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Karla Quevedo, Sarmad Said, Debabrata Mukherjee, Tariq Siddiqui
(Department of Internal Medicine, Division of Cardiology, Texas Tech University Health Science Center, El Paso, USA)
Med Sci Case Rep 2014; 1:11-14
Non-compaction cardiomyopathy (NCM) is a newly identified genetic myocardial disorder defined by unique features containing prominent myocardial trabeculations and profound endocardial recesses. An arrest of the normal endomyocard embryogenesis is thought to be the reason. Other congenital abnormalities are often associated with NCM. This type of cardiomyopathy involves usually the left ventricle, however few case reports demonstrated also biventricular involvement. Right ventricular noncompaction features have been described in several cases. Preponderant involvement of the right ventricle is very rare and has been described less frequently in the literature.
Case Report: Fifty-two years old male patient presented with progressive pressure-like chest discomfort, steadily fatigability and shortness of breath associated with physical activity started almost 2 months before the presentation. His past medical history is unremarkable and except for maternal diabetes mellitus his family history was insignificant. Initial laboratory workup was remarkable for an elevated BNP of 1719 pg/mL. Transthoracic echocardiogram demonstrated moderately dilated left ventricle, extensive biventricular trabeculations consistent with features of noncompaction. Left heart catheterization demonstrated normal coronaries and elevated left ventricular end diastolic pressure. Confirmatory cardiac MRI showed hypertabeculations in both ventricles with significant involvement of the right ventricle. Therapy with β-blocker, aspirin, ACE-inhibitor, statins and diuretic agents was initiated. Clinically the patient’s condition improved symptomatically, he was followed up on outpatient basis. His family has been recommended screening for myocardial non-compaction.
Conclusions: Myocardial noncompaction is a unique and very rare abnormality leading to life threatening events. If left ventricular noncompaction is suspected the right ventricle should also be examined carefully. The early detection allows an immediate and appropriate medical therapy resulting in a higher possibility of uneventful survival.
Keywords: Cardiomyopathy, Dilated, Echocardiography, Isolated Noncompaction of the Ventricular Myocardium