Fisher Rebecca, Hopkins Kali, Pant Krittika, Kapoor Arjun, Zaidi Ali N
Department of Cardiology, Mount Sinai Fuster Heart Hospital, One Gustave L. Levy Place, New York, NY 10029, USA.
Eur Heart J Case Rep. 2025 Mar 24;9(3):ytaf115. doi: 10.1093/ehjcr/ytaf115. eCollection 2025 Mar.
Some unrestrictive congenital shunts put patients at high risk for developing irreversible pulmonary vascular disease if not closed in the first year of life. Living at high altitude also places patients at risk for developing pulmonary hypertension. There are anecdotal data that living at high altitude may be protective against the development of shunt-related pulmonary vascular disease.
A 20-year-old female from Ecuador was found to have a large patent ductus arteriosus (PDA) and pulmonary hypertension after a murmur was auscultated. She had been living at altitude until 6 months prior. She was found to have a large PDA, systemic pulmonary artery (PA) pressure, and an elevated pulmonary vascular resistance index (PVRi) (33 wu ∗ m) with minimal shunting (Qp:Qs 1:1). Vasodilatory testing with inhaled nitric oxide (iNO) and 100% FiO2 showed continued systemic PA pressure but a dramatic decrease in PVRi (12.5 wu ∗ m) with an increase in left-to-right shunt (Qp:Qs 2:1). Living at high altitude causes hypoxic vasoconstriction. This vasoconstriction, which is typically reversible on descent to sea level, may decrease the left-to-right shunt and the irreversible downstream vascular changes that would normally occur in the setting of an unrestrictive PDA. Given the significant acute drop of this patient's vascular resistance with pulmonary vasodilator testing, she was started on oral pulmonary vasodilators. However, months later, the patient became increasingly symptomatic and repeat catheterization showed worsening cardiac function. Pulmonary vasodilators were stopped and the decision was made not to close the PDA.
Living at high altitude may be protective against the development of congenital shunt-related irreversible pulmonary vascular disease. However, in this case, it may not have been enough due to the complexity of anatomy.
一些非限制性先天性分流如果在出生后第一年未闭合，会使患者面临发生不可逆性肺血管疾病的高风险。生活在高海拔地区也会使患者有发生肺动脉高压的风险。有轶事数据表明，生活在高海拔地区可能对分流相关的肺血管疾病的发生有保护作用。
一名来自厄瓜多尔的20岁女性在听诊发现杂音后，被诊断为大型动脉导管未闭（PDA）和肺动脉高压。她一直生活在高海拔地区，直到6个月前。她被发现有大型PDA、体循环肺动脉（PA）压力升高以及肺血管阻力指数（PVRi）升高（33 wu∗m），且分流极小（Qp:Qs为1:1）。吸入一氧化氮（iNO）和100%氧气进行血管舒张试验显示，体循环PA压力持续存在，但PVRi显著下降（12.5 wu∗m），左向右分流增加（Qp:Qs为2:1）。生活在高海拔地区会导致缺氧性血管收缩。这种血管收缩通常在下降到海平面时是可逆的，可能会减少左向右分流以及在非限制性PDA情况下通常会发生的不可逆下游血管变化。鉴于该患者在肺血管舒张试验中血管阻力显著急性下降，开始给予口服肺血管扩张剂治疗。然而，数月后，患者症状逐渐加重，再次进行心导管检查显示心脏功能恶化。停用肺血管扩张剂，并决定不闭合PDA。
生活在高海拔地区可能对先天性分流相关的不可逆性肺血管疾病的发生有保护作用。然而，在本病例中，由于解剖结构的复杂性，这种保护作用可能还不够。
