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Sumario Vol. 43 - Nº 1 Enero - Marzo 2014


Noncompaction Cardiomyopathy

Silvia V. Agostino, Martín Robles, Ricardo L. Beigelman

Hospital Municipal Héctor Dagnillo
(1406) Marcos Paz, Provincia de Buenos Aires
E mail

Recibido 12-OCT-13 – ACEPTADO después de revisión el 27-DICIEMBRE-2013.
The authors declare not having conflicts of interest.
Rev Fed Arg Cardiol. 2014; 43(1): 52


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Female, 32-year-old patient, who consulted because of strong palpitations unrelated to any activity or specific situation. No cardiovascular risk factors. Her clinical parameters were: blood pressure 120/70, same regular pulse, 74 beats per minute. The physical examination did not yield significant elements. Laboratory tests and X-rays were also within normal limits. The ECG showed sinus rhythm with normal QRS width and isolated monomorphic premature ventricular contractions. Doppler echo displayed numerous trabeculations in the left ventricle with a predominant location in the interventricular septum (Figure 1) and in the inferior wall (Figure 2). The noncompaction-compacted myocardium ratio at the end of diastole was 3 in average. Between the trabeculations, the presence of color Doppler flow stood out. The left ventricle showed dimensions and systo-diastolic function within normal limits.

Figure 1.

 

Figure 2.

 

Noncompaction myocardium is a primary, genetic cardiomyopathy according to the new classification [1], characterized by the presence of an extensive layer of trabeculated myocardium with multiple and deep intertrabecular recesses on the endocavitary surface of the compacted portion of the ventricular wall [2]. It is probably secondary to a halt in the normal process of compaction in the ventricular wall, occurring between the 5th and the 8th week of embryonic life [3]. The prevalence estimated in the patients referred to echocardiography is 0.014% [4]. There is no specific treatment for this entity. Some authors suggest antiaggregation or anticoagulation because of the possibility of embolic events. In some patients, defibrillators were implanted by them presenting severe ventricular arrhythmias or family history of sudden cardiac death [5].

 

BIBLIOGRAPHY

  1. Maron B, Chair J, Towbin A, et al. Contemporary definitions and classification of the cardiomyopathies. Circulation 2006; 113: 1807-16.
  2. Mérida Álvarez O, Valdés García M, Castro Arca AM, et al. Dilemas actuales en el diagnóstico del VI no compacto. Rev Fed Arg Cardiol 2013; 42 (4): __-__.
  3. Sedmera D, ThomasPS. Trabeculation in the embryonic heart. Bioessays 1996; 18 (7): 607.
  4. Chrissoheris M, Ali R, Vivas Y, et al. Isolated noncompaction of the ventricular myocardium: Contemporary diagnosis and management. Clin Cardiol 2007; 30: 156-60.
  5. Oeschslin E, Attenhofer Jost CH, Rojas JR, et al. Long term follow up of 34 adults with isolated left ventricular noncompaction: A distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36 (2): 493-500.

 

Publication: Mach 2014

 
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