Aortico right atrial tunnel - Clinical presentation, transcatheter management, and follow-up from a large cohort of patients.

Background: Aortico right atrial tunnel (ARAT) is a rare extracardiac communication between the aorta and the right atrium with two anatomical types. A recent global review identified 59 patients. Methods: Patients with ARAT from two centers were analyzed for their demographics, symptoms, morphology, management, and follow-up thromboprophylaxis. Results: Among 21 patients including 8 males with a median age of 3 years (18 days-72 years) diagnosed as ARAT, 12 (57%) had posterior tunnels and 9 had anterior tunnels. Four patients had multiple exits. Eighteen tunnels were closed after arteriovenous circuit formation. Six patients (29%) weighing <10 kg presented early with heart failure. Transcatheter closure normalized the hemodynamics including in one infant after failed surgery. Two elderly patients (10%) above 60 years presented with angina and atrial fibrillation. The rest were asymptomatic. Occluders were positioned in the narrow proximal aortic end of the tunnel in all except two patients, where the distal atrial end was closed. All procedures were successful without complications. There was one late death after 1 year from subarachnoid hemorrhage. At a median follow-up of 96 months, all survivors were asymptomatic. Thromboprophylaxis with dual antiplatelets for 1-2 years followed earlier was recently changed to aspirin with Coumadin. Complete remodeling occurred when the proximal aortic end was closed, but partial persistence of the track was noted after distal closure. Conclusions: This largest cohort of ARAT showed the safety and efficacy of transcatheter closure even in neonates. The narrow proximal aortic end should be the target for closure rather than the distal atrial end to achieve complete remodeling.

Transcatheter Covered Stent Exclusion of Superior Sinus Venosus Defects.

BACKGROUND: Transcatheter correction of sinus venosus defects (SVDs) using balloon-mounted covered stents provides an attractive surgical alternative. Surgery may be complicated by superior vena caval or right upper pulmonary vein (RUPV) stenosis, sinus nodal dysfunction, and residual additional pulmonary veins. OBJECTIVES: Being a new intervention, technical modifications would simplify the procedure, improve universal applicability, and reduce or tackle complications. METHODS: Patients were included if balloon interrogation of cavoatrial junction confirmed closure of SVD and redirected RUPV to the left atrium. A single-center experience was analyzed to summarize the procedural modifications over 8 years. Transesophageal echocardiogram (TEE) on follow-up was done to identify residual shunt, RUPV flows, and stent thrombosis. RESULTS: A total of 100 patients including 9 children with a median age of 35 years (range, 4-69 years) underwent SVD closure after balloon interrogation. Among 57 patients balloon interrogated in the first 5 years, 70% underwent transcatheter closure, with 2 failures. RUPV occlusion caused the exclusions. Inclusions improved to 94% among the subsequent 65 balloon interrogations when RUPV protection was implemented, with 1 failure. Stent embolization caused the 3 failures warranting surgery. Recent modifications included limited transesophageal echocardiogram without anesthesia, avoiding venovenous circuit, interrogation with semicompliant balloons, trans-septal RUPV protection, overlapping stents to permit additional vein drainage to superior vena cava and tackle embolizations. There were no deaths. Minor complications included stent embolizations stabilized in catheterization laboratory in 2 patients, left innominate vein jailing in 2 patients, insignificant residual flows, and nonocclusive asymptomatic stent thrombosis in 4 patients. CONCLUSIONS: Procedural success was 97%. Recent modifications increased patient inclusions, decreased complications, and simplified the intervention.

Risk factors associated with device embolisation or malposition during transcatheter closure of patent ductus arteriosus.

BACKGROUND: Device embolisation is a serious adverse event during transcatheter duct closure. This study analyses risk factors for embolisation. METHODS: Demographic parameters, echocardiographic anatomy, haemodynamics, and procedural characteristics of consecutive duct closures in a tertiary centre over 8 years were analysed. Procedures complicated by embolisation were compared to uncomplicated procedures. RESULTS: Fifteen embolisations occurred during 376 procedures. All except one embolisation were in infants. The pulmonary artery: aortic pressure ratio was 0.78 ± 0.22. Embolisation was seen significantly more commonly in Type C tubular ducts. Vascular plugs were more significantly associated with embolisations. Logistic regression analysis showed device embolisation was significantly higher in age group of < 6 months compared to 6-12 months (p = 0.02), higher in those with tubular ducts versus conical ducts (p = 0.003), use of vascular plugs compared to conventional duct occluders (p = 0.05), and in duct closure with undersized devices (p = 0.001). There was no in-hospital mortality. Three patients needed surgical retrieval while others were successfully managed in catheterisation laboratory. CONCLUSIONS: Device embolisation complicates 4% of transcatheter duct closures, with need for surgery in one-fifth of them. Larger ducts with high pulmonary artery pressures in younger and smaller infants are more often associated with device embolisation. Tubular ducts are more prone for embolisation compared to usual conical ducts. Softer vascular plugs are often associated with embolisations. Intentional device undersizing to avoid vascular obstruction in small patients is a frequent risk factor for embolisation. Precise echocardiographic measurements, correct occluder choice, proper technique and additional care in patients with high pulmonary artery pressures are mandatory to minimise embolisations.

Single center experience of transcatheter closure of mitral and aortic Paravalvular leaks using the new rechristened rectangular Amplatzer PVL plug.

Amplatzer Paravalvular leak (PVL) plug is rectangular in shape, that might suit closure of crescentic PVL. Among 79 transcatheter PVL closures from a single center, a subgroup of 16 patients who received Amplatzer PVL plugs were analyzed. All procedures were successful, as the plug auto-oriented to the leak, without mechanical leaflet interference, though needing additional 31 devices. Two patients needed an elective re-intervention. NYHA class improved from III-IV before procedure to less than II after procedure. Complications were not directly related to the Amplatzer PVL plug. The rectangular shape for crescentic leaks, auto-orientation and non-interference with mechanical leaflets were good attributes.

Transcatheter Versus Surgical Closure of Acute Ruptured Sinus of Valsalva Aneurysms with Associated Ventricular Septal Defects.

Transcatheter ruptured sinus of valsalva aneurysm (RSOVA) closure is an alternative to surgery. When a restrictive ventricular septal defect (rVSD) coexists, there are doubts about support for an occluder and persistent left ventricular (LV) dilatation. We compared the outcomes of patients from a single centre with rVSD and RSOVA after surgery or transcatheter intervention in the past 8 years in this study. Location of rVSD did not alter the approach. Compliant balloon interrogation before transcatheter closure was occasionally used to assess significance of interventricular shunt or aortic regurgitation. Procedural success and LV dimensions before and after treatment were analyzed. Seventeen patients with a mean age of 36.64 ± 12.58 years were analyzed. 12/13 catheter procedures (92%) were successful, of whom eight patients had an outlet VSD. rVSD was closed with device in one patient with perimembranous rVSD, but unaddressed in others. Four patients underwent balloon interrogation. Surgery included the single patient who failed intervention. Transcatheter group had more acute symptoms and advanced functional class than surgical group, though not significant. At a median follow-up of 4.5 (1-7.5) years, the mean LV dimensions (50.3 ± 10.1 mm and 50.1 ± 3.1 mm, p = 0.46) were not different in the transcatheter and surgical groups. Mean end-diastolic volumes (110.5 ± 42. 1 ml and 98.5 ± 37.5 ml, p = 0.91) were not significantly different either. Transcatheter RSOVA closure success was not altered by rVSD despite their location underneath the aneurysms, including outlet rVSD with defect in aortoventricular junction. Persistent LV dilatation was not observed after catheter intervention even when the VSD was not closed.

Study of the effect of Occlutech Atrial Flow Regulator on symptoms, hemodynamics, and echocardiographic parameters in advanced pulmonary arterial hypertension.

Optimal sized balloon atrial septostomy improves hemodynamics in advanced pulmonary arterial hypertension. Occlutech Atrial Flow Regulator is designed to provide an 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 after Atrial Flow Regulator implantation in patients with syncope or right-heart failure. Patients with high-risk predictors of mortality during septostomy were scrutinized. Thirty-nine patients (9 children) with syncope (34/39) or right-heart failure (27/39) underwent Atrial Flow Regulator implantation without procedural complications. Six-minute walk distance increased from 310 ± 158.2 to 376.4 ± 182.6 m, 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) L/min/m2 and systemic oxygen transport increased from 546.1 ± 157.9 (256.2-910.5) to 637.2 ± 191.1 (301.3-1020.2) ml/min. 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. Five of seven patients with advanced disease and key hemodynamic predictors of mortality survived. Acute hemodynamic benefits in pulmonary arterial hypertension after Atrial Flow Regulator were improved cardiac output, systemic oxygen transport, and reduced 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.

Are early palliative procedures providing an adequate long-term benefit in young cyanotic infants from developing countries, despite advances in surgery and interventions?

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.

Simple Diagnostic Tools May Guide Transcatheter Closure of Superior Sinus Venosus Defects Without Advanced Imaging Techniques.

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.

Serum Sirolimus Levels After Implantation of Third Generation Drug Eluting Cobalt Chromium Coronary Stent in Ductus Arteriosus in Neonates with Duct-Dependent Pulmonary Circulation.

Ductal stenting (DS) palliates duct-dependent lesions using coronary stents. Sirolimus-eluting stents have replaced bare-metal stents in coronary interventions. Concerns exist about sirolimus levels in neonates. Therapeutic immunosuppressive sirolimus level is 5-15 ng/ml. After neonatal DS, drug levels were assessed at 24 h, 7 days and monthly thereafter till they were undetectable. Clinical course, ductal patency till their final corrective surgery was analyzed. The exact quantity of sirolimus in each stent was known. Twelve neonates with median age of 5.5 days received sirolimus-eluting stents, one stent in nine and two in the rest. The lesions were pulmonary atresia intact ventricular septum(PAIVS) in four, univentricular lesions with pulmonary atresia in four, biventricular lesions with pulmonary atresia in three and right ventricular rhabdomyoma in one neonate. If single stents up to 22 mm length, 24-h drug levels were less than 5 ng/ml. Even though 24-h levels were above 5 ng/ml in patients with single longer stent or two stents, it reduced to very low levels by seventh day. Two hospital deaths included rhabdomyoma with complete heart block and post-valvotomy cardiac failure for PAIVS. Stent patency after valvotomy for PAIVS exceeded three years. Patency was retained for 8-27 months till their elective corrective surgery in others. Sirolimus levels were acceptable at 24 h in all neonates receiving single stent under 22 mm length. In patients needing two stents, drug levels were in immunosuppressive range at 24 h but reduced rapidly within 7 days. The palliation provided by sirolimus-eluting DS was sufficiently long to provide clinical benefit.