Epidemiology and Mortality in Adult Congenital Heart Disease
Epidemiology and Mortality in Adult Congenital Heart Disease
To identify patients with CHD at risk for premature death in adulthood, we reviewed studies reporting the experience of large single tertiary care centers, studies reporting results from national registries and studies on heart transplantation in adults with CHD.
There are two studies elucidating the mortality and mode of death in adults with CHD. The study published by Oechslin et al. in 2000 reflects the experience of a large tertiary care center (Toronto Congenital Cardiac Centre for Adults, Toronto, ON, Canada). Among the 2609 patients in their database, 199 died and 197 patients, for whom adequate information was available, were analyzed. Of these, 164 out of 197 patients (83%) died from cardiovascular causes. By far the most common underlying congenital cardiac defects in these patients were shunt lesions with Eisenmenger physiology (48 out of 164; 29%), followed by patients with repaired tetralogy of Fallot (19 out of 164; 10%), patients with univentricular hearts (13 out of 164; 8%) and patients with subaortic right ventricles (10 out of 164; 6%). Mean age at death was 37 ± 15 years, with significant differences between different disease entities. The number of patients with Fontan palliation among those with single ventricle physiology was not reported. The study published by Verheugt et al. in 2011 reports data from the CONCOR Dutch national registry database of adults with CHD. Among 6933 patients in this database, 197 died during a follow-up of 24,865 patient-years at a median age of 49 years (range: 20–91 years). The majority of deceased patients had moderate or severe disease complexity (68%) and 77% died from cardiovascular causes. In all age categories, mortality among CHD patients was higher than in the general Dutch population. The most common individual congenital disease entities among deceased patients were atrial septal defects (34 out of 197; 17%), tetralogy of Fallot (23 out of 197; 12%), aortic stenosis/bicuspid aortic valves (18 out of 197; 9%) and patients with subaortic right ventricles (17 out of 197; 9%). Deaths among patients with univentricular hearts accounted for only 6% of all deaths (12 out of 197). The study does not report the rate of previous surgical repair or palliation among individual disease entities and does not report the number of patients with Eisenmenger physiology or residual cyanosis.
A study published by Nieminen et al. in 2007 reports a population-based analysis of postoperative survival in patients after reparative surgery for CHD. The study showed an increased mortality for CHD patients compared with the general population, particularly early after surgery. Most patients, however, died in childhood and relative survival during adulthood was not specifically analyzed.
The registry of the United Network for Organ Sharing (UNOS) does not record original anatomical diagnosis of the underlying congenital cardiac defects. Thus information about type and characteristics of adults with CHD undergoing heart transplantation are derived from single center reports. In all published series, the most frequent congenital heart lesions were single ventricle physiology with or without Fontan palliation, followed by patients with transposition complexes with subaortic right ventricles (complete transposition after atrial switch procedures or congenitally corrected transposition of the great arteries) and patients with repaired or palliated tetralogy of Fallot.
Patients at Risk of Premature Death in Adulthood
To identify patients with CHD at risk for premature death in adulthood, we reviewed studies reporting the experience of large single tertiary care centers, studies reporting results from national registries and studies on heart transplantation in adults with CHD.
Single Center Experience & National Registries
There are two studies elucidating the mortality and mode of death in adults with CHD. The study published by Oechslin et al. in 2000 reflects the experience of a large tertiary care center (Toronto Congenital Cardiac Centre for Adults, Toronto, ON, Canada). Among the 2609 patients in their database, 199 died and 197 patients, for whom adequate information was available, were analyzed. Of these, 164 out of 197 patients (83%) died from cardiovascular causes. By far the most common underlying congenital cardiac defects in these patients were shunt lesions with Eisenmenger physiology (48 out of 164; 29%), followed by patients with repaired tetralogy of Fallot (19 out of 164; 10%), patients with univentricular hearts (13 out of 164; 8%) and patients with subaortic right ventricles (10 out of 164; 6%). Mean age at death was 37 ± 15 years, with significant differences between different disease entities. The number of patients with Fontan palliation among those with single ventricle physiology was not reported. The study published by Verheugt et al. in 2011 reports data from the CONCOR Dutch national registry database of adults with CHD. Among 6933 patients in this database, 197 died during a follow-up of 24,865 patient-years at a median age of 49 years (range: 20–91 years). The majority of deceased patients had moderate or severe disease complexity (68%) and 77% died from cardiovascular causes. In all age categories, mortality among CHD patients was higher than in the general Dutch population. The most common individual congenital disease entities among deceased patients were atrial septal defects (34 out of 197; 17%), tetralogy of Fallot (23 out of 197; 12%), aortic stenosis/bicuspid aortic valves (18 out of 197; 9%) and patients with subaortic right ventricles (17 out of 197; 9%). Deaths among patients with univentricular hearts accounted for only 6% of all deaths (12 out of 197). The study does not report the rate of previous surgical repair or palliation among individual disease entities and does not report the number of patients with Eisenmenger physiology or residual cyanosis.
A study published by Nieminen et al. in 2007 reports a population-based analysis of postoperative survival in patients after reparative surgery for CHD. The study showed an increased mortality for CHD patients compared with the general population, particularly early after surgery. Most patients, however, died in childhood and relative survival during adulthood was not specifically analyzed.
Studies on Heart Transplantation in CHD
The registry of the United Network for Organ Sharing (UNOS) does not record original anatomical diagnosis of the underlying congenital cardiac defects. Thus information about type and characteristics of adults with CHD undergoing heart transplantation are derived from single center reports. In all published series, the most frequent congenital heart lesions were single ventricle physiology with or without Fontan palliation, followed by patients with transposition complexes with subaortic right ventricles (complete transposition after atrial switch procedures or congenitally corrected transposition of the great arteries) and patients with repaired or palliated tetralogy of Fallot.
