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Purpose: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) represent uncommon congenital malformations of the tricuspid valve. The purpose of this study is to report on current perinatal outcomes of EA/TVD in our center and to investigate clinical and fetal echocardiographic predictors of perinatal mortality. Methods and Results: We performed a retrospective study among fetuses diagnosed from January 2014 to December 2023. Clinical and echocardiographic data were obtained from hospital records of Research and Education Hospital. The primary outcome was perinatal mortality. Of 21 fetuses diagnosed, there were 1 lost to follow-up, 1 termination, and 7 demises. In the live-born cohort of 12 live-born patients, 2 died before discharge, yielding an overall perinatal mortality of 50%. The median gestational age at diagnosis was 23 for non-survivors and 24 weeks for survivors. Birth weight was lower in non-survivors (2430 g vs 2990 g). Tricuspid insufficiency severity varied insignificantly. Non-survivors exhibited higher rates of hydrops, functional atresia, and absent antegrade flow (p < 0.05). Two infants with severe tricuspid insufficiency and congenital abnormalities died postnatally. The limited dataset enables further analysis for a predictive model. Notably, all non-survivors displayed hydrops, functional atresia, and absent antegrade flow, hindering definitive determination of the most impactful parameter on survival estimation. Conclusion: Perinatal mortality remains notably elevated in fetuses with EA/TVD. The individuals at the highest risk are those with antegrade flow loss and functional atresia of pulmonary valve, this high-risk subgroup could benefit from targeted interventions, such as novel prenatal therapies or a more comprehensive perinatal approach involving optimized timing of delivery and postnatal interventional strategies. [ABSTRACT FROM AUTHOR]

Introduction: We aimed to investigate the incidence, prenatal factors and outcomes of twin‐to‐twin transfusion (TTTS) with right ventricular outflow tract obstruction (RVOTO). Material and methods: A systematic search was conducted to identify relevant studies published until February 2023 in English using the databases PubMed, Scopus and Web of Science. Studies reporting on pregnancies with TTTS and RVOTO were included. The random‐effect model pooled the mean differences or odds ratios (OR) and the corresponding 95% confidence intervals. Heterogeneity was assessed using the I2 value. Results: A total of 17 studies encompassing 4332 TTTS pregnancies, of which 225 cases had RVOTO, were included. Incidence of RVOTO at time of TTTS diagnosis was 6%. In all, 134/197 (68%) had functional pulmonary stenosis and 62/197 (32%) had functional pulmonary atresia. Of these, 27% resolved following laser and 55% persisted after birth. Of those persisting, 27% required cardiac valve procedures. Prenatal associations were TTTS stage III (53% vs 39% in no‐RVOTO), stage IV TTTS (28% in RVOTO vs 12% in no‐RVOTO) and ductus venosus reversed a‐wave (60% in RVOTO vs 19% in no‐RVOTO). Gestational age at laser and gestational age at delivery were comparable between groups. Survival outcomes were also comparable between groups, including fetal demise of 26%, neonatal death of 12% and 6‐month survival of 82% in RVOTO group. Findings were similar when subgroup analysis was done for studies including head‐to‐head analysis. Conclusions: RVOT occurs in about 6% of the recipient twins with TTTS, especially in stages III and IV and those with reversed ductus venosus a‐wave. The findings from this systematic review support the need for a thorough cardiac assessment of pregnancies complicated by TTTS, both before and after laser, to maximize perinatal outcome, and the importance of early diagnosis of TTTS and timely management. [ABSTRACT FROM AUTHOR]

Patients born with obstructed total anomalous pulmonary venous return have a high risk of morbidity and mortality in the neonatal period, which only increases when combined with single ventricle physiology and non-cardiac congenital anomalies such as heterotaxy syndrome. Despite advances in management of congenital heart disease, surgery within the first weeks of life to repair the pulmonary venous connection and establish pulmonary blood flow with a systemic-to-pulmonary shunt has historically led to disappointing outcomes. A multidisciplinary approach with pediatric interventional cardiology and cardiac surgery is required to reduce morbidity and mortality in this extremely high-risk patient population. Extending the time between birth and cardiac surgery can lessen postoperative complications and mortality risk, especially in patients with abnormal thoracoabdominal relationships. Our team was able to successfully utilize transcatheter stent placement in a vertical vein and patent ductus arteriosus to delay and stage cardiac surgeries in an infant born with obstructed total anomalous pulmonary venous return, unbalanced atrioventricular septal defect with pulmonary atresia and heterotaxy, thus reducing the morbidity and mortality associated with this diagnosis. [ABSTRACT FROM AUTHOR]

Congenital tracheo-esophageal fistula/esophageal atresia (TEF/EA) with concomitant pulmonary agenesis is exceedingly rare and has a high mortality rate. While there are several reported cases of successful repair, all but one patient had right-sided pulmonary agenesis. In the case of left-sided pulmonary agenesis, the patient had incomplete agenesis and underwent repair through a left thoracotomy. We present the first successful repair of TEF/EA with complete left-sided pulmonary agenesis. This patient also underwent elective pre-operative veno-venous extracorporeal membrane oxygenation (ECMO) and subsequent repair of the TEF/EA. We discuss the management, anesthesia risks, and role of periprocedural ECMO in pediatric patients who are high anesthetic risk. [ABSTRACT FROM AUTHOR]

To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared. Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7 %, 18.3 %, 26.1 % and 13.9 %. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2 m/s predicted PV pressure ≥40 mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11 % vs. PA-IVS 36 %) and lower rate of developing bi-ventricles (mild 100 % vs. moderate 95 % vs. critical 89 % vs. PA-IVS 36 %). Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome. [ABSTRACT FROM AUTHOR]

الملخص: أهداف البحث: تعتبر متلازمة تريزومي 18 أو متلازمة إدواردز اضطرابا كروموسوميا يتميز بشذوذات تنموية شديدة وعجز إدراكي. تعتبر المضاعفات القلبية من الأسباب الرئيسية للوفيات في هؤلاء المرضى، والدور الذي تلعبه التدخلات القلبية لا يزال محل جدل. عرض الحالة: نقدم حالة لطفلة حديثة الولادة كاملة المدة تعاني من تريزومي 18، ولدت عن طريق القيصرية الاختيارية. ظهر لدى الوليدة انسداد شرياني رئوي ومجموعة من التشوهات القلبية الأخرى، مما استدعى التدخل الفوري. على الرغم من التحديات الأولية، بما في ذلك نوبة وجيزة من انخفاض التشبع بالأكسجين بعد التدخل، استجابت المريضة بشكل إيجابي لتوسيع الصمام الرئوي بالبالون وإدخال قسطرة طارئة في قناة الشريان الشرياني، مما يوضح الفوائد المحتملة للتدخلات القلبية في مرضى تريزومي 18. المناقشة: تسلط هذه الحالة الضوء على التطبيق الناجح للتدخلات القلبية في مريض تريزومي 18، مما يتحدى فكرة الرفض العام لمثل هذه العلاجات لهذه الفئة. تشير نتائجنا إلى أن التدخلات الجزئية المختارة يمكن أن تحسن جودة الحياة وتثبت الحالة، مما يدعم الحاجة إلى المزيد من البحوث لوضع إرشادات واضحة للعلاج في هذه الديموغرافية. الاستنتاجات: تضيف هذه الحالة إلى الأدلة المتزايدة التي تدعم جدوى وفوائد التدخلات القلبية المحتملة في مرضى تريزومي 18، مما يدعو إلى نهج أكثر تخصيصا لمعالجة هؤلاء المرضى. Abstract: Background: Trisomy 18 (also known as Edwards syndrome) is a chromosomal disorder characterized by severe developmental anomalies and cognitive deficits. Cardiac complications are a leading cause of mortality in these patients, and the role of cardiac interventions remains controversial. Case Presentation: We report a case of a full-term baby girl with trisomy 18, born via elective cesarean section. The neonate presented with pulmonary atresia and a series of other cardiac abnormalities, necessitating immediate intervention. Despite the initial challenges, including a brief episode of desaturation post-intervention, the patient responded positively to a balloon pulmonary valvuloplasty and emergency patent ductus arteriosus stent insertion, illustrating the potential benefits of cardiac interventions in patients with trisomy 18. Discussion: This case highlights the successful application of cardiac interventions in a patient with trisomy 18, challenging the notion of universally denying such treatments to this population. Our findings suggest that selective interventions can improve quality of life and stabilize the condition, supporting the need for further research to establish clear guidelines for treatment in this demographic. Conclusion: This case adds to the growing evidence supporting the feasibility and potential benefits of cardiac interventions in patients with trisomy 18, advocating for a more individualized approach to treatment.

Choanal atresia is an uncommon condition with an incidence of 1:5,000–8,000 live births, affecting females more frequently and often associated with other malformations. This case report presents a 42-year-old female patient who was born with bilateral choanal atresia and intervened surgically for the first time at birth. However, the formed orifice was reobstructed a few months afterward, necessitating reoperation in adulthood. The purpose of this case report is to describe bilateral choanal atresia in detail, including its clinical presentation, epidemiology, diagnosis, pathogenesis, and therapeutic approach. It aims to enhance understanding of this rare but significant condition. [ABSTRACT FROM AUTHOR]

This document contains summaries of various studies on pediatric abdominal imaging. The studies cover a range of topics including hepatic steatosis in obese children, acute pancreatitis, Crohn's disease, and febrile urinary tract infections. The studies utilize techniques such as Acoustic Radiation Force Impulse and diffusion-weighted imaging. These studies contribute to the understanding and diagnosis of abdominal conditions in pediatric patients. The document also includes abstracts on specific studies and case reports, as well as practical guides and posters on pediatric radiology. Overall, these summaries provide a comprehensive overview of the field and can be a valuable resource for researchers and clinicians in pediatric radiology. [Extracted from the article]

Key Clinical Message: Inferior vena cava atresia is a rare condition with highly variable anatomy due to the complexity of caval embryology. When endovascular venovenous reconstruction is considered for severe persistent sequelae, multimodality imaging with CT and invasive venography is used to determine the appropriateness of intervention and for procedural planning. [ABSTRACT FROM AUTHOR]

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Congenital diverticulum is an uncommonly detected cardiac lesion, especially in infancy. However, its association with cyanotic congenital heart disease is extremely rare. In the current work, we report a case diagnosed in the neonatal period with tetralogy of Fallot and pulmonary valve atresia associated with a large congenital diverticulum originating from the right ventricle. [ABSTRACT FROM AUTHOR]

Patients with a single ventricle circulation continue to be a challenge for a heart transplant. The patients in this cohort, often in poor clinical condition with frequent hepatic and renal impairment having undergone previous multiple surgical procedures, are often allosensitive and consequently have an increased risk of post-transplant complications. Although the most recently published series results are improved, this group of patients, with preserved ventricular function, continues to have a higher mortality rate. Moreover, the operation can be complicated by anatomical differences among the pulmonary arteries. This case report presents a child with tricuspid atresia and pulmonary vascular resistance contraindicating Fontan surgery, unfavourable anatomy of the central pulmonary arteries and in poor clinical condition. An EXCOR ventricular assist device (Berlin Heart) was used for sub- pulmonary mechanical circulatory support and pulmonary bifurcation reconstruction, as a bridge to a transplant. [ABSTRACT FROM AUTHOR]

Brugada syndrome is a rare arrhythmogenic syndrome associated mainly with pathogenic variants in the SCN5A gene. Right ventricle outflow tract fibrosis has been reported in some cases of patients diagnosed with Brugada syndrome. Pulmonary atresia with an intact ventricular septum is characterized by the lack of a functional pulmonary valve, due to the underdevelopment of the right ventricle outflow tract. We report, for the first time, a 4-year-old boy with pulmonary atresia with an intact ventricular septum who harbored a pathogenic de novo variant in SCN5A, and the ajmaline test unmasked a type-1 Brugada pattern. We suggest that deleterious variants in the SCN5A gene could be implicated in pulmonary atresia with an intact ventricular septum embryogenesis, leading to overlapping phenotypes. [ABSTRACT FROM AUTHOR]

Objective: Ebstein’s anomaly (EA) is a rare congenital malformation associated with high perinatal mortality. In this study, we aimed to assess perinatal outcomes and factors associated with mortality in fetuses with EA. Material and Method: In this study, fetuses diagnosed with EA from 2016 to 2022 were included. Clinical information, ultrasonographic findings, and Doppler parameters were collected retrospectively. Result: A total of 14 fetuses with EA were included. Twelve patients had isolated EA, while one had unilateral renal agenesis and the other had mega cisterna magna. Amniocentesis was performed in six cases and normal results were obtained. Nine fetuses reached term pregnancy. All fetuses survived to birth. Three cases died in the first week after birth. Survivors were found to have lower tricuspid valve annulus diameter z score when compared with non-survivors (4.81±3.05 vs 6.97±1.26, respectively, p=0.05). Reversal pulmonary artery flow (2/11 vs 4/4 in survivors and non-survivors, respectively, p=0.01) and pulmonary atresia (1/11 vs 4/4 in survivors and non-survivors, respectively, p<0.01) were significantly more frequent in non-survivors. Conclusion: Perinatal mortality remains high in EA. Higher tricuspid valve annulus diameter z score, presence of reversal pulmonary artery flow, and pulmonary atresia during prenatal diagnosis are associated with poorer diagnosis. Future multicenter studies are warranted to identify risk factors and guide perinatal management. [ABSTRACT FROM AUTHOR]

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years. The primary outcome was procedural success which was defined as event-free survival (mortality, need for re-intervention, procedural failure) at 30 days post-procedure. The secondary outcome was defined by a composite of death, major adverse cardiovascular events, or need for re-intervention at 6 months and on long-term follow-up. We included 102 infants (DS, n = 53 and SAPS, n = 49). The median age at DS and SAPS was 4 days (IQR 2.0–8.5) and 8 days (IQR 4.0–39.0), respectively. The median weight at intervention was 3.0 kg (IQR 3.0–3.0) and 3.0 kg (IQR 2.5–3.0) in the two respective arms. Tetralogy of Fallot with pulmonary atresia was the most common indication for DS and SAPS. The 30-day mortality was significantly higher in SAPS group as compared with DS group (p < 0.05). However, 30-day major adverse cardiac events (MACE) rates were similar in both groups (p = 0.29). DS was associated with shorter duration of mechanical ventilation, duration of stay in the intensive care and hospital stay than with SAPS. At 6 months, there was no significant difference in terms of mortality or event-free survival. Long-term MACE-free survival was also comparable (p = 0.13). DS is an effective and safer alternative to SAPS in infants with duct-dependent pulmonary circulation, offering reduced procedure-related mortality and morbidity than SAPS. Careful study of ductal anatomy is crucial to procedural success. However, long-term outcomes are similar in both procedures. [ABSTRACT FROM AUTHOR]

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009–2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was − 3.06 (25%, 75%: − 4.00, − 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29–0.75, p < 0.001) and OR = 3.75 (95% CI 1.46–9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival. [ABSTRACT FROM AUTHOR]

22q11.2 deletion syndrome (22q11.2DS) shows significant clinical heterogeneity. This study aimed to explore the association between clinical heterogeneity in 22q11.2DS and the parental origin of the deletion. The parental origin of the deletion was determined for 61 individuals with 22q11.2DS by genotyping DNA microsatellite markers and single-nucleotide polymorphisms (SNPs). Among the 61 individuals, 29 (47.5%) had a maternal origin of the deletion, and 32 (52.5%) a paternal origin. Comparison of the frequency of the main clinical features between individuals with deletions of maternal or paternal origin showed no statistically significant difference. However, Truncus arteriosus, pulmonary atresia, seizures, and scoliosis were only found in patients with deletions of maternal origin. Also, a slight difference in the frequency of other clinical features between groups of maternal or paternal origin was noted, including congenital heart disease, endocrinological alterations, and genitourinary abnormalities, all of them more common in patients with deletions of maternal origin. Although parental origin of the deletion does not seem to contribute to the phenotypic variability of most clinical signs observed in 22q11.2DS, these findings suggest that patients with deletions of maternal origin could have a more severe phenotype. Further studies with larger samples focusing on these specific features could corroborate these findings. [ABSTRACT FROM AUTHOR]

Key Clinical Message Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative. Abstract Anomalous pulmonary artery originating from the ascending aorta (often called hemitruncus) is a rare congenital cardiac defect requiring immediate management in the neonatal period. We report a case of a rare variant of anomalous right pulmonary artery originating from the ascending aorta in combination with pulmonary atresia and tetralogy of Fallot. The above‐mentioned combination makes the surgical management of such cardiac defect exceedingly demanding.

Objective: To assess and compare the potential of transthoracic echocardiography (TTE) and cardiac computed tomography (CT) in determining severity of pulmonary artery hypoplasia in children with pulmonary atresia with ventricular septal defect (PA-VSD) during preoperative planning.Materials and methods: The study included 38 children (mean age, 1.5 years; 97% of the patients younger than 1 year) with a clinical diagnosis of PA-VSD. All the patients underwent TTE; 36 of them underwent cardiac CT, while 33 children had direct angiocardiography. During all diagnostic methods we performed morphometry of the pulmonary trunk (if any) and proximal and distal parts of right and left pulmonary arteries, calculated z score for every vessel, Nakata index, and McGoon ratio. All the results were compared with fiof direct angiography and/or intraoperative data. The interval between TTE, cardiac CT, and angiocardiography or open surgery was less than 10 days. The criterion for hypoplasia of the pulmonary artery and its branches was z score deviation less than (−2) from reference values. Nakata index less than 200 mm2/m2 and McGoon ratio less than 1.0 were considered contraindications for radical correction.Results: We found no statistically significant difference (P > .05) when comparing the potential of TTE and cardiac CT with direct angiography/intraoperative data in terms of morphometry of the proximal parts of the right and left pulmonary arteries and calculating z scores. All the methods demonstrated high diagnostic efficacy. We found statistically significant differences when comparing the potential of TTE and cardiac CT in pulmonary trunk angiometry: the results of pulmonary trunk measurement using TTE were statistically significantly different from the findings of cardiac CT and angiography/intraoperative data with significant underestimation of vessel diameters (P < .05). The results of measurements of the distal parts of the pulmonary artery using TTE were statistically significantly different from those of cardiac CT and direct angiography/intraoperative data with significant underestimation of vessel diameters (P < .05). There were no statistically significant differences between CT and direct angiography/intraoperative data in assessing the diameters of the distal branches of the pulmonary artery. When assessing McGoon ratio and Nakata index using TTE, we found statistically significant differences (underestimation of values) in comparison with direct angiography (P < .05). Comparison of McGoon ratios measured by TTE and cardiac CT also demonstrated their statistically significant underestimation by TTE (P < .05). The calculated McGoon ratio during cardiac CT did not differ significantly from the direct angiography findings.Conclusions: TTE cannot be considered the final method for preoperative assessment of the pulmonary artery system development in children with PA-VSD due to the limited visualization of the distal parts of the right and left pulmonary arteries and significant underestimation of the pulmonary trunk diameter and indices. Cardiac CT proved its high effectiveness in assessment of hypoplasia severity and can be an alternative to direct angiography.

This article explores the pathogenesis of ventricular hypoplasia in pulmonary atresia with intact ventricular septum (PA-IVS), a type of hypoplastic right heart syndrome. The study utilizes patient-specific induced pluripotent stem cells (iPSCs) and single-cell RNA sequencing to investigate the differentiation and proliferation of cardiomyocytes in PA-IVS. The findings suggest that impaired cardiomyocyte proliferation and abnormal lineage differentiation may contribute to the development of right ventricle hypoplasia in PA-IVS. Additionally, metabolic dysfunction and elevated mitochondrial activity are observed in PA-IVS iPSC-derived cardiomyocytes. Further research using 3-dimensional iPSC-derived cardiac organoids is recommended to better understand the specific defects in hypoplastic right heart syndrome. [Extracted from the article]

Open in new tab Download slide OBJECTIVES In this study, we aimed to compare infants with univentricular hearts who underwent an initial ductus stenting to those receiving a surgical systemic-to-pulmonary shunt (SPS). METHODS All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting or a surgical SPS between 2009 and 2022 were reviewed. Outcomes were compared after ductus stenting or SPS including survival, probability of re-interventions and the probability to reach stage II palliations. RESULTS A total of 130 patients were evaluated, including 49 ductus stenting and 81 SPSs. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19 patients. There was comparable hospital mortality (2.0% stent vs 3.7% surgery, P  = 0.91) between the groups, but shorter intensive care unit stay (median 1 vs 7 days, P  < 0.01) and shorter hospital stay (median 7 vs 17 days, P  < 0.01) were observed in patients with initial ductus stenting, compared to those with SPS. However, acute procedure-related complications were more frequently observed in patients with ductus stenting, compared with those with SPS (20.4 vs 6.2%, P  = 0.01), and 10 patients needed a shunt procedure after the initial ductus stent. The cumulative incidence of reaching stage II was similar between ductus stenting and SPS (88.0 vs 90.6% at 12 months, P  = 0.735). Pulmonary artery (PA) index (median 194 vs 219 mm2/m2, P  = 0.93) at stage II was similar between patients with ductus stenting and SPS. However, the ratio of the left to the right PA index [0.69 (0.45–0.95) vs 0.86 (0.51–0.84), P  = 0.015] was higher in patients who reached stage II with surgical shunt physiology, compared with patients with ductus stent physiology. CONCLUSIONS After initial ductus stenting in infants with univentricular heart, survival is comparable and post-procedural recovery shorter, but more acute stent dysfunctions and lower development of left PA are observed, compared to acute shunt dysfunctions. The less invasive procedure and shorter hospital stay are at the expense of more stent reinterventions. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12–19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31–63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10–53 mmHg) pre-procedure to 10 mmHg (IQR 5–16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5–44 min). The large sizes—mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120–300 mL). Patients were followed for a median period of 360 days (IQR 164–525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes. [ABSTRACT FROM AUTHOR]

Advances in fetal echocardiography including newer techniques like 4D spatio‐temporal image correlation technology has improved our understanding of fetal cardiac and extracardiac abnormalities. High resolution ultrasound combined with color Doppler and 3D rendering have contributed to an improved understanding of the fetal vascular system and its anomalies. This pictorial essay including ultrasound images and videos alongside their respective clay models, provides precise information of duct anatomy in fetuses with pulmonary atresia and aortic arch abnormalities. [ABSTRACT FROM AUTHOR]

Background: Both systemic-to-pulmonary shunt and right ventricle-pulmonary artery (RV-PA) connection are extensively applied to initially rehabilitate the pulmonary artery in pulmonary atresia with the ventricle septal defect (PA/VSD). However, which of these options is the most ideal for promoting pulmonary artery development and improving outcomes remains controversial. Methods: A total of 109 PA/VSD patients undergoing initial rehabilitative surgery at Guangdong Provincial People’s Hospital from 2010 to 2020 were enrolled in this study. A series of clinical data were collected to compare the perioperative and postoperative outcomes between systemic-to-pulmonary and RV-PA connection. Results: The mean duration of follow-up was 61.1 months in the systemic-to-pulmonary shunt group and 70.3 months in the RV-PA connection group (p > 0.05). The RV-PA connection technique resulted in a significantly higher PaO2, lower red blood cells (RBC), lower hemoglobin, and lower hematocrit (Hct) (p < 0.05). The cumulative incidence curve estimated a cumulative complete repair rate of 56 ± 7% after 5 years in the RV-PA connection group, significantly higher than 36 ± 7% after 5 years in the systemic-to-pulmonary shunt group (p < 0.05). The Kaplan-Meier curve revealed a similar estimated survival rate between the two groups (p = 0.73). The RV-PA connection was identified as an independent predictor for complete repair in the multivariable analysis (HR = 2.348, 95% CI = 1.131-4.873). Conclusions: The RV-PA connection is a more ideal initial rehabilitative technique than systemic-to-pulmonary shunt in treating PA/VSD as a consequence of comparable probability of survival but improved definitive complete repair rate. [ABSTRACT FROM AUTHOR]

Background: Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment. Case presentation: We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful. Conclusion: This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression. [ABSTRACT FROM AUTHOR]

The article discusses a case of a 25-year-old primigravida with a fetus showing mirror-image dextrocardia and thoracic-abdominal discordance, detected through fetal echocardiography. Three-dimensional high-definition live tissue virtual dissection, including HD-live tissue rendering and 3D/4D spatiotemporal image correlation HD-live color rendering, was utilized to comprehensively analyze the complex cardiac morphology.

Background: Right versus left kidney donor nephrectomy remains a controversial topic in renal transplantation given the increased incidence of right kidney vascular anomalies and associated venous thrombosis. We present the case of a 3‐year‐old pediatric recipient with urethral atresia and end‐stage kidney disease who received a robotically procured living donor right pelvic kidney with two short same‐size renal veins and a short ureter. Methods: We utilized a completely deceased iliac vein system (common iliac vein with both external and internal veins) to extend the two renal veins. Due to the distance between both renal veins, the external iliac vein was anastomosed to the upper hilum renal vein, and the internal iliac vein was anastomosed to the lower hilum renal vein. The donor's short ureter was anastomosed to the recipient's ureter end‐to‐side. Results: The patient had immediate graft function and there were no post‐operative complications. Renal ultrasound was unremarkable at 48 hours post‐transplant. Serum creatinine was 0.5 mg/dL at 3 months post‐transplant. Conclusion: We demonstrate the successful transplantation of a robotically procured right pelvic donor kidney with two short renal veins using a deceased donor iliac vein system for venous reconstruction without increasing technical complications. This technique of venous reconstruction can be used in right kidneys with similar anatomical variations without affecting graft function. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making. [ABSTRACT FROM AUTHOR]

Objectives: To determine the safety and efficacy of transcatheter arterial chemoembolization with CalliSpheres® beads loaded with arsenic trioxide (CBATO-TACE) in the first-line treatment of patients with large (5 cm ≤ maximum diameter < 10 cm) or huge (maximum diameter ≥ 10 cm) hepatocellular carcinoma (HCC). Methods: Patients were randomly allocated to the CBATO-TACE group and the conventional transcatheter arterial chemoembolization (cTACE) group. The primary endpoint was progression-free survival (PFS). The secondary endpoint was overall survival (OS), treatment response, and treatment-related adverse events (TRAEs). The extrahepatic collateral arteries, liver function, and liver fibrosis after the first TACE were also evaluated. Results: From September 2018 to September 2020, a total of 207 patients who underwent TACE were consecutively enrolled in this study. The median PFS was 9.5 months (range: 8.0 – 11.0) in the CBATO group, which was significantly longer than that in the cTACE group (6.0 months, range: 4.0–6.0) (p < 0.0001). Patients in the CBATO group had a median OS of 22 months (range: 20.0 – 27.0) compared with 16 months (range: 15.0 – 20.0) in the cTACE group (p = 0.0084). The most common TRAEs were fever (p = 0.043), and nausea and vomiting (p = 0.002), which were more observed in the cTACE group. In addition, the progressive disease time, pulmonary metastasis rate (p = 0.01), the mean number of extrahepatic collateral arteries (p = 0.01), and average number of TACE sessions (p = 0.025) were significantly decreased in the CBATO group. Conclusions: CBATO-TACE achieved better therapeutic outcomes and similar safety profile compared to cTACE in large or huge HCC patients. Furthermore, CBATO-TACE was able to reduce extrahepatic collateral arteries production and extrahepatic lung metastasis. Clinical relevance statement: Our study showed that CalliSpheres® beads loaded with arsenic trioxide (CBATO-TACE) were effective and safe for the treatment of large and giant HCC. In addition, CBATO-TACE can reduce lateral hepatic branch artery formation and extrahepatic pulmonary metastasis, which provides a new treatment approach for unresectable HCC. Key Points: • We compare long-term efficacy and safety of transcatheter arterial chemoembolization with CalliSpheres® beads loaded with arsenic trioxide (CBATO-TACE) and conventional transcatheter arterial chemoembolization (cTACE) in patients with large (5 cm ≤ maximum diameter < 10 cm) or huge HCC (maximum diameter ≥ 10 cm). • Compared with cTACE, CBATO-TACE significantly improved therapeutic outcomes, overall survival, and progression-free survival in patients with large or huge HCC. The safety assessment suggested that CBATO-TACE is a safe treatment that improves the quality of life and has good treatment adherence. [ABSTRACT FROM AUTHOR]

Background: Repeat surgery is common in adult congenital heart disease, and valve-related procedures are the most frequent indication for re-intervention. In these cases, problems such as advanced adhesion, deterioration and calcification of the prosthesis used, progression of cardiac dysfunction, and worsening of the general condition are often observed. Case presentation: We herein report a 43-year-old patient with repaired pulmonary atresia and ventricular septal defect who experienced repeated right heart failure and protein-losing enteropathy after multiple bioprosthetic tricuspid and pulmonary valve replacements. The patient was successfully treated with a fourth pulmonary valve replacement and third tricuspid valve replacement using a mechanical valve. During surgery, peeling off and removing the right ventricular outflow conduit was risky due to dense adhesion to the ascending aorta with extremely severe calcification; thus, the mechanical pulmonary valve was implanted to a more proximal position of the right ventricular outflow tract after removing the leaflet only and leaving the stent of the bioprosthetic valve within the conduit. The right heart failure and protein-losing enteropathy were relieved with this surgery, and the patient has remained in remission for over 5 years. Conclusion: Although severe adhesion and porcelain-like calcification caused by multiple surgical interventions were a major issue in this case, good surgical results were obtained. This method has a major advantage over conventional pulmonary valve replacement with right ventricle outflow tract reconstruction when the right ventricular outflow tract conduit shows severe adhesion and calcification. [ABSTRACT FROM AUTHOR]

Various transcatheter interventions for the right ventricular outflow tract (RVOT) have been introduced and developed in recent decades. Transcatheter pulmonary valve perforation was first introduced in the 1990s. Radiofrequency wire perforation has been the approach of choice for membranous pulmonary atresia in newborns, with high success rates, although complication rates remain relatively common. Stenting of the RVOT is a novel palliative treatment that may improve hemodynamics in neonatal patients with reduced pulmonary blood flow and RVOT obstruction. Whether this option is superior to other surgical palliative strategies or early primary repair of tetralogy of Fallot remains unclear. Transcatheter pulmonary valve replacement has been one of the biggest innovations in the last two decades. With the success of the Melody and SAPIEN valves, this technique has evolved into the gold standard therapy for RVOT abnormalities with excellent procedural safety and efficacy. Challenges remain in managing the wide heterogeneity of postoperative lesions seen in RVOT, and various technical modifications, such as pre-stenting, valve ring modification, or development of self-expanding systems, have been made. Recent large studies have revealed outcomes comparable to those of surgery, with less morbidity. Further experience and multicenter studies and registries to compare the outcomes of various strategies are necessary, with the ultimate goal of a single-step, minimally invasive approach offering the best longer-term anatomical and physiological results. [ABSTRACT FROM AUTHOR]

Congenital choanal atresia results from the developmental failure of the nasal cavity to connect posteriorly with the nasopharynx. Although congenital choanal atresia is a well-recognized developmental disorder, it is an uncommon condition with an overall incidence of approximately 1 per 10,000 live births. The authors aim to highlight that in very preterm neonates, early diagnosis of bilateral congenital choanal atresia needs a high index of suspicion to avoid life-threatening events. It is considered as one of the neonatal emergencies, and repair is recommended in the first few weeks of life. The authors report an uncommon presentation of a very preterm neonate born at 31 weeks of gestation, diagnosed to have bilateral congenital choanal atresia with repeated failure of a trial of extubation. A transnasal endoscopic repair was performed in the eighth week of life to relieve the life-threatening nasal obstruction. [ABSTRACT FROM AUTHOR]

We report a case of anomalous origin of the right coronary artery from the pulmonary artery in a patient with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. The diagnosis was made during a proposed hybrid approach to stent the native right ventricular outflow tract, and an alternative surgical strategy was created.

Abstract Background Pulmonary atresia and tetralogy of Fallot can require palliative surgery in the neonatal period due to severe hypoxia; however, limitations of established techniques include high failure rate and need for cardiopulmonary bypass. Herein, right ventricular outflow tract reconstruction on a beating heart using a Foley balloon catheter is described. Methods A retrospective review of patients who underwent right ventricular outflow tract reconstruction on a beating heart using a Foley balloon catheter at our institution between September 2018 and March 2022 was completed. During the procedure, a Foley balloon catheter was used to occlude the blood from the right ventricular inflow tract. Results Eight patients with pulmonary atresia and intact ventricular septum underwent an off-pump right ventricular outflow tract reconstruction. One patient with pulmonary atresia and ventricular septal defect, and two patients with tetralogy of Fallot underwent an on-pump right ventricular outflow tract reconstruction on a beating heart. The procedures were successful in all patients. Patent ductus arteriosus ligation without modified Blalock-Taussig shunt placement was performed in three patients with pulmonary atresia with intact ventricular septum and two patients with tetralogy of Fallot, ductus arteriosus was left open in four patients with pulmonary atresia with intact ventricular septum. All patients remained clinically well without serious complications. Conclusions Right ventricular outflow tract reconstruction on a beating heart using a Foley balloon catheter for pulmonary atresia and tetralogy of Fallot is a feasible alternative to catheter-based interventions or traditional surgical treatment, especially in patients with muscular infundibular stenosis or hypoplastic pulmonary annulus. Further studies with more cases are needed to verify feasibility and superiority of this approach.

Abstract Background Surgery for congenital heart disease has progressed by leaps and bounds in the last few decades, but the right ventricular outflow tract continues to pose a challenge to the congenital heart surgeon. Objectives We aim to describe the outcomes of patients with CHD who had surgical placement of right ventricle to pulmonary artery conduits with a focus on the risk factors for redo-surgery. Methods We performed a retrospective single-center clinical data review of patients who had RVOT surgery using RV-to-PA conduits Results Thirty-three patients (54.5% males) were included. The mean age at first conduit placement was 3.57 ± 3.18 years, mean conduit size conduit was 14.45 ± 3.85 mm. 51.5% of patients received Contegra tubes. On a mean follow-up of 2.07 ± 2.36 years, 45.5% of patients underwent RV-to-PA conduits redo replacement after 5.67 ± 3.25 years from the first surgery, 2 patients underwent re-intervention for the second time, 7 patients had transcatheter interventions on RVOT or branch PAs. The main mode of conduit failure was stenosis. The median survival without the need for surgical reintervention was 2.5 years for the non-contegra subgroup versus 3 years for the contegra subgroup (P = 0.59). we predicted that 100% of the study group would require redo surgery for conduit replacement within the first 11 years post-initial surgery. For every year of age increase at follow-up, the hazard ratio for redo surgery increases by a factor of 1.47. For every year of age increase at the time of first operation, the hazard ratio for redo surgery decreases by a factor of 0.7. Conclusions The use of conduits to treat the RV to PA discontinuity is a cornerstone in treating congenital heart diseases. Nevertheless, conduit failure and replacement are inevitable. In our experience the higher the age at the first conduit, the longer the re-intervention-free survival period.

Pulmonary atresia with ventricular septal defect is a congenital heart malformation in which a lack of continuity between ventricles and the pulmonary artery is accompanied by ventricular septal defect. We report a 3-year-old female patient, who presented with a history of shortness of breath and bluish lips. Computed tomography pulmonary angiography showed an absent main pulmonary artery, right pulmonary artery, left pulmonary artery and showed a defect that connects the right ventricle and left ventricle, which concluded a VSD (ventricular septal defect).

Congenital high airway obstructive syndrome (CHAOS) is a rare congenital anomaly, frequently caused by laryngeal or tracheal atresia, tracheal stenosis, and obstructing laryngeal cysts. This is a congenital malformation, often fatal, with an unknown prevalence. Laryngeal atresia is the most frequent cause. We report a case of an intrauterine diagnosis of CHAOS and ascites in a 17-week fetus delivered at 38 weeks of gestation without other associated malformations. A fetoscopic procedure was performed at 22 weeks of gestation. An attempt was made to perforate the affected area to ensure pulmonary fluid circulation and the ascites' resolution. After birth, a tracheostomy was performed. The patient was mechanically ventilated until 11 months of age, when she was discharged with no cerebral or other complications of immediate postnatal anoxia or episodes of respiratory arrest. A laryngotracheoplasty was performed at 2 years old, but decannulation was not possible due to certain complications. At 5 years old, a new surgical intervention was performed, which allowed decannulation 6 months later. [ABSTRACT FROM AUTHOR]

Background: Tetralogy 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 presentation: A 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. Conclusion: An 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. [ABSTRACT FROM AUTHOR]

Choanal atresia is a rare congenital disorder characterised by anatomical closure of the posterior choana in the nasal cavity due to failed recanalization during foetal development. The main aim of our study is to focus on our experience with choanal atresia and its management. In this study, we are discussing the prospective study of 12 cases of choanal atresia that came to a tertiary hospital from July 2017 to July 2022. All 12 choanal atresia cases underwent thorough evaluation, including history, blood investigations, nasal endoscopy, and CT scanning. Intra-nasal endoscopic choanoplasty with stenting was performed on all patients, followed by a 2-year follow-up, except for one case that missed follow-up after 6 months. All 12 cases were female, with their ages varying from newborn to 4 months old. In our study, 75% were unilateral and 25% were bilateral cases from newborn to 4 months old. The right was more prevalent in unilateral choanal atresia. The mixed type was the most commonly observed. The most common symptom was difficulty breathing and a running nose. All cases achieved an adequate patent airway. No intraoperative complications were noted in any case. All cases of choanal atresia can be diagnosed by a cold spatula test, failure to pass an intra-nasal catheter, and a CT scan is confirmatory. Surgical correction with endoscopic intranasal choanoplasty and stenting has less morbidity, a high success rate, and a lower recurrence rate. [ABSTRACT FROM AUTHOR]

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. This review includes (1) Fetal diagnosis, associated anomalies, and factors affecting outcomes in fetal congenitally corrected transposition of the great arteries (ccTGA), (2) The impact of Fontan-associated liver disease on heart and heart/liver transplant, (3) 1-year outcomes of the Harmony transcatheter pulmonary valve, (4) Risk factors associated with major adverse cardiac events in patients with pulmonary atresia and intact ventricular septum undergoing intervention, and (5) Benefits of pulmonary valve replacement in tetralogy of Fallot patients. [ABSTRACT FROM AUTHOR]

This article discusses a rare genetic condition called Kleefstra syndrome, which is characterized by intellectual disability, hypotonia, severe speech delay, and distinctive facial features. Approximately half of individuals with Kleefstra syndrome also have cardiac defects. The article presents a case report of a woman who was diagnosed with Kleefstra syndrome prenatally at 16 weeks. The fetus had pulmonary atresia with intact ventricular septum, which is a rare condition. The article highlights the importance of early fetal echocardiography and genetic testing in cases of structural abnormalities detected on ultrasound. [Extracted from the article]

Introduction: An aberrant right subclavian artery with a normal-sided left aortic arch is the most common congenital anomaly of the aorta. This study aims to determine the risk of cardiac anomalies associated with aberrant right subclavian artery in relation to aortic arch anomalies among pediatric patients. Material and methods: This prospective cross-sectional study was performed between 2008 and 2011. Patients with either clinical or echocardiographic findings of congenital heart disease were referred for complementary evaluation with computed tomography angiography. Results: Finally, 203 patients were included in this study. The prevalence of an aberrant right subclavian artery was presented in 24 patients (11.8%). Moreover, the most common aortic arch anomaly was coarctation 40 (19.7%). The only double outlet right ventricle cardiac abnormality was associated with a higher risk of an aberrant right subclavian artery (relative risk, 1.192; 95% CI, 0.179 – 7.95) relative to other aortic arch anomalies. Discussion: In conclusion, this study showed that the incidence of aberrant right subclavian artery in an extensive series of Aortic Arch Anomalies had been performed (11.8%). Furthermore, pulmonary atresia, total anomalous pulmonary venous return, aortic coarctation, cervical arch, aortic interruption, tubular hypoplasia, and pulmonary sling were significantly higher in the aberrant right subclavian artery group in contrast with Other Aortic Arch Anomalies. However, just double outlet right ventricle was associated with a higher risk of aberrant right subclavian artery. [ABSTRACT FROM AUTHOR]

Background: Surgery for congenital heart disease has progressed by leaps and bounds in the last few decades, but the right ventricular outflow tract continues to pose a challenge to the congenital heart surgeon. Objectives: We aim to describe the outcomes of patients with CHD who had surgical placement of right ventricle to pulmonary artery conduits with a focus on the risk factors for redo-surgery. Methods: We performed a retrospective single-center clinical data review of patients who had RVOT surgery using RV-to-PA conduits Results: Thirty-three patients (54.5% males) were included. The mean age at first conduit placement was 3.57 ± 3.18 years, mean conduit size conduit was 14.45 ± 3.85 mm. 51.5% of patients received Contegra tubes. On a mean follow-up of 2.07 ± 2.36 years, 45.5% of patients underwent RV-to-PA conduits redo replacement after 5.67 ± 3.25 years from the first surgery, 2 patients underwent re-intervention for the second time, 7 patients had transcatheter interventions on RVOT or branch PAs. The main mode of conduit failure was stenosis. The median survival without the need for surgical reintervention was 2.5 years for the non-contegra subgroup versus 3 years for the contegra subgroup (P = 0.59). we predicted that 100% of the study group would require redo surgery for conduit replacement within the first 11 years post-initial surgery. For every year of age increase at follow-up, the hazard ratio for redo surgery increases by a factor of 1.47. For every year of age increase at the time of first operation, the hazard ratio for redo surgery decreases by a factor of 0.7. Conclusions: The use of conduits to treat the RV to PA discontinuity is a cornerstone in treating congenital heart diseases. Nevertheless, conduit failure and replacement are inevitable. In our experience the higher the age at the first conduit, the longer the re-intervention-free survival period. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]