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Trajectory of left ventricular geometry and diastolic dysfunction in hereditary transthyretin cardiac amyloidosis

Tatsuya Akatsuka1, Naoki Fujimoto1, Masaki Ishiyama1

  • 1Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan.

ESC Heart Failure
|June 19, 2021
PubMed

Insights

Amyloid transthyretin (ATTR) cardiomyopathy causes heart changes over time. Early diagnosis is crucial for managing left ventricular (LV) stiffness and diastolic dysfunction in ATTR patients.

Area of Science:

  • Cardiology
  • Genetics
  • Neurology

Background:

  • Amyloid transthyretin (ATTR) deposition leads to left ventricular (LV) hypertrophy, diastolic dysfunction, and heart failure.
  • The progression of LV geometric and diastolic dysfunction changes in ATTR cardiomyopathy is not well-documented.

Observation:

  • A 79-year-old woman with a history of myocardial infarction presented with exertional dyspnea, progressive lower extremity weakness, and polyneuropathy.
  • Cardiac biopsy and genetic testing confirmed Val30Met hereditary ATTR cardiomyopathy.

Findings:

  • Over five years, the patient exhibited significant LV concentric remodeling and a reduced LV cavity size.
  • These changes resulted in increased LV stiffness and prolonged LV relaxation, indicative of worsening diastolic dysfunction.

Implications:

  • This case highlights the progressive nature of cardiac changes in ATTR cardiomyopathy.
  • Emphasizes the importance of early diagnosis and monitoring of LV geometry and diastolic function in patients with ATTR.
  • Understanding the time course of these changes can inform treatment strategies and improve patient outcomes.

Keywords:
AmyloidCardiac transthyretin amyloidosisLeft ventricular functionPressure-volume relationships

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