Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta.

We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair.

"Ground-cherry" appearance of the pulmonary trunk: Incidentally detected sinoatrial nodal branch-to-pulmonary trunk fistula.

We present a case of a 45-year-old man with atypical chest pain who underwent coronary computed tomography angiography which incidentally revealed a conglomerate of nondilated tortuous vessels along the inferior half of the surface of the pulmonary trunk, resulting in a "ground-cherry" like ridged appearance. Tracing these vessels proximally and distally revealed it to be arising from the sinoatrial nodal branch of the right coronary artery and distally draining into the left anterolateral aspect of the pulmonary trunk.

Partial anomalous pulmonary venous drainage to persistent left superior caval vein: Or is it a "levoatriocardinal vein"?

We report a case of a 3-month-old girl with pulmonary atresia and intact ventricular septum where computed tomography (CT) angiography demonstrated an anomalous venous channel connecting the left brachiocephalic vein to the left atrium and all the pulmonary veins draining the left lung were seen draining individually into this channel. The case highlights the differential diagnosis of such veno-venous connections between the systemic and pulmonary venous circulations as well as the growing role of CT angiography in the understanding of these venous anomalies in the setting of complex congenital heart diseases.

Superior sinus venosus defect with partial anomalous pulmonary venous return in a patient with tetralogy of Fallot.

We report a case of a 2-year-old boy with tetralogy of Fallot-pulmonary atresia where CT angiography additionally demonstrated the presence of superior sinus venosus defect with partial anomalous pulmonary venous return. Our case highlights the role of CT angiography in the preoperative evaluation in patients with tetralogy of Fallot in identifying such uncommon anomalies, which would help avoid postoperative complications and re-exploration in these patients.

Intrapulmonary course of superior caval vein associated with azygos lobe.

We report a case of a 4-month-old boy with tetralogy of Fallot where computed tomography angiography incidentally revealed the presence of a unique extra-mediastinal and intrapulmonary course of the superior caval vein within the anterior portion of the azygos fissure.

Atresia of the coronary sinus ostium with persistent left superior caval vein.

We describe a case of a 33-year-old woman with congenitally corrected transposition where computed tomography angiography incidentally detected ostial atresia of the coronary sinus with dilatation of the terminal parts of the middle cardiac vein and great cardiac vein and retrograde drainage of the coronary sinus into the persistent left superior caval vein, the intercommunicating vein, then to the right superior caval vein, and ultimately into the right atrium.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with isometric atrial appendages on multidetector CT.

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.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers on multidetector CT.

AIM: To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. MATERIALS AND METHODS: We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled. RESULTS: A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. CONCLUSIONS: Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia.

Proximal interruption of the right pulmonary artery: A rare anomaly.

We report a case of a 21-year-old woman with cyanosis where computed tomography (CT) angiography revealed a complete absence of the mediastinal part of the right pulmonary artery with the normal intrapulmonary vascular network, receiving supply from multiple collateral vessels. The case also highlights the various differential diagnoses and the role of CT angiography in arriving at the correct diagnosis.

Incidentally detected dual right coronary artery in patient with constrictive pericarditis.

We report a case of a 29-year-old man with constrictive pericarditis where CT angiography incidentally demonstrated a dual right coronary artery (RCA). The present case highlights the diagnostic criterion for dual RCA as well as the potential clinical implications of the anomaly.

A rare configuration of single coronary artery: Anomalous anterior interventricular artery arising from right coronary artery with absent left circumflex artery.

We report a case of a 49-year-old woman planned mitral valve replacement surgery where computerized tomography angiography incidentally detected a rare configuration of a single coronary artery resulting from a combination of anomalous origin of anterior interventricular artery from right coronary artery and absent left circumflex artery.

Cystic cardiac myxoma: a rare variant of cardiac myxoma in a woman with acute stroke.

BACKGROUND: Cardiac myxomas are the most common primary benign tumors of the heart usually presenting as an oval mass attached to the interatrial septum. Mild heterogenous enhancement is typically seen upon contrast administration. Myxomas are benign, however can present with embolic episodes necessitating prompt diagnosis and treatment. CASE PRESENTATION: A middle-aged woman presenting with acute ischemic stroke was detected to have a complex cystic mass in the left atrium on echocardiography. Further evaluation with cardiac magnetic resonance imaging narrowed a differential diagnosis to hemangioma, hemangioendothelioma, sarcoma, hydatid cysts, bronchogenic cysts, and cystic variant of cardiac myxoma. CTA demonstrated arterial supply from the left circumflex artery supplying the lesion. Following surgical excision, histopathology analysis suggested a cystic cardiac myxoma with secondary degeneration. CONCLUSION: Cystic masses in the heart are challenging to diagnose due to similar imaging characteristics, and misdiagnosis may lead to further major downstream complications such as stroke, hemorrhage, and hemodynamic alterations causing syncope. Therefore, surgical excision of a cystic cardiac mass is justified to establish a definitive histopathological diagnosis and prevent further downstream complications. We hereby report a rare case of cystic cardiac myxoma in the left atrium showing intense progressive enhancement in CMR and CTA in a middle-aged woman presenting with acute stroke. This case highlights an exceedingly rare variant of cystic cardiac myxoma.

Distinguishing 'dual' from 'duplicated' right coronary artery: Revisiting the nomenclature.

We report a case of a 53-year-old man with a "short RCA" seen coursing within the proximal part of the right atrioventricular (AV) groove and terminating in the mid-portion of the right AV groove and a "long RCA" seen to have a proximal course outside the right AV groove, over the free wall of the right ventricle, where it gave rise to the right ventricular and acute marginal branches before returning to the right AV groove in its distal course. The discussion highlights the need for revisiting the nomenclature of "dual RCA and drawing a distinction between "dual" and "duplicated" RCA.

Split right coronary artery with split posterior descending artery.

We report a case of an 87-year-old man where coronary CT angiography incidentally demonstrated a "split" right coronary artery (RCA) featuring a "split" posterior descending artery. This case focusses on the morphological description of this variant as well as its differentiation from a "dual" or "duplicated" RCA.

Sporadic occurrence of multiple paragangliomas causing carotid and coronary arterial encasement.

We describe a case of a 32-year-old man with a paraganglioma causing encasement of ostioproximal segments of the left internal carotid artery and left external carotid artery with concurrent presence of bilobulated mediastinal paraganglioma, with similar imaging characteristics, causing encasement of the coronary arteries.

Mesenteric ischemia in patients with COVID-19: an updated systematic review of abdominal CT findings in 75 patients.

BACKGROUND: Acute mesenteric ischemia (AMI) is a less common but devastating complication of COVID-19 disease. The aim of this systematic review was to assess the most common CT imaging features of AMI in COVID-19 and also provide an updated review of the literature on symptoms, treatment, histopathological and operative findings, and follow-up of these patients. METHODS: A systematic literature search of four databases: Pubmed, EMBASE, WHO database, and Google Scholar, was performed to identify all the articles which described abdominal CT imaging findings of AMI in COVID-19. RESULTS: A total of 47 studies comprising 75 patients were included in the final review. Small bowel ischemia (46.67%) was the most prevalent abdominal CT finding, followed by ischemic colitis (37.3%). Non-occlusive mesenteric ischemia (NOMI; 67.9%) indicating microvascular involvement was the most common pattern of bowel involvement. Bowel wall thickening/edema (50.9%) was more common than bowel hypoperfusion (20.7%). While ileum and colon both were equally involved bowel segments (32.07% each), SMA (24.9%), SMV (14.3%), and the spleen (12.5%) were the most commonly involved artery, vein, and solid organ, respectively. 50% of the patients receiving conservative/medical management died, highlighting high mortality without surgery. Findings on laparotomy and histopathology corroborated strikingly with CT imaging findings. CONCLUSION: In COVID-19 patients with AMI, small bowel ischemia is the most prevalent imaging diagnosis and NOMI is the most common pattern of bowel involvement. Contrast-enhanced CT is a powerful decision-making tool for prompt diagnosis of AMI in COVID-19, thereby potentially improving time to treat as well as clinical outcomes.