Unicommissural unicuspid aortic valve (UAV) presenting as ascending aortic aneurysm with aortic dissection.

We herein present an exceptionally rare instance of a unicommissural unicuspid aortic valve (UAV) manifesting with an aneurysmal ascending aorta complicated by a type A aortic dissection .

Innovative cardiovascular casting technique features the complex malformation of berry syndrome.

BACKGROUND: Prenatal diagnosis of Berry syndrome, a rare combination of cardiac anomalies including aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), interrupted aortic arch (IAA), hypoplastic aortic arch, or coarctation of the aorta (COA), poses a significant challenge. Due to the rarity of the disease, and the limited case reports available to features the complex malformation of Berry syndrome postpartum, this article introduces an innovative approach to visually showcase this unusual disease. The proposed method provides a comprehensive display of the structural deformities, offering valuable insights for clinical practitioners seeking to comprehend this condition. CASE PRESENTATION: In this report, we present a case where fetal echocardiography aided in diagnosing Berry syndrome, which was later confirmed through postpartum cardiovascular casting. Our experience highlights the importance of using the three-vessel view to diagnose APW and aortic origin of the right pulmonary artery. Additionally, obtaining true cross-sectional and sagittal views by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch is necessary to image IAA or coarctation of the aortic arch. CONCLUSIONS: Early and accurate prenatal diagnosis of Berry syndrome is feasible and our cardiovascular cast can perfectly display the microvascular morphology of the fetal heart, which may have great application prospects for postpartum diagnosis and teaching of complex cardiac abnormalities.

Three-dimensional high-definition live tissue virtual dissection of mirror-image dextrocardia with thoracic-abdominal discordance in a fetus.

Three-dimensional virtual dissection using high-definition live tissue rendering ultrasound tool of a 23-week gestation fetus with situs solitus, mirror image dextrocardia, ventricular septal defect, aortic override, and pulmonary atresia.

Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction.

Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit. Objective: To deliver the first heart valve implant that grows. Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury. Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves. Main Outcomes and Measures: Transplanted valve growth and hemodynamic function. Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves. Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

Multimodality imaging for comprehensive non-invasive diagnosis of aorto-left ventricular tunnel in infancy.

BACKGROUND: Aorto-left ventricular tunnel (ALVT) is a paravalvular communication between aorta and left ventricle. It is one of the rare congenital heart diseases which could present with heart failure. CASE PRESENTATION: A case of ALVT was diagnosed in infancy. Preliminary assessment was possible using conventional echocardiography; however, functional assessment and accurate anatomy of ALVT were further verified via variable imaging modalities starting from speckle tracking and three-dimensional echocardiography to cardiac CT angiogram. The tunnel was successfully repaired with uneventful recovery. CONCLUSIONS: Multimodality imaging can accurately assess cardiac function and demonstrate the anatomy of ALVT noninvasively to plan for successful intervention.

Evolution of surgical repair of intraseptal anomalous left coronary artery with myocardial ischaemia.

BACKGROUND: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes. METHODS: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3). RESULTS: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data. CONCLUSIONS: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

Evolution in the management of aorta to left ventricular tunnel in a national congenital cardiology centre.

An aorto-ventricular tunnel is a rare congenital cardiac defect, where a channel connects the lumen of the ascending aorta to the left or right ventricle. Four patients presented with an aorto-left ventricular tunnel over two decades at a median age of 8 months (range 0.1-10 months). Two patients (50%) had associated cardiac anomalies including hypoplastic left heart syndrome and left ventricular noncompaction/hypertrophic cardiomyopathy with aortic/pulmonary valve dysplasia in one patient each. Although traditionally surgical treatment has addressed this problem, management has evolved to transcatheter closure with excellent outcomes in appropriately selected patients at our national centre.

Anomalous Coronary Arteries: A State-of-the-Art Approach.

Congenital coronary anomalies are not an infrequent occurrence and their clinical presentation typically occurs during early years, though may be manifested only in adulthood. In the setting of anomalous aortic origin of a coronary artery, this is particularly concerning as it inflicts sudden loss of healthy young lives. Risk stratification remains a challenge and so does the best management decision-making in these patients, particularly if asymptomatic. Standardized approach to evaluation and management, with careful data collection and collaboration among centers, will likely impact future outcomes in this patient population, thus allowing for exercise participation and healthier lives.

Aortopulmonary window with anomalous ascending aortic origin of left pulmonary artery: Hitherto unreported pattern.

Anomalous ascending aortic origin of left pulmonary artery in association with aortopulmonary window has not been reported so far in literature and is the main highlight of the present case.

Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.

Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery of an Infant.

A female presented 2 weeks after birth with an unbalanced atrioventricular canal, double outlet right ventricle, mild pulmonary stenosis, and patent ductus arteriosus that eventually caused pulmonary over circulation. After pulmonary artery banding, she experienced myocardial ischemia, suggesting interference with coronary blood flow by the band that had been placed on the main pulmonary trunk. The band was removed and revised to bilateral branch pulmonary artery banding, and cardiac function improved. An anomalous left coronary artery from the underside of the right pulmonary artery was identified. Eight weeks later, the patient underwent coronary transfer and reimplantation of the left coronary artery into the aorta followed by main pulmonary artery banding. She subsequently underwent bidirectional Glenn.

Repair of an ascending aortic aneurysm associated with an anomalous right coronary artery origin.

BACKGROUND: Anomalous right coronary artery (RCA) origin off the ascending aorta, above the level of the sinus of Valsalva, is rare and can be associated with altered hemodynamics, decreased coronary perfusion, and presents unique considerations during intraoperative management in the surgical treatment of concomitant ascending aneurysmal disease. CASE PRESENTATION: A 58-year-old female with prior symptomatic visceral aneurysmal rupture and cerebrovascular disease presented with a 5 cm ascending aortic aneurysm, elevated aortic index, and increased rupture risk factors. She was preoperatively diagnosed with an anomalous, high RCA origin off the ascending aorta 15 mm above the sinotubular junction. She underwent successful ascending aortic repair using a 28 mm Dacron hemiarch graft with reimplantation of an RCA button. CONCLUSION: Our successful reconstructive surgical repair using an RCA button is effective management for high anomalous RCA take-offs from the ascending aorta and presents the first documented case of this rare coronary anomaly in the context of ascending aortic aneurysmal disease.

A rare case of tetralogy of Fallot-pulmonary atresia with absent right pulmonary artery and anomalous origin of left pulmonary artery from ascending aorta.

We report a case of a 5-year-old child with tetralogy of Fallot-pulmonary atresia with anomalous origin of left pulmonary artery from ascending aorta and right lung supplied by collateral arteries. This case highlights the role of preoperative CT angiography in differentiating this entity from a common arterial trunk with discontinuous pulmonary arteries.

Hemodynamics and Wall Shear Stress of Blood Vessels in Aortic Coarctation with Computational Fluid Dynamics Simulation.

The purpose of this study was to identify the characteristics of blood flow in aortic coarctation based on stenotic shape structure, stenosis rate, and the distribution of the wall load delivered into the blood vessels and to predict the impact on aneurysm formation and rupture of blood vessels by using a computational fluid dynamics modeling method. It was applied on the blood flow in abdominal aortic blood vessels in which stenosis occurred by using the commercial finite element software ADINA on fluid-solid interactions. The results of modeling, with an increasing stenosis rate and Reynolds number, showed the pressure drop was increased and the velocity was greatly changed. When the stenosis rate was the same, the pressure drop and the velocity change were larger in the stenosis with a symmetric structure than in the stenosis with an asymmetric one. Maximal changes in wall shear stress were observed in the area before stenosis and minimal changes were shown in stenosis areas. The minimal shear stress occurred at different locations depending on the stenosis shape models. With an increasing stenosis rate and Reynolds number, the maximal wall shear stress was increased and the minimal wall shear stress was decreased. Through such studies, it is thought that the characteristics of blood flow in the abdominal aorta where a stenosis is formed will be helpful in understanding the mechanism of growth of atherosclerosis and the occurrence and rupture of the abdominal aortic flow.

Can ectopic right coronary from the left sinus have a different course than intramural? A case of ectopic right with retroaortic course.

Retrocardiac course of an ectopic right coronary artery is newly described. The alternative (usual) course of an anomalous right coronary from the left sinus of Valsalva is pre-aortic and intramural, with stenosis. In the present exceptional case, there was no stenosis.

Cases of Ultrasound-Diagnosed Right Aortic Arch with Right Arterial Duct and the Treatment.

This paper aims to discuss the value of ultrasound to diagnose right aortic arch with right arterial duct. A retrospective analysis of fetal echocardiography characteristics of 10 fetuses who were diagnosed as right aortic arch with right arterial duct from December 2016 to March 2021 is made, and focus is put on the relationship between the aortic arch and arterial duct, and the position of aortic arch, arterial duct arch and trachea on the three vessels and trachea view (3VT). As a result, all 10 cases with right aortic arch and right arterial duct do not show aberrant left subclavian artery, and aortic arch with arterial duct are still connected as "V-shaped", and do not get vascular rings. In conclusion, 3VT can simply and clearly detect the right aortic arch, and the key to diagnosing the right aortic arch and right arterial duct is thorough inspection of the aortic arch, arterial duct, and trachea in their respective positions.

Anomalous Origin of the Right Pulmonary Artery From the Aorta and Congenital Tracheal Stenosis: Staged Repair in a Neonate.

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA), sometimes referred to as hemitruncus, is a rare malformation. We report a unique case of AORPA associated with Ebstein's anomaly and with congenital tracheal stenosis due to complete tracheal rings. The AORPA and tracheal stenosis were both successfully corrected in the neonatal period.

Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention.

Is Transposition of the Great Arteries Associated With Shortening of the Intrapericardial Portions of the Great Arterial Trunks? An Echocardiographic Analysis on Newborn Infants With Simple Transposition of the Great Arteries to Explore an Animal Model-Based Hypothesis on Human Beings.

Background The pathogenesis of transposition of the great arteries (TGA) as a congenital heart defect of the outflow tract with discordant ventriculoarterial connections remains an enigma. TGA usually have parallel great arteries suggesting that deficient torsion of the embryonic arterial heart pole might cause discordant ventriculoarterial connections. It has been speculated that deficient elongation of the embryonic outflow tract might prevent its normal torsion resulting in TGA. The aim of our study was to clarify whether the intrapericardial portions of the great arteries in human patients with TGA might be indeed shorter than in normal hearts. Methods and Results Thirty-four newborns with simple TGA and 35 newborns with normal hearts were analyzed by using images of the outflow tract in their echocardiograms and the following defined lengths of the great arteries were measured: aortic length 1, (AoL-1) and aortic length 2 (AoL-2) = distance between left and right aortic valve level and origin of the brachiocephalic artery, respectively. Pulmonary trunk length 1 (PTL-1) and pulmonary trunk length 2 (PTL 2) = distance between left and right pulmonary valve level and origin of left and right pulmonary artery, respectively. All measurements of the AoL were significantly shorter in TGA compared to normal hearts (AoL-1: 1.6±0.2 versus 2.05±0.1; P<0.0001; AoL-2: 1.55±0.2 versus 2.13±0.1; P<0.0001). With regard to the pulmonary trunk (PT), PTL-1 and PTL-2 were found to be shorter and longer, respectively, in TGA compared with normal hearts, reflecting the differences in the spatial arrangement of the PT between the 2 groups as in TGA the PT is showing a mirror image of the normal anatomy. However, the overall length of the PT between the 2 groups did not differ. Conclusions Our data demonstrate that, compared with normal newborns, the ascending aorta is significantly shorter in newborns with TGA whereas the overall length of the PT does not differ between the 2 groups. This finding is in accord with the animal model-based hypothesis that TGA may result from a growth deficit at the arterial pole of the embryonic heart.