Identification and Analysis of Atrial-Bronchial-Abdominal Disharmony in Patients with Isomeric Atrial Appendages.

Ideally, the morphology of atrial appendages should solely be used to identify and differentiate patients with isomeric right and left atrial appendages. However, in clinical practice, the segregation is often indirectly based on the arrangement of thoraco-abdominal structures. The correlation between thoraco-abdominal arrangement and atrial appendages, however, is imperfect. In this study, we sought to clarify the cardiovascular malformations in patients with isomeric atrial appendages with an emphasis on atrial-thoracic-abdominal disharmony. A retrospective review of all patients who underwent cardiac CT angiography between January 2014 and June 2023 and identified to have isomeric atrial appendages was performed. Of the 366 cases (median age: 2 years [interquartile range: 11 months-7 years]), 247 (67.5%) patients had isomeric right atrial appendages while 119 (32.5%) patients had isomeric left atrial appendages. In 316 (86.3%) patients, the thoraco-abdominal arrangement was as per atrial appendage morphology while the remaining 50 (13.6%) patients had disharmonious patterns. Compared to isomeric left atrial appendages, the disharmonious pattern was more frequent with isomeric right atrial appendages (5.9% vs. 17.4%; p 0.003). Irrespective of the type of isomerism, disharmony was mostly confined to the level of the abdomen. Not all patients with isomeric atrial appendages have a harmonious thoraco-abdominal arrangement. The atrial-bronchial-abdominal disharmony is more frequent with isomeric right atrial appendages and is mostly present at the level of the abdomen. A detailed sequential segmental analysis with an independent description of each organ system is, therefore, essential for the complete evaluation of patients with isomeric atrial appendages.

Low-dose, high-pitch, spiral (FLASH) mode versus conventional sequential method for coronary artery calcium scoring: A derivation-validation study.

Introduction: The present study sought to compare the diagnostic accuracy and radiation dose of ECG-gated, ultra-fast, low-dose, high-pitch, spiral (FLASH) mode versus conventional, ECG-gated, sequential coronary artery calcium (CAC) scoring in patients with suspected coronary artery disease (CAD). Methods: The study included 120 patients who underwent both conventional scanning and FLASH mode scanning and were subdivided into derivation and validation cohorts. In the conventional sequential (step-and-shoot) protocol, prospective ECG-gated, non-contrast acquisition was performed at 70% of R-R interval. The spiral (FLASH) mode utilized a high-pitch and high-speed gantry rotation scanning mode where acquisition of the entire heart was done within a single cardiac cycle with prospective ECG-gating at 70% of R-R interval. Results: Correlation between CAC scores derived from conventional (cCAC) and FLASH mode (fCAC) in derivation cohort was excellent (r=0.99; P<0.001). A linear regression model was used to develop a formula for deriving the estimated CAC score (eCAC) from fCAC (eCAC=0.978 x fCAC). In validation cohort, eCAC showed excellent agreement with cCAC (ICC=0.9983; 95%CI: 0.9972 - 0.9990). Excellent agreement for risk classification (weighted kappa=0.93898; 95%CI: 0.86833 - 1.0000) was observed with 95% (57/60) scores falling within the same risk category. Effective dose was significantly lower in FLASH mode (conventional, 0.58±0.21 mSv vs. FLASH, 0.34±0.12 mSv; P<0.0001). Conclusion: CAC scoring using FLASH mode is feasible with high accuracy and shows excellent agreement with conventional CAC scores at significantly reduced radiation doses.

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.

Dermoscopy of hypopigmented macules unveiling genetic diagnosis of tuberous sclerosis complex type 2 in an infant presenting with sacral chordoma.

A 2-month-old male with surgically resected sacral chordoma presented with multiple hypopigmented macules showing characteristic patchy, sharply demarcated areas of pigment network on dermoscopy. These dermoscopic findings were suggestive of the ash-leaf macules of tuberous sclerosis over other common hypopigmented macules in neonates. Chordomas presenting in early childhood in the sacral location have been reported as a rare manifestation of tuberous sclerosis complex. The combination of these findings led to a diagnosis of tuberous sclerosis, confirmed with the finding of a heterozygous TSC2 gene deletion; treatment with sirolimus resulted in regression of cardiac rhabdomyomas and hypopigmented macules.

An unusual case of duplicated left brachiocephalic vein with right sided aortic arch and aberrant origin of LSCA in a patient undergoing implantable cardioverter defibrillator (ICD) implantation.

BACKGROUND: Anomalous left brachiocephalic vein (ALBCV) is a rare venous anomaly. Double Left brachiocephalic vein is the rarest type of ALBCV anomaly. CASE REPORT: Here we report a case of gentleman with post myocardial infarction ventricular tachycardia who underwent ICD implantation, where we could not place the lead initially through left side. CT angiography revealed presence of a duplicated circumaortic left BCV. It's cranial limb coursing normally anterior to arch and compressed at its confluence with RBCV and the caudal limb with a subaortic course draining into the RSVC. We report this first case of double LBCV along with right sided aortic arch and aberrant origin of LSCA arising from Kommerel's diverticulum. CONCLUSION: This case highlights that interventional cardiologists should be aware of these venous anomalies for proper planning and implantation of CIED successfully via transvenous approach.

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.