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Background: Patienets with congenital diaphragmatic hernia comorbidity with congenital heart disease have a more severe prognosis than those with either condition alone. Here we report the clinical outcomes of congenital diaphragmatic hernia patients with congenital heart disease who have either two ventricles or a functional single ventricle. Methods: The subjects included 17 congenital diaphragmatic hernia patients with congenital heart disease who were treated at Osaka Women’s and Children’s Hospital. The severity of congenital diaphragmatic hernia was assessed by the observed-expected lung-to-head ratio. We assessed the details of clinical course in the patients. Results: A total of 238 patients with congenital diaphragmatic hernia were born between 1981 and 2021 at our hospital. Of these, a total of 21 patients (8.8 %) had congenital heart disease and seventeen patients were evaluated for the study. Fourteen patients had two ventricles and three patients had single ventricle. Of seventeen patients, eight patients were alive and nine patients were dead. There was no significant difference in congenital diaphragmatic hernia severity between the alive and dead patients. The patients who survived had two ventricles and received definitive heart repair operations. Although two patients with a functional single ventricle had mild congenital diaphragmatic hernia severity, they were managed using mechanical ventilation management. All of the patients with a functional single ventricle could not undergo Glenn or Fontan operation, and died. Conclusion: Long-term survival may be expected in the patients with two ventricles. Patients with a functional single ventricle might not survive for a long time if they required ventilation due to the respiratory dysfunction.

OBJECTIVES Atrial function in Fontan patients is unknown. Our goal was to report the relationship of atrial function with the cardiac index and atrial function and clinical outcome through longer follow-up periods. METHODS Twelve patients were followed up for over 20 years after their first Fontan operation. Atrial function, including the expansion index, atrial ejection fraction, passive ejection fraction and active ejection fraction, was examined using cardiac computed tomography. The relationship of atrial function with the cardiac index and failing Fontan patients was analysed. RESULTS Twelve Fontan patients were included. The median follow-up period after the first Fontan operation was 27 (range, 21-33) years, and the median age of those examined was 33.5 (range, 24-60) years. There were 6 male patients (50%). The cardiac index showed a significant positive correlation with the expansion index (P = 0.02), the atrial ejection fraction (P = 0.035), and the active ejection fraction (P = 0.013). The expansion index (39.2 ± 19.6 vs 64.1 ± 3.9), atrial ejection fraction (26.6 ± 10.9 vs 39.0 ± 1.5%), booster pump (15.6 ± 9.0 vs 31.3 ± 3.5) and cardiac index (2.1 ± 0.3 vs 2.5 ± 0.2 L/min/m2) were significantly lower in patients with a history of arrhythmia than in patients without a history of arrhythmia (P CONCLUSIONS Patients with atrial arrhythmia and signs of Fontan failure have lower atrial function than those without.

Background There are few reports of the outcomes of standardized surgical management addressing the etiologic and morphologic aspects of mitral valve malformation according to Carpentier's classification. This study aimed to evaluate the long-term outcomes of mitral valve repair in children according to Carpentier's classification. Methods Patients who underwent mitral valve repair at our institution between 2000 and 2021 were retrospectively reviewed. Preoperative data, surgical techniques, and outcomes were analyzed according to Carpentier's classification. The proportion of patients free of mitral valve replacement and reoperation was estimated using Kaplan-Meier analysis. Results Twenty-three patients (median operative age, four months) were followed up for 10 (range, 2-21) years. Preoperative mitral regurgitation was severe in 12 patients and moderate in 11 patients. Eight, five, seven, and three patients had Carpentier's type 1, 2, 3, and 4 lesions, respectively. Ventricular septal defect (N = 9) and double outlet of the great arteries from the right ventricle (N = 3) were the most commonly associated cardiac malformations. There were no cases of operative mortality or deaths during the follow-up. The overall five-year rate of freedom from mitral valve replacement was 91%, whereas the five-year rates of freedom from reoperation were 74%, 80%, 71%, and 67% in type 1, 2, 3, and 4 lesions, respectively. Postoperative mitral regurgitation at the last follow-up was moderate in three patients and less than mild in 20 patients. Conclusions Current surgical management of congenital mitral regurgitation is generally considered adequate; however, more complicated cases required a combination of various surgical techniques.