Hidden Coronary Artery Ostium and Sudden Death.

A 1-week-old girl died suddenly and unexpectedly. At autopsy the major finding was of a right dominant coronary artery circulation with an inapparent left coronary artery ostium. After careful examination, an anomalous origin of the left coronary artery was found with the ostium located in the non-coronary cusp immediately adjacent to the commissure of the non- and left coronary cusps. The ostium was of small caliber with an obliquely oriented artery (<45°) with no ostial ridges. The artery coursed anteriorly past the left coronary cusp between the aorta and the left atrial appendage to then follow its usual course inferiorly along the anterior aspect of the left ventricle. The reminder of the autopsy was unremarkable. Death was, therefore, attributed to an anomalous and hypoplastic left coronary artery (and ostium) with an acute angle of take-off. Tracing coronary arteries in the very young may be technically difficult due to their small size, thus identifying the location of ostia is important. This may be difficult when the ostium was located close to a commissure.

Accuracy of vascular tortuosity measures using computational modelling.

Severe coronary tortuosity has previously been linked to low shear stresses at the luminal surface, yet this relationship is not fully understood. Several previous studies considered different tortuosity metrics when exploring its impact of on the wall shear stress (WSS), which has likely contributed to the ambiguous findings in the literature. Here, we aim to analyze different tortuosity metrics to determine a benchmark for the highest correlating metric with low time-averaged WSS (TAWSS). Using Computed Tomography Coronary Angiogram (CTCA) data from 127 patients without coronary artery disease, we applied all previously used tortuosity metrics to the left main coronary artery bifurcation, and to its left anterior descending and left circumflex branches, before modelling their TAWSS using computational fluid dynamics (CFD). The tortuosity measures included tortuosity index, average absolute-curvature, root-mean-squared (RMS) curvature, and average squared-derivative-curvature. Each tortuosity measure was then correlated with the percentage of vessel area that showed a < 0.4 Pa TAWSS, a threshold associated with altered endothelial cell cytoarchitecture and potentially higher disease risk. Our results showed a stronger correlation between curvature-based versus non-curvature-based tortuosity measures and low TAWSS, with the average-absolute-curvature showing the highest coefficient of determination across all left main branches (p < 0.001), followed by the average-squared-derivative-curvature (p = 0.001), and RMS-curvature (p = 0.002). The tortuosity index, the most widely used measure in literature, showed no significant correlation to low TAWSS (p = 0.86). We thus recommend the use of average-absolute-curvature as a tortuosity measure for future studies.

High take off left main coronary artery accompanied by multicryptic left ventricle myocardium detected by cardiac computerized tomography in a young male: case report.

BACKGROUND: Myocardial crypts are discrete, narrow, blood filled invaginations within the left ventricular myocardium and high-take-off coronary artery are rare manifestations where coronary arteries originate above the sinotubuler junction. CASE PRESENTATION: A 41-year-old man with multiple coronary artery disease risk factors admitted to our outpatient department with progressive dyspnea and atypical chest pain. Physical examination revealed no pathological findings. His blood examination revealed only mild to moderately high IgE and LDL levels. Transthoracic echocardiography (TTE) was normal. His treadmill test was normal, yet in the 3rd stage of the test he had an atypically located chest pain which was relieved in the resting period. As he had multiple cardiovascular risk factors, we performed a coronary CT angiography to exclude coronary artery disease. Coronary CT angiography(CCTA) demonstrated multiple myocardial crypts, a muscular VSD like defect which were not detectable with TTE and a high take off left main coronary artery (LMCA). After CCTA, we repeated the TTE to investigate the crypts and VSD-like defect which were clear on CCTA, yet a precise TTE hardly showed crypts and didn't confirm a shunt between the left and right ventricle. We defined the defect as 'spontaneously closed muscular VSD'. None of these pathologies were clinically relevant with the patient's symptoms, thus pneumonology started a montelukast therapy for 1 year and we decided to follow up the patient, as multiple crypts may indicate an early phase hypertrophic cardiomyopathy. CONCLUSIONS: Considering that a high take-off LMCA is a congenital anomaly, encountering multiple crypts which are also congenital pathologies, is plausible, as congenital anomalies may accompany eachother. Echocardiography is a very useful, practical imaging tool but regrettably may be suboptimal due to various patient and method related reasons. Target combination of different cardiovascular imaging tools like echocardiography, cardiac CT(CCT), may be utilized in order to ensure a comprehensive diagnosis particularly.

FMD and SCAD: Sex-Biased Arterial Diseases With Clinical and Genetic Pleiotropy.

Multifocal fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection are both sex-biased diseases disproportionately affecting women over men in a 9:1 ratio. Traditionally known in the context of renovascular hypertension, recent advances in knowledge about FMD have demonstrated that FMD is a systemic arteriopathy presenting as arterial stenosis, aneurysm, and dissection in virtually any arterial bed. FMD is also characterized by major cardiovascular presentations including hypertension, stroke, and myocardial infarction. Similar to FMD, spontaneous coronary artery dissection is associated with a high prevalence of extracoronary vascular abnormalities, including FMD, aneurysm, and extracoronary dissection, and recent studies have also found genetic associations between the two diseases. This review will summarize the relationship between FMD and spontaneous coronary artery dissection with a focus on common clinical associations, histopathologic mechanisms, genetic susceptibilities, and the biology of these diseases. The current status of disease models and critical future research directions will also be addressed.

Primary adjunctive corticosteroid therapy is associated with improved outcomes for patients with Kawasaki disease with coronary artery aneurysms at diagnosis.

OBJECTIVE: Patients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outcomes in patients with CAA at diagnosis. DESIGN: Single-centre, retrospective review. PATIENTS: Patients with KD diagnosed within 10 days of fever onset and with baseline CA z-score ≥2.5. INTERVENTIONS: Primary treatment with IVIG (n=162) versus IVIG plus corticosteroids (n=48). MAIN OUTCOME MEASURES: Treatment resistance (persistent fever >36 hours after initial treatment), CAA regression rate. RESULTS: Of the 92 patients with KD who received corticosteroids at our institution from 2012 to 2019, 48 met the inclusion criteria for primary adjunctive therapy. The corticosteroid group was younger and had larger baseline CAAs compared with historical controls. Demographics and laboratory values were otherwise similar between groups. The corticosteroid group had a less treatment resistance (4% vs 30%, p=0.003) and a greater improvement in C reactive protein. After adjusting for baseline CA z-score, age and baseline bilateral versus unilateral CAA, the corticosteroid group had a higher odds of (OR 2.77 (1.04, 7.42), p=0.042) and a shorter time to CAA regression (HR 1.94 (1.27, 2.96), p=0.002). CONCLUSION: Primary adjunctive corticosteroid therapy is associated with decreased initial treatment resistance, greater improvement in inflammatory markers and higher likelihood of CAA regression in patients who have CAA at diagnosis. Multi-centre, randomised controlled trials are needed to confirm the benefits of corticosteroids in patients with CAA at diagnosis and to compare corticosteroids with other adjunctive therapies.

Dual right coronary artery: an unconventional presentation from Pakistan.

There is a significant variation in the mapping of epicardial coronary arteries. This variation may present as different anomalies. These anomalies are usually asymptomatic and can be present at birth. Some might become symptomatic during adulthood and subsequently, result in sudden death. The anomalies, in turn, present a challenge to clinicians during therapeutic intervention. Therefore, there is a growing need to have knowledge about the various forms of the coronary artery anomalies. In our case report, we present the case of a patient who had one such anomaly and provide a hint at one strategy that was used to tackle the challenge.

Intra-Right Ventricle Course of the Coronary Arteries on Computed Tomography Angiography.

BACKGROUND: The clinical relevance and anatomic characteristics of intracavitary coronary arteries coursing within the right ventricle (RV) are largely unknown. OBJECTIVES: The aim of our study was to assess the clinical and computed tomographic characteristics of patients diagnosed with intracavitary coronary arteries coursing within RV (intra-RV coronaries). METHODS: Electronic records from a single high-volume cardiac center were retrospectively screened for the presence of intra-RV coronaries among consecutive patients who underwent coronary computed tomography angiography (coronary CTA) from 2008 to 2019. RESULTS: Overall, 31,748 coronary CTA reports were evaluated, and 17 subjects with intra-RV coronaries were identified. None of these patients was referred for subsequent invasive coronary angiography. One patient underwent coronary artery bypass grafting, 1 patient had a history of percutaneous coronary intervention, and 1 patient had a concomitant coronary anomaly: left circumflex coronary artery originating from the right coronary artery. All of the involved coronaries (n = 17) were the left anterior descending coronary arteries (LADs). Typical segmental coronary course within RV was along the border between free RV wall and interventricular septum (beneath interventricular groove), often within trabeculae carneae of the RV. Only the midsegment and distal segment of the LAD traversed within the RV. The mean ± SD distance from the aorta to the coronary entrance into the RV was 74.5 ± 17.1 mm, whereas the mean ± SD intra-RV coronary length was 25.1 ± 14.0 mm. CONCLUSIONS: Intra-RV course of the coronaries in an adult CTA population is an infrequent anatomical variant involving LAD. It may require additional attention during interventional and surgical interventions.

Coronary Arterial Abnormalities in Hypoplastic Left Heart Syndrome: Pathologic Characteristics of Archived Specimens.

Palliation of patients with hypoplastic left heart syndrome remains challenging. Although coronary ischemia can be catastrophic, the prevalence and pathologies of anomalies of the coronary arteries remains unknown. We reviewed 119 specimens with the features of hypoplastic left heart syndrome, focusing our attention on the aortic root and the coronary arteries. We found 36 (30%) specimens with the combination of mitral and aortic atresia, 26 (22%) with mitral and aortic stenosis, and 57 (48%) with mitral stenosis combined with aortic atresia. In 29 specimens (24%), the coronary arteries were not located in the center of any sinuses, while in 24 specimens (21%) at least 1 coronary artery was located very proximal to a raphe or commissure, with potential for obstruction. The specimens with combined stenosis were more likely to have eccentric positions of the coronary arteries (11 specimens, 42%), compared to the 3 specimens with combined atresia (9%, P = 0.009). The specimens with combined stenosis were also more likely to have positioning at risk for obstruction (9 specimens, 35%), compared to those with combined atresia (3 specimens, 9%, P = 0.05). Coronary arterial fistulous communications were found in 11 specimens (9%), significantly more frequently in specimens with mitral stenosis and aortic atresia (9 specimens, 16%, P = 0.041). The origins of the coronary arteries in patients with hypoplastic left heart syndrome place them at potential risk for ischemia, with fistulous communications being a particular risk in those with mitral stenosis combined with aortic atresia.

Adult ALCAPA: from histological picture to clinical features.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. CASE PRESENTATION: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required re-do surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles. CONCLUSIONS: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the long-term follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment.

Kawasaki at the Extremes of Age: Thinking Outside the Box.

Kawasaki disease is an acute vasculitis syndrome that typically occurs in children aged 1 to 4 years. Because there is no specific diagnostic test for Kawasaki disease, the diagnosis is made clinically based on specific characteristic signs and symptoms. Cases in which patients fall outside of the typical age range are uncommon and often challenging to diagnose because they have atypical presentations. This is especially true in infants, who rarely meet all the clinical criteria required for diagnosis. Patients at the extremes of ages often have a delayed diagnosis, which can lead to worse cardiac outcomes. We describe the cases of a young infant and an older adolescent who present with Kawasaki disease. These cases illustrate the challenge of diagnosing Kawasaki disease in patients beyond the typical age range. Both patients were return visits to the emergency department after inpatient stays. When fever persists longer than 5 days, clinicians must have a high index of suspicion for Kawasaki disease in all pediatric age groups to prevent treatment delay and disease sequelae.

Left coronary artery atresia in the young: long-term follow-up without exercise restriction.

An 11-year-old male was presented with exertional chest pain and was diagnosed with atresia of the left main coronary artery. A stress nuclear perfusion imaging was negative at initial presentation, and a vasodilator stress cardiac MRI was again negative 5 years later. The patient has fully participated in competitive sports for 6 years with no occurrence of cardiac events.

[Anomalous origin of the right coronary artery from the pulmonary artery or coronary artery fistula: when the diagnosis is uncertain]. / Quando la diagnosi può essere incerta: origine anomala dell'arteria coronaria destra dall'arteria polmonare o fistola coronarica?

The anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital coronary anomaly with a reported incidence of approximately 0.002%. Usually, the diagnosis is made by echocardiography leaving computed tomography or angiography only to diagnostic completion in doubtful cases or for interventional procedures.Herein we report a doubtful case of a patient with a diagnosis of coronary fistula between the right coronary and the pulmonary artery that proved to be an anomalous origin of the coronary artery from the pulmonary artery. The patient underwent corrective surgery with translocation of the coronary artery on the aorta.