Utilization of Cardiac Graft with Single Coronary Artery for Orthotopic Heart Transplantation

Anomalous coronary arteries arise in a small subset of the population, with each configuration conveying a varying degree of long-term risk. In this report, we describe the discovery of an anomalous single coronary artery with the left main coronary artery arising from the right coronary ostium in a 40-year old male evaluated for cardiac donation. After evaluation, this heart was successfully procured and utilized for orthotopic heart transplantation.

447 Left ventricular non-compaction with coronary artery anomaly: incidental diagnosis after ST-elevation myocardial infarction

Left ventricular noncompaction (LVNC) is a rare congenital cardiomyopathy thought to be caused by arrest of normal endomyocardial morphogenesis. A 33-year-old male, smoker, dyslipidemic not on medical therapy, with family history for sudden cardiac death (SCD) presented to the ER for chest pain radiated to the left arm, unrelated to exertion. The physical examination was within normal limits. Blood tests showed increased cardiac enzymes levels (Troponin I hs 49 308.7 ng/l). The EKG showed STE in the anterior leads and diffuse ventricular repolarization anomalies, suggestive of anterior STEMI. The patient underwent coronary catheterization, with evidence of anomalous origin of the Cx from the RCA and critical stenosis on the proximal LAD, treated with PPCI and implantation of a DES with good angiographic result. The patient was transferred to the Cardiac Intensive Care Unit. TTE showed moderate LV dilatation, severe LV dysfunction (EF 30%) with apical, septal and anterior wall akinesis, and lateral wall hypertrabecularization with multiple prominent trabeculations and deep intertrabecular recesses communicating with the cavity, suggestive for LVNC. Cardiac MRI documented dilated LV with EF 34%, anterior and antero-septal wall akinesis (associated with increased T1 mapping values and areas of LGE after contrast injection, compatible with ischaemic outcomes), infero-lateral wall hypokinesia and LV free wall marked hypertrabecularization with a ratio of not compacted(T)/compacted(M) myocardium of 5 (Petersen criteria for LVNC diagnosis: T/M > 2.3 in telediastolic long-axis view). The patient was discharged in stable clinical conditions in DAPT(Cardioaspirin and Ticagrelor). At two months cardiologic follow-up the patient was asymptomatic and TTE confirmed a dilated LV with severely depressed EF (30%). In consideration of the post-ischaemic dilated cardiomyopathy with severely depressed EF and of the family history of SCD (father deceased at 54-years-old), the patient was admitted in our Cardiology Unit and he underwent subcutaneous ICD (sICD) implantation. He was discharged in stable conditions with remote home monitoring transmitter. The association between LVNC and anomalous coronary artery origin is rare. LVNC is sometimes associated with coronary artery disease, but only rare cases of acute myocardial infarction have been described in literature, with exceptionally rare cases of LVNC incidental diagnosis after STEMI. To the best of our knowledge, this is the first case of LVNC associated with anomalous coronary artery origin and STEMI reported in literature. Some authors hypothesized that a single gene responsible for both myocardial development and coronary endothelium could be involved in the pathogenesis of LVNC and at the same time predispose to coronary atherosclerosis. Further studies are necessary in order to assess the possible pathophysiological mechanisms that correlate LVNC and coronary atherosclerosis. According to European guidelines, LVNC in the absence of additional risk factors is not an indication for primary ICD implantation, and for arrhythmic risk stratification it’s recommended to follow the criteria used for non-ischaemic dilated cardiomyopathy. The anomalous origin of the Cx from the RCA has an incidence of 0.37%, and it is generally not linked to an increased risk of SCD. Involvement of RV in LVNC cannot be excluded even when RV appears normal on CMR, and if involved there is higher risk of perforation by the lead. sICD overcomes disadvantages of transvenous ICD in patients without a need for pacing therapy. In literature use of sICD is reported only in 14 patients with LVNC, mainly children and young adults.

234 Echocardiographic visualization of retroaortic anomalous coronary artery

A 65-year-old female was admitted to our hospital for sudden onset of typical chest pain at rest lasting few minutes. Her medical background included systemic hypertension, type 2 diabetes, dyslipidaemia, and mild obesity. Upon arrival in the emergency room, the electrocardiogram didn’t reveal signs of acute myocardial ischaemia and serial cardiac troponin T measurements were persistently negative. A transthoracic echocardiogram (TTE) was performed, showing mild ventricular hypertrophy, no regional wall motion abnormalities, and a preserved left ventricular ejection fraction. A highly echogenic tubular structure, located slightly on the atrial side of the atrioventricular groove was noted in multiple apical views. Its tubular shape was suggestive of a vascular structure, but its location was atypical for a normal vessel; indeed its persistence in more than an echocardiographic plane excluded an artefact. According to patient’s clinical history and her high cardiovascular risk profile she was referred for coronary angiography, demonstrating no critical stenosis but an anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva: the left main coronary artery (LMCA) arose from the right coronary cusp and then took a caudal posterior loop running posterior to the aortic root. In light of these findings we could associate the tubular structure seen at TTE to the retroaortic course of LMCA, a finding recently described as retroaortic anomalous coronary (RAC) sign. Among AAOCA, the retroaortic course of the LMCA is an uncommon diagnosis in adults, and its association with a single coronary origin is extremely rare. Although it has been usually considered a benign clinical entity, it is associated with an increased risk in morbidity and mortality during valve surgery. The presence of RAC sign at TTE was demonstrated to be highly suggestive of an anomalous coronary artery (specificity 93.9%) and strongly associated with retroaortic LMCA course at computed tomography angiography. 234 Figure B

Bicuspid aortic valve and anomalous right coronary artery from the opposite coronary sinus

We report on a patient with bicuspid aortic valve and anomalous right coronary artery from the opposite sinus without evidence of intramural course. Different authors support the universal presence of intramural course in patients with origin of the right coronary artery from the opposite sinus of Valsalva in normal heart. The occurrence of both bicuspid aortic valve and the absence of intramural course may not be accidental. This might suggest a developmental interaction between bicuspid aortic valve and anomalous coronary artery. Large observational study including characterisation by intravascular ultrasonography in patients with bicuspid aortic valve and anomalous coronary is needed.

High Risk Features of an Anomalous Origin of the Right Coronary Artery

Anomalous aortic origin of the coronary arteries (AAOCA) is a rare congenital abnormality. It is usually asymptomatic and often found incidentally during coronary angiography. However, it can also be discovered during the autopsy of young healthy adults who have suffered from sudden cardiac death (SCD). AAOCA represents the second most common cause of SCD in young athletes. Herein, we report a case of a 39-year-old patient with left-sided right coronary anomaly with multiple high-risk features who presented with life-threatening symptoms for SCD but normal electrocardiography, echocardiography, and cardiac markers. The coronary computed tomography revealed an anomalous coronary artery from the left sinus of Valsalva with a hypoplasic origin and a high-risk path between the aorta and the pulmonary artery with a short intramural path. He was surgically managed with a coronary artery bypass with an uneventful follow-up.

Assessment of the intramural segment of interarterial anomalous coronary arteries originating from the opposite sinus of Valsalva on CT angiography

Background Of the coronary anomaly variants, an anomalous coronary artery originating from the opposite sinus (AAOCA) with an interarterial course poses the highest the risk of sudden cardiac death. Assessment of high risk anatomical characteristics can be done with Computed Tomography Angiography (CTA). High risk features are a slit-like ostium, acute angle take-off, proximal narrowing and an intramural course. For an intramural course no clear-cut CT parameters exist. Purpose To deduct new CTA criteria to identify an intramural course as well as the length of the intramural segment based on peroperative findings. Material and methods Twenty patients were included that received unroofing surgery of the right or left AAOCA between 2010 and 2019. All patients had a pre-operative CTA (0.5–1mm slice-thickness) performed. The presence of the intramural segment was measured peroperatively by the surgeon and used as indicator for CTA evaluation. Using multiplanar reconstructions, CTA images were rotated perpendicular to the horizontal plane of the aortic valve annulus and AAOCA to assess the distance between the aortic and AAOCA lumen and the shape of the AAOCA. This was done at every 2mm for the length of the intramural course as described by the surgeon (Figure 1). Results Analysis of 20 patients (40% male, AAORCA n=17, age at diagnosis AAOCA 45.6±10.5 years), showed a mean intramural length of 11.5±2.4mm at surgery. The median distance between the aortic and AAOCA lumen was 0.76mm (IQR 0.72–0.97mm) for the intramural segment. At the distal end of the intramural part (indicated by no. 5 in Figure 1), the mean distance was 1.20mm±0.27mm. The median ratio between the antero-posterior and transverse diameter of the AAOCA lumen at the distal end of the intramural part was 0.94 (IQR 0.88–0.99). Along the intramural part (Figure 1, no. 1–4) this ratio was 0.56mm±0.11mm, indicating a more flattened ostial shape along the course of the vessel. Conclusions Results indicate that an aortic to AAOCA lumen distance of ≤0.76mm on CTA is suggestive of an intramural course. A distance between the aorta and AAOCA of ≥1.2mm combined with an antero-posterior to transverse diameter ratio of 0.94 of the AAOCA indicates that the intramural trajectory has ended. FUNDunding Acknowledgement Type of funding sources: None.

A Case of Malignant Course of Right Coronary Artery: Frequent Angina in Young Person

Introduction:Congenital anomalous coronary artery is a rare condition, but it might be the biggest pitfall for cardiologist. It might be silent until the patient reach young adult and has high intensity activity. Symptomatic anomalous course of coronary artery has wide spectrum from asymptomatic until the lethal one.Case Ilustration: We present a case of young adult with activity-triggered atypical chest pain and diagnose with anomalous origin of right coronary artery (RCA) from the left coronary sinus with inter-arterial course between the aorta and the main pulmonary artery that was detected by CT coronary angiography. This anomaly has been called malignant course RCA.Discussion:Coronary artery anomaly is a congenital condition. Most of the cases are remain asymptomatic. This condition also one of the most cause for sudden cardiac death because the coronary artery examination is not regularly done. Nevertheless, during high intense activity, it will be symptomatic and might be lethal.Conclusion:Diagnose coronary artery anomalies might be tricky and cardiologist must be aware with this. More devastating, no firm guideline in treatment of right anomalous coronary artery from opposite sinus.

A case of acute myocardial infarction with anomalous origin of the right coronary artery from the contralateral aortic sinus

Anomalous coronary anomalies are technically challenging. We describe the case of a 48-year-old male who suffered an acute inferior wall myocardial infarction due to thrombotic total occlusion in an anomalous right coronary artery arising from the contralateral aortic sinus.