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Myocardial infarction with non-obstructive coronary arteries in hypertrophic cardiomyopathy vs Fabry disease

      Highlights

      • MINOCA is 6-fold more frequent in Fabry Disease vs Hypertrophic Cardiomyopathy.
      • MINOCA may be considered a red flag for Fabry Disease.
      • In Fabry Disease, MINOCA is associated to a more advanced stage of cardiomyopathy.

      Abstract

      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.

      Keywords

      Abbreviations:

      CMD (coronary microvascular dysfunction), FD (Fabry disease), HCM (Hypertrophic Cardiomyopathy), LVEF (left ventricular ejection fraction), LVH (left ventricle hypertrophy), LVWT (left ventricular wall thickness), MINOCA (Myocardial infarction with non-obstructive coronary arteries)
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      References

        • Thygesen K.
        • Alpert J.S.
        • Jaffe A.S.
        • et al.
        Fourth universal definition of myocardial infarction (2018).
        Eur. Heart J. 2019; 40: 237-269
        • Agewall S.
        • Beltrame J.F.
        • Reynolds H.R.
        • et al.
        ESC working group position paper on myocardial infarction with non-obstructive coronary arteries.
        Eur. Heart J. 2017; 38: 143-153
        • Montone R.A.
        • Jang I.K.
        • Beltrame J.F.
        • et al.
        The evolving role of cardiac imaging in patients with myocardial infarction and non-obstructive coronary arteries.
        Prog. Cardiovasc. Dis. 2021 Sep-Oct; 68: 78-87
        • Tamis-Holland J.E.
        • Jneid H.
        • Reynolds H.R.
        • et al.
        American Heart Association interventional cardiovascular Care Committee of the Council on clinical cardiology; council on cardiovascular and stroke nursing; council on epidemiology and prevention; and council on quality of care and outcomes research. Contemporary diagnosis and management of patients with myocardial infarction in the absence of obstructive coronary artery disease: a scientific statement from the American Heart Association.
        Circulation. 2019 Apr 30; 139: e891-e908
        • Nordenskjöld A.M.
        • Baron T.
        • Eggers K.M.
        • et al.
        Predictors of adverse outcome in patients with myocardial infarction with non-obstructive coronary artery (MINOCA) disease.
        Int. J. Cardiol. 2018 Jun 15; 261: 18-23
        • Rakowski T.
        • De Luca G.
        • Siudak Z.
        • et al.
        Characteristics of patients presenting with myocardial infarction with non-obstructive coronary arteries (MINOCA) in Poland: data from the ORPKI national registry.
        J. Thromb. Thrombolysis. 2019 Apr; 47: 462-466
        • Camici P.G.
        Coronary microvascular dysfunction in patients with cardiomyopathies.
        Circ. Heart Fail. 2008; 1: 150-152
        • Tomberli B.
        • Cecchi F.
        • Sciagrà R.
        • et al.
        Coronary microvascular dysfunction is an early feature of cardiac involvement in patients with Anderson-Fabry disease.
        Eur. J. Heart Fail. 2013 Dec; 15: 1363-1373
        • Cecchi F.
        • Olivotto I.
        • Gistri R.
        • Lorenzoni R.
        • Chiriatti G.
        • Camici P.G.
        Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy.
        N. Engl. J. Med. 2003; 349: 1027-1035
        • Graziani F.
        • Lillo R.
        • Panaioli E.
        • et al.
        Massive coronary microvascular dysfunction in severe Anderson-Fabry disease cardiomyopathy.
        Circ. Cardiovasc. Imaging. 2019 Jun; 12e009104
        • Ommen S.R.
        • Mital S.
        • Burke M.A.
        • et al.
        2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association joint committee on clinical practice guidelines.
        Circulation. 2020 Dec 22; 142: e558-e631
        • Biegstraaten M.
        • Arngrímsson R.
        • Barbey F.
        • et al.
        Recommendations for initiation and cessation of enzyme replacement therapy in patients with Fabry disease: the European Fabry Working Group consensus document.
        Orphanet J. Rare Dis. 2015 Mar 27; 10: 36
        • Vitale G.
        • Ditaranto R.
        • Graziani F.
        • et al.
        Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy.
        Heart. 2022 Jan; 108: 54-60
        • Camici P.G.
        • Olivotto I.
        • Rimoldi O.E.
        The coronary circulation and blood flow in left ventricular hypertrophy.
        J. Mol. Cell. Cardiol. 2012 Apr; 52 (Epub 2011 Sep 5): 857-864https://doi.org/10.1016/j.yjmcc.2011.08.028
        • Chimenti C.
        • Morgante E.
        • Tanzilli G.
        • et al.
        Angina in Fabry disease reflects coronary small vessel disease.
        Circ. Heart Fail. 2008 Sep; 1: 161-169
        • Frustaci A.
        • Russo M.A.
        • Francone M.
        • Chimenti C.
        Microvascular angina as prehypertrophic presentation of Fabry disease cardiomyopathy.
        Circulation. 2014 Oct 21; 130: 1530-1531
        • Utsumi K.
        • Yamamoto N.
        • Kase R.
        • et al.
        High incidence of thrombosis in Fabry’s disease.
        Intern. Med. 1997 May; 36: 327-329https://doi.org/10.2169/internalmedicine.36.327