Novel use of a 3D printed heart model to guide simultaneous percutaneous repair of severe pulmonary regurgitation and right ventricular outflow tract aneurysm.

We describe percutaneous repair of severe pulmonary regurgitation and a right ventricular outflow tract pseudoaneurysm in a 19-year-old patient after repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. A 3D printed model of his heart was used to simulate percutaneous repair with a closure device in the aneurysm neck and a Venus P-valve in the right ventricular outflow tract. The encouraging findings from the simulation allowed us to plan the complex procedure effectively with a successful outcome and avoidance of surgery.

Doubly committed subarterial ventricular septal defect: A risk factor for aortic distortion in patients considered for percutaneous pulmonary valve implantation?

Percutaneous pulmonary valve implantation has gradually become the first line strategy for re-intervention for right ventricular outflow tract dysfunction during long-term follow-up after congenital cardiac surgery in many centers. We describe a case of a patient with double outlet right ventricle (Fallot's type) with a doubly committed subarterial ventricular septal defect, where the unique anatomy precluded percutaneous pulmonary valve implantation. © 2016 Wiley Periodicals, Inc.

Diagnosing rare cases of anomalous origin of the coronary arteries in tetralogy of Fallot using CT in an infant and an adult.

Major and minor coronary artery anomalies in tetralogy of Fallot is a well-described finding. The importance of determining the coronary distribution impacts upon the decision making for surgery and subsequent management. Traditionally, the coronary distribution is relied classically on echocardiography and cardiac catheterisation; however, they have well-known limitations. The use of CT as a first-line investigation modality for coronary artery distribution is discussed.

Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defects as an Alternative to Surgical Treatment.

BACKGROUND: The superior sinus venosus atrial septal defect (SVASD) is characterized by deficiency of the common wall between the superior vena cava (SVC) and the right upper pulmonary vein (RUPV), which is no longer committed to the left atrium. OBJECTIVES: This study sought to evaluate the potential for redirecting the SVC and RUPV flow to the right and left atria, respectively, by implantation of a covered stent in the SVC. METHODS: Review of 48 consecutive adult SVASD patients undergoing assessment for correction. Pre-procedural evaluation included cross-sectional imaging and ex vivo simulation using printed or virtual 3-dimensional models. RESULTS: Transcatheter correction was performed in 25 patients, with a further 6 awaiting stent implantation. Only 8 patients were deemed technically unsuitable. The procedure involved balloon test inflation in the anticipated stent landing zone with simultaneous transesophageal echocardiography and pulmonary venography to confirm defect closure and unobstructed pulmonary venous drainage, followed by deployment of a 10-zig covered Cheatham platinum stent. Stents of lengths between 5 and 8 cm were implanted. A second, uncovered stent was used for anchoring in 9 patients. The RUPV was protected with a high-pressure balloon during stent implantation to prevent pulmonary venous obstruction in 4 patients. The median follow-up period was 1.4 (interquartile range: 0.8 to 1.7) years, with no mortality. Stent embolization occurred in 1 patient; another required drainage of hemopericardium. Cardiac computed tomography after 3 months confirmed unobstructed pulmonary venous return. At latest follow-up, a residual shunt was present in 1 patient. CONCLUSIONS: Transcatheter correction of SVASD may be considered as an alternative to surgery in a substantial proportion of patients.

Congenital Heart Disease in East Africa.

Congenital Heart Disease (CHD) is an enormous problem in Low Middle Income Countries and particularly in sub-Saharan Africa. There is an estimated 500,000 children born in Africa with CHD each year with a major proportion of this in sub-Saharan Africa. The vast majority of these children receive sub-optimal or no care at all. In East Africa: Kenya, Tanzania, and Uganda have all attempted to create a CHD service for the last 20 years with minimal success due to various factors. Visiting cardiac missions have made considerable contributions in the development of CHD services in these countries, however there remains a significant number of children with lack of care. We explore the positive aspects of the current projects, the various factors that hinder growth in this area, and what can be done to promote CHD service growth in these countries.

Postoperative interventricular septal haematoma following tetralogy of Fallot repair and perimembranous ventricular septal defect repair.

Interventricular septal haematoma is a rare postoperative complication in congenital heart surgery. We present one case of a 6-month-old after tetralogy of Fallot repair and 1 case of a 10-month-old after ventricular septal defect repair. Both were noted to have interventricular septal haematoma on intraoperative transoesophageal and postoperative echocardiogram. Although multiple previous reports, mainly in adults, have suggested aggressive intervention, both these cases were managed conservatively, highlighting the management and evolution of a rare postoperative complication in the paediatric population.

ß-blockers in postoperative myocardial diastolic dysfunction: not a panacea.

Successful treatment with heart rate lowering medication has been used to treat adults with chronic myocardial dysfunction of various aetiologies for a number of years. There has been recent evidence for the successful use of ß-receptor blocking medication in highly selected group of infants with diastolic myocardial dysfunction. This case series demonstrates that while the use of ß-receptor blockers in infants early following initial treatment of congenital left heart obstructive lesions appears promising and safe adjunct to more conventional management, the medium-term and long-term care of these patients remains as challenging as before.

Right ventricular cavity near obliteration in neonatal severe biventricular hypertrophic cardiomyopathy.

A newborn presenting with cyanosis at day 9 of life was admitted to the local hospital. Initial local echocardiography confirmed a cardiac issue and the patient was transferred to a tertiary cardiac hospital. Further imaging confirmed a rare presentation of cardiomyopathy with severe right ventricular outflow tract obstruction. Surgery was performed but with postoperative haemodynamic instability complicated by incessant ventricular tachycardia. Following discussion with the family, care was withdrawn. Postmortem demonstrated a rare form of hypertrophic cardiomyopathy with right ventricular outflow tract obstruction not previously described in a neonate.