Pulmonary Atresia Intact Ventricular Septum Research Articles

Perfusion Strategy to Prevent Right Ventricular Decompression on Cardiopulmonary Bypass During Extracardiac Fontan for Right Ventricle-Dependent Coronary Circulation.

Early and long-term outcomes in patients with pulmonary atresia-intact ventricular septum undergoing staged univentricular palliations have been known to be adversely affected by the presence of right ventricle-dependent coronary circulation. We describe a surgical technique to circumvent the coronary insufficiency caused by acute decompression of the right heart.

Transcatheter atrial septal defect closure late after completion of biventricular circulation in patients with pulmonary atresia intact ventricular septum or critical pulmonary stenosis.

This study aimed to explore anatomical and hemodynamic features of atrial septal defect, which was treated by transcatheter device closure late after completion of biventricular circulation in patients with pulmonary atresia and intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). We studied echocardiographic and cardiac catheterization data, including defect size, retroaortic rim length, single or multiple defects, the presence of malalignment atrial septum, tricuspid and pulmonary valve diameters, and cardiac chamber sizes, in patients with PAIVS/CPS who underwent transcatheter closure of atrial septal defect (TCASD), and compared to control subjects. A total of 173 patients with atrial septal defect, including 8 patients with PAIVS/CPS, underwent TCASD. Age and weight at TCASD were 17.3 ± 18.3 years and 36.6 ± 13.9 kg, respectively. There was no significant difference in defect size (13.7 ± 4.0 vs. 15.6 ± 5.2 mm, p = 0.317) and the retro-aortic rim length (3.7 ± 4.3 vs. 3.6 ± 0.3.1 mm, p = 0.948) between the groups; however, multiple defects (50% vs. 5%, p < 0.001) and malalignment atrial septum (62% vs. 14%. p < 0.001) were significantly frequent in patients with PAIVS/CPS compared to control subjects. The ratio of pulmonary to systemic blood flow in patients with PAIVS/CPS was significantly lower than that in the control patients (1.2 ± 0.4 vs. 2.0 ± 0.7, p < 0.001); however, four out of eight patients with atrial septal defect associated with PAIVS/CPS had right-to-left shunt through a defect, who were evaluated by the balloon occlusion test before TCASD. The indexed right atrial and ventricular areas, the right ventricular systolic pressure, and mean pulmonary arterial pressure did not differ between the groups. After TCASD, the right ventricular end-diastolic area remained unchanged in patients with PAIVS/CPS, whereas it significantly decreased in control subjects. Atrial septal defect associated with PAIVS/CPS had more complex anatomy, which would be a risk for device closure. Hemodynamics should be individually evaluated to determine the indication for TCASD because PAIVS/CPS encompassed anatomical heterogeneity of the entire right heart.

Ductus arteriosus morphology in duct-dependent pulmonary circulation: CT classification and pattern in different ventricular morphology.

The objective was to study the ductus arteriosus morphology in duct-dependent pulmonary circulation and its pattern in different ventricle morphology using CT angiography. From January 2013 to December 2015, patients aged 6 months and below with duct-dependent pulmonary circulation underwent CT angiography to delineate the ductus arteriosus origin, tortuosity, site of insertion, and pulmonary artery anatomy. The ductus arteriosus were classified into type I, IIa, IIb, and III based on its site of origin, either from descending aorta, distal arch, proximal arch, or subclavian artery, respectively. A total of 114 patients and 116 ductus arteriosus (two had bilateral ductus arteriosus) were analysed. Type I, IIa, IIb, and III ductus arteriosus were seen in 13 (11.2 %), 71 (61.2%), 21 (18.1%), and 11 (9.5%), respectively. Tortuous ductus arteriosus was found in 38 (32.7%), which was commonly seen in single ventricular lesions. Ipsilateral and bilateral branch pulmonary artery stenosis was seen in 68 (59.6%) and 6 (5.3%) patients, respectively. The majority of patients with pulmonary atresia intact ventricular septum had type I (54.4%) and non-tortuous ductus arteriosus, while those with single and biventricular lesions had type II ductus arteriosus (84.9% and 89.7%, respectively). Type III ductus arteriosus was more common in biventricular lesions (77.8%). Ductus arteriosus in duct-dependent pulmonary circulation has a diverse morphology with a distinct origin and tortuosity pattern in different types of ventricular morphology. CT may serve as an important tool in case selection and pre-procedural planning for ductal stenting.

Abstract 14475: Use of Extracorporeal Membrane Oxygenation in Neonates With Pulmonary Atresia and Intact Ventricular Septum - A Multicenter Study

Introduction: Data are limited on use of extracorporeal membrane oxygenation (ECMO) in neonates with pulmonary atresia intact ventricular septum (PAIVS). We aimed to describe ECMO use, risk factors, and associated outcomes in this population. Methods: This is a retrospective analysis of neonates with PAIVS who received ECMO between 2009-2019 in 19 US hospitals affiliated with the Collaborative Research for the Pediatric Cardiac Intensive Care Society (CoRe-PCICS). Patient demographics and clinical, procedural, and imaging data were collected from the medical record. Variables significant on univariate analysis were entered into a logistic regression and a model built to calculate estimated risk of ECMO. A similar analysis compared patients who received ECMO and experienced inpatient mortality to those who did not. A receiver operator curve analysis was conducted to determine the accuracy of this risk score in predicting inpatient mortality. A p-value &lt;0.05 was statistically significant. Results: Of 295 neonates with PAIVS, 32 (11%) were supported with ECMO. By multivariable analyses, a higher left pulmonary artery z-score (OR 2.0; p-value &lt;0.01) and the presence of ventriculocoronary connections by cardiac catheterization (OR 3.4; p-value 0.03) was associated with an increased risk of receiving ECMO. The resulting risk score had an area under the curve of 0.71 for prediction of need for ECMO. Of these 32 ECMO patients, 15 (46%) experienced mortality. By univariate analyses, those who were supported with ECMO, and experienced inpatient mortality had a higher gestational age (p-value 0.04). No significant associations were identified in those supported with ECMO and any independent variable. Conclusions: In a multicenter cohort of patients with pulmonary atresia with intact ventricular septum, 11% received ECMO. Of those supported with ECMO, 46% experienced inpatient mortality. There are risk factors that can help in identifying those who may require ECMO.

Computed Tomographic Angiography Provides Reliable Coronary Artery Evaluation in Infants With Pulmonary Atresia Intact Ventricular Septum

Evaluate the use of coronary CTA as an initial assessment for determining Right Ventricle Dependent Coronary Circulation (RVDCC) in neonates with Pulmonary Atresia with Intact Ventricular Septum (PA IVS). Retrospective review of cases with coronary CTA and compare with available catheter angiography, pathology, surgical reports, and outcomes from Mar 2015 to May 2022. In our cohort of 16 patients, 3 were positive for RVDCC, confirmed by pathologic evaluation, and there was concordance for presence or absence of RVDCC with catheter angiography in 5 patients (4 negatives for RVDCC, 1 positive). Clinical follow up for the 8 patients that underwent RV decompression had no clinical evidence of myocardial ischemia. Our findings suggest that coronary CTA is reliable as first-line imaging for determination of RVDCC in neonates with PA IVS. These findings, if supported by further prospective study, may reserve invasive coronary angiography for cases with diagnostic uncertainty or at the time of necessary transcatheter interventions.

Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study.

Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT. Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality. Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p=.004). While waitlist mortality was similar between groups (p=.09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p=.03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p=.03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p < .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival. Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias.

A retrograde approach for transcatheter valvotomy procedure in infants with pulmonary atresia intact ventricular septum (PA-IVS): retrograde versus antegrade approach.

This study evaluates the retrograde approach compared to the antegrade approach in infants with PA-IVS who underwent transcatheter pulmonary valvotomy procedure at National Cardiovascular Center Harapan Kita, Jakarta, Indonesia. This is a single-centre retrospective study conducted from January 2017 to June 2019 consisting of infants undergoing transcatheter pulmonary valvotomy procedures from our centre. Among 3733 records of cardiac catheter procedure in paediatric patients during the last 3 years, there were 12 subjects with PA-IVS, where five subjects were done by antegrade approach and seven by retrograde approach. The retrograde approach is shown to excel the antegrade approach in terms of procedural time by 58.64 minutes (CI 95 % 32.97-84.29, p = 0.008) and PA-RV crossing time by 27 minutes (CI 95 % 14.01-39.99, p = 0.02). There was no significant difference in contrast used (120.23 ± 25.77 versus 150.27 ± 39.26 ml/BSA, p = 0.518), and right ventricle to pulmonary artery systolic pressure gradient after valvotomy (39.571 ± 5.814 versus 53.52 ± 29.15, p = 0.329) between the retrograde and the antegrade approach. The retrograde approach offered shorter procedural time and comparably satisfying results than the antegrade approach. The shorter procedural time was preferred due to the shorter duration of general anaesthesia, which may decrease the risk of neurodevelopmental deficits in the patient.

Very preterm and very low birthweight infant with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, and discontinuous pulmonary arteries.

Prematurity and low birthweight are associated with increased mortality in infants undergoing cardiac surgery. Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation carries one of the highest risks of mortality. We present a patient who was born at 28 weeks of gestation at 1.2 kg, with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, coronary artery atresia, and discontinuous pulmonary arteries, who successfully underwent palliation with a modified Blalock-Taussig shunt, pulmonary arterioplasty, and subsequently a bidirectional Glenn.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality.

Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences. This is a 30-year (1988-2018) retrospective study of patients with pulmonary atresia intact ventricular septum. Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock-Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001). Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

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 5-15ng/ml. After neonatal DS, drug levels were assessed at 24 h, 7days 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 5.5days 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 22mm length, 24-h drug levels were less than 5ng/ml. Even though 24-h levels were above 5ng/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 8-27months till their elective corrective surgery in others. Sirolimus levels were acceptable at 24 h in all neonates receiving single stent under 22mm length. In patients needing two stents, drug levels were in immunosuppressive range at 24 h but reduced rapidly within 7days. The palliation provided by sirolimus-eluting DS was sufficiently long to provide clinical benefit.

Prediction of biventricular repair by echocardiography in borderline ventricle

Objective:In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. This review evaluated ventricular development using echocardiography for a better prediction of biventricular repair in borderline ventricle.Data sources:Articles from January 1, 1990 to April 1, 2019 on biventricular repair in borderline ventricle were accessed from PubMed, using keywords including “borderline ventricle,” “congenital heart disease,” “CHD,” “echocardiography,” and “biventricular repair.”Study selection:Original articles and critical reviews relevant to the review's theme were selected.Results:Borderline left ventricle (LV): (1) Critical aortic stenosis: the Rhodes score, Congenital Heart Surgeons Society regression equation and another new scoring system was proposed to predict the feasibility of biventricular repair. (2) Aortic arch hypoplasia: the LV size and the diameter of aortic and mitral valve (MV) annulus should be taken into considerations for biventricular repair. (3) Right-dominant unbalanced atrioventricular septal defect (AVSD): atrioventricular valve index (AVVI), left ventricular inflow index (LVII), and right ventricle (RV)/LV inflow angle were the echocardiographic indices for biventricular repair. Borderline RV: (1) pulmonary atresia/intact ventricular septum (PA/IVS): the diameter z-score of tricuspid valve (TV) annulus, ratio of TV to MV diameter, RV inlet length z-score, RV area z-score, RV development index, and RV-TV index, etc. Less objective but more practical description is to classify the RV as tripartite, bipartite, and unipartite. The presence or absence of RV sinusoids, RV dependent coronary circulation, and the degree of tricuspid regurgitation should also be noted. (2) Left-dominant unbalanced AVSD: AVVI, LV, and RV volumes, whether apex forming ventricles were the echocardiographic indices for biventricular repair.Conclusions:Although the evaluation of echocardiography cannot guarantee the success of biventricular repair surgery, echocardiography can still provide relatively valuable basis for surgical decision making.

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices. Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.6 months (range: 2.7-51.7). Right ventricle coronary dependent flow to the left ventricular myocardium was excluded. All four patients had dual perfusion with competitive flow from the RV through the sinusoids to the coronary arteries. Devices used were: Amplatzer vascular plug II of 10-16 mm; 27 coils (diameter 5-15 mm) in the oldest patient. Right ventricular angiography after cavity obliteration showed no more significant coronary perfusion through the sinusoids. There were no complications or deaths. Only minor and transient changes in the levels of troponin were observed. Coronary angiography at pre-Fontan evaluation showed no progress of coronary abnormalities in two patients. In selected patients with functionally single left ventricle, obliteration of the hypertensive RV cavity by percutaneous devices is safe and abolishes the systolic pressure wave in coronary sinusoids. When performed early, this may halt coronary damage and avoid excess mortality.

Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.

In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited. To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies. Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia. This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potentialunder-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study.

In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1-year or prior to Glenn circulation). Eighty-one patients were included. At last follow-up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left-to-right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z-score (P=.017), ≥ moderate baseline TR (P=.045) and smaller baseline RV area (P<.001) were associated with larger increases in RV area. Baseline RV area ≥6cm2 /m2 had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8cm2 /m2 at follow up achieved 2V circulation. This finding was confirmed in a validation cohort from a separate center (N=25). Factors associated with achieving RV area ≥8cm2 /m2 included larger TV z-score (P=.004), ≥ moderate baseline TR (P=.031), and ≥ moderate postdecompression pulmonary regurgitation (P=.002). Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume-loading conditions signal increased capacity for growth sufficient for 2V circulation.

Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation.

Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long-term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure. We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. Demographic and procedural data were correlated with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biventricular circulation. The procedural success rate was 92% (33 out of 36) in this group with moderate right ventricular hypoplasia (tricuspid valve z score -4.2±3.0, 69.4% of patients with z score <-2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock-Taussig shunt. Overall reintervention-free survival was 53%, 30%, and 19% at 1, 6, and 12months postintervention. Despite no significant catch-up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circulation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%) CONCLUSION: Encouraging long-term results with biventricular circulation and functional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.

Major Aortopulmonary Collateral Arteries With Anatomy Other Than Pulmonary Atresia/Ventricular Septal Defect

Major aortopulmonary collateral arteries (MAPCAs) are frequently found in association with pulmonary atresia with ventricular septal defect (PA/VSD). However, some patients with MAPCAs do not have PA/VSD but have a variety of other "atypical" anatomic diagnoses. This was a retrospective review of patients with MAPCAs and atypical anatomy. The 50 patients with MAPCAs could be divided into two subgroups: (1) single ventricle anatomy (n= 33) and (2) two ventricle anatomy (n= 17). The 33 patients with MAPCAs and single ventricle included 15 with unbalanced complete atrioventricular canal (CAVC), 6 with pulmonary atresia-intact ventricular septum, and 12 with other forms of single ventricle. The initial cardiac operation included unifocalization/shunt in 24 patients and creation of aortopulmonary window or central shunt in 9 patients. There were seven operative and eight late deaths. Sixteen patients have had a bidirectional Glenn procedure and6 had a Fontan procedure. The 17 patients with MAPCAs and two ventricles included 5 with CAVC, 4 with corrected transposition, 3 with double outlet right ventricle, 3 with scimitar syndrome, and 2 with complex D-transposition. The initial cardiac operation included single-stage complete repair in 5 patients, unifocalization/shunt in 10 patients, and aortopulmonary window in 2 patients. There were two operative and two late deaths. Thirteen patients have achieved complete repair status. The data demonstrate the wide diversity of anatomy seen in patients with MAPCAs when evaluating diagnoses other than PA/VSD. Two-thirds of the patients had single ventricle and was associated with a relatively high mortality.

Pulmonary Atresia/Intact Ventricular Septum: Influence of Coronary Anatomy on Single-Ventricle Outcome

We investigated the influence of coronary artery abnormalities on outcome in patients with pulmonary atresia/intact ventricular septum (PA-IVS) for planned single-ventricle palliation. Catheterization and medical records were reviewed in patients with PA-IVS for planned single-ventricle palliation at our institution between 2000 and 2012. Primary outcome was death or transplantation. Patients with confirmed or strong suspicion of stenosis in 2 or more main coronary arteries or coronary ostial atresia were defined as having right ventricle-dependent coronary circulation (RVDCC); those with stenosis of 1 main vessel or normal anatomy were defined as having non-RVDCC. Of 58 patients with PA-IVS, 17 (30%) underwent single-ventricle palliation. Ten (59%) had RVDCC (3with ostial atresia) and 7 (41%) had non-RVDCC. Median follow-up time was 8.2 years (0 months-11.3 years), with 1 patient in each group lost to follow-up. Five patients with RVDCC died, including the 3 patients with ostial atresia, and 1 underwent transplantation at 6 months of life. No deaths occurred after second-stage palliation. Three of the 4 surviving patients with RVDCC completed a Fontan operation, and 2 of these patients had evidence of cardiac ischemia on follow-up. No deaths occurred among patients with non-RVDCC. Kaplan-Meier analysis demonstrated significantly better survival in patients with non-RVDCC (100%) than in patients with RVDCC (40%) (p= 0.026). In patients with PA-IVS undergoing single-ventricle palliation, RVDCC is associated with high early mortality, especially with coronary ostial atresia. There should be early consideration of transplantation inneonates with RVDCC. Patients with non-RVDCC undergoing single-ventricle palliation have excellent long-term outcomes, with no mortality seen in this series.

Unusual, Rare Case of Ascending Aortic Atresia and Right Ventricle Dependent Coronary Circulation in a Newborn

We describe a newborn with complex, congenital cardiac anomalies, most notably single ventricle physiology, interruption/atresia of the ascending aorta and a coronary circulation that was totally Right Ventricle (RV) dependent. This arrangement left the coronary supply in a very precarious situation, theoretically much worse than most patients with Pulmonary Atresia/Intact Ventricular Septum ( PA/IVS), since the entire coronary circulation was RV dependent. The patient underwent a palliative surgical correction, but expired the following day. We believe this is the first reported case in the literature with this constellation of lesions.