Defining the line between significantly excessive trabeculation versus apical hypertrophy. (QF_SA_118)

Saturday, February 1, 2025

8:30 AM – 8:40 AM East Coast USA Time

Location: Blue Room PreFunction

Presenting Author(s)

SM

Srijana Maharjan, MD

Resident Physician
Allegheny General Hospital

Primary Author(s)

SM

Srijana Maharjan, MD

Resident Physician
Allegheny General Hospital

Co-Author(s)

MM

Mariah Mascara, MD

Resident Physician
Allegheny General Hospital
SK

Sanchaya Khetrapal, MD

Resident Physician
Allegheny General Hospital
VF

Victor Farah, MD, FSCMR

Associate Program Director
Allegheny General Hospital

Description of Clinical Presentation: We present a case of a 84 y/o lady with PMH of atrial fibrillation, prominent pulmonary hypertension and diastolic congestive heart failure with NYHA class III presented for cardiac evaluation of her nonischemic cardiomyopathy. She initially presented with cardiogenic shock in 2015 requiring IV inotropes. She had a history of prominent apical hypertrophy since 2017 with left apical thrombus as well.

Diagnostic Techniques and Their Most Important Findings: Echocardiography is the initial diagnostic modality. Apical hypertrophic cardiomyopathy requires a minimum of LV thickness of 15 mm or 13-14 mm with positive family history or positive genetic testing. Left ventricular noncompaction or hyper-trabeculation relies on imaging criteria of that focus on the ratio of the trabecular layer versus non trabeculated layer.
Transthoracic echo showed obscured LV apex with no clear cavity, so imaging enhancing agent was utilized to evaluate the left ventricular apex and it revealed a large filling defect that represents a significantly thickened left ventricular apex with few islands of contrast close to the LV cavity and no real apical cavity or apical slit that is typically seen in apical hypertrophic cardiomyopathy. There were no spade-shaped contrast delineation of the cavity.
A cardiac MRI was performed and showed an LVEF of 49% with significant apical hypertrophy and no significant apical cavity. Late gadolinium enhancement LGE images showed patchy enhancement in the thick apical hypertrophied myocardium. Likely the diagnosis is apical hypertrophic cardiomyopathy rather than hyper trabeculated left ventricle.

Learning Points from this Case: LVNC does share morphologic similarity to HCM with trabeculations and myocardia crypts whereas HCM can be confused with LVNC due to increased myocardial thickness. Our patient exhibits overlapping phenotypic features of HCM and extensive LV apical noncompaction. Cardiac MRI was useful in discerning the likely exact etiology.