Clinical Case Session I16Anomalous origin of the coronary artery – 2 case reports17123 I-MIBG (123 I-metaiodobenzylguanidine) scintigraphy in myocardial noncompaction18Incidental detection of a paracardiac aortic paraganglioma via coronary angiography and cardiac CT : Hybrid revascularisation of the patient20Recurrent ventricular arrhythmias in a patient with cardiac sarcoid21An unusual cause for collapse

# 16 Anomalous origin of the coronary artery – 2 case reports {#article-title-2} An anomalous aortic originof the coronary arteryis rare with a reported incidence of 0.3–1.3 %. Anomalous left coronary arteryfrom the right sinus with an interarterial course has a prevalence of 0.17 % and is associated with a high risk of sudden cardiac death during or after strenuous exertion. The authors report the case of two patients with anomalous origin of the coronary arteries. The first case was a 45-year-old- female patient, with no relevant medical history, referred for cardiology consultation by tachycardia, she reported no other relevant cardiovascular symptoms. After a treadmill exercise test which was electrically positive for ischemia, a cardiac CT was requested to rule out coronary artery disease. Cardiac CT revealed an anomalous origin of the left coronary arteryfrom the right sinus of Valsalva with interarterial course between aorta and pulmonary artery. Second case was a 50-year-old male patient with history of smoking, referred for cardiology consultation by atypical chest pain. A Cardiac TC was requested and revealed anomalous origin of the right coronary above the sino-tubular junction, in the anterior wall of the ascending aorta with initial interartrial course between the great vessels. He also did a SPECT which was negative for ischemia. Both patients were presented to our referral center for Cardiac Surgery to discuss the possibility for surgical coronary revascularization. The authors wish to emphasize that Cardiac TC may be a useful and non-invasive tool to establish diagnosis and a surgical approach to rectify congenital coronary artery anomalies. # 17 123 I-MIBG (123 I-metaiodobenzylguanidine) scintigraphy in myocardial noncompaction {#article-title-3} Background and Aim: Isolated ventricular non-compaction (IVNC) is a rare condition caused by the arrest of myocardial compaction during embryogenesis resulting in multiple, deep, intertrabecular recesses communicating with ventricular cavity. Echocardiography shows a thin, outer (subepicardial) myocardial layer and a very thickened inner spongy zone on the endocardial side with deep recesses communicating with the ventricular cavity and filled with blood. IVNC is associated to a high risk of ventricular arrhythmias and sudden cardiac death (SCD), heart failure and systemic embolization. Patient and Methods: A 51 y old black male with familial history of SCD and with an already known condition of IVNC was admitted to our hospital for a heart evaluation including a 123 I-MIBG myocardial scintigraphy, that is regarded as an effective, safe and reliable method to evaluate the sympathetic innervation of the heart, and consequently, to assess the severity of heart failure. Scintigraphy was performed by i.v. injection of 320 MBq of the noradrenaline analogue 123 I-MIBG. Chest scans at 15 minutes and 4 hours from injection were performed. Regions of interest were drown on the heart (H) and mediastinum (M) and the H/M ratio, normalized per pixel, was than calculated together with the wash-out rate of 123 I-MIBG from myocardium. Results: As 123 I-MIBG demonstrated a significant reduction of the left ventricular sympathetic innervation with H/M ratio of 1.53 and 1.56 at respectively 15 min and 4 h and an increased wash out rate (31%) including decay correction. An ICD (Implantable Cardioverter Defibrillator) was implanted despite a not severely compromised left ventricular ejection fraction (LVEF) at echocardiography (42%). One week later the scintigraphic study the patient suffered a ventricular fibrillation interrupted by ICD discharge. Conclusions: The detection of sympathetic disorder at 123-I MIBG study resulted critical in the decision of implanting an ICD with life-saving demonstrated effect. 123 I-MIBG studies appear therefore especially useful in subjects with moderate LVEF reduction in which this test offers important insights on risk stratification and outcome. As suggested by the increase of 123 I-MIBG wash out and by the decrease of its uptake, a sympathetic heart innervation disorder seems to be associated to IVNC. The high frequency of malignant ventricular arrhythmias found in subjects with IVNC might therefore be partly consequent to reduced sympathetic innervation enhancing reentry phenomena due to anatomical abnormalities and to ischemic scarring. # 18 Incidental detection of a paracardiac aortic paragangliomavia coronary angiography and cardiac CT : Hybrid revascularisation of the patient {#article-title-4} Incidental detection of a paracardiac aortic paragangliomavia coronary angiography in a patient with a thrombotic coronary arterial occlusion. 55 year old man managed during acute myocardial infarction and incidentally paracardiac mass was seen via coronary angiography. Then transesophageal echocardiography showed the mass and multisliceCT scan confirmed the mass at posterior of the ascendan aorta. For thrombotic occlusion balloon angioplasty performed but stent was not deployed. He referred to surgery and mass mass excised with a two vessel coronary bypass graft. Paracardiac masses are very rare entities and paragangliomasare extremely rare. When a paracaridac mass detected, approach must be individualised and different imaging modalities should be used for precise diagnose before complete revascularisation. # 20 Recurrent ventricular arrhythmias in a patient with cardiac sarcoid {#article-title-5} A 60 year old woman was referred for management of non sustained broad complex tachycardia with trifasicular block and intermittent complete heart block. The echocardiogram showed septal thickening with normal LV function. The presentation was suggestive of cardiac sarcoid. She was unable to tolerate the planned cardiac MRI due to claustrophobia. A thoracic and abdominal CT was therefore performed and again this showed no evidence of lymphadenopathy or sarcoid. Whilst the CT was principally aimed at excluding extra cardiac sarcoid the CT did show an interventricular septal nodule. A dual chamber ICD was implanted. She was subsequently admitted with recurrent appropriate ICD shocks and incessant VT. Echocardiography demonstrated severe LV impairment with marked dysschrony. This was thought to be due to tachycardia mediated cardiomyopathy, high percentage of RV pacing and possible disease progression. The device was upgraded to a CRT-D with implantation of an LV lead. An RV septal biopsy confirmed cardiac sarcoid and prednisolone was started. She developed a cushingoidappearance but was continued on high dose therapy due to the burden of ventricular arrhythmias. Methotrexate was subsequently introduced as a steroid sparing agent. This was later reduced due to a deterioration in her liver enzymes and the prednisolone increased. During follow up there was a significant increase in her arrhythmia burden with recurrent VT and multiple ICD shocks. This was thought to be due to sarcoid disease progression, either as a manifestation of myocardial scarringor sarcoidosis related inflammation. If sarcoid inflammation was present more aggressive immunosuppressive therapy regime would be required. The patient was limited by proximal myopathy secondary to steroid therapy and it was important to clarify the need for further immunosupression. A tetrofosmin myocardial perfusion scanand fasting FDG PET CT scan was performed to clarify if there was any active cardiac disease. The resting myocardial perfusion imagesshowed almost absent uptake of the tracer in the infero septal region of the myocardium extending into the anterior septum and inferior wall with normal uptake elsewhere. There was almost full thickness myocardial damage in the septum and part of the inferior wall. There was no increase in FDG uptake in this region suggesting burnt out disease. The PET study was critical in establishing that there was no on-going active inflammatory cardiac sarcoid. This allowed the steroids to be weaned giving a significant improvement in the patient's muscle weakness and quality of life. # 21 An unusual cause for collapse {#article-title-6} A 25-year-old Nigerian gentleman presenting with collapse upon exertion; he had no relevant cardiac history but two similar episodes previously. Electrocardiography on admission showed sinus rhythm with tall R waves in the precordial leads, fixed early depolarisation in the anterior leads and T wave inversion in the lateral leads, associated with a raised troponin I. Coronary angiography showed an aberrant right coronary artery arising form the left coronary sinus. This was confirmed on cardiac MRI showing the course of the right coronary artery had an anomalous origin from the left coronary sinusand crossed anteriorly to the aorta and behind the pulmonary artery. Anomalous coronary anatomy has been associated with angina, myocardial infarction and sudden cardiac death; they are the second most common cause of sudden cardiac death in young athletes after hypertrophic cardiomyopathy. These anomalies can be investigated with MRI or CT, with CT coronary angiography being the current gold standard investigation. Management options include conservative therapy with beta-blockers, angioplasty or surgery. Patients who are symptomatic are generally offered surgical intervention, however management of asymptomatic patients is less certain.