Yasuo Murakami

Efficacy and safety of carvedilol for heart failure in children and patients with congenital heart disease

There have been few reports describing the use of carvedilol in children or patients with congenital heart disease. Therefore, its optimal regimen, efficacy, and safety in these patients have not been adequately investigated. Subjects were 27 patients with two functioning ventricles, for whom carvedilol was initiated (from December 2001 to December 2005) to treat heart failure. All patients had failed to respond to conventional cardiac medication. They consisted of 12 males and 15 females, aged 23 days to 47 years (median age: 2 years). Heart failure due to ischemia (myocardial infarction, intraoperative ischemic event) or due to myocardial disease (cardiomyopathy, myocarditis), and heart failure with atrial or ventricular tachyarrhythmia represented 70% of all cases. Carvedilol was initiated at a dose of 0.02–0.05 mg/kg/day, which was increased by 0.05–0.1 mg/kg/day after 2 days, 0.1 mg/kg/day after 5 days, and 0.05–0.1 mg/kg/day every month thereafter with a target dose of 0.8 mg/kg/day. This study retrospectively assessed the efficacy and adverse reactions based on changes of symptoms, cardiothoracic ratio (CTR), left ventricular ejection fraction (LVEF), and human atrial natriuretic peptide (hANP)/b-type natriuretic peptide (BNP) blood levels. The mean follow-up period was 10.2 months (range: 1–46 months). Twenty-six (96.3%) patients showed improvement in symptoms and were discharged from the hospital. However, the remaining one patient failed to respond and died. Significant cardiovascular adverse reaction was seen in none of the patients. The mean CTR decreased from 61.8% ± 5.3% before treatment to 57.6% ± 7.4% after treatment (P < 0.05, n = 25), and the mean LVEF improved from 41.4% ± 23.1% to 61.1% ± 10.1% (P < 0.05, n = 10), respectively. Mean hANP and BNP levels showed a decrease from 239.1 pg/ml to 118.3 pg/ml and a significant decrease from 437.9 pg/ml to 120.5 pg/ml, respectively (P < 0.05, n = 10). Improvements in these data were also demonstrated when analyzed individually among the pediatric group (aged younger than 18) and the congenital heart disease group. Initiation of carvedilol at a lower dose with more gradual dose escalation, compared with previously reported regimens, might have efficacy with low incidence of adverse effects in pediatric patients and patients with congenital heart disease. Carvedilol may be effective in treating heart failure in children due to ischemia, myocardial disease, and complicated by tachyarrhythmia.

Valvuloplasty for common atrioventricular valve regurgitation in cyanotic heart diseases

Valvuloplasty for common atrioventricular valvular regurgitation and bidirectional cavopulmonary shunt were successfully performed in 3 children with univentricular heart and double-outlet right ventricle. The free margins of opposite leaflets of the common atrioventricular valve were sutured together to restore coaptation of the leaflets, and Kay-Reeds or DeVegas annuloplasty was carried out. Postoperative examinations revealed significant reduction of atrioventricular valve regurgitation and improvement of general condition in all patients.

The degree of scooping of the interventricular septum influences electrocardiographic findings in atrioventricular septal defect

The aim of this study was to clarify those morphological features and hemodynamic stress factors which influence the electrocardiographic findings in atrioventricular septal defect. In 64 patients with the incomplete form of atrioventricular septal defect (separate valvar orifices) with usual atrial arrangement, the length from the left ventricular apex to the aortic valve (outlet dimension), to the so-called cleft (scoop dimension), and to the lowest point of the left atrioventricular valve (inlet dimension) were measured by biplanar cineangiography. The size of the ostium primum defect, the ventricular pressure ratio, and the pulmonary/systemic flow ratio were also evaluated. The results showed that the lower the scoop/outlet ratio (r=0.60, p

Multiple floppy valves with all cardiac valves prolapsing: Clinical course and treatment

SummaryTwo cases with prolapse of all four cardiac valves are described and compared with two similar ones previously reported. The severity and progression of regurgitation of each of the valves differed by case, despite having similar echocardiographic findings consistent with the diagnosis of multiple floppy valves. Two of the four patients had their aortic valve replaced because of severe regurgitation: the excised valves revealed myxomatous degeneration. None of the patients had any stigmata of Marfan or Ehlers-Danlos syndrome, except for the presence of hyperextensive joints. There may be an unknown collagen disorder that caused floppiness in all the valves.

Succinic acidemia: A new syndrome of organic acidemia associated with congenital lactic acidosis and decreased NADH-cytochrome c reductase activity

Two siblings with succinic acidemia, a hitherto unreported organic acidemia, were investigated. Succinic acidemia, lactic acidosis and respiratory distress were observed in one of the siblings, who died 37 days after birth, and succinic acidemia was also detected in the next sibling at the fetal stage. NADH-cytochrome c reductase activity was significantly low in both cases and NADH-ferricyanide reductase activity was also low in the fetal case, suggesting a complex I deficiency of the electron transport system in the mitochondrial membrane.

Does hypoplasia of one pulmonary artery preclude a definitive repair in pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle?

SummaryTwins with pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle associated with underdeveloped and discontinuous left pulmonary artery are described. Operations to connect the left pulmonary artery to the main pulmonary trunk, with subsequent biventricular repair assisted by bidirectional cavopulmonary anastomosis and atrial fenestration were performed. Follow-up catheterization proved spontaneous closure of the fenestration in one patient. Both patients have been in a satisfactory condition for 3 years since operation.

Pathological lesions causing pulmonary hypertension after closure of a ventricular septal defect

A 15-year-old boy with a ventricular septal defect, pulmonary hypertension, Downs syndrome, and extremely thickened media (ETM) of the small pulmonary arteries died of heart failure and pulmonary hypertension 13 years after intracardiac repair. Microscopic examination of lung specimens collected prior to the intracardiac repair and at the time of autopsy revealed that the ETM had remained unchanged and that the arteries connected to the vessels with ETM had become severely thickened. The present case shows that even a small percentage of arteries with ETM can cause pulmonary hypertension, and illustrates one of the mechanisms of how pulmonary hypertension can fail to be resolved after intracardiac repair.

Long-Term Outcomes of Definitive Surgical Repair for Congenitally Corrected Transposition of the Great Arteries

Long-Term Outcomes of Definitive Surgical Repair for Congenitally Corrected Transposition of the Great Arteries Yuki Nakamoto1),Tomomi Nishimura1),Kanako Kishiki1),Akio Inage1),Tomomi Ueda1), Tadahiro Yoshikawa1),In-Sam Park1),Yasuo Murakami1),Naoki Wada2),Makoto Ando2),Yukihiro Takahashi2) Departments of Pediatric Cardiology1) and Cardiovascular Surgery2), Sakakibara Heart Institute, Tokyo, Japan