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Apical Hypertrophic Cardiomyopathy: Diagnosis, Natural History, and Management

Pavlos Rouskas1, Sotiris Katranas1, Thomas Zegkos1

  • 1From the First Cardiology Department, AHEPA University Hospital, Thessaloniki, Greece.

Cardiology in Review
|July 3, 2023
PubMed

Insights

Apical hypertrophic cardiomyopathy (ApHCM) is a rare heart condition. Diagnosis uses echocardiography and cardiac MRI, with prognosis evolving from benign to similar to general hypertrophic cardiomyopathy (HCM).

Area of Science:

  • Cardiology
  • Cardiovascular Imaging

Background:

  • Apical hypertrophic cardiomyopathy (ApHCM) is a rare variant of hypertrophic cardiomyopathy (HCM).
  • Its prevalence varies geographically.
  • Distinct phenotypic characteristics differentiate ApHCM from other HCM forms.

Purpose of the Study:

  • To review diagnostic methods for ApHCM.
  • To compare ApHCM with other HCM types regarding natural history and prognosis.
  • To summarize management strategies for ApHCM.

Main Methods:

  • Echocardiography is the primary diagnostic tool.
  • Cardiac magnetic resonance (CMR) serves as the gold standard for challenging cases.
  • Review of existing literature on ApHCM diagnosis, prognosis, and management.

Main Results:

  • Echocardiography is the leading imaging modality for ApHCM diagnosis.
  • CMR is crucial for poor acoustic windows, equivocal findings, and suspected apical aneurysms.
  • Recent studies suggest ApHCM may have a similar adverse event incidence as general HCM, challenging its previously reported benign prognosis.

Conclusions:

  • ApHCM diagnosis relies on echocardiography and CMR.
  • The natural history and prognosis of ApHCM require further investigation.
  • Management strategies should be tailored, considering evolving prognostic data.

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