Naoki Wada

Single ventricle repair in children with Down's syndrome.

ObjectiveThere is a paucity of information regarding appropriate management of children with Down’s syndrome and a functional single ventricle. We report the results of bidirectional superior cavopulmonary shunts in six patients with Down’s syndrome with a functional single ventricle.MethodsBetween January 1991 and December 2004, we identified six patients with Down’s syndrome among 263 who had undergone bidirectional superior cavopulmonary shunts (BCPSs). There were four males and two females. The age at BCPS ranged from 1 to 12 years (mean 4.3 ± 3.9 years), and body weight varied between 8.2 and 29.4 kg (mean 13.8 ± 7.8 kg). All six patients had an unbalanced complete atrioventricular septal defect, with right ventricular hypoplasia present in five and left ventricular hypoplasia in one.ResultsThere were no operative deaths, but one case required takedown of the BCPS. Except for this case, postoperative courses were generally uneventful. The median duration of follow-up was 46 months (range 12–80 months). Only two of five survivors after BCPS underwent a subsequent Fontan procedure, and one of these patients died of pulmonary hypertension post-operatively. The remaining three patients appeared to have significant risk factors for the Fontan procedure due to severe common atrioventricular valve regurgitation or persistent pulmonary vascular obstructive disease, including one who has completely dropped out from the Fontan track.ConclusionDown’s syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down’s syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully.

Bilateral pulmonary artery banding for hypoplastic left heart syndrome and related anomalies

ObjectiveBilateral pulmonary artery banding is considered an option for initial palliation in high-risk patients with hypoplastic left heart syndrome or related anomalies. However, there are potential interim morbidities, including ductal constriction, compromised growth of the ascending aorta, and pulmonary artery stenosis at the banding site. In addition, there are still controversies regarding the optimal timing and choice of the second-stage operation.MethodsBetween February 2003 and October 2005, twelve high-risk patients with hypoplastic left heart syndrome or related anomalies underwent bilateral pulmonary artery banding. An atrial septal defect was created simultaneously when the septum was intact or the defect was restrictive. After the procedure, intravenous prostaglandin E1 was continuously administered.ResultsThe median weight was 3.0 kg (range 1.8–3.6 kg), and the median age was 16 days (range 0–27 days). Atrial septum defect creation was performed simultaneously in two patients. Ten of the twelve patients were discharged from the hospital. One patient died of progressive multiple organ failure and another patient died of cerebral hemorrhage. Eight patients developed various degrees of ductal constriction and required dose adjustment of prostaglandin E1. Two patients underwent ductal arteriosus stent placement: one was unsuccessful owing to the size mismatch between the stent and the ductus. The diameter of the ascending aorta remained unchanged until the time of the second-stage operation, except in two patients who had a sufficiently sized aorta at the time of pulmonary artery banding. Pulmonary arterial branch stenosis developed at the banding site in three patients. At the time of the second-stage operation, the Norwood procedure was performed in seven patients, biventricular repair in two, and Norwood plus Glenn procedure in one.ConclusionBilateral pulmonary artery banding is an option to salvage high-risk neonates with hypoplastic left heart syndrome or related anomalies. However, it is associated with high interstage morbidity, and patients may benefi t from early conversion to the Norwood operation.

Outcomes following surgical repair of aortic arch obstructions with associated cardiac anomalies

OBJECTIVE To assess outcomes of surgical repair of aortic arch obstructions with associated cardiac anomalies, we reviewed our institutional experience. METHODS Between 1995 and 2005, 90 consecutive patients with interrupted aortic arch (n=25) or coarctation of the aorta (n=65) and cardiac anomalies underwent biventricular repair. Coexisting cardiac anomalies included ventricular septal defect (VSD, n=69), transposition of the great arteries (n=11), Taussig-Bing heart (n=4), double-outlet right ventricle with subaortic VSD (n=1), atrioventricular septal defect (n=4), and an aortopulmonary window (n=1). Arch reconstruction was accomplished by a subclavian flap aortoplasty in 12 patients, end-to-end anastomosis in 23, and end-to-side anastomosis in 55. RESULTS The overall early and late mortalities were 5.6% (n=5) and 6.7% (n=6), respectively. The overall survival was 87%+/-3.7% at 10 years. Mortalities for VSD and for complex cardiac anomalies were 4.4% and 7.8%, respectively. Univariate analysis identified types of associated cardiac anomalies as a predictor for death, with the survival of patients with VSD significantly better than the other complex cardiac anomalies (p<0.05). There was a trend for single-stage repair to be associated with lower mortality compared with staged repair (p=0.06). No patients undergoing end-to-side arch reconstruction had a recurrent obstruction. CONCLUSIONS The type of associated intracardiac anomalies with arch obstruction influenced postoperative outcomes. Although both approaches could achieve good results, our data suggest that single-stage repair for arch obstruction with VSD may be associated with better survival, and is therefore recommended.

Left atrial free-floating ball thrombus with recent cardioembolic stroke

A 67-year-old man was admitted to a local hospital complaining of hemiparesis. Because of coexisting arrhythmia, he was examined by echocardiography and found to have an oval free-floating thrombus in the left atrium concomitant with mitral and aortic stenosis. He was transferred to our hospital for emergency surgery. He underwent an operation on the ninth day from the onset of neurological dysfunction, when a 2.5 x 2.5 x 3.0 cm ball thrombus was removed, and the diseased mitral and aortic valves were replaced. His postoperative course was uneventful, with no neurological sequelae. Though left atrial ball thrombus is rarely found in patients with mitral valve disease, when it is found, then immediate surgical intervention is recommended to avoid sudden death. However, there is a high risk that any cerebral lesion may worsen due to systemic heparinization. Therefore, the optimal time of surgery in a patient with a recent neurological deficit is controversial.

Ross procedure in an infant weighing 4.5 kg: eight years follow-up.

A 6 month-old male infant (weight: 4.5 kg) with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft (Ross procedure). The right ventricular outflow tract (RVOT) was reconstructed with a polytetrafluoroethylene (PTFE) -valved equine pericardial conduit. At the age of 5, re-RVOT reconstruction with an equine pericardial patch bearing a PTFE monocusp was required because of severe pulmonary stenosis resistant to 2 attempts of percutaneous transluminal pulmonary valvotomy. Currently, at the age of 8, the degree of aortic regurgitation is trivial and the pulmonary autograft is free of functional deterioration despite somatic growth.

Arterial switch operation using aortic homograft for transposition of the great arteries with pulmonary regurgitation.

We report a case of a 15 month-old boy who underwent the arterial switch operation using cryopreserved aortic homograft for transposition of the great arteries with pulmonary regurgitation, with coexisting right ventricular outflow tract obstruction precluding atrial switch operation. Follow-up echocardiography at 6 months showed trivial neoaortic valve regurgitation, no significant systemic outflow obstruction, with good cardiac function. In small children, the choice of material for left ventricular outflow tract reconstruction is one of the most crucial issues. Cryopreserved homograft has been one of the primary options for the aortic valve replacement in small children because of the ease of suturing and excellent hemostasis.