Outcomes of uncomplicated aortic valve stenosis presenting in infants

doi:10.1016/S0002-8703(03)00090-5

American Heart Journal

Volume 145, Issue 6, June 2003, Pages 1063-1070

https://doi.org/10.1016/S0002-8703(03)00090-5 Get rights and content

Abstract

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 ≥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.

Section snippets

Study population

From January 1985 to December 1996, 62 consecutive infants with isolated aortic valve stenosis who were <3 months of age were identified retrospectively from the Cardiology Database of the Division of Cardiology of the Hospital for Sick Children, Toronto, Ontario, Canada. To isolate the series to those patients optimally suited for a biventricular repair, we excluded patients with the following associated diagnoses: hypoplastic left heart syndrome, complex left ventricular outflow obstructions,

Patient characteristics

We identified 55 patients (45 male infants, 10 female infants) who met criteria for inclusion in this study. The median age at presentation was 6 days (range, birth to 2.7 months), and symptoms included a murmur only in 30 patients (55%), signs of congestive failure in 20 patients (36%), and poor perfusion or shock in 5 patients (9%). On the initial electrocardiogram (n = 53), 21 patients (40%) had results that were normal for their age, 22 patients (42%) had left ventricular hypertrophy, 8

Discussion

Only an estimated 10% to 15% of all patients with congenital aortic valve stenosis present within the first year of life.18 However, when neonates and infants do present with aortic valve stenosis, they face significant morbidity and mortality. From several natural history studies,14, 15 patients who are treated with medical therapy were found only to have an estimated 25% mortality rate, primarily because of ventricular failure. Interventions with transcatheter balloon dilation and surgical

References (42)

G.K. Lofland et al.
Critical aortic stenosis in the neonatea multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society
J Thorac Cardiovasc Surg
(2001)
M.P. Leung et al.
Critical aortic stenosis in early infancyanatomic and echocardiographic substrates of successful open valvotomy
J Thorac Cardiovasc Surg
(1991)
B.W. McCrindle
Independent predictors of immediate results of percutaneous balloon aortic valvotomy in children
Am J Cardiol
(1996)
M. Choy et al.
Percutaneous balloon valvuloplasty for valvar aortic stenosis in infants and children
Am J Cardiol
(1987)
J.J. Wheller et al.
Results of operation for aortic valve stenosis in infants, children, and adolescents
J Thorac Cardiovasc Surg
(1988)
A.G. Magee et al.
Balloon dilation of severe aortic stenosis in the neonatecomparison of anterograde and retrograde catheter approaches
J Am Coll Cardiol
(1997)
R.N. Justo et al.
Aortic valve regurgitation after surgical versus percutaneous balloon valvotomy for congenital aortic valve stenosis
Am J Cardiol
(1996)
R.T. Fedderly
Left ventricular outflow obstruction
Pediatr Clin North Am
(1999)
G.K. Lofland et al.
Critical aortic stenosis in the neonatea multi-institutional study of management, outcomes, and risk factors
J Thorac Cardiovasc Surg
(2001)
S.R. Gundry et al.
Prognostic factors in valvotomy for critical aortic stenosis in infancy
J Thorac Cardiovasc Surg
(1986)

H.P. Gildein et al.

Surgical commissurotomy of the aortic valveoutcome of open valvotomy in neonates with critical aortic stenosis

Am Heart J

(1996)

T.R. Karl et al.

Critical aortic stenosis in the first month of lifesurgical results in 26 infants

Ann Thorac Surg

(1990)

R.H. Beekman et al.

Balloon valvuloplasty for critical aortic stenosis in the newborninfluence of new catheter technology

J Am Coll Cardiol

(1991)

E.S. Egito et al.

Transvascular balloon dilation for neonatal critical aortic stenosisearly and midterm results

J Am Coll Cardiol

(1997)

O. Galal et al.

Follow-up results of balloon aortic valvuloplasty in children with special reference to causes of late aortic insufficiency

Am Heart J

(1997)

P. Moore et al.

Midterm results of balloon dilation of congenital aortic stenosispredictors of success

J Am Coll Cardiol

(1996)

J.A. Ettedgui et al.

Long-term results of survivors of surgical valvotomy for severe aortic stenosis in early infancy

J Thorac Cardiovasc Surg

(1992)

D. Kitchiner et al.

The progression of mild congenital aortic valve stenosis from childhood into adult life

Int J Cardiol

(1993)

R.S. Mosca et al.

Critical aortic stenosis in the neonatea comparison of balloon valvuloplasty and transventricular dilation

J Thorac Cardiovasc Surg

(1995)

J.A. Hawkins et al.

Late results and reintervention after aortic valvotomy for critical aortic stenosis in neonates and infants

Ann Thorac Surg

(1998)

E.P. Bauer et al.

Valvotomy for isolated congenital aortic stenosis in childrenprognostic factors for outcome

Thorac Cardiovasc Surg

(1992)

Cited by (22)

Contemporary experience with surgical treatment of aortic valve disease in children
2013, Journal of Thoracic and Cardiovascular Surgery
Surgical treatment of aortic valve (AoV) disease in childhood involves complex decisions particularly in very small patients. There is no consensus regarding the optimum surgical option. The objective of this review was to analyze a contemporary experience of AoV surgery in a large children's hospital.
A retrospective review of children (aged ≤18 years) undergoing AoV repair or replacement from June 1995 to December 2011 was carried out.
A total of 285 AoV operations (97 repairs, 188 replacements) were performed on 241 patients. Hospital survival for repair was 98% and for replacements was 97%. At follow-up of repairs, there were 16 (17%) reoperations and 3 (3%) late deaths. Follow-up of AoV replacements demonstrated 31 (16%) reoperations (homograft 27, autograft 3, mechanical 1) and 8 (4%) late deaths (homograft 5, autograft 2, mechanical 1). Freedom from reintervention or death (FRD) was found to be lower in repairs for infants (P = .048) and truncal valves (P < .05). For AoV replacements, infants and patients who had concomitant CHD or homografts (P < .0001) had lower FRD. Cox regression analysis for AoV replacements identified infants and homograft root replacements at a higher risk for death/reoperation.
AoV repairs and replacements were generally found to be associated with low death and reoperation rates at long-term follow-up. Infants had a lower freedom from reintervention or death after either an AoV repair or replacement, although truncal valve repairs and AoV replacement in patients with concomitant CHD were associated with lower valve survival. Among the valve options, homograft root replacement had a higher risk of death/reoperation and lowest freedom from reintervention or death.
Outcome of Surgical Commissurotomy for Aortic Valve Stenosis in Early Infancy
2007, Annals of Thoracic Surgery
Citation Excerpt :
The median peak systolic echo Doppler gradient reduction in our study group, 68 mm Hg, is comparable with what other studies have found. The reported reduction range is 25 to 52 mm Hg in surgically treated patients and 28 to 64 mm Hg in balloon-dilated patients [9, 13, 16–18]. We find that the gradient after open surgical commissurotomy keeps very well over time in most cases (Fig 2), in accordance with the findings of Hawkins and colleagues [5].
The method of treatment of aortic valve stenosis in early infancy is still controversial. This study was performed to evaluate short-term and long-term outcome in our center during a 14-year period.
Between 1991 and 2004, 64 consecutive patients younger than 3 months old underwent open surgical commissurotomy because of aortic valve stenosis. Median age was 18 days (range, 1 to 79 days), and median weight was 3.6 kg (range, 1.9 to 6.7 kg). Left ventricular function was good in 44 patients (69%), depressed in 12 (19%), and poor in 8 (12%). The study ended in July 2005. Median follow-up time was 4.1 years (range, 0.4 to 13.6 years).
The 30-day mortality was 3 of 64 patients and late mortality was 3 of 61, and the respective mortality in patients younger than 1 month old was 2 of 41 and 2 of 39. There was no early mortality after 1993 and no late mortality after 1999. Thirteen patients required reoperation. Median time to reoperation was 4.3 years (range, 0.2 to 11.3 years) and to aortic valve replacement (7 Ross and 1 homograft) was 6.9 years (range, 1.6 to 9.7 years). At the last follow-up, all had good left ventricular function and 57 of 58 had an ability index of 1.
Surgical commissurotomy for aortic valve stenosis during the first 3 months of life can be done with low mortality and morbidity. The risk for early recurrent stenosis or regurgitation is low, and the need for aortic valve replacement can, in most cases, be delayed until the child is older. The long-term functional ability is excellent.
Aortic Outflow Abnormalities
2006, Nadas' Pediatric Cardiology
Management options in neonates and infants with critical left ventricular outflow tract obstruction
2007, European Journal of Cardio-thoracic Surgery
Twenty years experience of surgical aortic valvotomy for critical aortic stenosis in early infancy
2006, European Journal of Cardio-thoracic Surgery
The influence of valve physiology on outcome following aortic valvotomy for congenital bicuspid valve in children: 30-Year results from a single institution
2005, European Journal of Cardio-thoracic Surgery

View all citing articles on Scopus

View full text

Clinical investigations: valvular and congenital heart diseaseOutcomes of uncomplicated aortic valve stenosis presenting in infants

Abstract

Background

Methods

Results

Conclusion

Section snippets

Study population

Patient characteristics

Discussion

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Am J Cardiol

Am J Cardiol

J Thorac Cardiovasc Surg

J Am Coll Cardiol

Am J Cardiol

Pediatr Clin North Am

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Am Heart J

Ann Thorac Surg

J Am Coll Cardiol

J Am Coll Cardiol

Am Heart J

J Am Coll Cardiol

J Thorac Cardiovasc Surg

Int J Cardiol

J Thorac Cardiovasc Surg

Late results and reintervention after aortic valvotomy for critical aortic stenosis in neonates and infants

Ann Thorac Surg

Valvotomy for isolated congenital aortic stenosis in childrenprognostic factors for outcome

Thorac Cardiovasc Surg

Clinical investigations: valvular and congenital heart disease
Outcomes of uncomplicated aortic valve stenosis presenting in infants