Pulmonary Atresia Research Articles

Unilateral pulmonary vein atresia.

This study aims to summarise the clinical characteristics of unilateral pulmonary vein atresia (UPVA) and compare the differences between Chinese cases and all published cases worldwide. We retrospectively enrolled 6 Chinese children with UPVA from January 2014 to January 2024 at a single centre. We reviewed their demographic data, clinical symptoms, laboratory tests, imaging examinations, treatment and prognosis to describe their clinical features. Additionally, the remaining 79 confirmed patients with UPVA, as described in 52 references, were also summarised. UPVA is sporadically distributed worldwide, with the highest number of reported cases in China (27/85) and the United States (20/85). In the overall cohort (85 cases), the patient median age at diagnosis was 5.2 years. The male-to-female ratio was 1:1. Right-sided UPVA was slightly more common, with a right-to-left ratio of 1.4:1. The most frequently reported clinical manifestations were recurrent pneumonia (79.2%), followed by recurrent haemoptysis (48.1%) and exercise intolerance (35.1%). Additionally, 10.4% of patients were asymptomatic. Congenital heart disease was observed in 34.1% of cases, and 20% of patients had comorbid pulmonary hypertension. The overall mortality rate was 8.9%. There were no statistically significant differences in the clinical characteristics of UPVA between Chinese patients and all published cases worldwide.

Surgical Repair of Tetralogy of Fallot With Pulmonary Atresia in Adults: Three Case Reports.

We present three cases of adults who underwent late repair of tetralogy of Fallot with pulmonary atresia (ToF-PA). All three patients underwent palliative procedures at an earlier age and were not considered candidates for corrective surgery due to borderline pulmonary artery anatomy. They developed symptomatic decline with increasing dyspnea, desaturation, and decreasing exercise tolerance; thus, they were reconsidered for complete surgical repair of ToF-PA in adulthood. Improvement in symptoms and oxygen saturation were achieved in all patients. With advances in surgical techniques and cardiopulmonary bypass support, it may be worthwhile to review patients who underwent palliative procedures earlier in life but previously were thought not to be surgical candidates. The discussion for potential operative management of these patients may be possible aftercareful reassessment of pulmonary artery anatomy and ventricular function.

Diagnostic characterization and morbi-mortality in pediatric patients with diagnosis of vascular ring

To analyze the clinical characteristics and natural history of pediatric patients with a diagnosis of vascular ring. We retrospectively studied the records of 110 patients of pediatric age, from January 2014 to December 2022, with the following variables: age, clinical manifestations, associated cardiac lesions, diagnostic method and surgical approach. Of the total, 60 (55%) were female and 50 (45%) were male. Diagnosis was made, on average, at 47 months of life. The vascular rings found were aberrant subclavian artery (82.8%), circumflex aorta (8.1%), double aortic arch (6.3%) and left pulmonary artery sling (1.8%). Ninety percent of the cases were associated with other congenital heart disease, mainly tetralogy of Fallot, pulmonary valve atresia and ventricular septal defect. Forty-five percent of the cases produced compression of structures and 77% of the patients were asymptomatic. Twenty-one percent of the patients received surgical treatment of which 35% presented postoperative complications: sepsis (26%), mediastinitis (4.3%) and cardiogenic shock (4.3%). Postoperative mortality was 17%. Within the natural history of symptomatic patients who remained in expectant management, 100% resolved their symptoms. In this series, the most frequent type of vascular ring was aberrant subclavian artery. Diagnosis is late due to the lack of specificity of its symptoms, being mainly respiratory. The symptoms of mild involvement resolve in most cases, associated with the growth of the affected structures and their laxity.

Role of tricuspid valve repair in pulmonary atresia with intact ventricular septum: fresh autologous pericardium as a viable option.

This study aimed to evaluate the surgical outcomes of tricuspid valve (TV) leaflet augmentation using fresh autologous pericardium (FAP) in patients with pulmonary atresia with intact ventricular septum (PA-IVS). Nine patients with PA-IVS underwent TV leaflet augmentation (TVLA) using FAP between January 2017 and April 2024. Native TV failure was defined as significant (≥moderate) tricuspid regurgitation and/or stenosis (mean pressure gradient >5 mmHg). Clinical outcomes, including both TV and right ventricle (RV) size and functions, were evaluated using echocardiography and magnetic resonance imaging. The median age and body weight at the time of surgery were 5.4 years and 21.2 kg, respectively. No operative mortality occurred. During the follow-up period, no late mortalities, reoperations or significant TV leaflet-related complications were observed; native TV failure was not observed in any patients, except one with significant tricuspid regurgitation. Biventricular repair was performed in all patients except one who had undergone preoperative one-and-a-half ventricle repair. After TVLA using FAP, the median TV annulus z-score remained within the normal ranges (preoperative -0.42 [-0.62 to 0.57] versus postoperative -0.51 [-0.95 to -0.13], mean difference: 0.231, 95% confidence interval: -0.338 to 0.800, P = 0.377) and tricuspid regurgitation improved significantly (mean difference: 2.111, 95% confidence interval: 1.510-2.712, P = 0.040). Postoperative magnetic resonance imaging showed that RV function, and cardiac output were preserved within acceptable ranges. TVLA using FAP could be a considerable and viable approach to optimize RV growth while preserving RV and TV functions in the long-term in young patients with PA-IVS.

Myocardial ischemia risk in Fontan candidates with pulmonary atresia with intact ventricular septum.

Sinusoidal communication (SC) is prevalent among Fontan candidates with pulmonary atresia with intact ventricular septum (PA/IVS), making it crucial in understanding its dynamics and potential impact on myocardial perfusion. This study aimed to assess the risk of myocardial ischemia in these patients. Sixteen Fontan candidates with PA/IVS were included in this study. All patients initially underwent Blalock-Taussig shunt (BTS), followed by bidirectional Glenn shunt in 15 cases, culminating in a total cavopulmonary connection (TCPC) at a median age of 24months. SC severity and risk of myocardial ischemia were assessed via angiography and scintigraphy, respectively. Changes in SCs across the procedural stages and their correlation with ischemic responses in scintigraphy were retrospectively analyzed. No deaths occurred during the study period. Ischemic events were observed in only one patient. SCs were observed in all 16 patients (100%). Significant regression of SCs occurred after BTS, particularly in coronary arteries with major SCs. However, no significant changes were found thereafter, particularly among coronary arteries without major SCs. No improvement or exacerbation of ischemic lesions was observed from pre- to post-TCPC. A positive correlation was revealed between angiographic SC grade and positive ischemic response on scintigraphy. In Fontan candidates with PA/IVS, an SC grade of 2 or higher was associated with a high risk of myocardial ischemia, emphasizing the need for careful monitoring and clinical management.

Analysis of 15 cases of ductus arteriosus stent placement without a guiding catheter through femoral artery approach

Objective: To investigate the feasibility of ductus arteriosus (DA) stent placement through femoral artery approach without guiding catheter. Methods: In this retrospective case study, the birth weight, intervention age, preoperative echocardiography, intraoperative angiography and other clinical data, as well as the follow-up status (Nakata index and final circulatory status, etc.) of 15 patients with congenital heart disease who are dependent on the DA for pulmonary circulation at Women and Children's Hospital, Qingdao University between January 2018 to June 2023 were collected. All pediatric patients underwent placement of a DA stent without a guiding catheter, the efficacy and safety of this approach were analyzed. Results: Among all 15 cases, there were 9 males and 6 females, with the birth weight of (3.3±0.3) kg and the intervention age of (15.0±0.1) d. Of which, there were 9 cases of pulmonary atresia with intact ventricular septum (PA-IVS), and 6 cases of critical pulmonary stenosis (CPS) with intact ventricular septum. The tricuspid annulus diameter before intervention was (8.8±0.8) mm, and the tricuspid annulus Z-score was -0.7±0.9. There were 3 cases of mild right ventricular dysplasia, 9 cases of moderate dysplasia, and 3 cases of severe dysplasia. All patients successfully completed the intervention without major complications. The narrowest diameter of the DA was (2.2±0.1) mm, the diameter of the DA stent was (3.9±0.3) mm, the preoperative percutaneous oxygen saturation (SpO2) was 0.83±0.03, and the postoperative SpO2 was 0.96±0.02. The follow-up time was (7.0±0.5) months. The Nakata index was (163±30) mm2/m2 before intervention, and was (173±34) mm2/m2 at the last follow-up. Biventricular circulation was successfully achieved in 14 patients, and the other one patient currently under close observation. Conclusion: The placement of a ductus arteriosus stent through femoral artery retrogradely without guiding catheter could serve as an effective and safe plan for neonates with PA-IVS or CPS accompanied by hypoplastic right heart syndrome.

Rehabilitation Strategy Should Not Be a Pretext for Suboptimal Repair for Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals are a challenging congenital anomaly to manage surgically with different centers and strategies producing wide-ranging outcomes. Over the past few decades despite diverging treatment pathways, there is an emerging consensus of how these patients "should" be treated. Quite often a combination of rehabilitation strategy and a unifocalization approach has to be tailored to each patient to address the anatomic and physiological variations that characterize this congenital heart defect. Irrespective of the surgical approach, the goal should be to have a complete repair with acceptable right heart pressure ensuring survival and a good quality of life.

Pathophysiology and management of adults with complex congenital heart disease after biventricular repair.

Pathophysiology and management of adults with complex congenital heart disease after biventricular repair.

Optimizing Anesthesia for a Pediatric Patient with Cyanotic Heart Disease: Pulmonary Atresia with MAPCAs

Pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs) is a rare and complex congenital heart anomaly marked by the absence of a direct connection between the right ventricle and the pulmonary arteries. The pulmonary blood supply originates via collateral arteries that arise from the aorta. Pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs) poses considerable difficulties in perioperative management, especially during general anesthesia for dental procedures in pediatric patients. This case report details the anesthetic management of a 5-year-old male with pulmonary atresia and MAPCAs undergoing dental extractions under general anesthesia. The anesthetic plan prioritized maintaining systemic blood pressure to ensure pulmonary perfusion, avoiding increases in pulmonary vascular resistance, and preventing myocardial depression. Anesthesia was induced with ketamine and fentanyl, while maintenance was achieved using sevoflurane and dexmedetomidine, with pressure-controlled ventilation to optimize hemodynamics. Postoperative recovery was uneventful, with the patient monitored in the pediatric intensive care unit. The management of patients with pulmonary atresia and MAPCAs requires careful planning and a multidisciplinary approach. A tailored anesthetic strategy emphasizing hemodynamic stability and oxygenation is critical. This report underscores the importance of understanding the pathophysiology of MAPCAs to optimize perioperative outcomes.

Tetralogy of Fallot With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries: Diagnostic Modalities-The Role of Computed Tomography, Cardiac Magnetic Resonance Imaging, and Three-Dimensional Modeling.

Cross-sectional imaging modalities facilitate the noninvasive acquisition of anatomic and hemodynamic data in patients with congenital heart disease. This is critical in patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF PA MAPCAs), who typically undergo multiple surgeries and interventional catheterizations over the course of their lifetime. Advances in cardiac computed tomography (CT) and cardiac magnetic resonance imaging (CMR) have enabled increased spatial resolution imaging and decreased scan times. High spatial resolution datasets provide a comprehensive evaluation of MAPCA anatomy including aortic origin location, lung lobes supplied, and the presence of stenoses. The physiologic data provided by CMR offers a noninvasive assessment of both anatomy and pulmonary blood flow, which can be utilized in decision-making and patient risk stratification. Furthermore, the three-dimensional (3D) datasets acquired by either imaging modality can be used to derive a 3D representation of patient-specific anatomy, visualized in a variety of formats including on-screen volume rendering, 3D prints, digital 3D models, and virtual reality. This article reviews the utility and added value of cardiac CT, CMR, and 3D modeling in the diagnosis and management of patients with TOF/PA/MAPCAs.

Pulmonary Atresia and Ventricular Septal Defect: Definitions, Nomenclature, and Classification.

This manuscript will provide information about pulmonary atresia + ventricular septal defect, including definitions, nomenclature, and classification, based on the 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). The 2021 IPCCC and ICD-11 provide the following definition for Tetralogy of Fallot with pulmonary atresia: Tetralogy of Fallot with pulmonary atresia is defined as "A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree." The 2021 IPCCC and ICD-11 provide the following definition for Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries: Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries is defined as "A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree and there are collateral blood vessels between the systemic and pulmonary arteries." "Tetralogy of Fallot + pulmonary atresia" is a specific type of "pulmonary atresia + ventricular septal defect" where the intracardiac malformation is more accurately defined. The presence or absence of major aortopulmonary collateral arteries (MAPCA[s]) does not change these definitions. Therefore, "tetralogy of Fallot + pulmonary atresia + MAPCA(s)" is a specific type of "pulmonary atresia + ventricular septal defect + MAPCA(s)" where the intracardiac malformation is more accurately defined. In the universe of patients with pulmonary atresia + ventricular septal defect, the anatomy and morphology of the pulmonary circulation to a large extent determines the surgical approach and overall outcome, with the intracardiac anatomy playing a secondary role. Based on the characterization of the pulmonary circulation, patients with pulmonary atresia + ventricular septal defect can be classified into three groups: In type A, pulmonary blood flow is provided by native pulmonary arteries (NPAs). In type B, pulmonary blood flow is provided by both NPA and MAPCA(s). In type C, pulmonary blood flow is provided only by MAPCA(s).

Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries: The Natural History and Late Presentation.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries represents a heterogenous and complex congenital heart defect. Treatment pathways vary widely depending on center expertise. The recommended approach in infancy is to achieve uniform pulmonary artery and capillary bed development, and a biventricular repair when feasible. The natural history of this condition is particularly relevant in low-middle income countries where early intervention is not possible due to poor access to cardiac surgical care. This group of patients may present in late childhood or as adults with symptoms of chronic cyanosis that may be associated with cardiac failure. The surgical approach to this group may range from complete correction to pulmonary artery rehabilitation or unifocalization, or consideration for heart-lung transplantation.

The Role of Collaboration in Prenatal Congenital Heart Disease Diagnosis: A Comparison of Maternal-Fetal Medicine Specialist and Pediatric Cardiologist Performance.

This study compared the accuracy of prenatal congenital heart disease (CHD) diagnosed by maternal-fetal medicine specialists (MFMs) and pediatric cardiologists (PCs), using postnatal cardiac findings as the reference standard. This retrospective analysis at Siriraj Hospital, Bangkok, Thailand, involved 125 pregnancies with fetal CHD diagnosed by MFMs and evaluated by PCs later. Prenatal CHD diagnoses by either MFM or PC were compared with postnatal diagnoses obtained through echocardiography, cardiac surgery/catheterization, or autopsy. Diagnostic accuracy was classified as (A) correct diagnosis, (B) minor differences not impacting clinical management or outcomes, or (C) major differences affecting prognosis or treatment. Cardiac sonography by MFM achieved diagnostic accuracies of 73.6% (A), 16% (B), and 10.4% (C), while fetal echocardiography by PC resulted in accuracies of 72% (A), 20% (B), and 8% (C). No statistically significant differences were found between MFM and PC in each category (P = .375-.832). The MFMs' accuracy was highest for tetralogy of Fallot (94.4%; 95% CI, 72.7-99.9%) and lowest for right atrial isomerism (71.4%; 95% CI, 29-96.3%) and pulmonary atresia with ventricular septal defect (57.1%; 95% CI, 18.4-90.1%). MFMs and PCs demonstrated high and comparable accuracy in prenatal CHD diagnosis. Although PCs tended to outperform MFMs in cases where misdiagnosis could significantly impact neonatal care and outcomes, MFMs can effectively perform primary screening for fetal CHD in all pregnancies. Collaboration with PCs remains essential when fetal CHD is suspected, particularly in complex cases.

Prenatal Opioid Use Disorder and the Risk of Congenital Anomalies in Offspring: A Population-Based Study.

To examine whether prenatal opioid use disorder (OUD) diagnosis is associated with the risk of congenital anomalies (CAs) in offspring. We conducted a population-based study of mother-newborn dyads comprising. 4143 761 births delivered in Canada from 2006 to 2021. We used robust Poisson regression to examine the association between prenatal OUD diagnosis and risk of non-chromosomal CAs, adjusted for maternal age, parity, multiple gestation, co-morbidities (including mental health disorders, chronic illnesses and other substance use disorders), and infant sex. We identified a total of 21, 638 births to persons who were diagnosed with prenatal OUD and 65, 992 (159.3 per 10,000) newborns with CAs. The overall risk of CAs was 2.3 times higher in infants born to birthing persons with a diagnosis of OUD (95% CI 2.2, 2.5). Compared to those without OUD diagnoses, births to persons with a diagnosis of OUD had a higher risk of specific types of congenital microcephaly (aRR 5.2, 95% CI 4.1, 6.6), cleft palate (RR 4.8, 95% CI 3.7, 6.1), pulmonary valve atresia with intact ventricular septum (aRR 2.7, 95% CI 1.1, 6.7), and atrial septal defect (aRR 3.1, 95% CI 2.8, 3.5), among others. In particular, infants born to those with an OUD diagnosis had a 1.8 (95% CI 1.4, 2.3)-fold increased risk of having severe congenital heart disease. Our findings suggest an association between prenatal OUD diagnosis and certain CAs in the offspring. Future research is necessary to better understand the role of socio-demographic factors on these associations.

Modified right ventricular overhaul procedure: Indications, techniques and outcome for pulmonary atresia with an intact ventricular septum

Modified right ventricular overhaul procedure: Indications, techniques and outcome for pulmonary atresia with an intact ventricular septum

Successful Management of Ventricular Tachycardia in an Adult Case of Unrepaired Pulmonary Atresia with Ventricular Septal Defect: A Case Report

Successful Management of Ventricular Tachycardia in an Adult Case of Unrepaired Pulmonary Atresia with Ventricular Septal Defect: A Case Report

Impact of Lesion Severity in Fetal Cardiac Counseling.

Approximately 1% of all live births in the USA are affected by congenital heart disease (CHD), the leading cause of congenital defect-related illness and infant death. Although technological innovations have improved CHD diagnosis in utero, variation among fetal cardiac counseling practices persists. Our study aims to evaluate physician counseling content based on cardiac defect complexity. We conducted an anonymous survey of providers who perform fetal cardiac counseling. Participants stated their counseling practices for 21 CHD lesions, choosing from postnatal surgical intervention, termination of pregnancy, and/or postnatal palliative comfort care. CHD lesions were divided by severity into three groups. Chi-square tests were performed to assess whether counseling content varied based on provider response to the statement "some life is always better than no life at all." There were 138 respondents, with postnatal surgical intervention counseling frequency consistent among all lesions. Inclusion of termination and palliative/comfort care in counseling increased in frequency as disease severity increased, with the former being counseled more frequently. For tetralogy of Fallot (p = 0.02), interrupted aortic arch (p = 0.03), hypoplastic left heart syndrome (p = 0.03), and pulmonary atresia with intact ventricular septum (p = 0.03), those providers who disagreed with the statement that "some life is always better than no life at all" were more likely to counsel termination of pregnancy and/or palliative care compared to their counterparts. Our study found that the interventions and options included in fetal cardiac counseling vary based on complexity. Moreover, physicians' personal beliefs tended to influence counseling practices in some moderate or severe defects.

Single-Stage Midline Unifocalization Is Associated With Excellent Outcomes in Infants of All Ages.

ObjectiveTetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months. Thus, we sought to evaluate surgical outcomes according to age at repair.MethodsWe performed a retrospective review of patients who underwent unifocalization from age 3 to 12 months. These patients had not undergone prior surgery at our institution or elsewhere and were also not protocoled into early surgery. Patients were divided in the following groups: 3.0 to 4.9 (n = 61), 5.0 to 5.9 (n = 56), 6.0 to 7.9 (n = 56), and 8.0 to 11.9 (n = 47) months. Competing risk regression analyses were performed.ResultsWe included 220 patients from 2001 to 2020. Baseline characteristics were not significantly different among the four groups. Overall, single-stage complete repair with bilateral unifocalization, ventricular septal defect closure, atrial septal defect closure, and right ventricular-pulmonary artery (PA) conduit placement was achieved at the index operation in 174 (79%) patients and did not differ across age groups. Early mortality was 4% (n = 9). At one year, 91% (200/220) of the entire cohort was fully septated. Comparing with group 1, group 4 was significantly less likely to undergo any PA reinterventions [hazard ratio (HR) 0.44, 95% CI 0.21-0.92, P = .028] or surgical PA reinterventions [HR 0.12, 95% CI 0.02-0.95, P = .044] following complete repair.ConclusionsGiven the excellent outcomes across all ages, surgical timing for single-stage unifocalization should be dictated by clinical and anatomic details, with potential advantage in select clinically appropriate older infants who appear to be at a lower probability of PA reinterventions following full septation.

Pulmonary Atresia with Intact Ventricular Septum, an Evolving Strategy in the Era of PDA Stenting: Single Center Experience.

Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate. We sought to review the management strategy of patients with PA/IVS at our institution to better understand what metrics can be used to guide management and initial interventions. The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). Survival was achieved in 93.1% with two patients (6.9%) expiring. The TV annulus z-score was significantly different between the three groups, with the 1V group having the smallest median TV annulus z-score at -4.04 (IQR -4.60- -3.60) and the 2V group having the largest median TV z-score at -1.4 (IQR -2.24- -0.12). Six patients underwent late right ventricular decompression. We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS.

Staged Approach: The Role of Delayed Repair Following Complete Unifocalization of Major Aortopulmonary Collateral Arteries with Ventricular Septal Defect and Pulmonary Atresia.

The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable-as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature. Furthermore, there are a small but important group of patients with poorly developed vessels (about 10%-15% of the population) and/or diminutive native pulmonary artery vasculature that require initial shunt procedures to promote rehabilitation and growth of vessels before unifocalization can be attempted. During unifocalization, patients not suitable for single stage repair can be identified by intraoperative flow studies and can be successfully managed with staged strategies that provide time for growth and reinterventions on the pulmonary vasculature. Over 85% of patients can achieve unifocalization. Deferring closure of the VSD to a subsequent procedure is safe and these cases have similar survival to primary repair. Some patients (15%-20%) may never achieve VSD closure with this strategy but can still maintain a good quality of life with a restrictive right ventricular to pulmonary artery conduit and open VSD.