Premature closure or restriction of foramen ovale (PCFO) is a rare congenital anomaly that can lead to a wide spectrum of cardiac malformations. This spectrum of secondary malformations appears to depend on the gestational timing of closure of the foramen ovale and to the degree of restriction. Earlier in the gestation, closure of the foramen has been associated with severe hypoplasia of the le...

Cerebellum is known to play an important role in coordination and motor functions. In some resent studies it is also considered to be involved in modulation of mood, cognition and psychiatric disorders. Dandy Walker Malformation is a congenital malformation that is characterized by hypoplasia or aplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle and enlargement of the p...

Coronary artery embolism is an uncommon cause of acute myocardial infarction (MI). We present a patient with pulmonary atresia and severe right heart hypoplasia who underwent a lateral tunnel Fontan procedure in childhood and presented with an acute ST-segment elevation MI at 19 years of age. In addition to the known risk of thrombotic complications associated with a Fontan circulation, potenti...

conclusions results suggest that these methods cannot be interchanged, and vvi showed significantly lower lv peak twist, peak twisting rate and peak untwisting rate. however, when lv twist and lv twisting rates were normalized to lv length, values were comparable for both imaging techniques. results twist degree was significantly lower in the vvi group than in the tdi group (p = 0.008, r = 0.56...

A 38-year-old woman had complex, congenital heart anomalies, including double-outlet right ventricle with mitral atresia, severe left ventricle hypoplasia, atrial septal defect, and severe pulmonary stenosis, for which she had not received appropriate treatment. She presented to our institution with an intense sudden-onset headache that had initiated while sleeping. Brain computed tomography in...

The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was u...

becker's naevus shows itself as a pigmented area of skin which is aquired , persistant and asymmetrical and sometimes shows evidence of increased androgen sensitivity. colocalization with other developmental anomalies is reported rarely. we report a 20 year old woman who developed an extensive and bilateral becker naevus since the age of 12 year and had left breast hypoplasia, too.

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were...

Oral-facial-digital syndrome type 1 (OFD1; OMIM# 311200) is an X-linked dominant ciliopathy caused by mutations in the OFD1 gene. This condition is characterized by facial anomalies and abnormalities of oral tissues, digits, brain, and kidneys. Almost all affected patients are female, as OFD1 is presumed to be lethal in males, mostly in the first or second trimester of pregnancy. Live born male...

BACKGROUND Right ventricular hypoplasia (RVH) is often caused by tricuspid valve atresia and pulmonary valve atresia. this condition leads to low right ventricular blood volume and right ventricular maldevelopment. But, in adults, the main cause of RVH may also be associated with alloplasia of the right coronary artery, which results in an insufficient blood supply to the right ventricular myoc...