Skill Checkup: A 68-Year-Old Man With Acute Decompensated Heart Failure, Worsening Dyspnoea and Lower Extremity Oedema

Marco Luigetti, MD, PhD

Disclosures

August 18, 2022

The Skill Checkup series provides a quick, case-style interactive quiz, highlighting key guideline- and evidence-based information to inform clinical practice.

A 68-year-old Black man in Europe was admitted to the hospital for acute decompensated heart failure (HF). He had a history of hypertension, paroxysmal atrial fibrillation and coronary artery disease that required angioplasty. He received an implantable cardioverter-defibrillator owing to recurrent non-sustained ventricular tachycardia. The patient reported worsening dyspnoea, weight gain, bilateral lower extremity oedema and orthopnoea.

Initial vital signs included an oral temperature of 36.9°C (98.4°F), a heart rate of 87 bpm and a blood pressure of 101/75 mm Hg. Respiratory rate was 22 breaths/min with an oxygen saturation of 97%. Physical examination was significant for an S3 gallop, markedly elevated jugular venous pressure and bibasilar crackles. Chest radiograph revealed pulmonary oedema. B-type natriuretic peptide level was 3206 pg/mL. Cardiac troponin T levels were persistently elevated, 400 ng/L. Serum creatinine was 1.29 mg/dL, and blood urea nitrogen was 45 mg/dL. Complete blood cell count was significant for an iron deficiency anaemia. A hepatic function panel revealed hypoalbuminaemia (2.2 g/dL), but no other significant abnormality.

Transthoracic echocardiography revealed grade 3 diastolic dysfunction with biatrial enlargement and severe concentric left ventricular (LV) hypertrophy (17.8 mm thick). Left ventricular systolic function was normal with a calculated ejection fraction of 55%. The 12-lead ECG showed normal QRS voltage.

The differential diagnosis of HF with concentric LV hypertrophy focused on genetic and acquired primary cardiomyopathies, including hereditary transthyretin amyloid cardiomyopathy (hATTR-CM). Genetic considerations included neutral septal hypertrophic cardiomyopathy (CM), mitochondrial myopathies, glycogen storage diseases (eg Danon disease) and Fabry disease.

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