Mortality in adults with congenital heart disease

Abstract

Aim: Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH).

Methods: Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken. Age, gender of deceased individuals, and frequency and cause of death in different congenital diagnosis groups was explored.

Results: Between 1991 and 2015, death occurred in 3.3% of the ACHD cohort. Median age at death was 32years (IQR 26-41.5) and 51% were male. The most frequent underlying cardiac conditions were Eisenmenger's syndrome (22%), pulmonary atresia and ventricular septal defect+/-major aorto-pulmonary collateral arteries (12%), Tetralogy of Fallot (10%), transposition of great arteries (TGA) with intact ventricular septum (8%), single ventricle (8%) and congenitally corrected TGA (5%). The cause of death was available from medical records in 60 (82%) of the 73 patients. The majority of deaths were due to cardiac causes (67%) including sudden death (40%), heart failure (13%), and documented ventricular arrhythmias (8%). The most common non-cardiac cause of death was sepsis (10%).

Conclusion: The majority of deaths in this group were due to cardiac causes with sudden death and heart failure being the most common. Identification of risk factors for sudden death might assist identification of patients who may benefit from preventative therapies including implantable cardiac defibrillator.

Keywords: Adult congenital heart disease; Heart defects; Mortality.