Myotonic dystrophy (MyD) is a rare progressive multisystemic-inherited neuromuscular disease. It is often associated with cardiovascular disorders which require surgical procedures. However, it high sensitivity to anesthetic and neuromuscular blocking agents may result in respiratory complications. Myotonic dysorder due to hypothermia and conductive disorder following open-heart surgery are also risk-factor of perioperative management, thus open-heart surgery for MyD has rarely been reported. We describe the perioperative management for a MyD patient with an atrial septal defect (ASD) and mitral regurgitation (MR), who successfully underwent cardiac surgery. Minimally invasive cardiac surgery (MICS) with reverse-L-shaped partial sternotomy were useful method to minimize postoperative respiratory problems.

A 60-year-old woman was admitted because of dyspnea and a cough. Computed tomography and transthoracic echocardiography showed a tumor in the left atrium. However, transesophageal echocardiography alone could show the tumor and its extension in the interior wall of the left atrium. Peripheral blood chemistry showed a high CA125 level. The first operation was carried out in order to perform a complete resection of the tumor which was 3.5×4.0×2.0cm, but the interior wall of the left atrium seemed normal. The CA125 level returned to within a normal range 80 days after the first operation. Histopathology showed the tumor had myxomatous changes and ring structure formations, but malignancy was also suspected. Transthoracic echocardiography performed 14 months after the first surgery showed a recurrence of the tumor, and subsequent transesophageal echocardiography showed the tumor and its invasion in the interior wall of the left atrium. A second operation was performed to resect the tumor, which had invaded a part of the left atrial interior wall. The histopathology showed the tumor was myxoid but had mitoses and foci of necroses. This tumor was consistent with a myxosarcoma. The patient died as a result of a recurrent tumor blocking the left atrium 20 months after the first surgery.

A 13-year-old girl with congenital mitral incompetence had undergone valvoplasty using the De Vega technique at age 5. The patient was referred by the pediatric department due to recurrence of mitral incompetence. Transesophageal echocardiography indicated regurgitation from A2 and P3, mild mitral leaflet tethering and left ventricular dilatation. Intraoperative findings showed valvular agenesis of the posterior leaflet around P3. No leaflet prolapse was observed at A2, but leaflet P2 had fallen to the left ventricular side compared with leaflet A2, thereby inducing regurgitation due to coaptation gap. In a procedure similar to folding plasty, leaflet P3 was folded down and sutured to the annulus extending up to the posteromedial commissure. This technique not only controlled regurgitation at P3 but also improved the coaptation between A2 and P2. Annuloplasty was conducted using a 28-mm Physio-ring. Folding plasty may be an effective surgical option for patients with congenital mitral incompetence because a broad valve orifice area can be maintained because there is no need for annular plication.

Recently, the demand for better cosmetic outcomes in pediatric cardiovascular operations has been growing. Between May 1998 and April 1999, six children aged 2 to 6 years with an ostium secundum type of atrial septal defect underwent reparative operations that used an approach consisting of a lower mid-line skin incision with full sternotomy. A 4.2-5.8cm vertical skin incision (mean, 4.9±0.3cm) was made between the level of the nipple and the xyphoid process. Comparison between this series and a group of weight-matched patients who underwent conventional operations revealed no significant differences in operation time (166.0±12.0vs. 147±8.4min), cardiopulmonary bypass time (33.2±4.0vs. 32.2±2.4min), aortic cross-clamp time (13.8±2.3vs. 12.3±1.3min), or the reduction in the hemoglobin concentration in blood on the first postoperative day (1.7±0.3vs. 2.9±0.6g/dl). The surgical wound was not associated with any complications in our series, including wound infection or subcutaneous hematoma. Our technique appears to be safe and provide satisfactory cosmetic outcome.

Here we present a long-term follow-up of 50 operative survivors, who underwent surgery between December 1975 and March 1994 for the placement of an extracardiac conduit. Twenty-six patients received conduits with various valves (VC group). The valves used were the Hancock valve in 9 patients, the St. Jude Medical valve in 5, and a valved roll made of equine pericardium in 10. Twenty-four patients received valveless Dacron conduits (NVC group). Another group of patients, also with discontinuity between the right ventricle and the pulmonary artery, who were operated on without the use of a conduit, is presented here for comparison (NCR group: 16 patients). The follow-up period for the NCR group was shorter than for the other groups. There were a total of 4 late deaths in the conduit groups, and none in the NCR group. Freedom from reoperation due to conduit stenosis was analyzed by the Kaplan-Meier method. In the VC group, freedom from reoperation at 5, 10, and 15 years, was 87.8%, 50.8%, and 31.2% respectively. In the NVC group, freedom from reoperation at 5, 10, and 15 years was 100%, 95.7%, and 60.4%. There were statistically significant differences between the values in these 2 groups. In the NCR group, only one patient (6.25%) underwent reoperation due to stenosis in the right ventricular outflow tract. Although the rate of freedom from reoperation was lower in the valveless conduit group than in the valved conduit group, the majority of patients who receive a conduit between the right ventricle and the pulmonary artery will eventually require reoperation. Avoiding the use of an extracardiac conduit, and creating continuity between the right ventricle and pulmonary artery with autologous tissue is a useful alternative and may reduce the need for reoperation.