Left Ventricular Noncompaction Cardiomyopathy in an Elderly Patient: A Case Report and Literature Review

Tetyana Okan1, Homayoon Lodeen2, Michael Abawkaw2

  • 1Department of Internal Medicine, Lviv National Medical University, Lviv, UKR.

Cureus
|June 1, 2023
PubMed

Insights

Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare genetic heart condition often diagnosed late. Early detection through echocardiography and advanced imaging is crucial for managing severe complications like heart failure and arrhythmias.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • Isolated left ventricular noncompaction cardiomyopathy (LVNC), a rare congenital disorder, results from impaired fetal myocardial development.
  • Classified as a genetic cardiomyopathy, LVNC affects 0.017-0.26% of adults, often leading to left ventricular dysfunction and high mortality.
  • Limited knowledge and diagnostic challenges contribute to the underdiagnosis of LVNC, particularly in adults.

Observation:

  • A 62-year-old male presented with palpitations, an irregular heartbeat, and a systolic murmur.
  • Electrocardiogram revealed atrial fibrillation and ST-T wave changes; echocardiography showed severe left ventricular hypertrophy, reduced ejection fraction (<30%), and prominent trabeculations.
  • Cardiac MRI confirmed LVNC, excluding ischemic heart disease.

Findings:

  • Transthoracic echocardiography (TTE) is the primary diagnostic tool, revealing characteristic features like a high noncompacted to compacted myocardium ratio (>2.5:1).
  • Cardiac magnetic resonance (CMR) imaging provides definitive diagnosis, complementing TTE findings.
  • The patient was treated with carvedilol, ramipril, verospiron, torasemide, rivaroxaban, and an implantable cardioverter-defibrillator was recommended.

Implications:

  • Delayed diagnosis of LVNC in adults is common due to its resemblance to more prevalent heart conditions.
  • Early diagnosis is critical for preventing life-threatening complications, including heart failure, thromboembolic events, and ventricular arrhythmias.
  • Further prospective studies are essential to enhance the management strategies and improve outcomes for patients with LVNC.

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