Transjugular melody valve placement in a small child with protein losing enteropathy.

The authors present a patient with situs inversus totalis, dextrocardia, and tetralogy of Fallot with complete AV canal who underwent pulmonary valve-sparing complete repair during infancy but developed progressive pulmonary outflow obstruction on serial follow-up. His right ventricular outflow tract (RVOT) was prestented as a precursor for Melody valve placement. After this, he developed protein losing enteropathy (PLE) which was unresponsive to medical management. Finally, he received transcatheter Melody valve percutaneously in the pulmonic position that lead to clinical and biochemical resolution of his PLE symptoms.

Transcatheter stenting of the systemic-to-pulmonary artery shunt: A 7-year experience from a single tertiary center.

BACKGROUND: Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS. METHODS: Retrospective review of all patients who underwent transcatheter SPS stenting from 1/2006 to 12/2013. RESULTS: Of 229 surgically implanted SPS, 25 transcatheter stent interventions were performed in 22 patients. The majority had pulmonary atresia (n = 9) or HLHS (n = 10). Their median age was 4 ms (range 10 days to 4 years) and median weight 4.9 kg (range 3-14). Nine had a central and 15 had a BT shunt with a median shunt size of 3.75 mm (range 3-6). The interval from shunt placement to intervention was 1.9 ms (range 4 days-3.8 years). The indication for intervention was increasing cyanosis in10 patients and delaying final repair in 9. Two patients were on ECMO at the time of intervention. The median shunt diameter increased from 2.3 to 4.1 mm and oxygen saturation from 72 to 85% (P < 0.001). No intra-procedural complications were encountered. One patient died from aspiration (autopsy demonstrated a patent shunt), 13 progressed to repair or next stage, 6 remain palliated with shunts as they are deemed unfit for the next stage and 2 are awaiting surgery. CONCLUSIONS: Stenting of systemic to pulmonary artery shunt is a safe and effective procedure and avoids surgical re-intervention. It can be performed both as a rescue procedure in patients with acute shunt occlusion and as an elective procedure to palliate patients not yet suitable for subsequent corrective or staged repair.

Tetralogy of Fallot with left superior vena cava and coronary sinus atrial septal defect: a rare association.

This report describes a rare case of Tetralogy of Fallot with associated left superior vena cava and coronary sinus atrial septal defect. The initial diagnosis was made by echocardiography. The patient underwent complete repair at 2 months of age. Her postoperative course was complicated by low cardiac output requiring ECMO. She was subsequently weaned off of ECMO and discharged home. She continues to do well on serial follow-up.

Spectrum of Cantrell's pentalogy: case series from a single tertiary care center and review of the literature.

The pentalogy of Cantrell (PC) was first described in 1958. It includes five anomalies: a deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, various congenital intracardiac abnormalities, and a defect of the lower sternum. Five patients showing the PC spectrum are reported. The report presents the prenatal diagnosis, the postnatal course, and the patients' outcome at a tertiary care center from June 2001 to May 2012. A literature review and the management plan for this group of patients also are discussed. All patient data were obtained via electronic medical records retrospectively after approval by the institutional review board at the home institution. The patients in the study were three males and two females. For all of the patients, a prenatal diagnosis had been determined. The mean gestational age at delivery was 36.6 weeks. One patient had associated cranial and spine malformations. All the patients had associated congenital heart disease but a normal karyotype. Four of the five patients died in the first year of life. The ages at death ranged from 0 to 259 days (mean, 46.2 ± 51.8 days). The patients who did not survive had withdrawal of care due to increased morbidity, associated complications, or parental wishes. The pentalogy of Cantrell is a wide spectrum of associations. Patients with the complete PC together with complex congenital heart disease or extracardiac malformations may have a poor prognosis. Incomplete PC cases may have a better outcome based on associated anomalies. Prenatal counseling plays a very important role in the decision-making process for the families and has a significant impact on the postnatal management. A multidisciplinary team approach is essential for successful postpartum outcomes.

Double-outlet right ventricle with an intact ventricular septum: a unique stage 1 palliation.

This report describes a case of double-outlet right ventricle with intact ventricular septum diagnosed in a newborn male. The initial diagnosis was made by echocardiography. The baby underwent a hybrid procedure including pulmonary artery banding and stenting of the patent ductus arteriosus. He subsequently underwent stenting of the atrial communication. The patient was discharged at 55 days of life with the intent to perform palliative repair at a later date.

Transcatheter Exclusion of a Large Pseudoaneurysm of a Systemic Right Ventricular Outflow Tract.

We describe a rare patient with a large pseudoaneurysm of the right ventricular outflow tract that had developed after placement of a right ventricle-pulmonary artery conduit. A 7-mm Amplatzer Septal Occluder device was used to exclude the pseudoaneurysm, allowing for safer subsequent surgical repair. (Level of Difficulty: Advanced.).

Endovascular Repair of Thoracic Aortic Pseudoaneurysms in Children.

Pediatric aortic pseudoaneurysms are rare and can result in life-threatening sequelae. We describe 2 cases of exclusion of descending thoracic aortic pseudoaneurysm by different approaches, chosen based on the anatomy and cause of the lesions. (Level of Difficulty: Beginner.).

Constrictive pericarditis in a patient with sinus venosus atrial septal defect and anomalous right upper pulmonary venous return.

This is a report of a 49-year-old male, who presented with typical signs and symptoms of constrictive pericarditis. He was diagnosed with sinus venosus atrial septal defect (ASD) and anomalous right upper pulmonary venous return during his adolescence, which was elected not to be repaired. During the attempted repair of the ASD it was noted there was a thick fibrous material covering the heart, which had progressed over time leading to frank constrictive pericarditis. His ASD spontaneously closed over time. There have been less than 10 cases reported with constrictive pericarditis of nonsurgical etiology in a patient with ASD, and none with sinus venosus ASD.