Pediatric Cardiac Surgery Foundation, Bangkok, Thailand.
World J Pediatr. 2010 Feb;6(1):13-31. doi: 10.1007/s12519-010-0002-9. Epub 2010 Feb 9.
This article aims to review recent advances in the diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease.
Articles on pulmonary arterial hypertension in congenital heart disease were retrieved from PubMed and MEDLINE published after 1958.
A diagnosis of primary (or idiopathic) pulmonary arterial hypertension is made when no known risk factor is identified. Pulmonary arterial hypertension associated with congenital heart disease constitutes a heterogenous group of conditions and has been characterized by congenital systemic-to-pulmonary shunts. Despite the similarities in histologic appearance of pulmonary vascular disease, there are differences between pulmonary arterial hypertension secondary to congenital systemic-to-pulmonary shunts and those with other conditions with respect to pathophysiology, therapeutic strategies, and prognosis. Revision and subclassification within the category of secondary pulmonary arterial hypertension based on pathophysiology were conducted to improve specific treatments. The timing of surgical repair is crucial to prevent and minimize risk of postoperative pulmonary arterial hypertension. Drug therapies including prostacyclin, endothelin-receptor antagonist, phosphodiesterase inhibitor, and nitric oxide have been evolved with promising results in neonates and children.
Among the different forms of congenital heart diseases, an early correction generally prevents subsequent development of pulmonary arterial hypertension. Emerging therapies for treatment of patients with idiopathic pulmonary arterial hypertension also improve quality of life and survival in neonates and children with congenital heart disease associated with pulmonary arterial hypertension. Heart and lung transplantation or lung transplantation in combination with repair of the underlying cardiac defect is a therapeutic option in a minority of patients. Partial repair options are also beneficial in some selected cases. Randomized controlled trials are needed to evaluate the safety and efficacy of these therapies including survival and long-term outcome.
本文旨在综述新生儿和先天性心脏病儿童肺动脉高压的诊断和治疗的最新进展。
从 1958 年后发表的 PubMed 和 MEDLINE 中检索到有关先天性心脏病肺动脉高压的文章。
当未发现已知危险因素时，可诊断为原发性（或特发性）肺动脉高压。与先天性心脏病相关的肺动脉高压构成了一组异质性疾病，其特征为先天性体肺分流。尽管肺血管疾病的组织学表现相似，但继发于先天性体肺分流的肺动脉高压与其他伴有肺动脉高压的疾病在病理生理学、治疗策略和预后方面存在差异。基于病理生理学对继发性肺动脉高压进行了修订和亚分类，以改善特定治疗。手术修复的时机至关重要，可预防和尽量减少术后肺动脉高压的风险。包括前列环素、内皮素受体拮抗剂、磷酸二酯酶抑制剂和一氧化氮在内的药物治疗在新生儿和儿童中取得了有希望的结果。
在不同形式的先天性心脏病中，早期纠正通常可预防随后发生的肺动脉高压。治疗特发性肺动脉高压的新兴疗法也改善了伴有肺动脉高压的先天性心脏病患儿的生活质量和生存率。心脏和肺移植或联合修复基础心脏缺陷的肺移植是少数患者的治疗选择。部分修复方案在某些选定的病例中也有益。需要进行随机对照试验来评估这些疗法的安全性和疗效，包括生存和长期结果。
