assessment of cardiac right‑ventricle functions plays an essential role in diagnosis of arrhythmogenic right ventricular dysplasia (arvd). among clinical tests, cardiac magnetic resonance imaging (mri) is now becoming the most valid imaging technique to diagnose arvd. fatty infiltration of the right ventricular free wall can be visible on cardiac mri. finding right‑ventricle functional paramete...

Tracheobronchial obstruction and unilateral hypoplasia of the pulmonary artery are serious barriers to successful Fontan completion. We describe a 5-year old girl with left bronchial obstruction, hypoplasia of the left pulmonary artery and a single ventricle (double inlet left ventricle). She had undergone external stenting to treat left bronchial stenosis at 3 years of age, bidirectional cavop...

A 14-month-old Hispanic female with a history of double-outlet right ventricle and developmental delay in the setting of recombinant chromosome 8 syndrome was referred for neurologic imaging. Brain MR revealed multiple abnormalities primarily affecting midline structures, including commissural dysgenesis, vermian and brainstem hypoplasia/dysplasia, an interhypothalamic adhesion, and an epidermo...

The paper presents the management of a child born with pulmonary valve atresia, a single (double-inlet) ventricle, right ventricular hypoplasia, and perimembranous septal defect. The first stage of treatment consisted in a Blalock-Taussig shunt. Control angiography performed 1 year after surgery confirmed that the anastomosis was correct, and there was no narrowing at the connection. The first ...

the majority of coronary artery fistulas (cafs) are congenital. the anomaly accounts for 0.4% of congenital heart defects and approximately 50% of pediatric coronary vasculature anomalies. twenty percent of people with congenital cafs have other concomitant cardiac anomalies, most frequently aortic and pulmonary atresia and patent ductus arteriosus. it is worthy of note that caf with the tetral...

background pulmonary arterial hypertension (pah) is a serious complication of unrepaired congenital left-to-right shunts. the final consequence is right ventricular (rv) systolic dysfunction and reversal of shunt. objectives the aim of this study was to evaluate the clinical course and paraclinical findings in a group of patients with pah associated with congenital heart disease (pah-chd). we a...

Dandy-Walker malformation is a rare congenital malformation, characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation 4th ventricle and enlargement posterior fossa. Postnatal hydrocephalus develops in approximately 70-90% patients. The relationship with postaxial polydactyl has been defined as possible autosomal recessive syndrome (OMIM 220220). Here, we present an in...

A 14-year-old male patient presented with cyanosis and tiredness. The patient had undergone a Glenn procedure at age 12 following the echocardiographic determination of a double inlet left ventricle, ventriculoarterial discordance, moderate valvular-subvalvular pulmonary artery stenosis, non-restrictive inlet ventricular septal defect and right ventricle hypoplasia; his oxygen saturation was 70...