Background: There is a recent interest in nonsurgical correction of superior sinus venosus defects. Patient selection is currently based on advanced imaging and printing technologies. Simple clinical tools to select patients will expand its applicability in developing countries. Methods: Defects caudally extending toward the oval fossa and right upper pulmonary veins draining beyond the cavoatrial junction on transesophageal echocardiography were excluded. Balloon interrogation of cavoatrial junction confirmed complete occlusion of the defect with unobstructed pulmonary venous drainage to left atrium. Single long covered stents or overlapping covered stents were used to exclude sinus venosus defects. Closure of left-to-right interatrial shunt without causing pulmonary vein occlusion was confirmed on follow-up imaging. Results: Forty-four patients selected after transesophageal echocardiography underwent balloon interrogation with monitoring of right upper pulmonary vein. Eighteen out of 44 patients were ineligible. Twenty-four eligible patients with closure of left-to-right interatrial shunt without pulmonary vein occlusion underwent covered stent exclusion using single long stents in 15 and overlapping stents in the rest, while 2 patients are awaiting the procedure. Four patients aged 6 to 16 years received stents that were 18 mm or larger. Three patients had stent embolization that required surgical correction in 2 but in the last patient was managed nonsurgically with an overlapping covered stent with good final outcomes. Procedure was successful in 22 patients. At a median follow-up of 20 months (range, 3–54 months), there were no adverse events. Follow-up imaging showed trivial left-to-right shunt in 4 and unobstructed pulmonary veins in all patients. Conclusions: Transesophageal echocardiography and balloon interrogation identified 60% of the patients with sinus venosus defects to be eligible for catheter closure. Overlapping stents are an alternative to custom-made long stents. Transesophageal echocardiography confirms procedural success on follow-up. Advanced imaging and printing technologies are not essential for successful outcomes and thus simple tests increase the feasibility in developing countries.

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Optimal sized balloon atrial septostomy improves hemodynamics in advanced pulmonary arterial hypertension(PAH). Occlutech Atrial Flow Regulator(AFR) is designed to provide a atrial septal fenestration diameter titrated according to the age and right atrial pressures. This observational study analyzed symptoms, exercise distance, oxygen saturations, hemodynamics and echocardiographic parameters in patients with syncope or right-heart failure after AFR implantation. Patients with high-risk predictors of mortality during septostomy were scrutinized. 39 patients (9 children) with syncope(34/39) or right-heart failure(27/39) underwent AFR implantation without procedural complications. 6-minute-walk distance increased from 310±158.2 to 376.4±182.6 meters, none developed syncope. Oxygen saturations reduced from 96.4±6.4% to 92±4.9% at rest and further to 80.3±5.9% on exercise. Right atrial pressures reduced from 9.4±5(2-27)mmHg to 6.9±2.6(1-12)mmHg, while cardiac index increased from 2.4±0.8(0.98-4.3) to 3±1(1.1-5.3) Litres/min/sq.metre and systemic oxygen transport increased from 546.1±157.9(256.2-910.5) to 637.2±191.1(301.3-1020.2) ml/minute. Echocardiographic improvement included significant reduction of pericardial effusion and inferior caval congestion at a median follow-up of 37 months. Overall survival improved except two early and one late deaths in high-risk patients. 5/7 patients with advanced disease and key hemodynamic predictors of mortality survived. Acute hemodynamic benefits in PAH after AFR are improving cardiac output, systemic oxygen transport and reducing right atrial pressures. Improvement of symptoms especially syncope, exercise duration and right ventricular systolic function as well as device patency were sustained on mid-term follow-up. Implantation was safe in all including young children without procedural complications. Mortality was noted only in patients who had high-risk predictors and patients at advanced stage of the disease.

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Objectives: Ductal stents, right ventricular outflow tract stents, and aortopulmonary shunts are used to palliate newborns and infants with reduced pulmonary blood flow. Current long-term outcomes of these palliations from resource-restricted countries are unknown. Methods: This single-centre, retrospective, observational study analysed the technical success, immediate and late mortality, re-interventions, and length of palliation in infants ≤5 kg who underwent aortopulmonary shunts, ductal, and pulmonary outflow stents. Patients were grouped by their anatomy. Results: There were 69 infants who underwent one of the palliations. Technical success was 90% for aortopulmonary shunts (n = 10), 91% for pulmonary outflow stents (n = 11) and 100% for ductal stents (n = 48). Early mortality within 30 days in 12/69 patients was observed in 20% after shunts, 9% after pulmonary outflow stents, and 19% after ductal stents. Late mortality in 11 patients was seen in 20% after shunts, 18% after outflow stents, and 15% after ductal stents. Seven patients needed re-interventions; two following shunts, one following outflow stent, and four following ductal stents for hypoxia. Among the anatomical groups, 10/12 patients with pulmonary atresia, intact ventricular septum survived after valvotomy and ductal stenting. Survival to Glenn shunt after ductal stent for pulmonary atresia, intact ventricular septum and diminutive right ventricle was very low in two out of eight patients, but very good (100%) for other univentricular hearts. Among 35 patients with biventricular lesions, 22 survived to the next stage. Conclusions: Cyanotic infants, despite undergoing technically successful palliation had a high inter-stage mortality irrespective of the type of palliation. Duct stenting in univentricular hearts and in pulmonary atresia with an intact ventricular septum and adequate sized right ventricle tended to have low mortality and better long-term outcome. Completion of biventricular repair after palliation was achieved only in 63% of patients, reflecting unique challenges in developing countries despite advances in intensive care and interventions.