Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry
Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry
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DOI：
10.1016/j.ijcard.2006.09.017
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发表时间：
2007-08-21
影响因子：
3.5
通讯作者：
Mulder, B. J. M.
中科院分区：
文献类型：
--
作者：
Duffels, M. G. J.;Engelfriet, P. M.;Mulder, B. J. M.
背景：与先天性心脏病相关的肺动脉高压（PAH）通常是大的全身-肺分流的结果，并且常常导致右心室衰竭和早期死亡。本研究的目的是确定国家先天性心脏病登记中的成年患者中 PAH 的患病率，并评估患者特征与 PAH 之间的关系。方法：从登记中确定患有与间隔缺损相关的 PAH 患者。记录性别、年龄、基础诊断、既往闭合、修复年龄和 NYHA 分类。 PAH 的定义是通过超声心动图评估估计的收缩肺动脉压 (sPAP) 大于 40 mm Hg。 结果：在所有 5970 名登记的成年先天性心脏病患者中，PAH 的患病率为 4.2%。在登记的 1824 名间隔缺损患者中，112 名患者 (6.1%) 患有 PAH。这些患者的中位年龄为 38 岁（范围 18-81 岁），其中 40% 为男性。这些患者中，58% 患有艾森曼格综合征。在先前闭合性间隔缺损的患者中，30 人患有 PAH（3%）。室间隔缺损 (VSD) 是 PAH 合并室间隔缺损患者中最常见的潜在缺损 (42%)。女性（优势比 = 1.5，p = 0.001）和 sPAP（优势比 = 0.04，p < 0.001）与功能等级下降独立相关。结论：PAH 在先天性心脏病成年患者中很常见。在我们的登记中，间隔缺损中 PAH 的患病率约为 6%。这些患者中有一半以上患有艾森曼格综合征，占 CONCOR 登记总人口的 1%。由于早期发现和干预，未来PAH的患病率是否会下降还有待观察。 (c) 2006 Elsevier Ireland Ltd. 保留所有权利。
Background: Pulmonary arterial hypertension (PAH) associated with congenital heart disease is usually the result of a large systemic-topulmonary shunt, and often leads to right ventricular failure and early death. The purpose of this study was to determine the prevalence of PAH among adult patients included in a national registry of congenital heart disease and to assess the relation between patient characteristics and PAH.Methods: Patients with PAH associated with a septal defect were identified from the registry. Gender, age, underlying diagnosis, previous closure, age at repair and NYHA classification were recorded. PAH was defined as a systolic pulmonary arterial pressure (sPAP) greater than 40 mm Hg, estimated by means of echocardiographical evaluation.Results: The prevalence of PAH among all 5970 registered adult patients with congenital heart disease was 4.2%. Of 1824 patients with a septal defect in the registry, 112 patients (6.1%) had PAH. Median age of these patients was 38 years (range 18-81 years) and 40% were male. Of these patients, 58% had the Eisenmenger syndrome. Among the patients with a previously closed septal defect, 30 had PAH (3%). Ventricular septal defect (VSD) was the most frequent underlying defect (42%) among patients with PAH and a septal defect. Female sex (Odds ratio= 1.5, p= 0.001) and sPAP (Odds ratio= 0.04, p < 0.001) were independently associated with a decreased functional class.Conclusion: PAH is common in adult patients with congenital heart disease. In our registry the prevalence of PAH in septal defects is around 6%. More than half of these patients have the Eisenmenger syndrome, which accounts for 1% of the total population in the CONCOR registry. Whether the prevalence of PAH will decrease in the future as a result of early detection and intervention remains to be awaited. (c) 2006 Elsevier Ireland Ltd. All rights reserved.