Cor Vasa 2002, 43(3):122-127
What is the fate of adults with chronic cyanosis in congenital heart disease?
- 1 Interní klinika 2. lékařské fakulty Univerzity Karlovy
- 2 Dětské Kardiocentrum
- 3 oddělení srdeční chirurgie, Fakultní nemocnice Motol, Praha, Česká republika
Aim:
The study was designed to establish the status and current potential for the treatment of a unique patient population surviving into adulthood with chronic cyanosis in congenital heart disease.
Group of patients:
We retrospectively evaluated 1,106 adults with congenital heart disease, examined at the Adult Congenital Heart Disease Counseling Center at the Department of Internal Medicine, Motol University Hospital, in the 1993-2001 period.
Results:
Overall, 77 (7 %) had cyanotic congenital heart disease with a resting O2 saturation below 90%. They were 40 women (52%) and 37 men with a mean age of 37 ± 11 (19-64) years. The mean resting O2 saturation was 78 ± 12%; average NYHA class was 2.9. Significant pulmonary hypertension was present in 17 patients (22%), restricted pulmonary flow associated with significant pulmonary artery stenosis or atresia in 55 patients (71%). Established diagnoses included a functionally common ventricle 40%, Eisenmenger's syndrome 17%, pulmonary atresia with interventricular septal defect 14%, tetralogy of Fallot without pulmonary artery atresia 10%, Ebstein's tricuspid valve anomaly 8%, corrected transposition of the large arteries with interventricular septal defect and pulomnary artery stenosis 5%, transposition of the large arteries with interventricular or interatrial septal defect 4%; there was one case of total anomalous pulmonary artery return (1%). A total of 19 patients (25%) died during follow-up. Ten patients had non-indicated venipuncture performed. Palliative surgery was undertaken in only a smaller proportion of patients (28; i.e., 36%) in childhood; most patients (49; i.e., 64%) did not undergo any cardiac surgical procedure. Following re-assessment, 19 patients (25%) were scheduled for surgery in adulthood and one for heart transplantation. Among these 20 patients, long-term elimination or improvement of cyanosis, and improvement of the quality of life were achieved in 17 (85% of those undergoing surgery, 22% of the whole group).
Conclusions:
With a well-balanced hemodynamic status and adaptation of the body to chronic hypoxemia, even patients with cyanotic congenital heart defect which had formerly been thought to be inoperable may survive to their middle age. Some of these patients are eligible for surgery improving the quality of their lives.
Keywords: Cyanotic congenital heart defects; Survival until adulthood; Surgical treatment
Published: March 1, 2002 Show citation