Background: Although progress in perioperative management and surgical treatment has improved the survival rate of patients with hypoplastic left heart syndrome (HLHS), the Fontan completion rate remains low and prognosis is poor. Analysis of prognostic factors is challenging because HLHS complicates hemodynamics in patients with typical HLHS without ventricular septal defect and nontypical HLHS. This study aimed to categorize typical HLHS into four groups with combinations of stenosis/atresia of the mitral valve/aortic valve and to investigate survival rates and mortality risk factors to improve Fontan completion and prognosis.

Methods: We enrolled 119 patients with HLHS [mitral valve atresia (MA)/aortic valve atresia (AA): 61 patients; MA/aortic valve stenosis (AS): 4; mitral valve stenosis (MS)/AA: 24; and MS/AS: 30)]. After excluding the MA/AS group because of a small number of patients, we investigated the survival rate, number of mortalities, and cause of death in the MA/AA, MS/AA, and MS/AS groups by using prenatal diagnosis, MS/AA, low birth weight, atrioventricular valvular insufficiency, noncardiac complications, restricted foramen ovale, and length of intensive care unit stay following initial surgery as variables.

Results: The overall 5-year survival rate for typical HLHS was 58％, which significantly varied by subtype (MS/AS: 72％; MA/AA: 59％; and MS/AA: 33％; p＝0.002). Multivariate analysis identified MS/AA (p＝0.002) and positive prenatal diagnosis (p＝0.03) as significant prognostic risk factors.

Conclusion: In typical HLHS, MS/AA was found to be an adverse prognostic factor. Because prognosis after total cavopulmonary connection was similar to those of other groups, preoperative diagnosis and treatment strategies before right heart bypass must be improved.

Background: Although vocal cord paralysis (VCP) is a well-known complication after cardiovascular surgery, there is little published information on VCP after cardiovascular surgery in neonates and early infants.

Methods: A total of 221 patients who were ＜2 months old and had undergone cardiovascular surgery at Chiba Children’s Hospital between October 2008 and September 2014 were included in this study. We retrospectively investigated the incidence, risk factors, and prognosis of VCP.

Results: Of the 221 patients, 14 (6.3％) exhibited postoperative VCP. Postoperative intubation period (p＝0.008), arch repair (p＜0.0001), Rastelli procedure (p＝0.006), and VSD closure (p＜0.0001) were identified as risk factors of VCP in univariate analysis, but arch repair was the only risk factor in multivariate analysis (p＝0.0005, OR 76.57, 95％CI 6.62–885.25). The paralyzed side was greater on the left side (left side only, 86％; bilateral, 14％), and 12 (92％) of 13 patients (1 died) demonstrated good recovery from difficulty of swallowing, stridor and/or hoarseness.

Conclusion: In neonates and early infants who have undergone arch repair treatment, postoperative VCP is one of the most important complications but is likely to improve spontaneously.

Background: The efficacy, pharmacokinetics, safety, and tolerability of bosentan (Tracleer^® 32 mg, new dispersible tablet for pediatric use) for pulmonary arterial hypertension (PAH) were investigated in Japanese children.

Methods: This was an open-label, multi-center, phase III, single-arm trial. Patients aged ＜15 years with PAH were administered a new pediatric formulation of bosentan (2 mg/kg twice per day). The efficacy of bosentan was evaluated during the first 12 weeks of the trial, and the subsequent period, including a post-marketing study, was evaluated by following a separate protocol. The primary outcome measure was a change in the pulmonary vascular resistance index (PVRI) from baseline to 12 weeks after starting treatment. Secondary outcome measures were changes in World Health Organization functional class (WHO-FC) every 12 weeks. Additionally, the pharmacokinetics of bosentan were evaluated by measuring blood concentrations, area under the curve (AUC), and time-to-peak blood concentrations (T_max). To assess safety and tolerability, adverse events were evaluated, including those leading to study drug discontinuation.

Results: In total, 6 subjects with a median age of 5.5 years (range, 1–13 years; 4 males, 2 females) were enrolled. The mean change±standard deviation from baseline to Week 12 in PVRI was 4.0±258.6 dyn·sec·m²/cm⁵, which indicated no significant change in PVRI relative to the baseline value. WHO-FC II was unchanged in all patients. The geometric mean C_max of bosentan and AUC_tau were 494 ng/mL and 2300 ng·h/mL, respectively. Safety and tolerability were satisfactory.

Conclusion: A new pediatric formulation of bosentan was confirmed to be safe and well-tolerated in Japanese children with PAH. There was no worsening in the mean PVRI and WHO-FC.

Fetal echocardiography is a well-established tool for prenatal diagnosis of many congenital heart diseases. However, there are few reports on the fetal diagnosis of cardiomyopathy. We herein report a case of siblings who were diagnosed as having familial dilated cardiomyopathy (DCM) by fetal echocardiography, pathological findings, and genetics analysis. Their father and father’s younger brother had DCM. Patient 1 was diagnosed at 21 weeks of gestation, and patient 2 at 27 weeks of gestation. Patient 1 was born by emergency cesarean section in the 27th week but died 46 minutes after birth. Patient 2 died in utero at 33 weeks of gestation. Pathological autopsy findings of both patients demonstrated endocardial elastofibrosis. Genetic analysis of both patients and their parents revealed a father-derived tropomyosin α-1 chain (TPM1) gene mutation and mother-derived desmoplakin (DSP) gene mutation. The prognosis of familial fetal DCM is poor, and careful family planning is necessary.

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is a rare congenital cardiac malformation. Clinical manifestations usually appear in infants or, more rarely, in newborns and include respiratory distress or congestive heart failure due to increased pulmonary resistance. Primary total correction of AORPA reportedly can result in excellent survival with a low incidence of reintervention. We report here two cases of staged repair of AORPA. Case 1 involved a 25-day-old girl who had a complicating respiratory syncytial (RS) virus infection. RS virus infection in children with congenital heart disease is associated with high mortality and morbidity, and cardiac surgery performed with cardiopulmonary bypass during symptomatic RS virus infection is associated with a high risk of postoperative complications, especially postoperative pulmonary hypertension. Therefore, we decided that the initial palliation should consist of right pulmonary artery banding, and total correction was achieved 2 months later. Case 2 involved an almost 2-month-old girl who initially underwent right pulmonary artery banding due to severe pulmonary hypertension, and total correction was achieved 13 days later. Both patients were discharged in good condition without any clinical symptoms. Thus, right pulmonary artery banding appears to be a good surgical option for patients with AORPA and complicated condition.