Left Ventricular Noncompaction Cardiomyopathy: “Spongy Heart”

1Lahey Clinic Medical Center, Burlington, MA
2Lahey Clinic Medical Center, Burlington, MA
3Lahey Clinic Medical Center, Burlington, MA

Meeting: Hospital Medicine 2013, May 16-19, National Harbor, Md.

Abstract number: 457

Case Presentation:

A 59‐year‐old man with a history of hypertension, hyperlipidemia, and type II diabetes mellitus presented with chest tightness, palpitations, and dizziness. He has a family history of premature coronary disease and sudden death. Physical examination was unremarkable. EKG suggests left ventricular hypertrophy and precordial T‐wave inversions. Cardiac biomarkers were negative and nonsustained ventricular tachycardia was noted on telemetry monitoring. Exercise stress echocardiography was negative for ischemia. Transthoracic echocardiography revealed mild concentric left ventricular hypertrophy with marked increased trabeculation of apical and lateral walls with normal ejection fraction. Cardiac MRI showed increased thickness and trabeculation of apical portion of myocardium of left ventricle suggesting diagnosis of noncompaction (Image 1). Electrophysiologic study — programmed stimulation was unable to induce ventricular tachycardia (VT). The patient was treated with a beta‐blocker and aspirin. Given his low‐risk profile, implantable cardioverter defibrillator (ICD) and anticoagulation were deferred. He is followed twice yearly and remains without heart failure, arrhythmia, or thromboembolic complications.

Discussion:

Left ventricular noncompaction (LVNC) is a rare primary genetic cardiomyopathy caused by arrest of normal embryogenesis of the endocardium and myocardium leading to prominent trabeculations and hypertrophy of the left ventricle giving spongy appearance. In children it is third most common primary cardiomyopathy however it is very rare in adults. In observational studies, LVNC has been found to in 0.014% of adults referred for echocardiography. Clinical presentations: — Phenotypic variation, may be asymptomatic. — Systolic or diastolic heart failure. — Arrhythmias/sudden death: VT (47%), atrial fibrillation (25%). — Thromboembolic events. Diagnosis: — 2‐D echocardiography reveals prominent trabeculations and deep intertrabecular recesses. There is usually hypertrophy of the endocardium in the apex and lateral walls. The ratio of noncompacted endocardium to compacted epicardium is >2:1 in LVNC and this helps distinguish it from other causes of increased trabeculation. Contrast ventriculography, CT, and MRI may also be used. — EKG is nonspecific. Management: — β‐blocker has a theoretical benefit. — ICD for primary prevention of sudden death: there are no specific guidelines beyond general guidelines for nonischemic cardiomyopathy. — Thromboprophylaxis is recommended for high‐risk patients. — Serial echocardiography and Holter monitoring. — Screening echocardiography of first‐degree relatives.

Conclusions:

LVNC is rare primary genetic cardiomyopathy thought to be caused by arrest of normal embryogenesis. Patient may present with heart failure, arrhythmias, or thromboembolic event. Echocardiography, contrast ventriculography, or MRI can be used for diagnosis. ICD and thromboprophylaxis should be considered in high‐risk patients.

Image 1. Cardiac MRI showing left ventricular noncompaction.

To cite this abstract:

Ganatra S, Udas P, Shah S. Left Ventricular Noncompaction Cardiomyopathy: “Spongy Heart”. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 457. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/left-ventricular-noncompaction-cardiomyopathy-spongy-heart/. Accessed September 20, 2019.

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