Key Information
Amyloidosis is characterised by the deposition of misfolded proteins in extracellular space. ATTR cardiomyopathy is known to be common in males over 60 years of age [1external link, opens in a new tab]. Wildtype ATTR (ATTRwt) and some genetic variants have been observed to be more frequent in persons of African heritage [2external link, opens in a new tab]. The curious question therefore arises of what is the true prevalence of this entity in our region? A review of the common causes of death in Ghana, the most recent figures being for the year 2015, does not include a record of amyloidosis [3external link, opens in a new tab]. However, we hypothesise that some of the cases recorded as “cardiac failure” (1.2%), and “all others” (57.5%), could be reclassified as systemic or organ-specific amyloidosis, taking into consideration the low level of awareness, limited diagnostic and therapeutic capabilities of our health system. Ahadzi et al. identified a probable case of cardiac amyloidosis using speckle-tracking strain analysis and subsequently using cardiac magnetic resonance imaging [4external link, opens in a new tab].
Diagnosis of amyloidosis is often delayed: 2 years or longer after the first symptoms in AL-CM, and more than 4 years in ATTR-CM. “Red flags” include nephrotic syndrome and heart failure with preserved ejection fraction (masquerader). Nine patients (mean age 68 years), all male, were seen at the Ho Teaching Hospital, between August 2023 and January 2024, and had been complaining of exertional dyspnoea (of different grades) and pedal edoema lasting for years. Physical examination was remarkable, in some cases, for raised jugular venous pressure and systemic congestion [5external link, opens in a new tab].
The 12-lead electrocardiography done on these patients was significant for varying degrees of atrioventricular block, QRS voltages discordant with left axis deviation, and the degree of “hypertrophy” on imaging. One patient had atrial fibrillation [6external link, opens in a new tab].
On transthoracic echocardiography, unexplained left ventricular (LV) thickness (≥ 12 mm) in all 9 patients (mean left ventricular mass index 139 gm-2, mean relative wall thickness 0.8), preserved LV ejection fraction (average of 61% by the Simpson method) and different grades of diastolic dysfunction were documented. Due to the unavailability of speckle-tracking strain imaging on our equipment, measurements based on this technology were not recorded [5external link, opens in a new tab, 7external link, opens in a new tab] ().