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Background Total anomalous pulmonary venous connection (TAPVC) to left superior vena cava (LSVC) is an extremely rare congenital heart disease, and its surgical management is very challenging. Case summary We report one such case of a 5-year-old south Asian male with double outlet right ventricle and unbalanced atrioventricular canal defect, where all the pulmonary veins were found opening into LSVC, which was then opening into the left side of the common atrium. Intraoperatively, the LSVC was transected just below the left internal jugular vein and left subclavian vein junction and left-sided bidirectional Glenn shunt done using 8 mm Dacron tube graft. Pulmonary veins were left draining through the LSVC into the common atrium. Right-sided Glenn shunt was completed as usual. Currently, the patient is year and half post-surgery and is doing well; school going on par with the peer group maintaining a room air saturation of 87%. Discussion Here, we report a successful surgical correction of TAPVC to LSVC in a child with univentricular physiology, however due to the paucity of data and rarity of such cases, optimal surgical management is yet to be defined. [ABSTRACT FROM AUTHOR]

Cardiac malformations are sporadically diagnosed in domestic species; however, little literature is available for this group of developmental anomalies in goats. We performed a retrospective study to catalog congenital cardiac conditions in goats submitted to the University of California-Davis, Veterinary Medical Teaching Hospital, Anatomic Pathology Autopsy Service. From 2000 to 2021, of 1,886 goat autopsies, 29 cases of cardiac malformations were identified (1.5%). Thirteen were ≤ 2-wk-old, 8 were 1-6-mo-old, and 8 were adults 2-9-y-old. The most common malformations were ventricular septal defect (VSD; 21 of 29), atrial septal defect or persistent foramen ovale (10 of 29), and double-outlet right ventricle (3 of 29). Nine cases had > 1 malformation, typically including a VSD. Conditions that had not been reported in the goat included double-outlet right ventricle (3), tetralogy of Fallot (1), cor triatriatum sinister (1), and mitral valve dysplasia (1). Two adult cases were incidental and not suspected clinically. Cardiac malformations occur not uncommonly in goats and should be considered in a wide age range.

Patients with hypoplastic left heart syndrome (HLHS) are born with an underdeveloped left heart. They typically receive a sequence of surgeries that result in a single ventricle physiology called the Fontan circulation. While these patients usually survive into early adulthood, they are at risk for medical complications, partially due to their lower than normal cardiac output, which leads to insufficient cerebral and gut perfusion. While clinical imaging data can provide detailed insight into cardiovascular function within the imaged region, it is difficult to use these data for assessing deficiencies in the rest of the body and for deriving blood pressure dynamics. Data from patients used in this paper include three-dimensional, magnetic resonance angiograms (MRA), time-resolved phase contrast cardiac magnetic resonance images (4D-MRI) and sphygmomanometer blood pressure measurements. The 4D-MRI images provide detailed insight into velocity and flow in vessels within the imaged region, but they cannot predict flow in the rest of the body, nor do they provide values of blood pressure. To remedy these limitations, this study combines the MRA, 4D-MRI, and pressure data with 1D fluid dynamics models to predict hemodynamics in the major systemic arteries, including the cerebral and gut vasculature. A specific focus is placed on studying the impact of aortic reconstruction occurring during the first surgery that results in abnormal vessel morphology. To study these effects, we compare simulations for an HLHS patient with simulations for a matched control patient that has double outlet right ventricle (DORV) physiology with a native aorta. Our results show that the HLHS patient has hypertensive pressures in the brain as well as reduced flow to the gut. Wave intensity analysis suggests that the HLHS patient has irregular circulatory function during light upright exercise conditions and that predicted wall shear stresses are lower than normal, suggesting the HLHS patient may have hypertension.

Purpose: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. Methods: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal–fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. Results: Ninety‐nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra‐cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty‐nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). Conclusion: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Many types of congenital heart disease are amenable to surgical repair or palliation. The procedures are often challenging and require specific surgical training, with limited real-life exposure and often costly simulation options. Our objective was to create realistic and affordable 3D simulation models of the heart and vessels to improve training. METHODS We created moulded vessel models using several materials, to identify the material that best replicated human vascular tissue. This material was then used to make more vessels to train residents in cannulation procedures. Magnetic resonance imaging views of a 23-month-old patient with double-outlet right ventricle were segmented using free open-source software. Re-usable moulds produced by 3D printing served to create a silicone model of the heart, with the same material as the vessels, which was used by a heart surgeon to simulate a Rastelli procedure. RESULTS The best material was a soft elastic silicone (Shore A hardness 8). Training on the vessel models decreased the residents' procedural time and improved their grades on a performance rating scale. The surgeon evaluated the moulded heart model as realistic and was able to perform the Rastelli procedure on it. Even if the valves were poorly represented, it was found to be useful for preintervention training. CONCLUSIONS By using free segmentation software, a relatively low-cost silicone and a technique based on re-usable moulds, the cost of obtaining heart models suitable for training in congenital heart defect surgery can be substantially decreased. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67–1.06) than in the univentricular-repair group (median 1.79, IQR 1.53–2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The goal of this retrospective multicentre study was to present late surgical outcomes of the treatment of children with double outlet right ventricle (DORV) coming from emerging countries. METHODS The Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated on in 9 hospitals that specialize in paediatric cardiac surgery. Data are collected from the Mécénat Chirurgie Cardiaque comprehensive database, with a strict postoperative follow-up. The patients included only those who had biventricular repair of DORV with 2 viable ventricles. According to the classification of the Eleventh Revision of the International Classification of Diseases, DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. RESULTS From January 1996 to January 2022, a total of 81 consecutive DORV biventricular repair operations were performed. There were 6 subtypes of DORV divided into 2 groups: DORV-committed ventricular septal defect (VSD): DORV-VSD (n = 25), DORV-Fallot (n = 34), DORV-transposition of the great arteries (n = 5); and DORV-non-committed (nc) VSD: DORV-ncVSD-no pulmonary stenosis (PS) (n = 7), DORV-ncVSD-PS (n = 5) and DORV-atrioventricular septal defect (AVSD)-PS (n = 5). Four Fontan patients were excluded. Three patients were lost to follow-up (3.4%). The overall perioperative mortality was 7.4% ± 2.6%, 6/81 (95% confidence interval: 2.8%–15.4%) ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10-year survival was 86%. The early mortality of DORV-ncVSD at 5.9% ± 2.4% (1/17) was similar to that of DORV-committed VSD at 7.8% ± 2.7% (5/64) (P = 0.79). There was a trend towards an optimal outcome for the arterial switch operation and the DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-ncVSD at 42% (5/12) (P = 0.001). There were low numbers in the complex groups. The number of Fontan cases was noticeably low. The aorta located entirely on the right ventricle represents the fundamental anomaly and the surgical challenge of DORV. CONCLUSIONS Overall survival at 10 years was 86%. This study shows a trend towards satisfactory early and late outcomes in BVR of simple DORV with committed VSD, compared to complex DORV with ncVSD. [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]

This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle.

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Double-outlet right ventricle (DORV) is a complex and rare congenital heart disease (CHD), which is usually symptomatic early in childhood and thus diagnosed early. Dextrocardia is another rare CHD that can occur either as an isolated cardiac abnormality or may be associated with other complex cardiac defects. However, the co-occurrence of DORV and dextrocardia is rare, especially in adult life. We present a 35-year-old Nigerian lady with situs inversus totalis and complex CHD, which included dextrocardia with multiple congenital intracardiac shunts, DORV occurring with L-malpositioning of the great vessels, atrial septal defect, and ventricular septal defect with partial anomalous pulmonary venous drainage, persistent left superior vena cava as well as severe pulmonary stenosis.

Open in new tab Download slide OBJECTIVES This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). METHODS All children born with DORV between 2003 and 2017 were identified in the Oslo University Hospital registry. Patients' characteristics, interventions, complications and deaths were recorded. Echocardiographic data were reviewed for classification according to current standards. We investigated time-dependent surgical reintervention and mortality using Kaplan–Meier analyses and determinants of treatment complications, reintervention and death using regression analyses. RESULTS Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. Six children received palliative care. With an intention to treat, a surgical route with the primary biventricular repair was followed for 62 children, staged biventricular repair for 15 and univentricular repair for 10 children. Major complications occurred in 1.0% and 6.2% of children following catheter or surgical intervention, respectively. No significant determinants of the complications were identified. Overall survival following treatment was 91.9%, 90.8%, 89.5% and 89.5% and corresponding freedom from surgical reintervention was 88.0%, 79.0%, 74.9% and 69.4% at 1, 2, 5 and 10 years, respectively. The presence of atrioventricular septal defect predicted an increased risk of mortality (hazard ratio: 7.16) but did not increase the risk of surgical reintervention. CONCLUSIONS In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death. [ABSTRACT FROM AUTHOR]

Background Children with congenital heart disease remain at risk of being lost to follow-up. They may present at a later stage with complications related to underlying condition that usually require in-depth phenotyping for optimal management. We describe an unusual case of a double outlet right ventricle (DORV) who presented three decades after initial intervention. Case summary A 32-year-old female sought attention for worsening breathlessness. Available data suggested that she had double outlet right ventricle (DORV) and underwent pulmonary artery (PA) banding in infancy. On examination, she was frail, clubbed and cyanosed with oxygen saturation reduced to 75% at rest. Blood tests demonstrated elevated haemoglobin (208 g/L) and thrombocytopenia (70 *109/L). Echocardiography was limited but demonstrated DORV with increased gradient across both outflow tracts. Cardiac magnetic resonance (CMR) imaging clearly demonstrated DORV with doubly committed interventricular communication, subvalvular aortic valve stenosis and supravalvular pulmonary stenosis due to a previous PA band. CMR was also remarkable for demonstrating severely impaired left ventricle and difficulty achieving optimal contrast between myocardium and blood pool on late enhancement images. She underwent relief of subaortic stenosis with immediate improvement in left ventricular (LV) function and PA plasty with application of a new PA band. Conclusion Lost to follow-up congenital patients present a particular challenge due to complications of chronic unusual physiology. This requires careful assessment by a multidisciplinary team to define optimal management strategy. [ABSTRACT FROM AUTHOR]

Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The management varies according to the literature. We present our experience with this rare complication. Methods: Echocardiography database were reviewed with the term ‘‘hematoma’’ or “hypoechoic mass” for patients who underwent congenital heart surgery from January 2018 to December 2021 at our institution to identify potential interventricular septal hematoma cases. Relevant data of the patients identified were collected. Focus was put on the presentation, management, outcomes according to patent medical charts and serial echocardiographic report data. Results: In total, there were 5 patients included. The mean age and weight at surgery were 5.5 ± 3.6 months and 5.5 ± 1.4 kg, respectively. Four patients were diagnosed with ventricular septal defect and the other one being double outlet of the right ventricle. While all patients had intraoperative transesophageal echocardiography, 80% (4 of 5) of Interventricular septal hematoma were revealed intraoperatively. Only one patient received hematoma drainage intraoperatively while the other 3 identified in the operating room were only closely observed. One after ventricular septal defect repair presented continuous dysfunction of the left ventricle at the last follow-up, while the others were doing well. All hematomas resolved completely with a mean time to interventricular septal hematoma resolution of 35.8 ± 16.9 days. Conclusion: Infants seem to be at a higher risk for Interventricular septal hematoma following congenital heart surgery. While the majority of interventricular septal hematoma has a benign postoperative course, some may result in ventricular dysfunction. Management strategies may be chosen on a case-by-case basis. [ABSTRACT FROM AUTHOR]

There are several choices for the correction of complex transposition of the great arteries and double outlet right ventricle not amenable to the Rastelli-type surgery, but outcome data are limited to small series. This study aims to report results after the aortic root translocation and en bloc rotation of the outflow tract procedures. This is a retrospective, multicentric, observational study. Clinical, anatomy, procedural, and detailed follow-up data (median, 4.43 years) were collected. A total of 70 patients (62.9% male; median age, 1 year; range 4 days to 12.4 years) were included: n = 43 in the aortic root translocation group and n = 27 in the en bloc rotation group. Those in the aortic root translocation group were older (P =.01) and more likely to have had previous procedures (P <.0001), but cardiac anatomy was similar in both groups. Aortic root translocation and en bloc rotation early mortality (30 days) was similar (4.7% vs 3.7%, P =.8). Late survival and freedom from any cardiac reintervention were 92.7% and 16.9% at 15 years overall, respectively. Freedom from right ventricular outflow tract/conduit reintervention was better in the en bloc rotation group than in the aortic root translocation group (100% vs 24.5%, P =.0003), but more patients in the en bloc rotation group had moderate (or worse) aortic valve regurgitation during follow-up (16% vs 2.6%, P =.07). Both aortic root translocation and en bloc rotation are valuable surgical options for the treatment of complex transposition of the great arteries and double outlet right ventricle. In the en bloc rotation group, there was better freedom from right ventricular outflow tract reinterventions, but a higher probability of aortic valve regurgitation. Identifying the main driving forces for these observed differences requires further study of these procedures. ART and EBR of the outflow tract procedures show overall good and comparable outcomes, with some differences in patient profile, freedom from reintervention, and residual valvular lesions. [Display omitted] [ABSTRACT FROM AUTHOR]

Ventricular septal defects (VSD) with outflow tract (OFT) malalignment are a common group of congenital heart diseases with varying severity. The developmental process of these defects is challenging to understand due to the complex nature of cardiac morphogenesis and the difficulties in visualizing the temporal and spatial changes that occur during pathogenesis. However, recent advancements in imaging techniques, such as high-resolution episcopic microscopy, have provided valuable insights into the normal septation of ventricular chambers and OFT alignment. Building upon this knowledge, we have utilized lightsheet microscopy, another innovative imaging method, to further investigate the developmental processes that lead to abnormal formation of the ventricular septum and the malalignment of arterial roots with the ventricular chambers. Our study highlights endocardial cushion hypoplasia and insufficient rotation of the outflow tract as two interrelated central factors contributing to the pathogenesis of these defects. This finding has the potential to enhance our understanding of the etiology of congenital heart diseases and may contribute to the development of improved diagnostic and therapeutic strategies in the future. [Display omitted] • E9.5 administration of dofetilide or deletion of Notch1 in cardiac lineages can induce OA/VSD or DORV/VSD in mouse embryos. • The reduction of endocardial cushion size at both outflow tract and atrioventricular canal is a key factor in causing the defects. • The outflow tract malalignment defects of OA and DORV reflect a spectrum of OFT rotation insufficiency. • Insufficient rightward expansion of the AVC tubercule also contributes to the final closure of the interventricular septum. [ABSTRACT FROM AUTHOR]

Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease. Three-dimensional printing is an emerging technology that is fundamentally affecting patient care, research, trainee education, and interactions among medical teams, patients, and caregivers. This paper first reviews key clinical cases where the technology has affected patient care. It then discusses 3-dimensional printing in trainee education. Thereafter, the role of this technology in communication with multidisciplinary teams, patients, and caregivers is described. Finally, the paper reviews translational technologies on the horizon that promise to take this nascent field even further.

Double outlet right ventricle (DORV) is a congenital cardiac malformation that occurs in 1%-3% of individuals with congenital heart defects. Cardiac magnetic resonance imaging (MRI) may play an important role in the anatomy of the ventricular septal defect, functional status of both ventricles, and in identifying any residual stenosis or regurgitation or coexistent anomalies. Here, we present a case of a 28-years-old woman who came to our emergency department with shortness of breath. The patient felt shortness of breath on exertion and improved with rest. Clinical examination showed no abnormalities except low oxygen saturation of 65%. After echocardiography and cardiac MRI were done, it was concluded that she had a double outlet right ventricle with peri membranous ventricular septal defect (VSD), pulmonary hypertension, and pericardial effusion. This study highlights the role of cardiac MRI in assessing DORV.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period. Herein, we report a 20‐week‐old fetus with a right aortic arch and an aberrant left subclavian artery with this rare combination. We report a case of a 20‐week fetus diagnosed having this rare combination of right aortic arch and aberrant left subclavian artery. The APVS with complete atrioventricular septal defects may represent another type of APVS. [ABSTRACT FROM AUTHOR]

Double outlet right ventricle (DORV) is a highly complex congenital heart disease (CHD) entity, gaining increasing interest due to the rapid progress of cardiac surgery. The number of patients operated for this congenital defect has been growing since 1980s and over following decades with active transitioning of this cohort into the adult medicine. However, the diversity of initial anomaly and performed interventions makes challenging the management of these patients. This is particularly important in the regions where specialized adult CHD cardiology still remains underdeveloped. In this review, we observe the basic principles of DORV nomenclature, main types of the operations and possible late complications. The article focuses on adult patients and offers illustrations from clinical practice. [ABSTRACT FROM AUTHOR]

This article presents an anatomical variation of the very high take-off right coronary artery from the ascending aorta. Clinical care is complicated by the patient's double outlet right ventricle and right aortic arch associated with this situation.

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

Background: The most prevalent cyanotic congenital cardiac condition is the tetralogy of Fallot (TOF). A congenital heart defect is a flaw in the structure of the heart that is present at birth. The normal flow of blood through the heart is disrupted by this type of cardiac defect. It accounts for about 6% to 10% of all congenital heart defects. The congenital heart condition double outlet right ventricle (DORV) occurs when two blood arteries do not attach to the heart. It can affect 1 to 3% of people with congenital cardiac disease. Case Presentation: A newborn male child was brought by her parents to the paediatric ward with a chief complaint of shortness of breath, cyanosis, hypoglycaemia, and convulsion. In the case of Tetralogy of Fallot with Double outlet right ventricle. The primary therapeutic interventions were given to the newborn patient and he was treated with antibiotics and vasodilators. Conclusion: The newborn male old child with complaints of shortness of breath, cyanosis, hypoglycaemia, and convulsion and diagnosed with tetralogy of Fallot with double outlet right ventricles. After the proper therapeutic interventions and treatment, the client's condition is improved. He has been able to maintain oxygen saturation up to 88-94% and reduced hypoglycaemia and convulsion, cyanosis. [ABSTRACT FROM AUTHOR]

Aim: The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial appendages as detected on multidetector computed tomography (CT) angiography Materials and Methods: The CT angiography studies performed at a tertiary medical center from January 2014 to December 2021 were retrospectively evaluated to identify patients with left atrioventricular valve atresia and patent aortic root with isometric atrial chambers. The morphology of the atrial appendage and extent of pectinate muscles was used to identify the type of isomeric atrial chambers. A comprehensive assessment of discordant arrangement of organs and the variations in venoatrial connections was done. Results: Left atrioventricular valve atresia in the presence of patent aortic root and isomeric atrial appendages was identified in eight patients (four males). The mean age at diagnosis was 7 years. Right isomerism of the atrial appendages was seen in five patients while three patients had left isomerism. Disharmonious patterns of abdominal visceral arrangement were seen in two patients. Atrial and ventricular septal defects were seen in all patients. The most common pattern of ventricular arterial connection was DORV (double outlet right ventricle) (n = 4). While five patients had normally related great arteries, three had aorta anterior to the pulmonary trunk. Right ventricular outflow obstruction was seen in seven patients. Right aortic arch with mirror image branching was seen in four patients. Coronary artery anomalies were seen in four patients. Conclusions: The presence of left atrioventricular valve atresia and patent aortic root with isomeric atrial appendages is a rare congenital anomaly frequently associated with anomalous systemic and pulmonary drainage, conotruncal anomalies, and right ventricular outflow obstruction. Hence, comprehensive CT‐based evaluation is indispensable in providing detailed anatomy and aids in presurgical planning. [ABSTRACT FROM AUTHOR]

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

To address various problems with conventional surgical procedures for transposition of the great arteries with left ventricular outflow obstruction, the aortic translocation procedure has been reconsidered as an alternative surgical procedure. We developed another innovative translocation technique, the half-turned truncal switch operation, to make use of various anatomical characteristics. The truncal block involving both semilunar valves is harvested en bloc and anastomosed to the opposite ventricular outflow tract after a half-turn. Temporarily detached coronary arteries are re-anastomosed to the reversed aortic wall defects. The key advantage of this half-turned truncal switch operation is the creation of hemodynamically faultless right and left ventricular outflow tracts including competent valve functions and a coronary circulation ensured by the posteriorly translocated aortic valve, the maximally used autologous pulmonary valve, and anastomosis of the coronary arteries to the confronting aortic wall defects. [ABSTRACT FROM AUTHOR]

Abstract Background Congenital heart diseases are occasionally encountered in the bovine species. Ventricular septal defects (VSD) and atrial septal defects (ASD) are reported to be the most common; however, a vast collection have been reported [1, 2]. Congenital heart diseases is thought to represent less than 3% of all congenital abnormalities in calves [3]. Various cardiac anomalies arise due to defective embryologic development such as defects of the septae or the cardiac chambers [2]. The exact aetiology of these congenial heart anomalies remains to be fully elucidated [4]. VSDs appear to be the most common congenital cardiac anomaly in calves. Other diseases can be subdivided into cyanotic (e.g. ASD or patent ductus arteriosus) and non-cyanotic (e.g. tetralogy of fallot or eisenmengers complex) [5, 6]. An exceptional presentation of an array of congenital anomalies was identified in a Friesian heifer calf. To the authors’ knowledge this concurrent collection of congenital abnormalities has never been reported in this species. Case presentation A 3-day old Friesian heifer presented with a history since birth of regurgitation post feeding. The main finding on clinical examination was tachypnoea with a holosystolic murmur. Echocardiography identified a VSD, patent foramen ovale (PFO) (both with left to right blood flow) and tricuspid insufficiency. The calf was subsequently euthanised and underwent gross post-mortem examination. A persistent right aortic arch (PRAA) was identified. The cardiac anomalies identified on the echocardiogram were confirmed along with additional abnormalities; double outlet right ventricle (DORV), partial transposition of the great vessels, pulmonic stenosis, hypoplasia of the right branch of the pulmonary artery and right ventricular hypertrophy. The final diagnosis was Tetralogy of Fallot with DORV, PFO and PRAA. The lungs appeared oedematous and congested due to cardiac malfunction and cranioventral aspiration pneumonia. Free serous fluid was identified in the thoracic cavity. Unilateral renal agenesis of the left kidney was an incidental finding but is of note due to its coexistence with the cardiac abnormalities. Conclusions This is an unusual case as it features numerous congenital abnormalities that appeared to negate each other allowing capability with life. To the authors’ knowledge, this collection of concurrent cardiac anomalies has not been previously reported in bovines.

A patient with repaired double outlet right ventricle presented during early gestation with heart failure symptoms due to severe right ventricle–pulmonary artery conduit stenosis and insufficiency. In the first trimester, she underwent transcatheter therapy with Melody pulmonary valve implantation with excellent hemodynamic results and completed pregnancy without significant maternal complications. (Level of Difficulty: Advanced.)

Double outlet ventricle is a type of conoventricular anomaly. The great arteries arise predominantly (more than 50%) from one of the ventricle. The aortomitral discontinuity varies from minimum discontinuity to complete bilateral conal tissue. Both right and left ventricles can have double outlet though double outlet right ventricle (DORV) is the most common anomaly in this group of abnormalities. Double outlet left ventricle (DOLV) is relatively rare. There are multiple types of DORV based on the site of ventricular septal defect and great artery relationship and presence or absence of ventricular outflow obstruction. The correct anatomical diagnosis requires a detail segmental approach in echocardiography. The echocardiographic features of DORV reviewed in detail followed by a brief discussion on DOLV.

We report a case of a 1-year-old boy with double outlet right ventricle where computed tomography (CT) angiography incidentally demonstrated the separate origin of the left external and internal carotid arteries directly from the left-sided aortic arch with absence of a left common carotid artery. The case highlights the underlying embryological hypothesis as well as the importance of CT angiography in identifying anomalies of the aortic arch and arch vessels in the setting of complex congenital heart defects. [ABSTRACT FROM AUTHOR]

A 35-year old man sustained fracture of cervical vertebrae in an automobile accident and pre-operative routine echocardiography showed double outlet right ventricle (DORV) with infundibular and valvar pulmonary stenosis, mitral atresia, hypoplastic left ventricle, intact ventricular septum, dysplastic tricuspid valve, secundum atrial septal defect and L-malposition of great vessels. Functionally univentricular hemodynamics allowed survival in adulthood.