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Echocardiographic follow-up of children with isolated discrete subaortic stenosis
Kadir Babaoglu1, Ayse Guler Eroglu, Funda Oztunç
1Department of Pediatrics, Division of Pediatric Cardiology, Kocaeli University Medical Faculty, Izmit/Kocaeli, Turkey. babaogluk@yahoo.com
Insights
This study on pediatric subaortic stenosis (SAS) found that stenosis and aortic regurgitation (AR) often progress over time, necessitating careful monitoring and timely surgical intervention for moderate cases.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Echocardiography
Background:
- Isolated discrete subaortic stenosis (SAS) is a congenital heart defect requiring evaluation of its progression and impact.
- Aortic regurgitation (AR) is a common complication of SAS, influencing patient outcomes and management strategies.
- Understanding the natural history of SAS and AR is crucial for optimizing treatment decisions in pediatric patients.
Purpose of the Study:
- To evaluate the progression of stenosis severity in children with isolated discrete SAS.
- To assess the onset and progression of aortic regurgitation (AR) in this patient cohort.
- To analyze surgical outcomes for pediatric patients undergoing intervention for SAS.
Main Methods:
- Retrospective review of medical records for 108 children diagnosed with isolated discrete SAS.
- Echocardiographic assessment of peak systolic instantaneous gradient (PSIG) to quantify stenosis severity.
- Longitudinal follow-up data analysis for patients managed medically and surgically, including AR assessment.
Main Results:
- A significant increase in mean PSIG was observed in medically managed patients over time (p < 0.001).
- Progression of stenosis was noted in patients initially classified with very mild, mild, and moderate SAS.
- Aortic regurgitation (AR) prevalence increased from 50% at initial evaluation to 77% during follow-up; 91% of surgical patients had AR preoperatively.
Conclusions:
- Pediatric patients with isolated discrete SAS demonstrate significant progression of stenosis and development/worsening of AR over time.
- Annual noninvasive follow-up is recommended for patients with very mild or mild SAS.
- Patients with moderate SAS require careful evaluation for potential surgical intervention due to the risk of progression to severe stenosis and moderate AR.
Abstract:
This study evaluates the progression of stenosis, onset and progression of aortic regurgitation (AR), and the results of surgical outcomes in children with isolated discrete subaortic stenosis (SAS). The medical records of 108 patients (mean age, 5.5 +/- 3.8 years; range, 3 days to 18 years) with isolated discrete SAS were reviewed. Patients with lesions other than AR were excluded. Very mild stenosis was defined as Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25-49 mmHg, moderate stenosis as 50-75 mmHg, and severe stenosis as more than 75 mmHg. Seventy-eight of 108 patients were followed for 2 months to 14 years (mean, 4.8 +/- 3.7 years; median, 5 years) with medical treatment alone. In these patients, the mean PSIG at last echocardiogram was higher than the mean PSIG at initial echocardiogram (39 +/- 19 vs 31 +/- 12 mmHg, respectively; p < 0.001). Among 24 patients with very mild stenosis at initial echocardiogram, 10 had mild and 2 had moderate stenosis after a mean period of 5.6 years. Among 46 patients with mild stenosis at initial echocardiogram, 11 had moderate and 5 had severe stenosis after a mean period of 4.1 years. Only 1 patient among the 8 patients with moderate stenosis at initial echocardiogram had severe stenosis after a mean period of 2.7 years. Thirty-nine patients (50%) had AR (13% trivial, 33% mild, and 4% moderate) at initial echocardiogram. After a mean period of 4.8 years, 77% of the patients had AR (10% trivial, 53% mild, 9% mild-moderate, and 5% moderate). Twenty-four patients underwent surgery. Preoperatively, mean Doppler PSIG and AR incidence were 64 +/- 17 mmHg and 91% (22/24), respectively. The mean Doppler PSIG was 30 +/- 19 mmHg and AR was present in all of the patients a mean period of 4.1 years after surgery. Two patients underwent reoperation for recurrent SAS and AR. Patients with very mild or mild stenosis may be followed noninvasively every year. One patient of the 8 patients with moderate stenosis progressed to severe stenosis, and moderate AR developed in 2 patients after a mean of 2.7 years. We recommend that patients with moderate stenosis undergo careful evaluation to determine whether surgery is necessary due to the severity of stenosis and AR.
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