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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 (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.

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.

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 β-receptor blocking medication in highly selected group of infants with diastolic myocardial dysfunction. This case series demonstrates that while the use of β-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.