Ductal Stenting in Low-Resource Environments.

Duct-dependent pulmonary circulation has traditionally been addressed by the Blalock-Taussig-Thomas shunts (BTTS). Recently, catheter-based alternatives such as ductal stenting have emerged as a particularly advantageous option, especially in resource-constrained settings. This article delves into the nuances of ductal stenting within low-resource environments, highlighting its relative ease of application, reduced morbidity, and cost-effectiveness as key factors in its favor. Comparisons in mortality between the two procedures are however likely to be confounded by selection biases. Ductal stenting appears to be particularly beneficial for palliating older infants and children with cyanotic congenital heart disease and diminished pulmonary blood flow who present late. Additionally, it serves as a valuable tool for left ventricular training in late-presenting transposition with an intact ventricular septum. A meticulous pre-procedure echocardiographic assessment of anatomy plays a pivotal role in planning access and hardware, with additional imaging seldom required for this purpose. The adaptation of adult coronary hardware has significantly enhanced the technical feasibility of ductal stenting. However, challenges such as low birth weight and sepsis specifically impact the performance of ductal stenting and patient recovery in low-resource environments. There is potential for systematic application of quality improvement processes to optimize immediate and long-term outcomes of ductal stenting. There is also a need to prospectively examine the application of ductal stenting in low-resource environments through multi-center registries.

Simultaneous Double Balloon Dilatation for Supravalvar Pulmonary Obstruction After Arterial Switch Operation.

The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7-3.8) years; 9.8(8.1-15.1) kgs underwent the procedure at 14(8-44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12-14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9 mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2 mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9 months, IQR 7-22, range 5-73, others are free of re interventions with median RVOT gradient of 42, IQR 27-49, range 21-55 mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results.

Three-dimensional/four-dimensional spatiotemporal image correlation morphology of the ductus arteriosus in fetuses with pulmonary atresia undergoing neonatal ductal stenting.

Background: The value of prenatal identification of morphology of ductus arteriosus in fetuses with congenital heart defects (CHD) with pulmonary atresia and duct-dependent pulmonary circulation (DDPC) in planning neonatal ductal stenting procedure is untested. The aim of the study is to analyze the utility of three-dimensional/four-dimensional (3D/4D) spatiotemporal image correlation (STIC) fetal echocardiography in delineating the morphology of ductus arteriosus in fetuses with DDPC undergoing neonatal ductal stenting. Methods: In this retrospective study (2017-22), prenatal imaging of pulmonary artery (PA) anatomy, aortic arch sidedness, and morphology of ductus arteriosus (ductal origin was classified as vertical/horizontal and ductal course as tortuous/straight) was done using 3D/4D STIC imaging and volume datasets. Prenatal findings were correlated with angiographic findings during stenting and the degree of agreement was calculated. Results: We included 27 fetuses with a prenatal diagnosis of CHD with DDPC who underwent neonatal ductal stenting. The accuracy of prenatal assessment of PA anatomy, branch PA stenosis, and arch sidedness was 100%, 92.6%, and 88.9%, respectively. The accuracy of prenatal assessment of ductal origin and course, compared with angiography, was 85.2% and 88.9%, respectively. Prenatal imaging had a diagnostic accuracy of 100% for vertical straight and horizontal tortuous ducts, 84.6% for vertical tortuous, and 67% for horizontal straight ducts. Duct stenting was successful in 25 (92.6%) babies; two died after the procedure from stent occlusion. Conclusion: Fetal echocardiography using 3D/4D STIC imaging enables accurate delineation of the morphology of ductus arteriosus in fetuses with DDPC, thereby aiding parental counseling and planning neonatal ductal stenting.

Rescue right ventricular outflow tract stenting for refractory hypoxic spells.

BACKGROUND: While right ventricular outflow tract stenting (RVOTS) has become an acceptable alternative to palliative surgery in Tetralogy of Fallot (TOF) and similar physiologies, its utility for relief of refractory hypoxic spells is unclear. METHODS: Patients who underwent RVOTS for emergency relief of refractory hypoxic spells were identified. Specific modifications to enable expeditious RVOTS included use of stent delivery systems (guiding catheter or long sheath) upfront to minimize catheter exchanges; using coronary wires to cross RVOT initially; stabilizing the catheter with a wire in the aorta while crossing RVOT with a second wire. RESULTS: From 2015 to 2022, 11 patients underwent RVOTS for hypoxic spells refractory to medical management. Their median age was 27 days (IQR 8.5-442.5); weight 3.27 kg (2.7-8.96); 9 males. Median pulmonary annulus Z score was -4.13 (IQR-4.85 to -0.86). Thirteen stents with median diameter 5 (4-6.5) mm and length 19 (16-19.75) mm were implanted, fluoroscopy time:13.6 (11-26.3) min; procedure time (60, 30-70 min). All were ventilated. Oxygen saturations improved from 45% (40-60) to 90% (84-92); (p < 0.0001) with no major complications. Postprocedure ventilation was needed for 21 (20-49) hours and 4 required diuretic infusion for pulmonary over-circulation. Four needed re-stenting 13 days to 5 months later. At median follow-up of 7 (4-17) months; 2 died from unrelated causes, 3 underwent surgery (two correction and one aorto-pulmonary shunt) and 6 await surgery. CONCLUSION: RVOTS enables safe, expeditious and effective short-term palliation for refractory hypoxic spells. Specific technical modifications facilitate safety, ease and swiftness.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

BACKGROUND: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. METHODS/DESIGN: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DISCUSSION: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

Diagnosis and management of junctional ectopic tachycardia in children.

Junctional ectopic tachycardia (JET) is more common in its postoperative form. A thorough understanding of its etiology, pathophysiology, and management strategies is essential. Classically, postoperative JET is considered to arise from surgical trauma. Genetic susceptibility and an intrinsic morphologic/functional defect in the conduction system inherent in congenital heart diseases likely play a significant role. The devastating effects on postoperative hemodynamics warrant prompt attention. A multipronged management approach with general measures, pharmacotherapy, and pacing has decreased morbidity and mortality. Amiodarone and procainamide remain the preferred drugs, while ivabradine appears promising. Carefully planned randomized trials can go a long way in developing a systematic management protocol for postoperative JET.

Outcomes of Blalock-Taussig shunts in current era: A single center experience.

OBJECTIVES: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity. DESIGN: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression. RESULTS: Median age was 8 (0-126) days, weight 3.1(1.7-5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent had a sternotomy approach for shunt placement with 70% receiving a 3.5 mm graft. Mean graft index (shunt cross sectional area [mm2 ]/BSA [m2 ]) was 44.39 ± 8.04 and shunt size (mm) to body weight (kg) ratio 1.1 ± 0.2. Hospital mortality was 12%, with an interval mortality of 6%. Shunt thrombosis/stenosis occurred in 23% and pulmonary over circulation in 30%, while shunt reoperation was required in 12% and catheter intervention in 8% of the cohort. At 1-year, survival was 82.0% (95% CI [72.7%, 88.4%]), and survival free of major morbidity 61.4% (95% CI [50.7%, 70.5%]). Duct dependency predisposed to mortality (P = .01, HR 6.74 [1.54, 29.53]) and composite outcome (mortality and major morbidity) (P = .04, HR 2.15, CI [1.036, 4.466]) and higher graft index to mortality (P = .005, HR 1.07 [1.02, 1.12]). CONCLUSIONS: The commonest indication for a MBTS in the current era was single ventricle palliation. Morbidity and mortality was considerable, partly explained by the higher at risk population. Alternative methods to maintain pulmonary blood flow in place of a MBTS requires further investigation.

The warden procedure through a posterolateral thoracotomy approach.

The Warden procedure for the correction of a right-sided partial anomalous pulmonary venous connection to the high superior vena cava is well established. It has the advantages of avoiding sinoatrial node dysfunction and pulmonary and systemic venous obstruction. In the case related here, a 3-year-old girl presented with a superior vena cava type of sinus venosus atrial septal defect and an anomalously draining right upper pulmonary vein, with bilateral superior venae cavae. Our approach to the Warden procedure was through a right posterolateral thoracotomy, which provided additional advantages.

Pulmonary artery banding for univentricular heart beyond the neonatal period.

BACKGROUND: It is standard practice to band the pulmonary artery at 2 to 4 weeks of age in patients with univentricular hearts with increased pulmonary blood flow. The behavior of patients banded beyond the neonatal period has not been well elucidated. PATIENTS AND METHODS: This was a retrospective chart review of 32 consecutive patients (one neonate) who underwent pulmonary artery banding for functionally univentricular heart. The mean age at banding was 5.7 ± 6.0 months, and 34.4% were over 6-months old. RESULTS: Mortality was 15.6%. The mean systolic pulmonary artery pressure decreased from 43.6 ± 9.7 to 29.6 ± 7.0 mm Hg. The mean pre-discharge echocardiographic band gradient was 60.6 ± 13.6 mm Hg (mean systemic systolic pressure 73.7 ± 11.0 mm Hg) and systemic oxygen saturation was 81.7% ± 5.8%. At a mean follow-up period of 44.9 ± 30.0 months, 6 patients were lost to follow-up, 13 had undergone bidirectional Glenn shunt, and 7 had Fontan operations. Pulmonary artery mean pressure was 17.2 ± 4.6 mm Hg at pre-Glenn catheterization. Of the 5 patients who had not undergone further surgery, only one was inoperable. All were in functional class I or II. CONCLUSION: Pulmonary artery banding beyond the neonatal period in suitable patients with univentricular hearts provides reasonable palliation in the intermediate term, with a significant number successfully undergoing Fontan stages.

Branching vertical vein with multiple sites of obstruction in supracardiac total anomalous pulmonary venous connection.

We present the case of an infant with total anomalous pulmonary venous connection and a branching vertical vein with multiple points of narrowing, draining the confluence into the innominate vein. The embryology and clinical relevance of this interesting anatomy is discussed.

Prosthetic reconstruction of bicuspid pulmonary valve in tetralogy of Fallot.

BACKGROUND: Various techniques have been described for preserving pulmonary valve function in tetralogy of Fallot repair. In selected substrates, the pulmonary valve can be bicuspidized and preserved using polytetrafluoroethylene pericardial membrane. METHODS: This study was a retrospective review of 20 patients aged 11 months to 31 years with tetralogy of Fallot and a bicuspid pulmonary valve (with anteroposterior cusps) who underwent intracardiac repair from August 2010 to January 2013. The anterior cusp was augmented using polytetrafluoroethylene pericardial membrane to preserve the valve hinge. A transannular patch was used in all cases. Data relating to surgical outcome, intensive care unit course, pulmonary regurgitation, and right ventricular outflow tract gradient were collected. RESULTS: There was no mortality. Predischarge pulmonary regurgitation was ≤grade 2 in 18 (90%) patients. One patient had an outflow gradient >40 mm Hg. On follow-up of 3-24 months, there was no increase in outflow gradient; 18.7% had progression of pulmonary regurgitation. CONCLUSION: In selected substrates, this technique is associated with minimization of pulmonary regurgitation and an excellent functional outcome. The utility of this technique needs to be validated over a longer time scale in a larger series.

Bilateral anomalous pulmonary venous connection to bilateral superior caval veins.

A four-year-old girl presented with superior vena cava (SVC) type of sinus venosus defect, right upper pulmonary vein draining into right SVC-right atrium junction, left upper lobe pulmonary veins draining into the lower part of persistent left SVC (LSVC), and a patent ductus arteriosus. The anomalous pulmonary venous drainage to LSVC was overlooked in the preoperative evaluation and was found intraoperatively. Warden procedure was performed for right-sided veins. Lower LSVC draining the anomalous pulmonary veins was anastomosed to the left atrial appendage. The short stump of LSVC was diverted to left pulmonary artery. Anomalous pulmonary venous connections to LSVC are rare. Embryology of venous development is analyzed with respect to this rare anomaly, and options for surgical correction are discussed.

Single-stage surgical correction of combination lesions through thoracotomy.

Coarctation of the descending aorta is associated with significant cardiac lesions in up to 50% of the patients. Under such circumstances, surgical management requires consideration of the nature of the associated lesion which usually requires a median sternotomy for surgical correction. Coarctation of the aorta (COA) is, therefore, rarely associated with lesions that are amenable to surgical correction through a thoracotomy incision. In this case report, we report a case of COA with significant associated cardiac disease that was repaired through a left thoracotomy incision. A 13-year-old boy was found to have COA and also partial anomalous pulmonary venous drainage of the left upper pulmonary vein to the innominate vein. Both the lesions were surgically corrected simultaneously through a thoracotomy.

Percutaneous closure of a large aortic paravalvular leak using two duct occluder devices.

A 21-year-old male presented with severe aortic paravalvular leak. He had undergone three cardiac surgeries and also had chronic kidney disease. It was decided for a trans-catheter closure owing to the risks of a fourth surgery and co-morbidity. The device was sized based on angiogram, balloon sizing and two dimensional transesophageal echo. There was significant residual leak after deployment of first device. Hence the defect was re-crossed and two duct occluder devices were positioned across the leak from two arterial access. After confirming position and satisfactory reduction in paravalvular leak, the devices were released in tandem. There was near abolition of leak. The patient is asymptomatic at three months follow up. Larger paravalvular leaks are better addressed with two devices of smaller size rather than a single large device. Technical considerations while deploying multiple devices are discussed.

Right ventricular exclusion and univentricular palliation for failed one and a half ventricle repair for Ebstein's anomaly.

A 20 year-old male was diagnosed to have Ebstein's anomaly with severe right ventricular dysfunction. He was taken up for 1.5 ventricle repair. Post procedure, there was difficulty in weaning from cardiopulmonary bypass due to progressive right ventricular dilatation compromising the systemic output. An atrial septectomy did not help. Progressive right ventricular dilatation compressing the left ventricle, demonstrated on transoesophageal echocardiogram, prompted us to perform a right ventricular exclusion and univentricular palliation. The patient was successfully weaned off cardiopulmonary bypass and had a smooth postoperative recovery. Judicious use of right ventricular exclusion and univentricular palliation could be an effective bailout strategy in difficult surgical scenarios in Ebstein's anomaly.

Spontaneous recovery of complete atrioventricular block complicating acute anterior wall ST elevation myocardial infarction.

BACKGROUND: Complete atrioventricular block complicating acute anterior wall ST elevation myocardial infarction (MI) is classically considered one of the worst prognostic indicators. METHODS: We present the case of a gentleman who developed complete atrioventricular block during the course of acute anterior wall ST elevation MI, and had spontaneous resolution of the same. Mechanisms of spontaneous resolution of complete atrioventricular block in the setting of acute MI are discussed. Attention is drawn to a subgroup of patients, albeit a minority, who have a better prognosis owing to reversible causes than classically expected and seen. RESULTS: Clinical features suggested that this patient had reocclusion of the infarct-related artery after thrombolysis on presentation and spontaneous reperfusion. CONCLUSION: Coronary angiography provides invaluable information for decision making in such clinical scenarios. Complete atrioventricular block due to reversible ischaemia produced by reocclusion of an infarct-related artery should be reversible by percutaneous coronary angioplasty of the infarct-related artery. We suggest that reversible causes be considered before attributing atrioventricular block to irreversible damage, which would require a permanent pacemaker implantation. This would be more significant in most of the developing world, where resources are scarce.