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We report on a patient with protein-losing enteropathy (PLE) after undergoing total cavopulmonary connection (TCPC) operation who was introduced to subcutaneous immunoglobulin (SCIG) home therapy as immunoglobulin G (IgG) replacement therapy. The patient was a 21-year-old man with hypoplastic left heart syndrome who developed edema and ascites secondary to PLE 5 years after the TCPC operation. When he was 15-yearsold, intravenous immunoglobulin (IVIG) was administered for the treatment of hypogammaglobulinemia. SCIG home therapy was introduced when he was 20 years old. The serum IgG level did not increase with the initial SCIG dose (8 g/week); therefore, we increased the SCIG dose (16 g/week). After SCIG administration, the serum albumin level was reduced from 2.7 to 2.4 g/dL, IgG level increased from 370 to 484 mg/dL, and the total duration of the hospital stay was shortened from 4.7 to 1.2 d/month. With regard to adverse events, a local reaction was observed at the subcutaneous infusion site, but this improved with time. These findings suggest that SCIG home therapy is effective in increasing and maintaining serum IgG levels and avoiding hospitalization. The appropriate SCIG dose for patients with PLE is unknown. We believe that this should be considered on a case-by-case basis depending on the severity of each case.

Intracardiac thrombosis may occur in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Most thrombi are treated with warfarin, but some are treated with novel oral anticoagulants (NOAC). We describe a case of a 30-year-old man with a thrombus that was successfully dissolved by treatment with dabigatran etexilate. The patient was diagnosed with ARVC at the age of 15 years. At the age of 16, he was prescribed carvedilol and enalapril for the treatment of heart failure. However, at the age of 30, echocardiography detected a thrombus in the right ventricle. Although oral warfarin and intravenous unfractionated heparin were initiated, the size of the thrombus did not change, and on day 3 of therapy initiation, we switched from warfarin to dabigatran etexilate. On day 35, the thrombus was eliminated. Dabigatran etexilate may be effective in the treatment of intracardiac thrombosis in patients with ARVC. Adequate anticoagulation is needed to prevent severe complications such as thromboembolism or sudden death.

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BackgroundThe aim of this study was to investigate the prediction of postnatal prognosis using fetal and perinatal data in patients with primary congenital dilated cardiomyopathy (PCDCM), and to estimate the incidence of this disease.MethodsWe examined correlations between fetal or perinatal data and postnatal clinical course in a multicenter retrospective study of eight patients with PCDCM. Incidence was calculated in a population‐based study.ResultsAll patients developed heart failure at a median of 8 days (range, 0–43 days), and six patients died or required extracorporeal artificial heart therapy at a median of 67 days (range, 0–92 days). The cardiothoracic area ratio from fetal echocardiography, the Apgar score, and the standard deviation of birth weight correlated significantly with the date at onset of heart failure. However, no data correlated with survival. Cumulative incidence of PCDCM was calculated as 1.21 per 100 000 total births (95% confidence interval, 0.37 to 2.06).ConclusionsPrimary congenital dilated cardiomyopathy has a poor prognosis, but cardiothoracic area ratio from fetal echocardiography, body weight at birth, and Apgar score correlate with the timing of the onset of heart failure, and these indicators might therefore be useful for peri‐ and postnatal management.