Vall Camell Maria, Rodríguez-Fanjul Javier, Bautista Rodríguez Carles, Pradda Freddy Hermogenes, Caffarena-Calvar Jose María, Iriondo Sanz Martín, Sánchez-de-Toledo Joan
Servicio de Pediatría, Hospital Sant Joan de Déu-Clínic, Universidad de Barcelona, Barcelona, España.
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.
An Pediatr (Engl Ed). 2019 Nov;91(5):336-343. doi: 10.1016/j.anpedi.2018.10.020. Epub 2019 Apr 2.
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.
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.
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.
Coronary artery to right ventricle fistulae may not be a contraindication for biventricular 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.
新生儿室间隔完整的肺动脉闭锁及重度肺动脉狭窄涵盖了广泛的疾病谱，包括右心室显著发育不良和冠状动脉右心室瘘的病例，后者可能被视为右心室减压的禁忌证。本研究的目的是回顾这些患者20年的中长期结局，并确定两组之间的差异因素，包括患有冠状动脉瘘的患者。
我们通过识别1996年1月至2018年1月期间所有被诊断为室间隔完整的肺动脉闭锁及重度肺动脉狭窄的患者，进行了一项描述性回顾性研究。我们收集并分析了有关右心室形态、手术治疗、经皮介入及中长期结局的数据。
共收治51例患者。随访期间共有9例患者（17.6%）死亡。所有死亡患者均无冠状动脉右心室瘘。42例幸存者的中位随访时间为8.9年（1 - 16年）。根据纽约心脏协会分类的最新修订版，总体样本的功能分级为1.2。重度肺动脉狭窄幸存者的功能分级为1.1，室间隔完整的肺动脉闭锁幸存者的功能分级为1.6。有无冠状动脉右心室瘘并无差异。
冠状动脉右心室瘘可能并非双心室策略的禁忌证。与室间隔完整的肺动脉闭锁患者相比，重度肺动脉狭窄患者的结局更好。早期积极开放肺动脉瓣的策略与良好的总体生存率相关，且与良好的功能分级相关。
