Surgery for congenital mitral valve disease in the first year of life

Abstract

Between 1980 and 1993, 20 patients less than 1 year of age underwent operations for congenital mitral valve disease. Ten patients had congenital mitral incompetence and 10 had congenital mitral stenosis. Mean age was 6.6 +/- 3.4 months and mean weight was 5.6 +/- 1.5 kg. Atrioventricular canal defects, univentricular heart, class III/IV hypoplastic left heart syndrome, discordant atrioventricular and ventriculoarterial connections, and acquired mitral valve disease were excluded. Indications for operation were intractable heart failure or severe pulmonary hypertension, or both. Associated lesions, present in 90% of the patients, had been corrected by a previous operation in seven. In congenital mitral incompetence there was normal leaflet motion (n = 3), leaflet prolapse (n = 2), and restricted leaflet motion (n = 5). In congenital mitral stenosis anatomic abnormalities were parachute mitral valve (n = 4), typical mitral stenosis (n = 3), hammock mitral valve (n = 2), and supramitral ring (n = 1). Mitral valve repair was initially performed in 19 patients and valve replacement in one with hammock valve. Concurrent repair of associated lesions was performed in 12 patients. The operative mortality rate was zero. There were six early reoperations in five patients for mitral valve replacement (n = 4), a second repair (n = 1), and prosthetic valve thrombectomy (n = 1). One late death occurred 9 months after valve replacement. Late reoperations for mitral valve replacement (n = 2), aortic valve replacement (n = 1), mitral valve repair (n = 2), subaortic stenosis resection (n = 1), and second mitral valve replacement (n = 1) were performed in five patients. Actuarial freedom from reoperation is 58.0% +/- 11.3% (70% confidence limits 46.9% to 68.9%) at 7 years. After a mean follow-up of 67.6 +/- 42.8 months, 94% of living patients are in New York Heart Association class I. Doppler echocardiographic studies among the 13 patients with a native mitral valve show mitral incompetence of greater than moderate degree in one patient and no significant residual mitral stenosis. Overall, six patients have mitral prosthetic valves with a mean transprosthetic gradient of 6.2 +/- 3.7 mm Hg. These results show that surgical treatment for congenital mitral valve disease in the first year of life can be performed with low mortality. Valve repair is a realistic goal in about 70% of patients and possibly more with increased experience. Reoperation rate is still high and is related to complexity of mitral lesions and associated anomalies, but late functional results are encouraging.

Balloon dilatation of congenital mitral stenosis in a critically ill infant

“…Upon transfer, 2D and Doppler echo examination revealed severely restricted mobility and opening of the MV leaflets with concomitant LV inflow obstruction on color Doppler. Two closely spaced papillary muscles with short chordal attachments to the mitral leaflets were noted, conforming to the features described as typical symmetric hypoplastic mitral valve [5]. The mitral annulus measured 13 mm in diameter.…”

“…Mitral valve repair procedures such as surgical commisurotomy and valve replacement in children carry inordinately high risks, especially in infants [7], although among the more recent series Uva et al [5] reported zero operative mortality for congenital mitral valve anomalies operated during infancy. Despite improved operative mortality, however, late morbidity continues to limit the success of surgical management in this disease.…”

Using hospital mortality rates to judge hospital performance: a bad idea that just won't go away

“…Comparing practices in different places has provided crucial insights, from Semmelweis's discovery of antisepsis to more recent evidence about the optimal age for surgery to repair congenital mitral valve disease. 21 However, controlled studies of this type are entirely different from single site investigations called down on organisations by regulatory bodies. In Mid Staffordshire "concerns about mortality" prompted first the HCC investigation, 22 then two reviews commissioned by the Department of Health, 23 24 and finally an independent inquiry set up by the secretary of state for health.…”

Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

“…In Carpentier's series 3 , the age of the congenital mitral insufficiency group was 6.1±3.2 years and in the congenital mitral stenosis group 5.1±3.2 years. In the report by Uva and colleagues 11 , from MarieLannelongue Hospital, the congenital mitral insufficiency group was 7.4±2.7 months old, and the congenital mitral stenosis group was 5.8±3.9 months old. The factors that explain the lower age of the congenital mitral stenosis patients at the time of surgery are 1) the stenotic lesion is less tolerated than the insufficiency; 2) the association with higher and more severe intracardiac anomalies; 3) the abnormalities of the subvalvular apparatus are more frequent in these patients.…”