MYOCARDIAL INFARCTION AS TYPICAL PRESENTATION OF NONCOMPACTION CARDIOMYOPATHY

The main clinical presentation of noncompaction myocardium (NCM) as nosologic unit are thromboembolic syndrome, heart failure, mostly ventricular arrhythmias and ischemia itself, related to insufficient myocardium blood supply under the noncompacted layer. Also, there are only sporadic cases of myocardial infarction described (MI), including “idiopathic”, in NCM.

Aim. To check the prevalence of acute MI in patients with NCM, the specialties of clinical picture, diagnostics, and its probable specific mechanisms, influence on prognosis and its ways of prevention.

Material and methods. Totally, 10 patients included, 7 males and 3 females, mean age — 46,3±15,8 y. o., (30 to 76 y. o.) among 85 patients with verified NCM diagnosis, set in accordance with harmonized visual criteria, and developed MI on this background. In 4 cases the NCM is confirmed with three visualizing criteria (EchoCG, MSCT, MRI of the heart), in 4 other — with the two. Mean follow-up was 10,5 [1,75; 32,25] months — from 1 month to 1 year. All patients underwent ECG, ambulatory ECG recording by Holter, EchoCG, assessment of antibodies against various heart antigens, PCR for DNA of parvovirus and B19, as well as herpes group, in blood; 7 coronary arteriographies, 7 MSCT of the heart, measurement of Troponin (n=7), morphological investigation of the heart with PCR-diagnostic of viral infection (n=6), MRI (n=5) and myocardium scintigraphy with 99mТс (n=6).

Results. In 4 among 10 patients the development of MI was the first presentation of NCM. Prevalence of coronary atherosclerosis in those with MI and NCM was 20%, however in most cases development of MI was not related with coronary atherosclerosis. Intracardiac thrombosis was verified in 60% of patients with MI, embolism to other organs in 30%. The following mechanisms of MI established: 1) thromboembolism to coronary arteries if thrombi are present in the left chambers of the heart (atrium a. w.a. ventricle), verified in 1 patient at autopsy, and is suspected in other five; 2) concomitance of myocarditis, incl. viral, with microvasculitis development and thrombosis of intramyocardial arteries and focal necrosis in ischemized myocardium (n=6); 3) thrombosis of coronary arteries with presence of hemodynamically significant atherosclerosis (probably 1 patient); 4) sudden worsening of blood supply under noncompacted layer under the circumstances of low cardiac output by secondary origins.

Conclusion. MI is typical and unrare complication of NCM: its prevalence reached 11,8% in separate registry of 85 patients with NCM syndrome. Four probable mechanisms of MI (necrosis) in NCM (embolism, thrombosis, myocarditis, microcirculation disorder) might be comorbid. The development of MI leads to serious worsening of the baseline systolic dysfunction and ventricular rhythm disorders: mortality among MI patients with NCM is 20% with the mean time of follow-up 10,5 months. As a preventive matter against MI in NCM should be concerned the anticoagulants at least for atrial fibrillation and in systolic dysfunction, on-time diagnostics and myocarditis management.

1. Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990; 82(2): 507-13.

2. Dusek J, Ostádal B, Duskova M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol. 1975; 99(6): 312-7.

3. Engberding R, Bender F. [Echocardiographic detection of persistent myocardial sinusoids]. [Article in German]. Z Kardiol. 1984; 73(12): 786-8.

4. Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies. Circulation. 2006; 113(14): 1807-16.

5. Kaski JP, Elliott P. ESC Working Group. The classification concept of the ESC Working Group on myocardial and pericardial diseases for dilated cardiomyopathy. Herz. 2007; 32(6): 446-51

6. Baldi M, Sgalambro A, Nistri S et al. Clinical and genetic features of left ventricular noncompaction: a continuum in cardiomyopathies. [Article in Italian] G Ital Cardiol (Rome). 2010; 11(5): 377-85.

7. Stöllberger C, Keller H, Finsterer J. Disappearance of left ventricular hypertrabeculation/ noncompaction after biventricular pacing in a patient with polyneuropathy. J Card Fail. 2007; 13(3): 211-4.

8. Pfammatter JP, Paul T, Flik J, et al. Q-fever associated myocarditis in a 14-year-old boy. [Article in German] Z Kardiol. 1995; 84(11): 947-50.

9. Sen-Chowdhry S, McKenna WJ. Left ventricular noncompaction and cardiomyopathy: cause, contributor, or epiphenomenon? Curr Opin Cardiol. 2008; 23(3): 171-5.

10. Stöllberger C, Hamedanchi A, Finsterer J. Myopathy, apical hypertrophic cardiomyopathy and left ventricular noncompaction within the same family. Acta Cardiol. 2009; 64(1): 35-40.

11. Ker J, Du Toit-Prinsloo L, Van Heerden WF, et al. Subendocardial fibrosis in left ventricular hypertrabeculation-cause or consequence? Clin Med Insights Cardiol. 2011; 5: 13-6.

12. Fazio G, Visconti C, D’Angelo L, et al. Delayed MRI hyperenhancement in noncompaction: sign of fibrosis correlated with clinical severity. AJR Am J Roentgenol. 2008; 190(4): W273.

13. Kurita T, Matsuoka K, Hoshida K, et al. Unique myocardial fibrosis pattern by late gadolinium enhanced magnetic resonance imaging in a patient with isolated noncompaction of the ventricular myocardium. Circ J. 2010; 74(2): 381-2.

14. Stöllberger C, Finsterer J, Blazek G, et al. Coronary angiography in noncompaction with and without neuromuscular disorders. Ir J Med Sci. 2011; 180(3): 667-72.

15. Everett ME, Kirkpatrick JN, Lang RM. Noncompaction of the myocardium complicated by coronary artery embolism. J Am Soc Echocardiogr. 2005; 18(2): 194-6.

16. Yavuzgil O, Gürgün C, Soydas Cinar C, et al. Anterior myocardial infarction in an adult patient with left ventricular hypertrabeculation/noncompaction. Int J Cardiol. 2006; 106(3): 394-5.

17. Swinkels BM, Boersma LV, Rensing BJ, et al. Isolated left ventricular noncompaction in a patient presenting with a subacute myocardial infarction. Neth Heart J. 2007; 15(3): 109-11.

18. Correia E, Santos LF, Rodrigues B, et al. Noncompaction of the myocardium in a patient with acute myocardial infarction. [Article in English, Portuguese]. Arq Bras Cardiol. 2010; 94(5): e62-4, e125-7.

19. Güvenç TS, Erer HB, Altay S, et al. “Idiopathic” acute myocardial infarction in a young patient with noncompaction cardiomyopathy. Cardiol J. 2012; 19(4): 429-33.

20. Paiva M, Pinho T, Sousa A, et al. Embolic complication of left ventricular non-compaction as an unusual cause of acute myocardial infarction. Rev Port Cardiol. 2012; 31(11): 751-4.

21. Pulignano G, Tinti MD, Tolone S, et al. Noncompaction and embolic myocardial infarction: the importance of oral anticoagulation. Rev Port Cardiol. 2015; 34(7-8): 497.e1-4.

22. Toufan M, Shahvalizadeh R, Khalili M. Myocardial infarction in a patient with left ventricular noncompaction: a case report. Int J Gen Med. 2012; 5: 661-5.

23. Fettouhi H, Tamdy A, Ellouali F, et al. [Convulsives crisis revealing left-ventricular noncompaction with apical myocardial infarction]. Ann Cardiol Angeiol (Paris). 2011; 60(3): 159-64.

24. Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001; 86(6): 666-71.

25. Stöllberger C, Gerecke B, Finsterer J, et al. Refinement of echocardiographic criteria for left ventricular noncompaction. Int J Cardiol. 2013; 165(3): 463-7.

26. 2013 ESC guidelines on the management of stable coronary artery disease: the Task Force on the management of stable coronary artery disease of the European Society of Cardiology. Task Force Members. Eur Heart J. 2013; 34(38): 2949-3003.

27. http://eurheartj.oxfordjournals.org/content/early/2015/12/09/eurheartj.ehv320

28. Dobranici M, Buzea A, Popescu R, et al. Genetic disorder or toxoplasma myocarditis: a case report of dilated cardiomyopathy with hypertrabeculation in a young asymptomatic woman. J Med Life. 2012; 5(1): 110-3.

29. Brodskiĭ AV, Nadzhafova KN, Kovalev IuR, et al. A clinical case of diagnosis of left ventricular noncompaction myocardium in a patient with infectious myocarditis. Ter Arkh. 2014; 86(4): 75-9.

30. Patil KG, Salagre SB, Itolikar SM. Left ventricular non-compaction with viral myocarditis: a rare presentation of a rarer disease. J Assoc Physicians India. 2014; 62(3): 261-3.

31. Cho HJ, Ma JS. Left ventricular non-compaction progression to dilated cardiomyopathy following acute myocarditis in an early infant twin. Minerva Pediatr. 2015; 67(2): 199-202.

32. Blagova OV, Nedostup AV, Sedov VP, et al. [Noncompaction myocardium as a primary phenomenon or consequence of myocardial dysfunction: clinical masks of the syndrome]. Kardiologia. 2012; 52(11): 17-26.

33. Blagova OV, Pavlenko EV, Nedostup AV, et al. The Left Ventricular Noncompaction Syndrome In 45 Adults: Clinical Variants, Follow-Up And Outcomes. J Atrial Fibrillation, October 2015, Special Issue. Venice Arrhythmias. www.jafib.com, va_oral.1.

34. Caforio AL, Pankuweit S, Arbustini E, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2013; 34(33): 2636-48.

35. Basic D, Gupta S, Kwong RY. Parvovirus b19-induced myocarditis mimicking acute myocardial infarction: clarification of diagnosis by cardiac magnetic resonance imaging. Circulation. 2010; 121(7): e40-2.

36. Liapounova NA, Mouquet F, Ennezat PV. Acute myocardial infarction spurred by myopericarditis in a young female patient: Coxsackie B2 to blame. Acta Cardiol. 2011; 66(1): 79-81.

37. Iwanaga N, Nakamura S, Fukuda Y, et al. A fatal case of acute myocardial infarction following the improvement of influenza A(H1N1)pdm2009-related acute myocarditis. Intern Med. 2014; 53(18): 2153-7.

38. Francone M, Chimenti C, Galea N, et al. CMR sensitivity varies with clinical presentation and extent of cell necrosis in biopsy-proven acute myocarditis. JACC Cardiovasc Imaging. 2014; 7(3): 254-63.

39. Wan J, Zhao S, Cheng H, et al. Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left ventricular non-compaction. J Cardiovasc Magn Reson. 2013; 15: 20.

40. Dodd JD, Holmvang G, Hoffmann U, et al. Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. AJR Am J Roentgenol. 2007; 189(4): 974-80.

41. Barbukhatty KO, Boldyrev SY, Rossokha OA, et al. A rare case of coronary artery bypass grafting in a patient with left ventricular noncompaction. Ann Thorac Surg. 2010; 90(6): 2047-9.

42. Stöllberger C, Wegner C, Finsterer J. CHADS2 — and CHA2DS2VASc scores and embolic risk in left ventricular hypertrabeculation/noncompaction. J Stroke Cerebrovasc Dis. 2013; 22(6): 709-12.

43. Erer HB, Güvenç TS, Kemik AS, et al. Troponin and anti-troponin autoantibody levels in patients with ventricular noncompaction. PLoS One. 2013; 8(2): e57648.

44. Doesch C, Schimpf R, Haneder S, et al. Patient with hypertrophic cardiomyopathy with apical aneurysm and thrombus presenting with progressive congestive heart failure. Hellenic J Cardiol. 2015; 56(3): 258-9.

45. Morita Y, Kato T, Okano M, et al. A rare case of hypertrophic cardiomyopathy with subendocardial late gadolinium enhancement in an apical aneurysm with thrombus. Case Rep Radiol. 2014; 2014: 780840

46. Stöllberger C, Ormin V, Finsterer J. Fatal outcome of aortic-valve endocarditis in noncompaction/hypertrabeculation. Int J Cardiol. 2011; 150(2): e46-7.