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J Thorac Cardiovasc Surg 2007;133:572-573
© 2007 The American Association for Thoracic Surgery
Brief Communication |
University of Kansas Hospital, Kansas City, Kan.
* Address for reprints: James L. Vacek, MD, 3901 Rainbow Blvd, MS 4023, Kansas City, KS 66160. (Email: jlvacek{at}mac.md).
Congenital mitral stenosis is an uncommon congenital heart defect, typically presenting with symptoms in early childhood.1,2
This condition remains a surgical challenge, with a trend toward early single-stage complete repair.3 We present the case of a 15-year-old patient given a diagnosis of severe mitral stenosis and pulmonary hypertension. The patient underwent successful complex mitral valve repair with mitral valve ring annuloplasty and neochordae placement and achieved dramatic improvement in symptoms and quality of life.
A 15-year-old boy with a history of asthma was transferred to our hospital with apparent status asthmaticus. He was initially treated for a 5-lobe pneumonia with intravenous antibiotic therapy. He was noted to have a loud holodiastolic murmur, best heard at the left lower sternal border. An echocardiogram was obtained that showed severe mitral stenosis (pressure half-time, 284 cm/s; mitral valve area, 0.8 cm2), significant tricuspid regurgitation, and an estimated systolic right ventricular pressure in excess of 100 mm Hg. His congestive heart failure responded favorably to diuresis; however, his shortness of breath continued, and he had difficulty attending school.
Follow-up echocardiography indicated a significant decrease in estimated right ventricular and pulmonary artery systolic pressure (60-65 mm Hg). Severe mitral stenosis was again evident, along with significant tricuspid valve regurgitation. His pressure half-time was 238 ms, with a peak gradient of 49 mm Hg and an estimated mitral valve area of 0.9 cm2. The posterior leaflet was tethered to the free wall of the left ventricle, and the anterior leaflet was tethered to the tips of the papillary muscles, with essentially no chordae tendineae. The leaflets were normal in appearance. The valve orifice was fixed and severely restrictive.
At the time of the operation, the echocardiographic diagnoses were confirmed. The patient underwent a complex mitral valve repair within weeks of his initial presentation by using the following techniques: (1) transection of trapped chordae tendineae to both the anterior and posterior mitral valve leaflets from both papillary muscles, leaving normal-sized and normal-appearing pliable leaflets without any chordae tendineae; (2) mitral valve ring annuloplasty with a no. 26 Cosgrove annuloplasty ring; and (3) placement of multiple neochordae (5-0 Gore-Tex polytetrafluroethylene sutures; W.L. Gore and Associates, Flagstaff, Ariz), 2 to the anterior leaflet and 3 to the posterior leaflet. Neochordae were used because of the congenital absence of functional chordae.
After the procedure, the patient had a dramatic reduction in tricuspid regurgitation. He was able to resume activities normal for an adolescent boy without shortness of breath. A postoperative echocardiogram was obtained. The echocardiogram demonstrated no residual mitral stenosis and a trivial jet of mitral valve regurgitation. The Doppler estimated right ventricular systolic pressure was 49 mm Hg. Moderate tricuspid valve regurgitation was noted. Mitral valve flow on Doppler scanning was 178 cm/s. Our patient continued to be asymptomatic at 1-year follow-up.
In this case the mitral stenosis resulted from subvalvular fusion and marked foreshortening of the chordae tendineae. This tethered the anterior leaflet to both papillary muscles and the posterior leaflet to the posterior wall of the left ventricle, resulting in a severe conical fixed and restricted valve orifice. This was believed not to be rheumatic because the leaflets were completely normal.
The variety of congenital mitral valve abnormalities ranges from repairable cleft leaflets to restrictive and challenging lesions of isolated mitral stenosis.3 Most patients with isolated congenital mitral stenosis have severe symptoms in early childhood.1,2 These patients often require early surgical management. When symptoms are mild or even moderate, the operation is delayed in the hope that in the future an adult-sized device can be used.1 In patients with congenital mitral valve disease, reconstructive surgery is the primary goal. When necessary, valve replacement can be performed safely with relatively low morbidity and mortality and can provide relief of symptoms for many patients.1,2,4 However, mitral valve repair is preferable when feasible to limit implantation of prosthetic material and reduce the need for future reoperation. Also, mitral valve repair reduces the need for long-term anticoagulation and might provide more physiologic correction of the lesion. Our report is notable because of the novel use of neochordae to both leaflets for the repair.
References
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