Outcomes of uncomplicated aortic valve stenosis presenting in infants.
BACKGROUND
The management of uncomplicated aortic valve stenosis presenting with critical obstruction in infants continues to be associated with significant morbidity and mortality. However, not all infants have critical obstruction, and outcomes spanning the broader spectrum of disease severity are less well defined.


METHODS
In a 12-year period, 55 infants (<3 months of age) were seen with aortic valve stenosis and with anatomy suitable for biventricular repair. Clinical, echocardiographic, angiographic, management, and outcome data were reviewed.


RESULTS
Status at presentation (median age 6 days) included signs of congestive heart failure in 20 patients, cardiovascular collapse in 5 patients, and an asymptomatic heart murmur in 30 patients. The initial echocardiogram showed reduced left ventricular function in 26% of patients, with a mean peak instantaneous gradient of 69 +/- 30 mm Hg in patients with normal function. There were 5 deaths (9%), all in patients with poor ventricular function. The initial intervention was balloon valvotomy in 24 patients and surgical valvotomy in 20 patients, with 11 patients having no intervention to date. The freedom-from-intervention rate was 69% at age 1 week, 58% at 1 month, 36% at 3 months, and 28% at 1 year. Patients without cardiovascular collapse, normal left ventricular function, and gradients <60 mm Hg at presentation (n =1 9) had better survival and longer freedom from intervention than patients with poor ventricular function or gradients >or=60 mm Hg (n = 36, P =.0001).


CONCLUSIONS
Most infants with aortic valve stenosis receive intervention, although this may be safely delayed in selected patients with lower initial gradients and good left ventricular function.
Baram
Shaul
S
Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
McCrindle
Brian W
BW
Han
Ra K
RK
Benson
Lee N
LN
Freedom
Robert M
RM
Nykanen
David G
DG
D001021:Aortic Valve / Q000503:physiopathology / Q000601:surgery*; D001024:Aortic Valve Stenosis / Q000150:complications / Q000601:surgery / Q000628:therapy*; D002404:Catheterization / Q000379:methods* / Q000401:mortality; D002985:Clinical Protocols; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D018487:Ventricular Dysfunction, Left / Q000401:mortality / Q000503:physiopathology