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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.

This case study looks at a common pathway in the management of a patient with truncus arteriosus. The chapter follows the various considerations in decision-making and the conundrums in contemplating an intervention in the right ventricular outflow tract. The chapter explores the evidence available in determining an intervention and the suitability in consideration for a percutaneous approach. It further explores the various conduits used in the creation of a ‘right ventricle to pulmonary artery’ conduit and the resulting challenges associated with them, the pre-procedural workup, the benefits and risks in the percutaneous approach, and the various valves (specifically the Melody® valve) currently available.

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.

Objectives This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing

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.

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.

Background The technique of transcatheter patent ductus arteriosus (PDA) closure has evolved in the past two decades and is increasingly used in smaller patients. We sought to report a multicentre experience of transcatheter PDA occlusion in infants weighing Methods A multicentre, retrospective analysis of children weighing Results A total of 361 patients underwent attempted transcatheter PDA closure. The mean weight at catheterization was 4.8 ± 1.0 kg and mean age was 5.7 ± 2.7 months. Successful device closure was achieved in 321(89%) patients without major complication. Device placement was attempted but failed in in 13 cases. Device malposition or embolization post release occurred in the remaining 27 cases. Of these, 11 patients had surgical retrieval of the embolized device and PDA ligation; 6 patients were referred for surgery at a later date after transcatheter retrieval of device and 10 patients had ductal closure with a different device after transcatheter retrieval of the initial device. In the 331 patients who had transcatheter PDA occlusion, 34 had coils and 297 had occluders, including Amplatzer Duct Occluder (ADO) (n = 197); ADO II (n = 51); ADO II Additional Sizes (n = 42); Amplatzer Vascular Plug (n = 6); Occlutech Duct Occluder (n = 1). Complete occlusion was achieved in a 312(94%) patients at either catheterization or last available follow-up. Three of 19 patients had mild residual flow which persisted at less than a year post procedure; but follow up data was not complete in the rest. There were 17 cases of mild left pulmonary artery (LPA) stenosis related to device impingement with velocity >2m/s; 11 of which resolved with time, 5 had persistent mild LPA stenosis at last review with Doppler velocity of 2.5m/s or less; and 1 underwent LPA stenting. Mild aortic obstruction was recognised in 10 patients during catheterisation or on post procedure echocardiogram, 7 of which resolved; one had persistent increased velocity at the arch with no diastolic tail at last review and 2 had incomplete follow up data. Other complications include loss of pulse requiring TPA (n = 5) temporary reduction in pulse requiring heparin infusion (n = 16); bleeding requiring transfusion (n = 2); anaesthesia or airway related (n = 1); small pericardial effusion (n = 1); haemopericardium requiring percutaneous drainage (n = 1); tricuspid valve injury (n = 1); and cerebrovascular event (n = 1). There were 13 deaths in the cohort which were unrelated to the procedure. The causes of death were mainly related to underlying chronic lung disease and pulmonary hypertension. Conclusions Transcatheteter closure of PDA can be safely accomplished in selected infants who are less than 6 kg despite the manufacturer’s recommended weight limit of 6 kg for most ductal occluders. The embolization rate (7%) in this group appeared to be higher than published series in infants >6kg, therefore thorough delineation of ductal anatomy and attention to device selection are vital to the success of the procedure.