Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection

Background: Vertical vein is left patent in some cases of supra-cardiac total anomalous pulmonary venous connection (TAPVC) when there is hemodynamic instability due to noncompliant left atrium and ventricle. After the peri-operative period, this results in features of pre-tricuspid shunt. Materials and Methods: Three cases with patent vertical vein following repair of supra-cardiac TAPVC presented with features of pre-tricuspid shunt on follow-up. Trans-catheter closure of patent vertical vein was performed using vascular plug in all three subjects. Results: The procedure was technically successful in all the patients. There was a complication related to catheter tip breakage in one of them, which was successfully managed. There was no impingement on pulmonary vein in any of the patients. Conclusion: Patent vertical vein following TAPVC repair results in features of pre-tricuspid shunt. Transcatheter closure of the patent vein is feasible

Critical thrombotic obstruction of transverse aortic arch in a neonate

A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography showed a large organized thrombus in the transverse arch causing obstruction to theaortic arch and carotids with partial recanalization. Patient underwent surgical thromboendarterectomy with arch reconstruction. The evaluation did not reveal any hematological abnormalities and suspected to be due to fetal thromboembolism. Patient improved well, and no neurological deficits were observed during follow-up

Isolated agenesis of the right pulmonary veins with pulmonary sequestration

We report two cases of agenesis of the right pulmonary veins (PVs) associated with sequestration of the right lung with systemic to pulmonary collateral. Both the children were referred for evaluation of recurrent lower respiratory tract infections. Transthoracic echocardiographic evaluation showed reversal of flow in the right pulmonary artery (PA) and absent right PVs. Cardiac catheterization confirmed the diagnosis of agenesis of the right PVs. Transcatheter closure of collaterals was performed in view of significant shunt from systemic to PA. Both the patients are asymptomatic on follow-up

Symptomatic improvement using everolimus in infants with cardiac rhabdomyoma

Background: Cardiac rhabdomyoma (CR) often shows spontaneous regression and needs close follow-up. These tumors may be associated with tuberous sclerosis complex (TSC), caused by the disinhibition of m-TOR protein. Objective: The aim of the study is to observe the efficacy of everolimus in infants with significant CR. Materials and Methods: This is a single-center prospective observational study including infants with significant CR causing either clinical symptoms or obstruction to the blood flow. Everolimus was administered at a dose of 4.5 mg/M2/wk till the symptomatic improvement. Liver and renal function tests were monitored during treatment. Results: There were six cases of suspected CR included in the study. Median age and weight of patients were 5 days (range: 1–90 days) and 3.2 kg (range: 2.2–4.5 kg), respectively. One patient was excluded after surgical excision biopsy during concomitant closure of associated large perimembranous ventricular septal defect confirmed it as a fibroma. The remaining all five cases showed regression of tumor during mean follow-up of 6.1 ± 5.1 months. One child developed varicella infection necessitating temporary discontinuation of medicine during follow-up. One case had sudden death at 4 months of age. Conclusion: Everolimus appears to be useful in selected cases of symptomatic CR. Multicentric studies are needed to determine its safety and efficacy in larger population

Unexpected difficulty during transcatheter device closure of atrial septal defect associated with right aortic arch

Right aortic arch (RAA) associated with isolated atrial septal defect (ASD) is very rare. We report successful closure of ASD associated with RAA using a 26-mm atrial septal occluder in a 30-year-old male patient. The impingement of right descending aorta in RAA caused malposition of the device in the left atrium. Deployment of the device through the right upper pulmonary vein successfully closed the defect. Follow-up evaluation by computerized tomography scan and echocardiogram showed no pulmonary venous obstruction

Emergency renal artery stenting in acute anuric renal failure in children with Takayasu's arteritis

We report on emergency percutaneous renal artery stenting in two children who developed acute anuria due to bilateral renal artery stenosis and near-total occlusion. In both children, urine output could be reestablished following the procedure. One patient died from persistent severe cardiac failure. Emergency stenting is feasible, and may buy time for future procedures

Transcatheter closure of large right pulmonary artery-to-left atrial fistula

We report the successful transcatheter closure of right pulmonary artery fistula to left atrium in a six-year-old boy, who had presented with cyanosis and shortness of breath. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy. The fistula was closed successfully by a transcatheter trans-septal approach using an 18/20 duct occluder