Severe coronary artery ectesia in a paediatric patient with Noonan syndrome presenting for transcatheter pulmonary valve placement.

Coronary ectasia is a very rare phenomenon seen in Noonan syndrome with only a few documented case reports. We describe a 14-year-old with Noonan syndrome and tetralogy of Fallot with described coronary artery ectasia since infancy who presented for possible transcatheter pulmonary valve placement and was found to have severe ectasia of bilateral coronary arteries.

Biatrial Thrombi on a Gore Septal Occluder Despite Early Epithelialization.

ASD and PFO device-related thrombus is an uncommon phenomenon that can be seen both early- and late-post-closure. Typical risk factors include atrial fibrillation, hypercoagulable diseases/states, and lack of device endothelialization. We present an interesting case of a 15-year-old female who underwent PFO device closure after a renal infarction and was found to have biatrial device thrombus 3-month post-closure.

Combination Chemotherapy in Severe Pulmonary Vein Stenosis-A Case Series.

Pulmonary vein stenosis results from a proliferative process that leads to the progressive obstruction of venous return to the left atrium. It is often resistant to catheterization and surgical based interventions and is frequently fatal when encountered in its severe form. Here, we describe three patients with severe, primary pulmonary vein stenosis that was progressing despite aggressive conventional management strategies.

An unusual case of coronary artery compression that did not preclude successful transcatheter pulmonary valve placement.

During transcatheter pulmonary valve placement, coronary compression observed during simultaneous right ventricular outflow tract angioplasty and coronary angiography typically contraindicates valve implantation. We present a unique patient with tetralogy of Fallot who underwent successful transcatheter Melody valve placement despite coronary compression observed during right ventricular outflow tract balloon angioplasty.

Hybrid Pulmonary Artery Stenting at the Bidirectional Glenn or Fontan Operation in Patients With Single Ventricle Congenital Heart Disease.

Background: Stenosis of the retroaortic pulmonary artery is common in patients with single ventricle heart disease. Intraoperative hybrid stenting at the bidirectional Glenn or Fontan operation can treat this lesion and avoid a complex surgical arterioplasty.

Methods: Patients who underwent intraoperative stent implantation to the retroaortic pulmonary artery during the bidirectional Glenn or Fontan operation at our center between January 2005 and July 2014 were retrospectively identified.

Diagnosing Hirschsprung's disease: increasing the odds of a positive rectal biopsy result.

Background/purpose: Rectal biopsies are performed as a definitive means of diagnosing Hirschsprung's Disease (HD) in children presenting with constipation. The authors hypothesized that key features in the history, physical examination, and radiographic evaluation would allow us to avoid unnecessary rectal biopsies.

Methods: A retrospective analysis was conducted on patients undergoing rectal biopsy between 1995 and 2001.