Ventricle-dependent Coronary Circulation Research Articles

Percutaneous transient occlusion of the transtricuspid flow: a new method to evaluate the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with an intact ventricular septum is characterised by heterogeneity in right ventricle morphology and coronary anatomy. In some cases, the presence of ventriculocoronary connections may promote coronary artery stenosis or interruption, and aortic diastolic pressure may not be sufficient to drive coronary blood flow. This requires a correct evaluation (currently done by angiography) which depends on whether the patient can be offered decompression of the right ventricle. To date, there is no objective method to do so, so we designed a percutaneous, transitory technique with the purpose of occluding the transtricuspid anterograde flow. The manoeuverer was performed in a 25-day-old female with pulmonary atresia with intact ventricular septum, right ventricle at suprasystemic level, and selective coronarography was not conclusive, the anterior descendant with stenosis in its middle third and from this point, thinner with to-fro flow. Occlusion was performed with a balloon catheter. We re-evaluated the coronary flow and the normalised anterior descendant flow. We hope that with this new method, we can give a more accurate diagnosis and determine the cases in which the coronary circulation is truly not right ventricle dependent to offer a greater number of patients biventricular or 1.5 ventricular repairs and thereby improve their quality of life and survival, the ones that turn out to be right ventricular dependant; offer them an early reference for cardiac transplant or in case it is not available to consider univentricular palliation knowing that this probably would not reduce the risk of ischaemia and/or death over time.

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.

Fetal pulmonary valvuloplasty for pulmonary atresia with intact ventricular septum: a single-center clinical experience.

Fetal pulmonary valvuloplasty for pulmonary atresia with intact ventricular septum: a single-center clinical experience.

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.

Right ventricle-dependent coronary circulation diagnosed by non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

Duct Stenting vs. Modified Blalock-Taussig Shunt: New Insights Learned From High-Risk Patients With Duct-Dependent Pulmonary Circulation.

BackgroundAs no data were available on the comparison of outcomes between modified Blalock-Taussig shunts (MBTs) vs. duct-stenting (DS) in patients with pulmonary atresia (PA) and an increased ductal tortuosity and in patients with pulmonary atresia and intact septum (PA-IVS) with right ventricle-dependent coronary circulation (RVDCC), we aimed to perform a single-center retrospective evaluation.MethodsBetween 2010 and 2019, 127 patients with duct-dependent pulmonary circulation (DDPC) underwent either MBTs (without additional repairs) (n = 56) or DS (n = 71). The primary endpoint was defined as arriving at the next planned surgery (Glenn or biventricular repair) avoiding one of the following: (1) unplanned surgery or unplanned perforation of the pulmonary valve (PVP) with a stent, (2) procedure-related permanent complications, and (3) death. Two subgroups were considered: (1) patients who had a ductal curvature index (DCI) >0.45 (n = 32) and (2) patients with PA-IVS and RVDCC (n = 13). Ductal curvature index (DCI) was measured in all the patients to assess the tortuosity of the ducts. Patients with DCI >0.45 were considered as being in a high-risk group for the duct-stenting; a previous study showed that the patients with a DCI < 0.45 had a better outcome when compared with those with a DCI> 0.45.ResultsThe primary outcome was achieved equally in the two groups (77.5% in DS, 75% in MBTs). Hospital deaths, need for ECMO, and the occurrence of major complications was more frequent in the group with MBTs with an Odds Ratio (OR) of 5, 0.8, and 4, respectively, and a 95% Confidence Interval (CI) 1.1–22.6, 0.7–0.9, and 1.6–10.3, respectively, and a P-value < 0.05. For the two subgroups, the primary outcome was achieved in 64% of patients with a DCI >0.45 who received MBTs compared to 20% in those with DS (OR 3.5, 95% CI 1.2–10, P 0.005). While 74.1% of the patients with PA-IVS and RVDCC after DS had achieved the primary outcome, all patients with MBTs had an impaired outcome (OR 3.5, 95%CI 1–11.2, P 0.004).ConclusionMBTs showed a better outcome in patients with tortuous ducts compared to DS. DS seems to be superior in patients with DDPC with DCI <0.45 and patients with PA-IVS with RVDCC.

Pulmonary atresia with intact ventricular septum: Intended strategies

ObjectiveNeonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies. MethodsRetrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies. ResultsOf 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of −1.59 (interquartile range, −3.03 to 0.21) and −5.12 (interquartile range, −5.60 to −4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001). ConclusionsPulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation.

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.

Right Ventricle-Dependent Coronary Circulation: Location of Obstruction Is Associated With Survival

Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium. We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC. Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio= 3.63; 95% confidence interval, 1.01-13.00; P= .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P= .217). The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC.

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.

Myocardial strain abnormalities in fetuses with pulmonary atresia and intact ventricular septum.

Global and regional myocardial deformation have not been well described in fetuses with pulmonary atresia and intact ventricular septum (PA/IVS). Speckle-tracking echocardiography (STE), an angle-independent technique for assessing global and regional strain, may be a more sensitive way of determining ventricular systolic dysfunction compared with traditional 2D echocardiography. The aim of this study was to assess myocardial deformation in fetuses with PA/IVS compared with control fetuses and to determine if, in fetuses with PA/IVS, strain differs between those with and those without right ventricle-dependent coronary circulation (RVDCC). This was a retrospective analysis of fetuses with PA/IVS examined at two medical centers between June 2005 and October 2017. Left ventricular (LV) and right ventricular (RV) regional and global longitudinal strain (GLS) and strain rate were obtained using STE, and comparisons were made between fetuses with PA/IVS and gestational age (GA)-matched controls. Postnatal outcome was assessed, including the presence of RVDCC. Fifty-seven fetuses with PA/IVS and 57 controls were analyzed at a mean GA of 26.5 ± 5 weeks. LV-GLS was significantly decreased in fetuses with PA/IVS compared with controls (-17.4 ± 1.7% vs -23.7 ± 2.0%, P < 0.001). LV strain rate was also significantly decreased (-1.01 ± 0.21/s vs -1.42 ± 0.20/s, P < 0.001). Fetuses with PA/IVS had decreased strain in all segments. Similarly, RV strain was significantly decreased in fetuses with PA/IVS (-11.6 ± 3.8% vs -24.6 ± 2.5%, P < 0.0001). Thirty-six patients had postnatal cardiac catheterization performed to define coronary anatomy; 10 fetuses had RVDCC. Fetuses with RVDCC had decreased LV strain compared with those without (-15.8 ± 1.2% vs -17.9 ± 1.7%, P = 0.009). RV strain was also decreased in fetuses with RVDCC vs those without (-7.0 ± 2.9% vs -12.1 ± 3.2%, P = 0.0004). Fetuses with PA/IVS have decreased global and regional LV and RV strain compared with controls. The finding of decreased LV strain may be due to altered ventricular mechanics in the context of a hypertensive right ventricle and/or abnormal coronary perfusion. Moreover, fetuses that were found to have RVDCC postnatally had decreased LV and RV strain compared with those that did not. These results encourage further investigation to assess whether fetal ventricular strain could be a prenatal predictor of RVDCC. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Left ventricular function after a Fontan operation in patients with pulmonary atresia with an intact ventricular septum.

In patients with pulmonary atresia with an intact ventricular septum (PA/IVS), there are no reports about the impact of sinusoidal communication (SC) on left ventricular (LV) performance after a Fontan operation; therefore, the purpose of this study was to fill this gap in the literature. We performed a single-centre, retrospective study of 46 patients with PA/IVS. Nineteen patients who underwent the Fontan procedure were enrolled and divided into 2 groups: those with SC (SC group) and those without SC (N group). The 2 groups were compared in terms of postoperative cardiac function. Thirteen patients were in the SC group (4 patients with right ventricle-dependent coronary circulation) and 6, in the N group. Although 2 of the patients with right ventricular (RV)-dependent coronary circulation showed focal asynergy of the LV wall, others showed no findings of myocardial infarction. The patients' preoperative age and age during the observation period after the Fontan operation did not differ. There was no difference in LV ejection fraction, cardiac index and RV and pulmonary artery pressure before and after the Fontan operation. Preoperative RV volume in the N group was greater than that in the SC group. Brain natriuretic peptide levels were higher in the SC group after the Fontan operation. On ventricular efficacy analysis, contractility was lower (1.8 ± 0.32 vs 2.5 ± 0.40 mmHg/ml/m2, P = 0.001) and ventricular efficacy was worse (1.0 ± 0.15 vs 0.86 ± 0.11, P = 0.046) in the SC group during the postoperative period. Compared to patients with PA/IVS without SC, patients with PA/IVS with SC had a low cardiac contractility and decreased cardiac output efficiency after the Fontan procedure.

Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.

The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. The size of the tricuspid valve and right ventricular cavity as well as the presence of right ventricle-dependent coronary circulation help to dichotomize between biventricular repair versus univentricular palliation or heart transplantation, as well as predicting the expected survival. The delineation and understanding of these features help to dictate both medical and surgical management.

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.

Short-and mid-term outcomes of transcatheter intervention for critical pulmonary stenosis and pulmonary atresia with intact ventricular septum in neonates

To assess the safety and efficacy of transcatheter intervention for critical pulmonary stenosis (CPS) and pulmonary atresia with intact ventricular septum (PA/IVS) in neonates. From June 2006 to September 2011, 27 neonates including CPS in 19 patients and PA/IVS in 8 patients underwent transcatheter intervention. All patients had membranous stenosis or atresia without severe Ebstein's anomaly and severe right ventricle and pulmonary valve hypoplasia, without right ventricle-dependent coronary circulation in PA/IVS. The mean age was (16.8 ± 9.9) d. The mean weight was (3.3 ± 0.5) kg. Two of them were premature neonates, the weight was 2.3 kg and 2.5 kg, respectively. The procedural success, early outcome, complication rates, midterm results and pulmonary regurgitation were retrospectively studied. Twenty-six patients were successfully treated with transcatheter intervention. Right ventricular pressure fell from (112.0 ± 21.0) mm Hg (1 mm Hg = 0.133 kPa) to (50.4 ± 15.9) mm Hg (P < 0.001). The ratio of right ventricular pressure and aortic pressure fell from 1.7 ± 0.1 to 0.7 ± 0.3 (P < 0.001). One patient died early of PA/IVS. Complication occurred in 5 patients. Hemopericardium occurred in 3 patients, tachyarrhythmia in 2 patients. Five patients needed prolonged prostaglandin E(1) infusion for 3 to 14 days because of desaturation after the procedure. No patient needed surgery in neonatal period. At a mean follow-up of (33.5 ± 18.3) months (from 6 months to 5 years), 21 patients had no further transcatheter or surgical intervention. Four patients with CPS had moderate to severe residual pulmonary stenosis after the procedure, 3 of them underwent a second balloon dilation at 3 months of follow-up, the other one was waiting for the second balloon dilation. One patient with PA/IVS was waiting for a bidirectional Glenn procedure because of chronic right ventricular failure. Mild pulmonary regurgitation occurred in 18 patients (69.2%), and moderate pulmonary regurgitation in 8 patients (30.8%). Transcatheter intervention for CPS and PA/IVS in neonates is safe and effective. It can avoid neonatal surgery. Some patients may require repeat balloon valvuloplasty in infant period. In most patients surgical or transcatheter intervention could be avoided and mild pulmonary regurgitation was the common finding in midterm follow-up.

Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum

To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum. Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome. Tertiary-care pediatric hospital. Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z-scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs. In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.

Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum

We report the case of a newborn with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation. He died several weeks after a Blalock-Taussig procedure because of a progressive stenosis of the main coronary artery. We present echocardiographic and dual-source CT images of the stenosis, with autopsy correlation. To our knowledge, CT images of this quality have never been reported in a newborn. This case illustrates the extreme difficulty in prognosticating the outcome for these patients and underlines the need for a detailed neonatal coronary mapping to assess right ventricle-dependent coronary circulation.

Aneurysm of Left Ventricular Apex in a Patient With Pulmonary Atresia, Intact Ventricular Septum

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5][![Graphic][6] ][6] A 12-month-old girl was referred for left ventricular (LV) aneurysm. She had pulmonary atresia, intact ventricular septum with right ventricle–dependent coronary circulation (

Individualized surgical management of pulmonary atresia with intact ventricular septum

To delineate individualized surgical management strategy for the optimal management of pulmonary atresia with intact ventricular septum (PA/IVS). Between 1997 to 2005, 52 consecutive patients with PA/IVS, 31 male and 21 female, aged 2-9, divided into 2 groups according if right ventricle-dependent coronary circulation (RVDCC) existed, underwent individualized surgical treatment. The patients in Group I (with: RVDCC, n = 6) underwent single ventricle repair. Forty-six patients were in Group II (without RVDCC), 13 of which with severe right ventricular hypoplasia (tricuspid valve Z value < -4)underwent single ventricle repair and 33 of which were with mild to moderate right ventricular hypoplasia (Z value > -4). One critical neonate underwent systemic- pulmonary artery shunt. The other 32 of the 33 patients received right ventricular decompression procedures firstly, and 6 of them presented optimal hemodynamics and oxygenation and achieved biventricular repair. If the patients presented unstable hemodynamics or hypoxemia after right ventricular decompression procedure, an additional shunt was added. 13 young infants (0-2 months) underwent additional systemic-pulmonary artery shunt, and 13 patients (> 3 months-old) underwent additional bidirectional cavopulmonary shunt (1.5 ventricular repair)The patients were followed up for 1-10 years. There were 1 early death in Group I and 7 early deaths in Group II. The total early mortality was 15%. A successful definitive repair was achieved in 26 cases (50%). Follow-up reported 3 late deaths. Individualized surgical management based on the presence of RVDCC, right ventricular hypoplasia grading, right ventricular growth potential, hemodynamic situation, and oxygen saturation after the decompression procedure is helpful to improve the surgical results of PA/IVS