Kinsella John P, Ivy D Dunbar, Abman Steven H
Department of Pediatrics, Division of Neonatology, The Children's Hospital and the University of Colorado School of Medicine, Denver, CO 80218-1088, USA.
Semin Perinatol. 2005 Apr;29(2):123-8. doi: 10.1053/j.semperi.2005.04.008.
Pulmonary hypertension complicates the course of many newborns with congenital diaphragmatic hernia. In the most severe cases, the fetal condition of markedly elevated pulmonary vascular resistance persists after birth and is associated with hypoxemic respiratory failure and severe disturbances in cardiac performance. Late pulmonary hypertension (weeks to months after birth) is increasingly recognized in this population, and chronic pulmonary vascular abnormalities (months to years after birth) are now being discovered. In this review, we will discuss the pathophysiology of acute, late, and chronic pulmonary hypertension in patients with congenital diaphragmatic hernia. We will also review the role of currently available pulmonary vasoactive drugs in the management of pulmonary hypertension in this population.
肺动脉高压使许多先天性膈疝新生儿的病程复杂化。在最严重的病例中，出生后肺血管阻力显著升高的胎儿状况持续存在，并伴有低氧性呼吸衰竭和心脏功能严重紊乱。该人群中晚期肺动脉高压（出生后数周数月）越来越受到关注，慢性肺血管异常（出生后数月至数年）现也被发现。在本综述中，我们将讨论先天性膈疝患者急性、晚期和慢性肺动脉高压的病理生理学。我们还将综述目前可用的肺血管活性药物在该人群肺动脉高压管理中的作用。
