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Aims Sudden cardiac death (SCD) is an appalling complication of hypertrophic cardiomyopathy (HCM). There is an ongoing discussion about the optimal SCD risk stratification strategy since established SCD risk models have suboptimal discriminative power. The aim of this study was to evaluate the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for SCD risk stratification compared to the European Society of Cardiology (ESC) SCD risk score and traditional risk factors in an >10-year follow-up. Methods and results Two hundred and twenty consecutive patients with HCM and LGE-CMR were enrolled. Follow-up data were available in 203 patients (median age 58 years, 61% male) after a median follow-up period of 10.4 years. LGE was present in 70% of patients with a median LGE amount of 1.6%, the median ESC 5-year SCD risk score was 1.84. In the overall cohort, SCD rates were 2.3% at 5 years, 4.8% at 10 years, and 15.7% at 15 years, independent from established risk models. An LGE amount of >5% left ventricular (LV) mass portends the highest risk for SCD with SCD prevalences of 5.5% at 5 years, 13.0% at 10 years, and 33.3% at 15 years. Conversely, patients with no or ≤5% LGE of LV mass have favourable prognosis. Conclusions LGE-CMR in HCM patients allows effective 10-year SCD risk stratification beyond established risk factors. LGE amount might be added to established risk models to improve its discriminatory power. Specifically, patients with >5% LGE should be carefully monitored and might be adequate candidates for primary prevention implantable cardioverter-defibrillator during the clinical long-term course.

Background: Coronary spasm is an established cause for myocardial infarction with unobstructed coronary arteries, and can be diagnosed using intracoronary acetylcholine testing. However, it has been questioned whether such testing is feasible and safe in the acute phase. The aim of this study was to assess the frequency of coronary spasm and the safety of the acetylcholine test in patients with myocardial infarction with unobstructed coronary arteries compared to patients with stable angina and unobstructed coronaries. Methods: One hundred and eighty selected patients (52% women, mean age 62±13 years) with either myocardial infarction with unobstructed coronary arteries ( n=80) or stable angina and unobstructed coronaries ( n=100) were enrolled from 2007–2018. All patients underwent the acetylcholine test according to a standardised protocol immediately after diagnostic angiography. Apart from assessment of clinical, demographic and risk factor data, side effects and complications during the acetylcholine test were recorded. Results: Overall, epicardial spasm was found in 26% with a higher prevalence among the myocardial infarction with unobstructed coronary arteries compared to the stable angina patients (35% vs 19%, p=0.017). Microvascular spasm was found in 42% with a higher prevalence among the stable patients compared to the myocardial infarction with unobstructed coronary arteries cohort (53% vs 29%, p=0.0014). There were no statistically significant differences in the rate of side effects (16% vs 14%, p=0.674) or complications (1% vs 2.5%, p=0.438) between the two groups. None of the patients experienced irreversible complications. Conclusion: Coronary spasm is a frequent cause for myocardial infarction with unobstructed coronary arteries. Spasm provocation testing using acetylcholine is feasible in such patients in the acute phase. The complication rate during acetylcholine testing in myocardial infarction with unobstructed coronary arteries patients is low and comparable to patients with stable angina.

Background Approximately 10% of patients with acute myocardial infarction do not have a culprit lesion. Such patients have been labelled as MINOCA (myocardial infarction with non-obstructive coronary arteries) and several pathophysiological etiologies have been described as potential explanations. This includes spontaneous coronary dissection, tako-tsubo-syndrome and coronary spasm. The latter can be diagnosed during invasive provocative testing. The aim of this study was to assess the frequency of coronary spasm and the safety of intracoronary provocation testing using acetylcholine in MINOCA patients compared to patients with stable angina and unobstructed coronary arteries. Methods Between 2007 and 2018 180 consecutive patients with either MINOCA or stable angina and unobstructed coronary arteries were enrolled. MINOCA was defined as acute onset of chest pain with either ST-segment elevation on the ECG or significant high sensitive troponin T elevation but no relevant epicardial stenosis (<50%) according to the current ESC guidelines. All patients underwent intracoronary acetylcholine provocation testing (ACH-test) in search of coronary spasm according to a standardized protocol immediately after diagnostic coronary angiography. Apart from systematic assessment of clinical, demographic and risk factor data, data regarding complications during the ACH-test were meticulously recorded. Results Eighty patients with MINOCA and 100 consecutive patients with stable angina were recruited (52% women, mean age 62±13 years). Overall, 59% had hypertension and 20% had diabetes. Comparison of clinical, demographic and risk factor data did not reveal any statistically significant differences except for a female preponderance in the stable patients (61% vs. 40%, p=0.007). The ACH-test revealed a coronary vasomotor disorder in 68% of cases. In 32% of cases the ACH-test was either inconclusive or negative. Epicardial spasm was found in 31% of patients with a higher prevalence among the MINOCA patients compared to the stable angina patients (41% vs. 23%, p=0.002). Microvascular spasm was found in 37% with a higher prevalence among the stable angina patients compared to the MINOCA cohort (49% vs. 23%, p=0.002). Assessment of complications during the ACH-test revealed that 13 MINOCA patients and 15 stable angina patients had minor complications such as intermittent atrioventricular block, sinusbradycardia, paroxysmal atrial fibrillation, ventricular ectopic beats or transient hypotension. Comparison of minor complications between the two groups did not reveal statistically significant differences (16% vs. 15%, p=0.839). None of the patients experienced any irreversible complications. Conclusion Coronary spasm is a frequent cause for MINOCA. Intracoronary spasm provocation testing using acetylcholine is feasible in such patients. The complication rate during ACH-testing in MINOCA patients is low and comparable to patients with stable angina. Acknowledgement/Funding Berthold-Leibinger-Foundation, Ditzingen, Germany