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. All three patients were initiated on combination chemotherapy with imatinib and sirolimus, drugs which have been previously shown to independently have potential benefit against PVS. Soon after the initiation of these therapies, all three patients experienced a stabilization of their disease process and clinical improvement. All three patients remain alive, with tolerable side effects from the medications. Although early in our experience and with only a small number of patients, combination chemotherapy with imatinib and sirolimus shows promise and merits further investigation as a therapeutic option for this aggressive disease.

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. RESULTS: Thirteen patients were included with a median weight of 8 kg (5.6-14.4 kg) and age of 6 months (4 months-3.8 years). All had undergone Norwood operation, and eight (62%) had hypoplastic left heart syndrome. Eight (62%) underwent stent placement during bidirectional Glenn and five (38%) during Fontan operation. Ten patients had one stent placed, and three had two overlapping stents. The median diameter of the stenotic vessel was 3 mm (2.0-5.5 mm) and diameter of the balloon used for stent expansion was 7 mm (5-10 mm). Two complications occurred including pulmonary hemorrhage from presumed wire perforation and left main stem bronchus compression requiring stent removal. No patient required stent intervention in the postoperative period, and all were discharged from the hospital. At a median follow-up of 1.3 years (2 months-7.1 years), six patients underwent interval dilation to account for somatic growth. CONCLUSIONS: Hybrid stenting of the retroaortic pulmonary artery at the bidirectional Glenn or Fontan operation is an effective treatment of pulmonary artery stenosis and prevents the need for a complex surgical arterioplasty.

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. Patients with HD were identified (n = 50), and a concurrent cohort of patients with idiopathic constipation (IC; n = 50) was selected. Pertinent features in patients with HD versus those with IC were cross tabulated using Pearson Chi2 testing (significance was P <.05). RESULTS: Sixty percent of patients with HD and 15% of patients with IC experienced onset of symptoms in the first week of life. HD patients more frequently experienced delayed passage of meconium (P <.05), abdominal distension (P <.05), vomiting (P <.05), and transition zone on contrast enema (P <.05). All patients with HD had one or more of these significant features. In contrast, only 64% of patients with IC had one or more of these features. The classic triad of symptoms (ie, delayed passage of meconium, vomiting, and abdominal distension) was present in 18%, and one or more of these symptoms was present in 98% of HD patients. In contrast, only 60% of patients with IC had a history of delayed passage of meconium, vomiting, or abdominal distension. CONCLUSIONS: A history of delayed passage of meconium, abdominal distension, vomiting or the results of a contrast enema identified all patients with HD and excluded HD in approximately 36% of patients with idiopathic constipation. The authors have shown that key features in a patient's history, physical examination, and radiologic evaluation can differentiate between HD and IC. In a child presenting with constipation and none of the above features, it is not necessary to perform a rectal biopsy to exclude HD.