1 Department of Pediatrics, Vanderbilt University School of Medicine, and Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee, USA.
Pulm Circ. 2013 Sep;3(3):454-66. doi: 10.1086/674438. Epub 2013 Nov 19.
Abstract Infants with forms of pulmonary hypertension (PH) that persist or develop beyond the first week of life are an understudied group of patients with up to 40%-60% mortality. The clinical management of the progressive PH that develops in these infants is challenging because of the nonspecific signs and symptoms of clinical presentation, the limited diagnostic sensitivity of standard echocardiographic techniques, and the lack of proven therapies. The signaling mechanisms that underlie the structural and functional abnormalities in the pulmonary circulation of these infants are not yet clear. The ability to improve outcomes for these patients awaits technological advances to improve diagnostic capabilities and therapeutic discoveries made in basic science laboratories that can be tested in randomized clinical trials.
患有持续性或出生后第一周后出现的肺高血压（PH）的婴儿是一组研究较少的患者群体，其死亡率高达 40%-60%。由于临床表现无特异性体征和症状、标准超声心动图技术的诊断灵敏度有限以及缺乏经过验证的治疗方法，这些婴儿中出现的进行性 PH 的临床管理极具挑战性。这些婴儿的肺循环结构和功能异常的信号机制尚不清楚。提高这些患者的治疗效果需要依靠技术进步，以提高诊断能力，并在基础科学实验室中发现可在随机临床试验中进行测试的治疗方法。
