Sarcomere gene variants did not improve cardiac function in pediatric patients with dilated cardiomyopathy from Japanese cohorts.

Dilated cardiomyopathy (DCM) is a progressive myocardial disorder characterized by impaired cardiac contraction and ventricular dilation. However, some patients with DCM improve when experiencing left ventricular reverse remodeling (LVRR). Currently, the detailed association between genotypes and clinical outcomes, including LVRR, particularly among children, remains uncertain.

Cilostazol treats transient heart failure caused by ATP1A3 variant-associated polymicrogyria.

Background: Some patients with ATP1A3 variant-associated polymicrogyria have recurrent transient heart failure. However, effective treatment for the transient cardiac condition remains to be elucidated.

Case Report: The patient started experiencing focal motor onset seizures in 12 h after birth, revealing bilateral diffuse polymicrogyria.

Fetal case of rare association of hypoplastic left heart syndrome and absent atrial septum accompanied by mixed form of supracardiac total anomalous pulmonary venous connection.

Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion.

A double-barrelled aorta with high aortic Arch.

A double-barrelled aorta was detected in a female newborn with 22q11.2 deletion syndrome. Double-barrelled aorta had been previously described as persistence of the fifth pharyngeal arch, but its existence continues to be debated.

The Value of Interleukin-6 among Several Inflammatory Markers as a Predictor of Respiratory Failure in COVID-19 Patients.

Patients with coronavirus disease 2019 (COVID-19) develop severe respiratory failure within a short period during the clinical course. It is essential to predict respiratory deterioration in the short term. We investigated the use of inflammatory markers to predict respiratory distress within three days from their analysis in COVID-19 patients.

Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures.

Successful laparoscopy-assisted repair of a rectovaginal fistula after low anterior resection for rectal cancer: a report of two cases.

Background: Rectovaginal fistula (RVF) after low anterior resection for rectal cancer is troublesome and refractory. Although various surgical procedures have been previously described, no definitive procedure has shown a satisfactory outcome. We present two consecutive Japanese patients who underwent successful surgery for an RVF after low anterior resection.

Electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy in school-aged children.

It can be difficult to distinguish children with early-stage arrhythmogenic right ventricular cardiomyopathy (ARVC) from those with benign premature ventricular contraction (PVC). We retrospectively evaluated six school-aged children with ARVC and compared with those of 20 with benign PVC. The median age at initial presentation was 11.

Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10.

Mitochondrial acyl-CoA dehydrogenase 9 (ACAD9) deficiency is one of the common causes of respiratory chain complex I deficiency, which is characterized by cardiomyopathy, lactic acidemia, and muscle weakness. Infantile cardiomyopathy is the most common phenotype and is usually lethal by the age of 5 years. Riboflavin treatment is known to be effective in ~65% of the patients; however, the remaining are unresponsive to riboflavin and are in need of additional treatment measures.

The Arg192His mutation in a 13-year-old girl with left ventricular noncompaction.

Left ventricular noncompaction (LVNC) is a distinct cardiomyopathy that is morphologically characterized by a two-layered myocardium, numerous prominent trabeculations, and deep intertrabecular recesses communicating with the left ventricular cavity. We present a case report regarding the identification of a new mutation in in a patient with LVNC using next-generation sequencing. A 13-year-old girl who had no family history of cardiac disease was hospitalized with dyspnea after exercise and electrocardiographic abnormalities during a school screening.

Successful non-operative management of traumatic extrahepatic portal venous injury without intraperitoneal hemorrhage: a case report.

Case: A 52-year-old woman was admitted to our hospital with hypotension after falling from the fifth floor of an apartment building. Contrast-enhanced computed tomography showed liver injury with extravasation of contrast material from the hepatic artery, and extrahepatic portal venous injury with extravasation and pseudoaneurysm. Intra-abdominal hemorrhage was not observed, and bleeding was confined to the retroperitoneal space.

[A Case Report of Reoperation for Intractable Obstruction of the Stomach after Surgery for Esophageal Cancer].

Although esophageal reconstruction using the stomach is common in surgery for esophageal cancer, this procedure sometimes results in delayed gastric emptying and reflux. This is a case report of reoperation for intractable obstruction of the stomach after initial esophageal surgery in a 59-year-old man. The obstruction was resistant to conservative management.

[A case report of distal gastrectomy with thrombectomy after NAC for Gastric cancer].

This is a case report of gastric cancer with a tumor embolus in the portal vein of a 76-year-old male. Both computed tomography (CT) and upper gastrointestinal endoscopy were performed. The diagnosis was gastric cancer with an accompanying tumor embolus in the portal vein, specifically in the superior mesenteric vein.

Radiofrequency catheter ablation of idiopathic left anterior fascicular ventricular tachycardia in children.

Background: Idiopathic ventricular tachycardia of left anterior fascicular origin (IVT-LAF) is a rare condition, and radiofrequency catheter ablation (RFCA) therapy has not been reported in children.

Objective: This study aimed to evaluate the procedures and outcomes of RFCA for pediatric IVT-LAF.

Methods: Pediatric IVT-LAF cases for which RFCA was performed between June 2006 and May 2012 at our hospital were reviewed.

[A case of severe hand-foot syndrome caused by capecitabine].

A 55-year-old woman underwent total mastectomy and axillary lymphnode dissection in 2001. Widespread lymphnode metastasis was found histologically (26/33). Neither PgR nor ER was positive.