Daniel Tamisier¹, Damien Bonnet², Bari Murtuza¹, Pascal R Vouhé¹, Olivier Raisky¹

Affiliations

¹ Department of Pediatric Cardiac Surgery, University Paris Descartes and Sick Children Hospital, Paris, France.
² Department of Pediatric Cardiology, University Paris Descartes and Sick Children Hospital, Paris, France.

PMID: 28633393
DOI: 10.1093/ejcts/ezx194

Abstract

Objectives: To determine the early, intermediate and long-term outcomes of pulmonary atresia with ventricular septal defect (PA/VSD) Types I, II and III initially palliated by a right ventricle to pulmonary artery (RVPA) connection.

Methods: We performed a retrospective study from 2000 to 2014 that included 109 patients with PA/VSD who had undergone an RVPA connection (tetralogy of Fallot and PA/VSD Type IV excluded). The end-points of this strategy were adequate pulmonary artery tree post-palliation, second palliation, biventricular repair, right ventricular pressure post-biventricular repair and late reoperation. Mean follow-up was 5.4 years (1 day to 14-78 years).

Results: Early mortality after an RVPA connection was 2.7% (3 of 109). The interstage mortality rate was 6.6% (7 of 106). Eighty-four (77%) patients had a biventricular repair and 8 patients (7%) are awaiting repair. Overall survival was 90% at 1 year and 81% at 10 years. The RVPA connection allowed significant growth of the native pulmonary artery with a Nakata index of 101 mm2/m2 before the RVPA connection and 274 mm2/m2 after (P = 0.001). Twenty-nine reinterventions for restrictive pulmonary blood flow have been done (9 before 2 months and 20 after 2 months). Of the 84 patients who had a repair, 22 patients (26%) initially had a right ventricular pressure greater than 40 mmHg. Twenty-eight patients (33%) required late reoperation.

Conclusions: Hospital deaths after the RVPA connection were low. The procedure allowed good growth of the native pulmonary artery. Biventricular repair was possible in a large number of cases. The late morbidity rate remains significant. Early reinterventions could be avoided by appropriate calibration. This technique appears to be suitable for any type of PA/VSD with central pulmonary arteries.

Keywords: Major aortopulmonary collaterals; Palliative surgery; Pulmonary atresia with ventricular septal defect; RVPA connection.

MeSH terms

Abnormalities, Multiple*
Anastomosis, Surgical
Cardiac Surgical Procedures / methods*
Female
Follow-Up Studies
Forecasting*
France / epidemiology
Heart Septal Defects / diagnosis
Heart Septal Defects / mortality
Heart Septal Defects / surgery*
Heart Ventricles / abnormalities
Heart Ventricles / diagnostic imaging
Heart Ventricles / surgery*
Hospital Mortality / trends
Humans
Infant
Male
Palliative Care / methods*
Pulmonary Artery / abnormalities
Pulmonary Artery / diagnostic imaging
Pulmonary Artery / surgery*
Pulmonary Atresia / diagnosis
Pulmonary Atresia / mortality
Pulmonary Atresia / surgery*
Reoperation
Retrospective Studies
Survival Rate / trends
Treatment Outcome

Supplementary concepts

Pulmonary Atresia With Ventricular Septal Defect