Percutaneous management of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis
Por:
Jose Manuel Vall Torrego, Rodríguez-Fanjul J, Bautista Rodríguez C, Prada F, Caffarena-Calvar JM, Iriondo-Sanz M and Sanchez deToledo J
Publicada:
1 nov 2019
Ahead of Print:
2 abr 2019
Categoría:
Pediatrics, perinatology and child health
Resumen:
Introduction: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae.
Patients and methods: We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes.
Results: 51 patients were admitted. A total of 9 patients (17.6%) died during the followup. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of follow up in the 42 survivors was 8.9 years (1-16).
The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae.
Conclusions: Coronary artery to right ventricle fistulae may not be a contraindication for biven-tricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class. (C) 2018 Published by Elsevier Espana, S.L.U. on behalf of Asociacion Espanola de Pediatria.
Filiaciones:
Jose Manuel Vall Torrego:
Servicio de Pediatría, Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España
Rodríguez-Fanjul J:
Servicio de Transporte Pediátrico, Servei Emergències Mèdiques (SEM), Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España
Unidad de Cuidados Intensivos Pediátricos, Servicio de Pediatría, Hospital Universitario de Tarragona Joan XXIII, Tarragona, España
Bautista Rodríguez C:
Servicio de Cardiología Infantil, Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España
Servicio de Cardiología Infantil, Royal Brompton Hospital, Londres, Reino Unido
Prada F:
Servicio de Cardiología Infantil, Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España
Caffarena-Calvar JM:
Servicio de Cirugía Cardíaca Infantil, Hospital Sant Joan de Déu-Clínic, Barcelona, España
Iriondo-Sanz M:
Servicio de Neonatología. Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España
Sanchez deToledo J:
Servicio de Cardiología Infantil, Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España
Servicio de Cuidados Críticos, University of Pittsburgh School of Medicine, Children's Hospital Pittsburgh of UPMC, Pittsburgh, Estados Unidos
Green Submitted, gold
|