Unidad de Cardiopatías Congénitas del Adulto, Hospital Universitario La Paz, Madrid, Spain; Department of Cardiology and CIBERCV, Hospital General Universitario Gregorio Marañón, Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain.
Unidad Intercentro de Cardiopatías Congénitas del Adulto, Hospital Universitario Virgen Macarena, Sevilla, Spain.
Int J Cardiol. 2017 Oct 15;245:119-124. doi: 10.1016/j.ijcard.2017.06.060. Epub 2017 Jun 19.
The impact of gender and aging on relative survival and causes of death in adults with congenital heart disease (ACHD) are not well known.
Single center observational longitudinal study of 3311 consecutive ACHD (50.5% males) followed up to 25years. Patients were divided by the age at last follow-up into three groups: <40, 40-65 and >65years old. Their vital status was verified by crosschecking the Spanish National Death Index. Regression model for relative survival from reference population was performed. Cause of death was classified according to the International Classification of Diseases (ICD-10). Patients who died from cardiovascular (CV) causes were further investigated on a case-by-case basis.
During a cumulative follow-up time of 37,608 person-years 336 patients died (10%). Age-adjusted relative survival in females was significantly worse than in males (hazard ratio [HR] 1.25; 95% confidence interval [CI] 1.0-1.6; p=0.046), and sex-adjusted relative survival improved across the three group of ages (HR 0.98; 95% CI 0.97-0.99; p<0.001). There was a temporal decline of CV deaths with aging in both genders (p<0.001). The leading cause of CV death was heart failure but sudden death prevailed in subjects <40years (p=0.004). While sudden death progressively declined with aging heart failure significantly increased (p<0.001).
Women with CHD fare worse than men. There are a decline in CV deaths and a major temporal shift in the causes of CV deaths with aging. Heart failure surpasses sudden death as the primary cause of death in survivors over 40years.
性别和年龄对先天性心脏病（ACHD）成人患者的相对生存率和死亡原因的影响尚不清楚。
对 3311 例连续 ACHD 患者（50.5%为男性）进行了单中心观察性纵向研究，随访时间长达 25 年。根据最后一次随访时的年龄，将患者分为三组：<40 岁、40-65 岁和>65 岁。通过交叉核对西班牙国家死亡指数来核实患者的生存状态。根据参考人群的相对生存率回归模型进行分析。根据国际疾病分类（ICD-10）对死亡原因进行分类。对死于心血管（CV）原因的患者进行了进一步的个案调查。
在 37608 人年的累计随访期间，336 名患者死亡（10%）。女性的年龄调整后相对生存率明显低于男性（危险比 [HR] 1.25；95%置信区间 [CI] 1.0-1.6；p=0.046），且随着年龄的增长，男女两性的相对生存率均呈上升趋势（HR 0.98；95% CI 0.97-0.99；p<0.001）。在两性中，随着年龄的增长，CV 死亡呈下降趋势（p<0.001）。CV 死亡的主要原因是心力衰竭，但在<40 岁的患者中，猝死占主导地位（p=0.004）。随着年龄的增长，猝死逐渐减少，心力衰竭显著增加（p<0.001）。
患有 CHD 的女性比男性预后差。随着年龄的增长，CV 死亡人数减少，CV 死亡原因发生重大时间转变。心力衰竭超过猝死成为 40 岁以上患者的主要死亡原因。
