Chida-Nagai Ayako, Sagawa Koichi, Tsujioka Takao, Fujimoto Takanori, Taniguchi Kota, Sasaki Osamu, Izumi Gaku, Yamazawa Hirokuni, Masaki Naoki, Manabe Atsushi, Takeda Atsuhito
Department of Pediatrics, Hokkaido University, Kita14, Nishi5, Kita-Ku, Sapporo, Hokkaido, 060-8648, Japan.
Department of Pediatric Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
Heart Vessels. 2020 Sep;35(9):1307-1315. doi: 10.1007/s00380-020-01604-1. Epub 2020 Apr 13.
Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully.
先天性心脏病相关肺动脉高压（CHD-PAH）是先天性心脏病患者的主要并发症之一。及时关闭左向右分流通常会使肺血流动力学恢复正常，但少数患者在幼儿期预后严重。我们推测，肺动脉高压广泛的病理机制可以解释重度CHD-PAH的临床状态。利用电子病历，我们回顾性分析了6例患有重度CHD-PAH且对治疗抵抗的肺动脉高压的婴儿。所有患者均患有先天性畸形综合征。开始使用肺血管扩张剂后，6例患者中有5例出现并发症，包括肺水肿和间质性肺疾病（ILD），4例患者出现肺泡出血。接受类固醇治疗后，4例患者的临床状况有所改善，但2例患者死亡。其中1例死亡患者的尸检结果显示存在反复肺泡出血、肺静脉高压、ILD和PAH。根据这些CHD-PAH患者的临床病程及文献，CHD-PAH可伴有肺血管阻塞性疾病（PVOD）/肺毛细血管瘤病（PCH）、ILD和/或肺泡出血。CHD-PAH的严重程度可能取决于遗传疾病、呼吸道感染和上气道狭窄。此外，肺血管扩张剂可能与PVOD/PCH和ILD的发生有关。当CHD-PAH患者出现意外恶化时，临床医生应考虑与PVOD/PCH和/或肺部疾病相关的并发症。此外，对于患有CHD-PAH的儿科患者，应谨慎选择初始联合治疗方案。
