Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK.
First Department of Cardiology, Hippokration Hospital, National and Kapodistrian Athens University, Athens, Greece.
Heart. 2018 Dec;104(23):1963-1969. doi: 10.1136/heartjnl-2017-312881. Epub 2018 May 18.
Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain.
All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis.
A total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, p<0.0001).
Arrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.
约 5%-10%的先天性心脏病（CHD）成人患者会发展为肺动脉高压（PAH），这会影响预期寿命和生活质量。心律失常在这些患者中很常见，但它们的发生率及其对预后的影响仍不确定。
在 2007 年至 2015 年间，我们对一家三级中心的所有与 CHD 相关的 PAH（PAH-CHD）成年患者进行随访，以观察新发的房性或室性心律失常。使用 Cox 分析评估与心律失常相关的临床变量及其与死亡率的关系。
共纳入 310 例患者（平均年龄 34.9±12.3 岁，36.8%为男性）。大多数患者患有艾森曼格综合征（58.4%），15.2%有先心病修复史，三分之一的患者患有唐氏综合征。基线时，14.2%的患者有既往心律失常史，主要为室上性心律失常（86.4%）。在中位随访 6.1 年期间，64 例患者至少发生一次新发心律失常事件（发生率为每年 3.47%），主要为室上性心动过速或心房颤动（78.1%的患者）。心律失常伴有症状者占 75.0%。PAH-CHD 类型、疾病严重程度标志物和既往心律失常与随访期间的心律失常有关。心律失常是死亡的强烈预测因素，即使在调整了人口统计学和临床变量后也是如此（HR 3.41，95%CI 2.10 至 5.53，p<0.0001）。
心律失常在 PAH-CHD 中很常见，与不良的长期预后相关，即使在专科中心进行治疗也是如此。
