Risk factors for aortic valve dysfunction in children with discrete subvalvar aortic stenosis

doi:10.1016/j.amjcard.2004.05.005

The American Journal of Cardiology

Volume 94, Issue 4, 15 August 2004, Pages 459-464

https://doi.org/10.1016/j.amjcard.2004.05.005 Get rights and content

Abstract

Aortic regurgitation (AR) is a known complication of discrete subvalvar aortic stenosis (DSS), and its detection often triggers referral for surgery. However, risk factors for aortic valve dysfunction in children with DSS remain incompletely defined. The primary goal of this study was to determine independent risk factors for moderate or severe AR at mid-term follow-up in patients with DSS. Clinical records and echocardiograms of 220 patients with DSS (109 patients had DSS resection and 111 had no surgery) were analyzed. The primary outcome variable was AR grade (based on the width of the vena contracta) at latest follow-up. Age at diagnosis, gender, and duration of follow-up (median 7.2 years, range 1 to 20.4) did not differ significantly between medical and surgical patients. By multivariate analysis, independent risk factors for moderate to severe AR (n = 30) were older age at diagnosis of DSS (odds ratio [OR] for age ≥17 years 5.13, p = 0.024), previous balloon or surgical aortic valvuloplasty (OR 19.6, p <0.001), and a longer follow-up period (OR for 1-year increase 1.15, p = 0.032). Excluding patients with previous surgical or balloon aortic valvuloplasty, a higher maximal Doppler gradient was an independent risk factor for moderate to severe AR (OR for peak gradient ≥50 mm Hg 10.8, p = 0.001). Independent predictors of low-risk patients (none or trivial AR and peak gradient ≤30 mm Hg) included thin and mobile aortic valve leaflets (OR 7.86, p = 0.006) and an associated ventricular septal defect (OR 2.18, p = 0.019). These clinical and echocardiographic variables can be used to stratify risk of aortic valve dysfunction in patients with DSS and aid in timing of surgical resection.

Section snippets

Subjects

The computer database of the Department of Cardiology, Children's Hospital (Boston, Massachusetts) was searched for all patients with any form of subvalvar aortic stenosis. Patients were included in the study if they fulfilled the following criteria: (1) diagnosis of DSS, (2) normal segmental cardiac anatomy (atrial situs solitus, D-ventricular loop, normally related great arteries, and atrioventricular and ventriculoarterial concordance), (3) ≥2 echocardiographic examinations available for

Subjects

Of the 1,187 patients identified with any form of subvalvar aortic stenosis, 220 patients fulfilled inclusion criteria. There were nearly equal numbers of patients who underwent surgical resection of DSS (n = 109) and those who were followed medically (n = 111). The groups did not significantly differ in terms of gender, age at diagnosis, or duration of follow-up. Table 1 lists demographic and anatomic characteristics of the study cohort. Compared with the medical group, patients who underwent

Discussion

Aortic valve regurgitation has long been recognized as a complication of DSS.6, 7, 8 Surgical and postmortem observations have demonstrated thickening, distortion, and tethering of the valve leaflets by the subvalvar pathology, observations that have led clinicians to recommend early surgical resection of DSS to preserve aortic valve function.6, 9, 10, 11 However, before the advent of high-resolution echocardiography, most patients with DSS were diagnosed when the disease was in advanced

Acknowledgements

We thank Peter Lang, MD, for his valuable comments and Alon Geva for his critical review and editing of the manuscript.

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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines
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2018, Diagnosis and Management of Adult Congenital Heart Disease: Third Edition
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The authors present a case of a 40-year-old female patient with fixed subaortic stenosis (SAS). This defect is classified into a group of diseases characterized by the presence of obstruction in the left ventricular outflow tract, which collectively includes stenotic lesions located in the region extending from the anatomical outflow tract of the left ventricle distally to the descending aorta. Obstructions are named according to their relationship to the aortic valve (subvalvular, valvular, and supravalvular) and their common denominator is an increase in the left ventricular afterload. Fixed subaortic stenosis may be either a focal stenotic lesion formed by a fibrous membrane, or a narrow muscular or fibromuscular tunnel diffusely tapering the outflow tract of the left ventricle. Individuals with SAS usually present with other congenital heart diseases such as a bicuspid aortic valve or perimembranous ventricular septal defect. Surgical resection of SAS is already indicated in asymptomatic individuals with severe SAS (peak gradient >50 mmHg). It is also advisable to consider surgery in some cases of less severe obstruction such as in those with coexistent moderate aortic regurgitation, ventricular septal defect, or in women planning pregnancy. In many cases, however, recurrence of SAS requires secondary surgical treatment. In the focal type of SAS, it is possible to indicate percutaneous balloon dilatation in some cases.
Outcomes of Subaortic Obstruction Resection in Children
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Studies of long-term outcomes of discrete subaortic stenosis are rare. Therefore, we reviewed the long-term outcomes of fibromuscular resection in children with subaortic stenosis over 26 years from a single institution.
We conducted a retrospective review of all children (n=72) who underwent resection of subaortic obstruction for discrete subaortic stenosis between 1989 and 2015.
Median age at surgery was 5.0 years (2.7-7.6 years). There were no operative deaths but three late deaths (4.2%, 3/72). Overall Kaplan-Meier survival at 10 years was 93.0 ± 3.9% (95% CI: 79.6, 97.7). Peak instantaneous left ventricular outflow tract Doppler gradient decreased from 74.2 ± 36.7 mmHg (16.0-242.0 mmHg) preoperatively to 12.8 ± 7.4 mmHg (2.6-36.0 mmHg) postoperatively (p<0.001). Mean left ventricular outflow tract Doppler gradient decreased from 42.4 ± 17.2 mmHg (12.0-98.0) preoperatively to 7.5 ± 2.7 mmHg (1.4-19.3 mmHg) postoperatively (p<0.001). However, over the mean follow-up period of 7.8 ± 6.1 years (0.1-25.2 years), 29.0% (20/69) of patients had recurrence and 18.8% (13/69) required reoperation at median time of 4.8 years (3.1-9.1 years) after the initial repair. Freedom from reoperation at 10 years was 71.1 ± 7.1% (95% CI: 54.6, 82.3). Risk factors for reoperation were age less than five years at initial repair (p=0.036) and extension of the membrane to the aortic valve (p=0.001). Aortic insufficiency was present in 54.2% (39/72) of patients preoperatively. Progression of aortic insufficiency occurred in 38.9% (28/72). Involvement of the aortic valve at initial repair was associated with need for subsequent aortic valve repair or replacement (p=0.01).
Resection of subaortic obstruction is associated with low mortality and morbidity. Recurrence and reoperation rates are high and progression of aortic insufficiency following subaortic resection is common. Therefore, these patients warrant close follow-up into adult life.
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2017, Diagnosis and Management of Adult Congenital Heart Disease
Aortic Stenosis Morphology
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Risk factors for aortic valve dysfunction in children with discrete subvalvar aortic stenosis

Abstract

Section snippets

Subjects

Subjects

Discussion

Acknowledgements

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Am Coll Cardiol

Ann Thorac Surg

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol