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.

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.

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.

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.