Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his res...

OBJECTIVE To report on the use of sildenafil for pulmonary hypertension treatment of a newborn patient after cardiac surgery. DESCRIPTION A female, full term newborn infant with diagnosis of double outlet right ventricle, pulmonary hypoplasia and subaortic ventricular septal defect, was submitted to Blalock surgery in the first week of life. In postoperative the newborn had pulmonary hyperten...

Objective: To describe cardiac tomography findings of an apparently new, presumably congenital, left ventricular (LV) abnormality noted consistently in three patients. Patients: Three patients presenting with non-specific symptoms including fatigue, shortness of breath, or chest discomfort were evaluated with cardiac tomography for cardiac structure and function. Results: Findings from the thre...

The malformed heart to be described was removed at necropsy from a 9-day old Bantu infant, and sent to the Department of Anatomy for study. Unfortunately neither the clinical nor necropsy records could subsequently be traced and the only information available was that the cause of death was thought to be partial atelectasis. The main vessels branching off the arterial trunk were identified for ...

OBJECTIVE To describe cardiac tomography findings of an apparently new, presumably congenital, left ventricular (LV) abnormality noted consistently in three patients. PATIENTS Three patients presenting with non-specific symptoms including fatigue, shortness of breath, or chest discomfort were evaluated with cardiac tomography for cardiac structure and function. RESULTS Findings from the thr...

A 12-lead ECG was obtained soon after the birth of a pair of omphalo-thoracopagus (Figure 1) conjoined twins. Before birth, they had been diagnosed with two separate hearts that shared a wide communication through the independently defined right atria. Other anomalies including a common liver with two gallbladders and two stomachs with common jejunum and ileum beyond the duodenum were also note...

Patients with Joubert syndrome 2 (JBTS2) suffer from a neurological disease manifested by psychomotor retardation, hypotonia, ataxia, nystagmus, and oculomotor apraxia and variably associated with dysmorphism, as well as retinal and renal involvement. Brain MRI results show cerebellar vermis hypoplasia and additional anomalies of the fourth ventricle, corpus callosum, and occipital cortex. The ...

The molar tooth sign is seen in very few conditions and is a very rare paediatric central nervous system congenital anomaly. Molar tooth sign is the result of cerebellar vermis hypoplasia, thick and maloriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. In Joubert syndrome this is seen in about 85% of patients. We present a case of 13 months old baby boy with r...

Congenital diaphragmatic hernia is a very serious congenital defect associated with high mortality rate. The syndrome is characterized by migration of abdominal viscera to thoracic cavity a variable degree of pulmonary hypoplasia associated with pulmonary hypertension caused by alteration of pulmonary vessels diameter increased muscularisation of pulmonary arteries and decreased compliance of t...

Received March 20, 2017; revised manuscript received July 16, 2017; accepted August 1, 2017; released online September 1, 2017 Time for primary review: 45 days Department of Cardiovascular Medicine (K.M., S.F., K.S., A.T., T.O., N.N., S.Y.), Department of Pediatric Cardiology (H.O.), National Cerebral and Cardiovascular Center, Suita, Japan Mailing address: Shigefumi Fukui, MD, PhD, Department ...