BACKGROUND Patients with simple shunt lesions, such as atrial septal defect (ASD), ventricular septal defect (VSD) and persistent arterial duct (PDA) remain at risk of developing pulmonary hypertension (PH) even after correction of their cardiac defect. We aimed to assess the contemporary prevalence of PH in a well characterized nationwide group of patients based on the German National Register for Congenital Heart Defects. METHODS AND RESULTS We included all patients >16 years of age with an isolated diagnosis of ASD, VSD or PDA. Only patients with previous surgical or interventional closure of the defect were included. Patients with genetic syndromes were excluded. Out of 49,597 CHD patients in the register we identified 825 patients with closed, isolated simple defects (52% ASD, 41% VSD, 7% PDA). Of these, 25 (3%) developed PH after a median follow-up of 16 years from defect closure. The risk of PH increased significantly with age at follow-up (p < 0.0001) and age at repair (p < 0.0001) on logistic regression analysis Patients with PH were significantly more likely to be symptomatic (59% vs. 9% in NYHA class ≥2, p < 0.0001) and had significantly higher mortality (hazard ratio 13.4, p < 0.0001) compared to the remaining patients. CONCLUSIONS Based on data from the German National Register CHD Register we report a PH prevalence of 3.0% in patients with corrected, simple lesions. Patients with PH were more symptomatic and had significantly increased mortality risk. Life-long surveillance and low threshold for workup is recommended to ascertain diagnosis of PH, which has important prognostic and clinical implications.

中文翻译：

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单纯性先天性心脏缺陷患者分流闭合后的肺动脉高压。

背景技术具有简单分流病变的患者，例如房间隔缺损（ASD），心室间隔缺损（VSD）和持续性动脉导管（PDA），即使在纠正其心脏缺损之后也仍然存在发生肺动脉高压（PH）的风险。我们旨在根据德国国家先天性心脏病缺陷登记表评估全国范围内特征明确的一组患者的PH患病率。方法和结果我们纳入了所有16岁以上的ASD，VSD或PDA的独立诊断患者。仅包括先前通过手术或介入方式闭合缺损的患者。遗传综合征患者被排除在外。在注册的49597名CHD患者中，我们确定了825名闭合，孤立的单纯缺陷患者（52％ASD，41％VSD，7％PDA）。这些，有25名（3％）患儿在闭合缺陷后进行了16年的中位随访后出现了PH。在Logistic回归分析中，随着随访年龄（p <0.0001）和修复年龄（p <0.0001），PH的风险显着增加。PH的患者有症状的可能性更高（59％，而在NYHA级别为9％） ≥2，p <0.0001），与其余患者相比，死亡率显着更高（危险比13.4，p <0.0001）。结论根据德国国家注册的CHD注册数据，我们报告了已纠正的简单病变患者的PH患病率为3.0％。PH患者的症状更严重，死亡率风险显着增加。建议进行终生监测和低检查阈值，以确诊PH值，这对预后和临床都有重要意义。