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Outcomes of uncomplicated aortic valve stenosis presenting in infants
Shaul Baram1, Brian W McCrindle, Ra K Han
1Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Infants with aortic valve stenosis (AVS) often require intervention, but those without severe symptoms or with good heart function may safely delay treatment. This study clarifies outcomes across the AVS severity spectrum in infants.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Echocardiography
Background:
- Aortic valve stenosis (AVS) in infants can lead to severe complications.
- Outcomes for infants with less critical AVS are not well-defined.
Purpose of the Study:
- To define the spectrum of disease severity and outcomes in infants with aortic valve stenosis.
- To identify predictors of intervention and survival.
Main Methods:
- Retrospective review of 55 infants (<3 months) with AVS over 12 years.
- Analysis of clinical, echocardiographic, and management data.
Main Results:
- 20 infants had heart failure, 5 cardiovascular collapse, 30 asymptomatic murmurs.
- 5 deaths (9%) occurred, all with poor ventricular function.
- Patients with lower gradients (<60 mm Hg) and normal ventricular function had better outcomes.
Conclusions:
- Most infants with AVS undergo intervention.
- Delayed intervention is safe for selected infants with good ventricular function and lower gradients.
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).
Conclusion:
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.