Hypoplastic left heart syndrome: Duration of survival without surgical intervention

Background Mortality rate for heart transplantation for patients with hypop lastic left heart syndrome (HLHS) has improved, but there is a considerable wait until a suitable donor is available. Thus it is important to examine the duration of survival and risk factors for early death in patients with HLHS who did not undergo surgical intervention. Methods and Results Twenty-six consecutive patients were studied retrospect ively. Duration of survival and the 14 following variables were investigate d: date of birth, body weight at birth,cardiothoracic ratio, ascending aort a diameter, interatrial communication size, coarctation of the aorta, tricu spid regurgitation, anatomic subtype (patency) of mitral and aortic valve, arterial blood gas Findings (pH, PaO2, SaO(2), PaCO2, base excess), and ST depression in the electrocardiogram. Twenty patients survived <60 days (gro up A) and 6 patients survived beyond 60 days (group B). The duration of sur vival (mean [SD]) was 60 (151) days overall (1 patient is currently alive a t 783 days). The long-term survivors (beyond 60 days) increased significant ly after 1991 (P <.05). Coarctation of the aorta was a significant risk of early death (<60 days) (P <.05). Interatrial communication size was signifi cantly smaller in group B than in group A (P <.05). The mean pH and base ex cess were significantly lower in group A than in group B. The other 9 varia bles showed no significant difference between the 2 groups. Conclusions There was a significant correlation of long-term survival with stabilized ductal blood flow without coarctation of the aorta, adequate res triction of interatrial communication without severe hypoxemia, and no meta bolic acidosis.