Left Ventricular Myxoma Occluding the Suprarenal Abdominal Aorta in an Infant.

Myxoma is the most common primary cardiac tumor in adults; however, it is extremely rare in infants. Acute occlusion of the abdominal aorta by a cardiac myxoma is also rare. We report the case of an infant with acute occlusion of the suprarenal abdominal aorta by a left ventricular myxoma. The patient underwent successful catheter embolectomy of the abdominal aorta and surgical resection of the cardiac myxoma. This is a very rare case report of the combination of infantile left ventricular myxoma and acute occlusion of the abdominal aorta.

Preoperative management for tricuspid regurgitation in hypoplastic left heart syndrome.

BACKGROUND: Tricuspid regurgitation (TR) is an important finding in hypoplastic left heart syndrome (HLHS). The aim of the present study was to investigate changes in the degree of TR after the preoperative management of HLHS with mechanical ventilation, and whether the improvement of TR under preoperative management would affect the short-term prognosis of Norwood operation. METHODS: Clinical and echocardiographic data of 55 patients with HLHS who underwent preoperative echocardiography and management at Department of Pediatrics, Okayama University Graduate School of Medicine and Dentistry, were retrospectively reviewed. Preoperative management with mechanical ventilation was performed in 31 of these patients. Based on echocardiography the 55 patients were divided into a trivial-mild TR group (group A) and a moderate-severe TR group (group B). RESULTS: After preoperative management, TR improved in six of the 31 patients and was unchanged in 25; in five of the nine group B patients, TR before management improved to the level of group A after management. In-hospital deaths after surgery included 17 of the 48 patients in group A and five of the six in group B, but there was only one death in the five patients for whom TR improved from group B to group A level. CONCLUSION: Preoperative management of HLHS resulted in an improvement in the degree of TR. The short-term prognosis was better for the patients in group A than those in group B, and so preoperative management is useful for HLHS patients, especially those with moderate or severe TR at admission, with the exception of severe dysplasia of tricuspid valve.

Repair of a dysplastic tricuspid valve using artificial chordae: case report.

Atrioventricular valve repair with artificial chordal replacement has been widely used for congenital and acquired mitral valve abnormalities, but not for tricuspid valve abnormalities. A case is presented of dysplastic tricuspid valve that was successfully repaired using artificial chordae. A 2-year-old female presented with poor weight gain. Echocardiography revealed severe tricuspid regurgitation due to dysplastic tricuspid valve, poor coaptation by prolapse of the anterior leaflet, and tethering of the septal leaflet by short chordae. The prolapsed anterior leaflet was repaired with three pairs of 6-0 expanded polytetrafluoroethylene sutures. The short chordae of the septal leaflet were detached, and the septal and posterior leaflets were sutured together. Trivial tricuspid regurgitation was noted postoperatively. There was no tricuspid regurgitation during the follow up period of three years. The present case provides further evidence that artificial chordal replacement is a useful technique even for small children with congenitally abnormal tricuspid valves.

Coarctation of the thoraco-abdominal aorta associated with mucopolysaccharidosis VII in a child.

Herein we describe a case of atypical coarctation of the thoraco-abdominal aorta associated with mucopolysaccharidosis VII in a 4-year-old girl. Aortography showed diffuse narrowing of the descending aorta. An extra-anatomic bypass was constructed using an 8-mm Dacron graft (Meadox Medicals Inc, Oakland, NJ) between the proximal and distal portion of the descending aorta. Balloon angioplasty was necessary to treat stenoses in the infrarenal abdominal aorta. Two years postoperatively at age 6, aortography revealed no stenosis at the anastomotic sites or in the prosthesis, but the hypoplastic segment of the descending aorta between the anstomoses was completely occluded.

Total right ventricular exclusion improves left ventricular function in patients with end-stage congestive right ventricular failure.

BACKGROUND: We developed a total right ventricular (RV) exclusion procedure for the treatment of isolated congestive RV failure. The objective of the present study was to elucidate the effects of a complete removal of RV volume overload (RVVO) on the surgically created single left ventricle (LV). METHODS AND RESULTS: Three adults (2 arrhythmogenic RV dysplasia, 1 Ebstein) and 5 children (all Ebstein) in NYHA class IV underwent the procedure. The RV free wall was resected from the heart, and the tricuspid orifice was closed. Pulmonary blood supply was obtained by a cavopulmonary connection in 6 patients and a systemic-pulmonary shunt in 2. The LV function was evaluated by 2-dimensional echocardiography 1 month after the surgery. All patients are alive. The paradoxical movement of the interventricular septum and geometry of the LV expressed by its eccentricity (2.1 to 1.2, P<0.01) were normalized after the operation in all 8 patients. LV end-diastolic volumes (59% to 109% of normal value, P<0.01), indexed maximal left atrial area (6.5 to 10.5 cm2/m2, P<0.01), LV ejection fraction (27% to 62%, P<0.01), and cardiac index (2.1 to 3.3 L/min/m2, P<0.05) all significantly increased. CONCLUSIONS: Removal of the RVVO by means of the total RV exclusion procedure provides effective volume loading, restores a cylindrical shape, and improves contractile function of the LV, thus leading to increased systemic output.

Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome.

OBJECTIVE: Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle-pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome. METHODS: Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle-pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 5 patients and 5 mm in 14. RESULTS: All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle-pulmonary artery shunt. Thirteen patients underwent a stage II Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage I) ranged from 26% to 43% (n = 13, mean, 33%). CONCLUSION: Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle-pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.

Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure.

OBJECTIVE: To prevent possible deleterious effects of right ventricular volume overload on cardiorespiratory function, we developed a total right ventricular exclusion procedure for the treatment of end-stage isolated congestive right ventricular failure. METHODS: Since 1996, this procedure has been performed in 5 patients in New York Heart Association functional class IV: 2 adults with arrhythmogenic right ventricular dysplasia and 3 children with Ebstein anomaly. The entire right ventricular free wall was resected along the atrioventricular groove and then parallel to the interventricular septum, sparing the pulmonary valve and a skeletonized right coronary artery. The orifice of the tricuspid valve was closed with either a polytetrafluoroethylene patch or with its leaflets. The defect of the right ventricular free wall was covered with a polytetrafluoroethylene patch in the 2 patients with arrhythmogenic right ventricular dysplasia and directly closed with the remnant of the free wall in the 3 children with Ebstein anomaly. After resection of a redundant right atrial wall, coronary sinus blood flow was rerouted into the left atrium through an atrial septal defect. A total cavopulmonary connection was constructed in 4 patients and a bidirectional superior cavopulmonary anastomosis in 1 infant. The heart was controlled with a DDD pacemaker in 3 patients. RESULTS: The patients were extubated at a mean of 14 hours postoperatively (range, 1-38 hours). There were no early or late deaths. At follow-up, ranging from 8 to 57 months, the mean cardiothoracic ratio had decreased from 74% +/- 7% before the operation to 52% +/- 6% (P <.01). All patients are in functional class I. Neither of the patients with arrhythmogenic right ventricular dysplasia have had attacks of ventricular tachycardia nor are they using antiarrhythmic medication. CONCLUSIONS: The total right ventricular exclusion procedure provides effective decompression of the lung, as well as the left ventricle, and may result in more effective volume loading of a surgically created single ventricle with increased systemic output. We believe that this new surgical option offers rescue treatment for isolated end-stage right ventricular failure in critically ill patients.