Department of Cardiology, Rigshospitalet, Copenhagen, Denmark; Department of Pediatrics and Adolescent Medicine, Section of Pediatric Cardiology, Rigshospitalet, Copenhagen, Denmark.
Int J Cardiol. 2013 Sep 30;168(2):1458-65. doi: 10.1016/j.ijcard.2012.12.055. Epub 2013 Jan 18.
Long-term survival after the Fontan procedure shows excellent results but is associated with a persistent risk of arrhythmias and exercise intolerance. We aimed to analyze the current burden of clinically relevant arrhythmia and severe exercise intolerance in Danish Fontan patients and furthermore, to estimate the future burden from analysis of mortality and the current burden related to age.
All Danish citizens with Fontan completion from 1981 to 2009 were identified (n=235). Surviving patients performed exercise test, Holter monitoring, echocardiography, pulmonary function test, and blood sampling and medical history was retrieved from medical records.
Twenty-six (11%) patients died or had heart transplantation (HTx) after a mean (± SD) post-Fontan follow-up of 8.3 ± 5.7 years. Excluding perioperative deaths (n=8), a linear probability of HTx-free survival was observed and estimated to 99.1% per year. Prevalence of clinically relevant arrhythmia and severe exercise intolerance increased significantly with age and was found in 32% and 85% of patients ≥ 20 years, respectively. Thus, from survival data and logistic regression models the future prevalence of patients, clinically relevant arrhythmia and severe exercise intolerance were estimated, revealing a considerable augmentation. Furthermore, resting and maximum cardiac index, resting stroke volume index and pulmonary diffusing capacity decreased significantly with age while diastolic and systolic ventricular function was unchanged.
The prevalence of clinically relevant arrhythmia and severe exercise intolerance increased significantly with age in Danish Fontan patients. The future Fontan burden was estimated showing an increase in the prevalence of older patients, clinically relevant arrhythmia, and severe exercise intolerance.
Fontan 手术后的长期存活率表现出优异的结果，但仍存在持续性心律失常和运动不耐受的风险。我们旨在分析丹麦 Fontan 患者目前与临床相关的心律失常和严重运动不耐受的负担，并通过分析死亡率和与年龄相关的当前负担来预测未来的负担。
从 1981 年至 2009 年，确定了所有接受过 Fontan 手术的丹麦公民（n=235）。存活患者进行了运动试验、动态心电图监测、超声心动图、肺功能检查和血液采样，并从病历中检索了病史。
在 Fontan 手术后平均（±SD）8.3±5.7 年后，26 名（11%）患者死亡或接受了心脏移植（HTx）。排除围手术期死亡（n=8）后，观察到 HTx 无生存概率，估计每年为 99.1%。临床相关心律失常和严重运动不耐受的患病率随年龄显著增加，≥20 岁的患者分别为 32%和 85%。因此，根据生存数据和逻辑回归模型，预计未来患者、临床相关心律失常和严重运动不耐受的患病率将会增加，呈现出显著的增加趋势。此外，静息和最大心脏指数、静息每搏量指数和肺弥散能力随年龄显著下降，而舒张和收缩心室功能保持不变。
丹麦 Fontan 患者的临床相关心律失常和严重运动不耐受的患病率随年龄显著增加。未来的 Fontan 负担预计会增加，包括年龄较大的患者、临床相关心律失常和严重运动不耐受的患病率增加。
