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Management of Cardiac Amyloidosis With Concomitant Left Ventricular Outflow Tract Obstruction

Vrinda Gupta1, Muhammed A Rahim1, Claire Brookmeyer2

  • 1Department of Internal Medicine, The Johns Hopkins Hospital, Baltimore, Maryland, USA.

JACC. Case Reports
|July 11, 2025
PubMed

Insights

Cardiac amyloidosis (CA) can be misdiagnosed as hypertrophic obstructive cardiomyopathy (HOCM). Early screening for CA in HOCM patients is crucial for timely diagnosis and management.

Area of Science:

  • Cardiology
  • Cardiovascular Medicine
  • Amyloidosis Research

Background:

  • Cardiac amyloidosis (CA) is a severe heart failure cause with poor prognosis.
  • Left ventricular outflow tract (LVOT) obstruction in CA can mimic hypertrophic obstructive cardiomyopathy (HOCM).

Observation:

  • Seven patients (4 AL-CM, 3 ATTR-CM) initially presented with HOCM between 2022-2024.
  • Diagnostic delays for CA ranged from 0 to 34 months.
  • AL-CM patients presented with advanced disease (Mayo stage III).

Findings:

  • Six patients required modification of LVOT obstruction therapies.
  • Three patients underwent septal reduction therapy.
  • Management of CA with LVOT obstruction is complicated by medication intolerance and orthostatic hypotension.

Implications:

  • Consider screening for CA in older adults presenting with HOCM.
  • Improved diagnostic strategies are needed to reduce delays in CA diagnosis.
  • Specialized management approaches are required for CA patients with LVOT obstruction.

Keywords:
cardiac amyloidosiscardiac magnetic resonancehypertrophic obstructive cardiomyopathyleft ventricular outflow tract obstruction

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