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In patients with rapid RV dilatation, restrictive RV physiology might be frequently noted at the initial MRI assessment. Therefore, careful follow-up may be necessary in patients with restrictive RV physiology to determine the optimal timing of pulmonary valve implantation.

OBJECTIVES: We investigated the results of a revision of a previous Fontan connection to total cavopulmonary connection (TCPC) in patients with failing Fontan circulation. METHODS: From July 1998 to April 2013, 21 patients who had failing Fontan circulation underwent revision of the previous Fontan operation to TCPC. The median age at TCPC conversion was 17.9 years (range, 4.6-38.1 years) and the median interval between initial Fontan operation and TCPC was 13.8 years (range, 2.1-25.4 years). Th

Certain congenital heart defects, which present at birth as complex morphologic defects, are actually the result of a relatively simple primary lesion and the subsequent acquired development of a complex secondary lesion during gestation. Moreover, fetal heart approach during gestation can prevent simple cardiac lesions from such development. Specific structural lesions can be diagnosed before 12 weeks of gestation by transvaginal fetal echocardiography, and animal experiments have shown that di

In patients with Duchenne muscular dystrophy (DMD), death secondary to cardiac or respiratory failure typically occurs in the second or third decade without treatment. Although cardiac dysfunction is treated with standard heart-failure strategies, it remains insufficient in DMD children. The purpose of this study was to evaluate the efficiency of cardiac medication and noninvasive ventilator support in DMD cardiomyopathy children with analyzing echocardiographic data. Forty-eight DMD children pa

Transcatheter device closure of a secundum atrial septal defect (ASD) is followed by a noticeable change in the left atrium (LA) and left ventricle (LV) over a long-term follow-up. This study aimed to assess the remodeling of the atrial and ventricular myocardium with respect to diastolic function in adult ASD patients. Around 38 asymptomatic patients (age: 48.6 ± 17.1 years, range: 23–69 years) diagnosed with ASD during routine health check-up and who underwent ASD device closure with the Ampla

OBJECTIVE: Extracardiac pericardial-flap lateral tunnel Fontan operation has the theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. METHODS: Clinical data were reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was 2.8+/-1.5 years and the body weight was 12.3+/-3.2kg. Extracardiac

OBJECTIVES: There is no concrete predictor of the change of pulmonary arterial pressure after surgical closure of an atrial septal defect (ASD) in patients with pulmonary arterial hypertension (PAH). The aim of this study was to investigate the role of preoperative room air arterial oxygen saturation (SaO2) (arterial blood gas data) as a predictor of postoperative PAH. METHODS: The medical records of 36 patients [>20 years, mean pulmonary arterial pressure (mPAP) ≥25 mmHg] who underwent surgical