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Myocardial infarction with non-obstructive coronary arteries in hypertrophic cardiomyopathy vs Fabry disease
Francesca Graziani1, Rosa Lillo2, Elena Biagini3
1Department of Cardiovascular Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Insights
Myocardial infarction with non-obstructive coronary arteries (MINOCA) is rare in hypertrophic cardiomyopathy (HCM) but significantly more common in Fabry disease (FD). MINOCA can serve as an indicator for FD, aiding in its differentiation from HCM.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Prevalence and predictors of MINOCA in Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are poorly understood.
- This study investigates MINOCA in large cohorts of HCM and FD patients.
Purpose of the Study:
- To assess and compare the prevalence and predictors of MINOCA in adult patients with HCM and FD.
- To identify factors associated with MINOCA in these patient groups.
Main Methods:
- A multicenter, retrospective study included 2870 adult patients with HCM and 267 with FD.
- Exclusion criterion was documented obstructive coronary artery disease; MINOCA was defined per guidelines.
- Clinical, ECG, and echocardiographic data were collected at initial evaluation.
Main Results:
- MINOCA occurred in 0.5% of HCM patients and 7.5% of FD patients (p < 0.001), a 6-fold higher frequency in FD.
- In FD, MINOCA was associated with left ventricle hypertrophy (LVH) (17.7% vs 1.7%, p < 0.001).
- ECG voltage criteria for LVH and maximal LV wall thickness independently predicted MINOCA in FD.
Conclusions:
- MINOCA is significantly more frequent in FD patients compared to HCM patients.
- MINOCA may serve as a crucial indicator for FD, assisting in differential diagnosis from HCM.
Background:
Little is known about prevalence and predictors of myocardial infarction with non-obstructive coronary arteries (MINOCA) in Fabry disease (FD) and hypertrophic cardiomyopathy (HCM). We assessed and compared the prevalence and predictors of MINOCA in a large cohort of HCM and FD patients.
Methods:
In this multicenter, retrospective study we enrolled 2870 adult patients with HCM and 267 with FD. The only exclusion criterion was documented obstructive coronary artery disease. MINOCA was defined according to guidelines. For each patient we collected clinical, ECG and echocardiographic data recorded at initial evaluation.
Results:
Overall, 36 patients had MINOCA during a follow-up period of 4.5 ± 11.2 years. MINOCA occurred in 16 patients with HCM (0.5%) and 20 patients with FD (7.5%; p < 0.001). The difference between the 2 groups was highly significant, also after adjustment for the main clinical, ECG and echocardiographic variables (OR 6.12; 95%CI 2.80-13.3; p < 0.001). In the FD population MINOCA occurred in 17 out of 96 patients with left ventricle hypertrophy (LVH, 17.7%) and in 3 out of 171 patients without LVH (1.7%; OR 12.0; 95%CI 3.43-42.3; p < 0.001). At multivariable analysis, voltage criteria for LVH at ECG (OR 7.3; 95%CI 1.93-27.7; p = 0.003) and maximal LV wall thickness at echocardiography (OR 1.15; 95%CI 1.05-1.27; p = 0.002) maintained an independent association with MINOCA. No major significant differences were found in clinical, ECG and echocardiographic findings between HCM patients with or without MINOCA.
Conclusions:
MINOCA was rare in HCM patients, and 6-fold more frequent in FD patients. MINOCA may be considered a red flag for FD and aid in the differential diagnosis from HCM.
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