Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5–21) and 19.5 kg (4.2–73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days–4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.

Background: Balloon angioplasty (BA) for aortic coarctation in neonates and infants remains controversial due to high recurrence rate and vascular complications. Aim: This study aimed to determine the safety and outcome of percutaneous treatment of coarctation in neonates and infants and to share the initial experience of strategy of prepartial dilatation with high-pressure noncomplaint balloon before final targeted dilatation using low-pressure compliant balloon. Materials and Methods: Retrospective analysis of records of all neonates and infants aged <6 months who underwent BA either using only low-pressure balloon (Group A) or those with prepartial dilatation using high-pressure noncomplaint balloon followed by low-pressure compliant balloon (Group B) between July 2017 and February 2020 was performed. Demographic, clinical, echocardiographic, interventional, and follow-up data were collected for all. Results: A total of 51 patients (41.2% neonates) were included in the study. Median age was 1 month 14 days (60.8% girls) and mean weight was 3.6 ± 1.5 kg. The mean peak trans-coarctation gradient was 53 ± 12 (34–80) mmHg. The final pressure gradient dropped to <10 mmHg in all cases of Group B and only in 26.3% (5) patients of Group A (P < 0.001). Recoarctation rate was 25.5% (13) overall and was significantly higher in Group A patients (P < 0.001), in those with borderline/mildly hypoplastic arch (P = 0.04) and in those with postprocedure gradient between 10 and 20 mmHg (P = 0.02). Median time to re-coarctation was significantly delayed in Group B (P < 0.001). There were no major complications or mortality in either group. Conclusions: BA in neonates and young infants has an excellent short and mid-term safety and efficacy. The recoarctation rate is significantly reduced as well as delayed with prepartial dilatation using high-pressure noncompliant balloon.

Background: Device closure of multiple atrial septal defects (MASDs) is frequently done using a single centrally deployed septal or cribriform occluder, but multiple devices are needed for large defects separated more than 6 mm. There is a concern about complications while using multiple devices, especially in children. Methods: Patients who received multiple devices for closure of MASD were grouped according to their age and analyzed for procedural techniques, immediate and late complications. MASDs closed by a single device were not included. Balloon sizing was done when echocardiographic images were suboptimal before simultaneous device deployment through two venous accesses or sequential deployment through one access. Duration and number of antiplatelet drugs and residual flows were analyzed on follow-up. Results: Twenty-five patients received multiple devices. Balloon interrogation was performed in 16/18 adults but only in 2/7 children. Device size was 2–5 mm larger than echocardiographic defect size or equal to balloon waist. There were no procedural failures; 7/25 showed small postprocedural residual flows. Complications including embolization in one, arrhythmia in one, and cobra deformity in two were managed successfully. On a median follow-up of 5.5 years (1–12 years), residual flows disappeared in 4/7 and there were no major late complications. Conclusions: Use of multiple devices for closing MASD is feasible with good technical success. Echocardiography and balloon interrogation are the keys for success. Simultaneous deployment is often needed and sequential delivery is feasible rarely if the defects are far apart. Minor residual leaks are common but improve on follow-up. There are no significant new complications on long-term follow-up.

Background: Data on congenital systemic arteriovenous fistulas are largely based on individual case reports. A true systemic arteriovenous fistula needs to be differentiated from other vascular malformations like capillary or venous hemangiomas, which are far more common. Objectives: We sought to identify the varied symptoms, diagnostic challenges, describe interventional treatment options, and postulate an embryological basis for this uncommonly described entity. Methods: This is a descriptive study of a cohort of systemic arteriovenous fistulas seen in the department of pediatric cardiology at a tertiary cardiac institute from 2010 to 2020, with prospective medium-term follow-up. A total of seven cases were identified. The diagnosis was confirmed by computed tomographic imaging, magnetic resonance angiography, or conventional angiography. Results: All were successfully closed using duct occluders or embolization coils with no recurrence in six cases over a median duration of follow-up of 48 months (interquartile range: 16; 36–52 months). Four of the seven cases underwent follow-up imaging using echocardiography or ultrasound. Conclusion: The incidence of congenital systemic arteriovenous fistulas is low and accounted for 0.009% of pediatric outpatients seen over 10 years at our institute. The spectrum of clinical presentation varies from an innocuous swelling or a pulsating mass to frank heart failure. Strong clinical suspicion and advanced imaging modalities have helped identify some hitherto undescribed connections. Large malformations with multiple communications may persist or recur despite transcatheter closure.

Introduction: Bioresorbable stent has the theoretical advantage in the pediatric age group; however, experience in the literature is very limited. Objectives: This pilot study sought to evaluate the feasibility, safety, and performance of iron bioresorbable scaffold (IBS Angel™) as short-term palliation in lesions with noncomplex patent ductus arteriosus (PDA). Materials and Methods: Nine neonates with duct dependent but dual-source pulmonary blood flow (PBF) were included. Major stent-related complications, in-hospital course, stent patency up to 6 months, and unplanned re-interventions were studied, as well the percentage of stent material resorbed in patients in whom the stents were explanted at the surgery. Results: IBS Angel™ was successfully implanted in all nine patients (mean weight range 3.4 ± 0.4 kg). Six were pulmonary atresia with an intact ventricular septum and 1 critical pulmonary stenosis patients where PDA stenting was done together or after balloon dilation. The mean procedure and fluoroscopy times were 89 ± 39 min and 16.3 ± 6.9 min, respectively. There were no major complications such as stent thrombosis or embolization and there were no in-hospital deaths. Post procedure overshunting and prolonged ventilatory support was a prominent feature. The median ventilation days was 3 (1–11 days). One patient died after 1 month due to respiratory syncytial virus pneumonia at the referring hospital. At 6 months follow-up, four had patent stents and four had blocked or restrictive stent flow. One patient received re-stenting at 4 months for restrictive stent flow. In three patients where microcomputed tomography of explanted stent was available, resorption of iron was 15% at 6 months and >65% at 16 months. Conclusions: Ductal stenting with IBS Angel™ is feasible and safe in selected patients. Because of early luminal loss, its use may be recommended for lesions with dual-source PBF with noncomplex PDA morphology that requires a short duration of palliation.

Introduction: Transcatheter pulmonary valve implantation has been an effective treatment for dysfuntional right ventricular tract outflow tract (RVOT). Defining a landing zone before the intervention is crucial in patients with native RVOT. Improper sizing and undefined landing zone will lead to embolization. Methods: It is a retrospective observational study from August 2020 to December 2020 in native RVOT. Three patients who had significant Right ventricle dilatation were analyzed. The multi-slice computed tomography (MSCT) with magnetic resonance imaging and angiography data of all patients before the procedure were analyzed. All patients underwent an angiogram in the same sitting, before the procedure to assess the landing zone, valve diameter as well as the risk for coronary compression. We chose a valve based on valve area 23%–25% more than the area at the waist during balloon sizing. Results: All three patients underwent successful valve implantation. Valve sizes used were 27.5 mm in one and 32 mm in the other two. The mean RVOT gradient postprocedure was 11.5 mm Hg and pre procedure was 43 mmHg. There were no complications during the procedure or at a mean follow-up of 3.6 months. Conclusion: The balloon sizing gives the true narrowest diameter in comparison with MSCT, and increasing this area by 23%–25% will give the appropriate valve size for successful implantation.

Percutaneous transcatheter closure of fossa ovalis atrial septal defect (FOASD) is an established procedure. When femoral venous approach is unfeasible due to interrupted inferior vena cava (IVC) or occluded femoral veins, other interventional methods through transjugular, transhepatic approach, or surgical closure can be performed. We report two cases of transhepatic device closure of FOASD associated with IVC interruption successfully performed without any complications.

Transcatheter closure of patent ductus arteriosus (PDA) is now the standard of care with some exceptions. Best treatment for peripheral pulmonary artery (PA) stenosis in small children is still debatable. Surgical augmentation, balloon dilatation, and stenting are the available options with each having its own risks and limitations. Here, we are reporting complete transcatheter management of three cases of congenital rubella syndrome who had PDA and left branch PA stenosis by device closure and stent placement, with successful outcome in two cases and complication, leading to surgery in one. We also share our understanding of case selection for a better outcome.

Superior sinus venosus defect is characterized by varying degrees of override of the atrial septum by the superior vena cava and anomalous right upper pulmonary vein (RUPV) drainage. The defect is seen posterosuperior to the oval fossa and is traditionally corrected by a surgical patch that routes the anomalously draining RUPV to the left atrium. A few postoperative patients develop stenosis or occlusion of the caval or pulmonary vein and/or sick sinus syndrome secondary to vascular injury. This has kindled a recent interest in nonsurgical correction of these defects by the use of a covered stent to reroof the defect. The complex anatomy of this defect often necessitates the use of advanced imaging tools and postprocessing. Countries with restricted resources do not have easy access to technologies such as three-dimensional printed models and advanced processing like virtual reality or holography. This “How to do it?” article, applicable in these countries, explains the current understanding of this intervention, patient selection, step-by-step explanation of the evaluation, and stent implantation.

Dysfunctional right ventricular outflow tracts after a repair for tetralogy of Fallot using a transannular patch offer limited nonsurgical opportunities due to their large dimensions. A discrete subannular narrowing between a dilated right ventricle and the enlarged pulmonary trunk was a potential anatomical target for the creation of a landing zone using a prestent in a young male with severe pulmonary regurgitation and moderate stenosis. Asymmetric expansion of the prestent in the angulated outflow tract led to distal stent embolization that was stabilized by another telescoping stent before successful valve implantation. This manuscript details the unique challenges posed by a dilated regurgitant outflow tract for implanting a pulmonary valve.

Patients with single right ventricle and tricuspid regurgitation have increased morbidity and mortality rates. The outcomes of surgical tricuspid valve repair and replacement are dismal. Although some centers prefer valve replacement with a bioprosthetic valve in the tricuspid position for the pediatric population, these valves have limited longevity and will eventually need to be replaced. Unfortunately, single ventricle patients are fragile and unlikely to tolerate a second replacement. Percutaneous valve-in-valve (ViV) implantation is an acceptable alternative with low morbidity and mortality. Studies have also shown acceptable short- and mid-term outcomes. We report a case of Melody ViV placement in the tricuspid position in a patient with hypoplastic left heart syndrome through the fenestration of a Fontan conduit.

The use of the Melody^® (Medtronic, Minneapolis, MN, USA) biological prosthesis in mitral position has shown acceptable short-term results. Furthermore, it allows its expansion with a balloon when the patient grows up, and this procedure can be performed by a venous catheterization through transseptal approach through an interatrial communication or puncturing the septum. Patients with complex congenital heart disease undergoing multiple surgical and percutaneous interventions may present with vascular complications such as thrombosis of the femoral venous system that make percutaneous access impossible. In this situation, the transhepatic approach is a very useful alternative to access the heart.

Extreme pulmonary artery hypoplasia in cyanotic malformations precludes palliative surgeries. When aortopulmonary collaterals (APC) in such patients are also hypoplastic, their unifocalization to create a neopulmonary vasculature is also hampered. Stent angioplasty of the outflow or collateral arteries may reduce hypoxia but is challenging in tortuous and atretic tracts. Fusion imaging overlays anatomical data from computed tomography during adult structural interventions, but its use is not often reported in young children with complex cyanotic malformations. This report shows utility of fusion imaging in pulmonary atresia with extremely hypoplastic pulmonary arteries and stenotic APC to guide stenting of outflow tract and collaterals.

Atrioventricular block (AVB) is an infrequent but life-threatening complication of transcatheter closure of atrial septal defect (ASD), accounting for 0.1%–6.2% of cases in large series. It has been related to unfavorable defect anatomy as well as size and intrinsic stiffness of the occluding device. In this setting, the new GORE^® cardioform ASD occluder (GCA) device could be an appealing technical advance in ASD treatment. We report a case of complete AVB after ASD closure with an Amplatzer septal occluding (Abbott, Plymouth MN, USA) device successfully treated by its percutaneous retrieval and “rescue” deployment of GCA device few months later.

Coronary arteries coursing behind the aortic root may get compressed when nitinol septal occluders are used to close an atrial septal defect. Hence, echocardiographic recognition of a retroaortic linear vessel is important during preinterventional evaluation. While the left circumflex arising from the right coronary artery is the most common cause, a similar finding is sometimes observed in a single left or right coronary artery and rarely with small sinus nodal branches from the left circumflex artery. Complex three-dimensional relations between the defect and the aortic root may be understood only after a postdeployment selective coronary angiography. Two patients with anomalous retroaortic left circumflex from the right coronary artery underwent uneventful device closure with clearly documented separation between the edges of the occluder and the anomalous vessel. Follow-up imaging and exercise testing confirmed the safety of the intervention. A selective postdeployment and postrelease coronary angiography are mandatory in every patient with retroaortic coronaries.

Herein, we report successful device closure of aorto-right ventricular tunnel (ARVT) in a 2 year-old-boy presenting with recurrent respiratory tract infection and poor weight gain. He was initially diagnosed with coronary arteriovenous fistula after the clinical examination and echocardiogram. However, his cardiac catheterization revealed ARVT. Although most of the aorto-ventricular tunnels are managed by surgical intervention, we successfully managed our case with transcatheter intervention.

Aortic arch interruption is a rare cardiac malformation. In some cases, it is part of a more complex congenital heart disease. Survival of extremely low birth weight infants with this cardiac disease is very rare. Surgical correction is the only treatment. Ductal stenting as a bridge procedure in small affected infants is a good alternative.

The right ventricular outflow tract (RVOT) stenting is used for the treatment of Fallot-type lesions when not amenable to complete correction or when surgical palliation carries a substantial risk. Despite the increasing clinical application, there is a lack of data that describe the RVOT morphology after stenting. This article elucidates post-RVOT stenting and in-stent stenosis, which is thought to be a zonal phenomenon, in this case, predominantly occurring proximally, in the portion of the stent apposing the RVOT infundibulum.

Modified Blalock‒Taussig shunt is a commonly performed palliative cardiac surgery. Complications like early or late occlusion have been reported in approximately 10% of patients. Thrombosis, stenosis, or kinking are possible mechanisms that lead to shunt obstruction which may be life-threatening and need urgent intervention in form of medical, surgical, or interventional catheterization. Traditionally, these patients are managed in the operating room with shunt revision but with advancing techniques, percutaneous interventions are being increasingly deployed to salvage these patients effectively. We describe our experience of two cases of blocked modified Blalock‒Taussig shunt, which was successfully managed by stenting the shunt.

Pulmonary arteriovenous malformations after the Kawashima procedure causing severe hypoxemia are treated by Fontan surgery that redirects hepatic venous blood to the pulmonary circulation. Alignment of the hepatic venous conduit toward both pulmonary arteries is crucial for their regression. Persistent hypoxemia due to nonregressed malformations in one lung often warrants a repeat surgery to redirect hepatic venous flows. Catheter-based redirection of Fontan flows is not commonly performed as metallic stents and devices may predispose to thrombus formation in the circuit. This report highlights a patient with persistent arteriovenous malformations in the left lung leading to severe disabling hypoxemia that was managed in the catheterization laboratory with an innovative strategy to redirect Fontan flows toward the affected left lung.

Interrupted aortic arch (IAA) associated with an aortopulmonary window (APW) is a rare cardiac malformation that needs an immediate diagnosis after birth and surgical treatment to avoid irreversible pulmonary lesions. Herein, we describe a case of successful staged treatment of premature neonate using many operative procedures. The first stage was stenting of patent ductus arteriosus with banding of pulmonary artery branches using a hybrid approach. At the age of one, he underwent total correction of malformation using Gore-Tex patch. Two procedures of implantation and redilatation of the previously implanted stents were performed in the following years. In the 8-year follow-up, the patient has been in good clinical condition, without cardiac symptoms. To the best of our knowledge, this is the first case report representing such a long-term follow-up of treatment of IAA accompanied by APW.

Transcatheter closure of patent ductus arteriosus (PDA) for preterm infants is increasingly performed after the Food and Drug Administration approval of the Amplatzer Piccolo Occluder device (Abbott Inc., Abbott Park, IL, USA) in the United States. We report three babies in whom transcatheter closure of PDA was unsuccessful; however, the PDA closed spontaneously in 1–5 days after the transcatheter attempt. The PDA remained closed during follow-up at 3 weeks, 10 weeks, and 17 months, respectively. Mechanical stimulation of the PDA by the wire during attempted device closure likely induced the PDA closure. Further studies are needed to evaluate whether this is a useful alternative therapy in this patient population.

Proximal stent migration in setting of transcatheter management of coarctation of the aorta is a rare complication, which may require emergency surgery. Herein, we report a successful transcatheter management of proximal stent migration in a high surgical risk, a 14-year-old girl, which caused partial occlusion of both the descending aorta and the left subclavian artery.

Acquired aortopulmonary fistula (APF) in the setting of repaired congenital heart disease is extremely rare but potentially fatal, so timely diagnosis and treatment are critical. We present a case of an 8-year-old female with a history of complex Taussig-Bing anomaly, who underwent an arterial switch procedure with LeCompte maneuver and ventricular septal defect closure early in life. The patient developed neopulmonary stenosis and branch pulmonary artery (PA) stenosis, for which she underwent patch augmentation and balloon dilatation of the left PA. The patient presented with a fistula between the ascending aorta and the left branch PA, confirmed by echocardiography and cardiac catheterization. She underwent repair of the APF with a homograft patch reconstruction of the ascending aorta.