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Operative treatment of hypertrophic obstructive cardiomyopathy and aortic valve disease in infants
J A van Son1, J Hambsch, T Bossert
1Herzzentrum, University of Leipzig, Germany.
Insights
This study combined septal myectomy with the Ross-Konno procedure in infants with severe hypertrophic obstructive cardiomyopathy (HOCM) and aortic valve disease. The novel approach improved left ventricular function and resolved mitral valve issues, offering a valuable technique for complex pediatric cardiac cases.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Congenital Heart Disease
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) in infants is rare, with poorly defined surgical indications.
- Traditional septal myectomy may be insufficient for HOCM with concentric left ventricular hypertrophy and aortic valve disease.
Purpose of the Study:
- To evaluate a combined surgical approach for severe HOCM in infants.
- To improve outcomes in pediatric patients with HOCM, left ventricular hypertrophy, and aortic valve disease.
Main Methods:
- Three infants underwent combined septal and free wall resection with a Ross-Konno procedure.
- The procedure addressed left ventricular hypertrophy, aortic stenosis/regurgitation, and systolic anterior mitral valve motion.
Main Results:
- Significant reduction in left ventricular septum and free wall thickness.
- Increased left ventricular end-diastolic volume and improved diastolic function.
- Resolution of systolic anterior mitral valve motion and elimination of outflow tract gradients.
Conclusions:
- The combined Ross-Konno and resection procedure is a valuable technique for severe pediatric HOCM with aortic valve disease.
- This surgical approach enhances left ventricular function, restores aortic valve function, and resolves mitral valve abnormalities.
Background:
The indications for operative intervention for symptomatic hypertrophic obstructive cardiomyopathy (HOCM) in infancy and childhood are not well defined because of the rarity of the lesion. The traditional surgical procedure consists of septal myectomy. In an attempt to further improve the outcome of HOCM associated with concentric left ventricular hypertrophy and aortic valve disease in infancy, we have combined resection of the left ventricular septum and free wall with a Ross-Konno procedure.
Methods:
Three infants (aged 3, 4, and 10 months) with HOCM (left ventricular aortic gradients of 75, 95, and 110 mmHg), associated concentric left ventricular hypertrophy, and valvar aortic stenosis (n = 1) or combined valvar aortic stenosis and regurgitation (n = 2) underwent extensive resection of fibroelastosis and subendocardial myocardium of the left ventricular septum and free wall in combination with a Ross-Konno operation. All three patients had marked systolic anterior motion of the mitral valve. The length of the incision into the ventricular septum was 1.8, 2.0, and 2.3 cm.
Results:
In all three patients this procedure resulted in a marked reduction of width of the left ventricular septum (median 9 mm vs 14 mm preoperatively) and the left ventricular posterior free wall (median 8 mm vs 12 mm preoperatively) and an almost twofold increase of the left ventricular end-diastolic volume (median 13.5 cm3 vs 7.0 cm3 preoperatively). The neo-aortic valve functioned normally. Systolic anterior motion of the anterior leaflet of the mitral valve had completely resolved in two patients and had markedly regressed in the remaining patient. At follow-up of 15, 17, and 26 months, two patients had absence of a left ventricular outflow tract gradient and the third patient had a residual sub-valvar gradient of 15 mmHg.
Conclusions:
The reported procedure may be a valuable technique in severe forms of hypertrophic cardiomyopathy associated with aortic valve disease. The operation results in enlargement of the left ventricular stroke volume and improvement of the left ventricular diastolic function, restores aortic valve anatomy and function, and abolishes or decreases systolic anterior motion of the mitral valve.