Rhabdomyoma is a benign tumor that comes from skeletal tissue, it classified as cardiac and extracardiac according to the location. Extracardiac rhabdomyomas most frequently originate in head neck at larynx pharynx, being parapharyngeal space an exceptional We present clinical case of 86-year-old man asymptomatic patient with laterocervical mass years evolution, has increased its size progressi...

Goldmuntz et al. (1) have reported the frequency of 22q11 deletions in a prospectively ascertained sample of 251 patients with conotruncal defects. Deletions were found in 17.9% of the patients, including 50% with interrupted aortic arch (IAA), 34.3% with truncus arteriosus (TA), and 15.9% with tetralogy of Fallot (TOF). Although this study was designed to determine the frequency of deletions i...

OBJECTIVE Extracardiac pericardial-flap lateral tunnel Fontan operation has the theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. METHODS Clinical data were reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November...

BACKGROUND Patients with surgically palliated total cavopulmonary connection are at risk for recurrent atrial arrhythmia requiring catheter ablation. Transcatheter procedures for those with extracardiac conduits (extracardiac-total cavopulmonary connection) are perhaps the most challenging because of exclusion of the venous circulation from the arrhythmia substrate. Puncture through the inferio...

BACKGROUND There exists large geometric variability among total cavopulmonary connections (TCPC) because of the patient-specific anatomies and the chosen surgical procedure. In this study we present quantitative comparison of the geometric characteristics of the extracardiac and intraatrial Fontan anatomies, the two commonly used TCPC procedures. METHODS A method of centerline approximation o...

BACKGROUND Persistent left superior vena cava (PLSVC) is a variant of systemic venous return which is observed in 0.3% of autopsies in the general population and in 4-8% of patients with congenital heart disease. AIMS To evaluate associated cardiac, extracardiac and chromosomal anomalies in prenatally diagnosed cases of PLSVC and to review their outcome. STUDY DESIGN Retrospective comparati...

Introduction. Rhabdomyomas of the head and neck are exceptionally rare benign mesenchymal tumors. Rare cases have been reported to involve other sites of the body including the head and neck regions. Case Presentation. We report a case of voluminous extracardiac adult rhabdomyoma affecting adult patients and initially seen as slowly growing, indolent neoplasms. The patient is a seventy-year old...

Connexin43 knockout mice die neonatally from conotruncal heart malformation and outflow obstruction. Previous studies have indicated the involvement of neural crest perturbations in these cardiac anomalies. We provide evidence for the involvement of another extracardiac cell population, the proepicardial cells. These cells give rise to the vascular smooth muscle cells of the coronary arteries a...

OBJECTIVE To describe the prenatal sonographic appearances in cases of absent pulmonary valve syndrome and the importance of investigating the presence of 22q11 deletion. METHODS We describe 2 cases, which were referred because of a suspicion of a cardiac malformation. In both cases, a large anechoic mass emerging from the right ventricle was visualized and identified as an aneurysmal dilatat...

The diagnosis of structural heart disease before birth is associated with a poor prognosis. Of 222 continuing pregnancies seen in a 10 year period, there has been a 79% mortality. This is inconsistent with published results and current concepts of the outcome for children with cardiac malformation. Of the 222, death occurred in intrauterine life in 57, 87 died as neonates, and 31 in infancy or ...