Aortopulmonary Collateral Arteries Research Articles

High-definition flow showing the major aortopulmonary collateral arteries between the descending aorta and left/right pulmonary artery.

High-definition flow showing the major aortopulmonary collateral arteries between the descending aorta and left/right pulmonary artery.

Paragangliomas and syringomyelia in Tetralogy of Fallot-A case report and literature review.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, paragangliomas, and syringomyelia are uncommon diseases. Furthermore, in the absence of any genetic link and with less than five reported adult patients surviving unrepaired rare form of Tetralogy of Fallot, our case shows noteworthiness. The possibility of definitive treatment of these conditions is rendered unsafe due to this persistent defect. Thus, management and ongoing survival of this patient remains complex and challenging.

The Indispensable Role of Cardiac Catheterization Before Surgical Intervention of Pediatrics Undergoing Tetralogy of Fallot Total Correction; A Single Center Experience

Background: Inadequate pulmonary blood flow in the tetralogy of Fallot (ToF) can lead to the development of major aortopulmonary collaterals, interfering with surgical repair. Objectives: We aimed to depict the crucial role of cardiac catheterization before the surgical intervention of pediatrics undergoing ToF total correction. Methods: This retrospective cohort study was conducted on consecutive pediatric patients under two years of age who underwent ToF total correction surgery. All of the patients were diagnosed with ToF with doubled times trans-thoracic echocardiography, and the patients with high suspicion of the presence of major aortopulmonary collateral arteries (MAPCAs) underwent cardiac catheterization before ToF total correction surgery. Conversely, if the catheterization approach failed, they underwent ToF total correction surgery simultaneously with MAPCA closure. On the other hand, the patients with no evidence of MAPCA in the echocardiographic findings (as considered undiagnosed patients) underwent ToF total correction surgery. Results: Two hundred fifty eligible pediatrics who underwent ToF total correction surgery participated. The ICU stay (P < 0.001), hospital stay (P < 0.001), and weaning time from mechanical ventilation (P < 0.001) in patients with correct diagnosis before the ToF surgery were statistically lower than misdiagnosed open MAPCA patients who underwent the surgical approach of the ToF group. Some intra-operative and postoperative parameters showed no statistical differences between patients in the two groups. We had three mortality in the patients whose trans-thoracic echocardiography (TTE) findings were undiagnosed as there was no evidence to rely on the presence of open MAPCA. Conclusions: Based on this study's findings, we strongly recommend that all ToF patients undergo cardiopulmonary catheterization before the surgical approach.

A retrospective study: Long term prognosis in adults with PA-VSD-MAPCAs

BackgroundPulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment. MethodsA prospectively established database of 169 PA-VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison. ResultsThe overall mean survival for patients with PA-VSD-MAPCAs was 38.5 years (95%-CI: 33.1–43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1–49.6) versus the other groups (p < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167). ConclusionsPatients with PA-VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach.

Durability of Aortic Homografts in Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention (P < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate.

PA-VSD Without MAPCA(s): Review of Long-Term Outcomes and Reinterventions.

Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.

Giant Morgagni hernia and aorto-pulmonary collaterals in a Loeys-Dietz patient undergoing surgery for aortic root aneurysm and mitral valve prolapse.

The case of a Loeys-Dietz syndrome patient undergoing mitral valve repair and composite aortic root and valve replacement is here described: preoperative CT scan unravelled a previously misdiagnosed Morgagni hernia (anterior diaphragmatic), containing omentum only, compressing the right ventricle. Intraoperatively, an abnormal oxygenated blood backflow into the left ventricle was observed, postoperatively found to be caused by major aorto-pulmonary collateral arteries. This is the 1st case of Morgagni hernia and systemic-pulmonary shunt ever reported associated with Loeys-Dietz syndrome. These congenital features may be important in both phenotyping and surgical management.

Major aortopulmonary collateral arteries: clinical, echocardiographic, and radiologic characteristics.

Major aortopulmonary collateral arteries (MAPCAs) are rare congenital anomalies with significant clinical implications, often associated with congenital heart diseases like tetralogy of Fallot (TOF) and pulmonary atresia (PA). This study aimed to investigate the clinical, echocardiographic, and radiologic characteristics of MAPCAs in patients with congenital heart diseases admitted to our clinic between 2016 and 2023. A retrospective analysis of 46 cases was conducted using chest computed tomography exams performed on a dual-source 128-slice CT scanner. Clinical data and radiologic characteristics were collected and analysed. The study revealed a strong correlation between congenital heart diseases and the presence of MAPCAs, with TOF, PA, and ventricular septal defect (VSD) being the most common, and it indicated that these collaterals may exist with non-diagnosed congenital heart disease. Tricuspid regurgitation and aortic insufficiency were the predominant echocardiographic findings. Radiologically, MAPCAs primarily originated from the descending aorta (type II) in 85% of cases, and their sizes ranged from ≤ 3 mm to > 10 mm, with an average of 5 mm. This study provides comprehensive insights into the clinical and radiologic aspects of MAPCAs in patients with congenital heart diseases. The findings emphasise the importance of early detection and intervention for better managing of these complex conditions and the need for further research.

Association of right aortic arch and agenesis of ductus arteriosus in prenatal tetralogy of Fallot spectrum and its clinical implications.

In our center, we observed an increased frequency of right aortic arch (RAA) with an agenesis of the ductus arteriosus (ADA) in prenatally diagnosed tetralogy of Fallot (ToF) and its variations. This study aimed to determine whether there is an association of RAA and ADA in fetuses with ToF. Distribution of genetic anomalies and impact on postnatal outcome were further evaluated. Single-center retrospective observational study including pregnancies with prenatal diagnosis of ToF from 2010 to 2023. All cases were subdivided into ToF with pulmonary stenosis (PS) and pulmonary atresia (PA). Clinical and echocardiographic databases were reviewed for pregnancy outcome, genetic anomalies, and postnatal course. The cohort included 169 cases, 124 (73.4%) with ToF/PS and 45(26.6%) with ToF/PA. Agenesis of the ductus arteriosus was significantly associated with RAA in both subtypes of ToF (p=0.001) compared to left aortic arch and found in 82.5% (33/40) versus 10.7% (9/84) of fetuses with ToF/PS and in 57.1% (8/14) versus 12.9% (4/31) of fetuses with ToF/PA. In both ToF/PS and ToF/PA, RAA/ADA versus RAA/patent DA revealed a significantly higher risk for the presence of genetic abnormalities, especially microdeletion 22q11.2, major aorto-pulmonary collateral arteries and a shorter time to complete surgical repair. We demonstrated a significantly increased frequency of RAA/ADA in patients with prenatally diagnosed ToF. Although this association revealed no significant impact on overall survival, the prenatal detection of RAA/ADA has implications for counseling, genetic evaluation and postnatal management.

Scimitar Syndrome in a Pediatric Cohort.

Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome. We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography. Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up. All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.

Pulmonary flow-study can predict in-hospital prognosis of unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries

ObjectivesWith the development of perioperative treatment, the results of the unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries have been significantly improved. However, the in-hospital recovery is quite different individually. Therefore, it is essential to find prognostic indicators to avoid unsatisfactory recovery. DesignThis was a case-control study. SettingThe study was conducted in the national center for cardiovascular diseases in China. ParticipantsPediatric patients between 2014 and 2022. InterventionsNone. Measurements & main resultsA total of 19 patients were included. The possible prognostic indicators included were commonly used clinical data. Unsatisfactory postoperative recovery was defined as mechanical ventilation≥ 7 days and/or in-hospital death. Satisfactory postoperative recovery was defined as mechanical ventilation<7 days and survival at discharge. We separated patients into two groups and compared the peri-operative data through univariable analysis. There were 8 patients in unsatisfactory recovery group (including 1 death) and 11 patients in satisfactory recovery group. Among all the possible prognostic indicators, through univariable analysis, pulmonary arterial pressure in pulmonary flow study was statistically different (p = 0.027 < 0.05). The ROC curve showed that the area under curve and cut-off values in predicting satisfactory recovery were 0.841 and 22 mmHg; the corresponding sensitivity and specificity were 100% and 72.7%. There was no statistical difference between the two groups in ventricular septal fenestration and pulmonary hypertension targeting drugs. ConclusionA pulmonary arterial pressure <22 mmHg in pulmonary flow study may avoid unsatisfactory in-hospital recovery after unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries.

Clinical Presentation and Therapy of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular septal defect, (2) an obstruction of the right ventricular outflow tract (usually infundibular and valvular pulmonary stenosis with a small pulmonary valve annulus and supravalvular stenosis, (3) an aorta that "overrides" the ventricular septal defect, and (4) right ventricular hypertrophy. TOF represents 4-8% of congenital heart defects. Specific variations of TOF include all forms of pulmonary atresia with VSD and absent pulmonary valve syndrome. In addition, the left and right main pulmonary arteries may be stenotic or hypoplastic. In these cases, there may be major aortopulmonary collateral arteries (MAPCAs) which are vessels arising from the aorta or the subclavian arteries that supply segments of the pulmonary arterial tree. Additional variations include an ASD (Pentalogy of Fallot), a right aortic arch, and coronary abnormalities.

Clinical Presentation and Therapy of Truncus Arteriosus.

Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the heart and an additional ventricular septal defect and (Fig. 50.1). This great artery is positioned above the ventricular septal defect and gives rise to the coronary arteries, the pulmonary arteries, and the aortic arch. Historically, TA has been classified by Collet and Edwards in three types, where in type I there was a common pulmonary artery truncus, in type II the left and right PA arise separately but close to each other, in type III both PA arise independently; in addition, there was a type IV that was later characterized as pulmonary atresia with VSD and major aortopulmonary collateral arteries arising from the descending aorta.

A Noninvasive Circulating Signature of Combined Right Ventricular Pressure and Volume Overload in Tetralogy of Fallot/Pulmonary Atresia/Major Aortopulmonary Collateral Arteries.

Background: Despite surgical advances, children with tetralogy of Fallot/pulmonary atresia/major aortopulmonary collaterals (TOF/PA/MAPCAs) are subject to chronic right ventricular (RV) pressure and volume overload. Current diagnostic tools do not identify adverse myocardial remodeling and cannot predict progression to RV failure. We sought to identify a noninvasive, circulating signature of the systemic response to right heart stress to follow disease progression. Methods: Longitudinal data were collected from patients with TOF/PA/MAPCAs (N = 5) at the time of (1) early RV pressure overload and (2) late RV pressure and volume overload. Plasma protein and microRNA expression were evaluated using high-throughput data-independent mass spectroscopy and Agilent miR Microarray, respectively. Results: At the time of early RV pressure overload, median patient age was 0.34 years (0.02-9.37), with systemic RV pressures, moderate-severe hypertrophy, and preserved systolic function. Late RV pressure and volume overload occurred at a median age of 4.08 years (1.51-10.83), with moderate RV hypertrophy and dilation, and low normal RV function; 277 proteins were significantly dysregulated (log2FC ≥0.6/≤-0.6, FDR≤0.05), predicting downregulation in lipid transport (apolipoproteins), fibrinolytic system, and extracellular matrix structural proteins (talin 1, profilin 1); and upregulation in the respiratory burst. Increasing RV size and decreasing RV function correlated with decreasing structural protein expression. Similarly, miR expression predicted downregulation of extracellular matrix-receptor interactions and upregulation in collagen synthesis. Conclusion: To our knowledge, we show for the first time a noninvasive protein and miR signature reflecting the systemic response to adverse RV myocardial remodeling in TOF/PA/MAPCAs which could be used to follow disease progression.

Bridge to Surgery with Pulmonary Valve Perforation and Right Ventricular Outflow Tract Stenting in an Infant with PA-VSD

Pulmonary atresia (PA) with ventricular septal defect (VSD) is a rare congenital heart disease. Blood flow to pulmonary arteries is provided by patent ductus arteriosus or major aorto-pulmonary collateral arteries (MAPCAs). Palliative interventions are required to maintain the blood supply to the pulmonary arteries in order to achieve for the complete surgical repair. In this case report, we present a patient who underwent pulmonary valve perforation and right ventricular outflow tract (RVOT) stenting as an alternative to a systemic-pulmonary artery shunt (SPS). Subsequently, surgical repair was performed ten months later.

Aortic valve replacement in a 41-year-old woman with uncorrected tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries: a case report

BackgroundTetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare.Case presentationA 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE). As vegetation gradually increased despite intravenous antibiotic administration, she was scheduled for urgent aortic valve replacement under CPB. Pulmonary blood flow was primarily provided by the MAPCAs originating from the descending aorta. Intra-aortic balloon occlusion for MAPCAs was performed to ensure a bloodless surgical field. Aortic valve replacement was successful.ConclusionAn adult with uncorrected TOF developed acute IE and subsequently had successful cardiac surgery under CPB. Understanding TOF physiology with PA and MAPCAs, particularly pulmonary blood flow through MAPCAs, is crucial.

Outcomes after surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: a series of 104 cases

Objective: To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies. Methods: A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged (M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results: In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95%CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95%CI: 89.9% to 100%). Conclusions: Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Clarifying the Pulmonary Arterial Morphology and Pulmonary Blood Supply in Patients with Tetralogy of Fallot and Pulmonary Atresia on Computed Tomography Angiography

Aim. The present study sought to characterize the pulmonary arterial morphology and pulmonary blood supply in patients of tetralogy of Fallot and pulmonary atresia (TOF-PA) on CT angiography. Materials and Methods. We retrospectively reviewed our departmental database to identify patients with TOF-PA evaluated using CT angiography. The images were analysed to detect the presence of main and branch pulmonary arteries and pulmonary arterial confluence, presence of major aortopulmonary collateral arteries (MAPCAs), laterality and relation with pulmonary arterial morphology, and presence of patent arterial duct and associated intra- and extracardiac anomalies. Results. TOF-PA was identified in 177 patients (114 (64.4%) males; median age: 9 months). Pulmonary arteries were confluent in 142/177 (80.2%) patients. According to Somerville classification, type II pulmonary atresia was the most frequent pattern seen in 127/177 (71.8%). Based on McGoon’s ratio, pulmonary arteries were adequate for surgery in 123/177 (69.5%) patients. Patent arterial duct was present in 84/177 (47.5%) patients while MAPCAs were present in 124 (70.1%) patients, of which 72/124 (58.1%) patients had at least 1 essential MAPCA supplying either lung. According to Congenital Heart Surgeons’ Society classification, type B pulmonary arterial anatomy was the most prevalent, seen in 103/177 (57.6%) patients. Conclusion. TOF-PA is associated with marked morphologic variability in the pulmonary arterial arborization to supply the lungs. Cardiac CT angiography can accurately delineate the pulmonary arterial morphology, sources of pulmonary blood supply, and associated cardiovascular anomalies in patients with TOF-PA which aids in planning appropriate surgical management including decisions regarding the need for unifocalization of MAPCAs.

T-Remodeling of the Pulmonary Artery Bifurcation for Pulmonary Artery Origin Stenosis.

Various surgical techniques are utilized for reconstructing hypoplastic pulmonary arteries (PAs) in patients with conotruncal anomalies and at times, may be susceptible to restenosis and reoperation. We reviewed our experience with a simple technique of T-shaped remodeling of the PA bifurcation. Between 2005 and 2019, 31 patients underwent T-remodeling of central PAs by a single cardiac surgeon. The PA bifurcation was opened cranially, and the opening was augmented with an oval-shaped patch effectively transforming the V-shaped bifurcation into a T-shaped bifurcation. Both origins of the PAs were enlarged, even in the instance of single PA origin stenosis. Median age at time of T-remodeling was 17 months (range: 7 weeks to 14 years). The following cardiac morphologies were observed: tetralogy of Fallot (n = 12, 39%), pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (n = 8, 26%), truncus arteriosus (n = 6, 19%), pulmonary atresia with VSD (n = 3, 9.7%), and transposition of the great arteries (n = 2, 6.5%). Thirteen patients (42%) had previous central shunt, and eight patients (26%) had previous modified Blalock-Taussig shunt. There were no operative mortalities. Immediately after T-remodeling, echocardiographic estimates of right ventricle to PA gradient decreased from 42 [interquartile range 28-58] mm Hg to 20 [12-36] mm Hg (P = .03). Freedom from reoperation on the PA bifurcation for the entire cohort was 100% at one year, 88% (95% CI 68%-96%) at five years and 82% (57%-93%) at ten years. T-remodeling for PA origin stenosis is a safe procedure with excellent freedom from reoperation that is easily reproducible and applicable to patients with all cardiac morphologies.

Pulmonary atresia and ventricular septal defect: How accurate is the fetal echocardiography, and do the major aortopulmonary collateral arteries matter?

To assess the accuracy of prenatal echocardiography in defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD). The second aim is to compare the perinatal and postnatal outcomes of different pulmonary blood supply types. The cases prenatally diagnosed with PAVSD between 2017 and 2022 in a single tertiary fetal medicine center were identified on the electronic database. Fetal echocardiography reports and images were reviewed retrospectively. Postnatal outcomes were acquired from the hospital records of relevant pediatric cardiology and cardiovascular surgery clinics. Fetal echocardiography results were compared with postnatal results. Perinatal and postnatal outcomes were compared between the different pulmonary vascular supply types. Among the 24 PAVSD cases, six were diagnosed with major aortopulmonary collateral arteries (MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA) dependent pulmonary supply, and seven were diagnosed with double pulmonary supply (MAPCA + DA) on prenatal echocardiography. Seventeen cases were live-born and have undergone postnatal investigations. Fetal echocardiography was 88.2% accurate about the type of pulmonary supply. The accuracy of fetal echocardiography regarding pulmonary vascular anatomy was 82.3%. Postoperative survival was 69.2%. Mortality before surgery and postoperative survival did not differ between pulmonary supply groups. Survival was impaired by the extracardiac anomalies. The need for early interventions was significantly higher in the DA group. Pulmonary vascularization in PAVSD can be defined precisely on fetal echocardiography. The source of pulmonary blood supply does not impact postnatal short-term outcomes significantly but it impacts the management. The associated anomalies highly contribute to postnatal mortality.