CARDIOVASCULAR JOURNAL OF AFRICA • Volume 35, No 3, September – October 2024 186 AFRICA hypertension. He had also developed bilateral carpal tunnel syndrome four years earlier, for which he had carpal tunnel release surgery. Three years prior to the index presentation, he was diagnosed with HFpEF after presenting with suggestive symptoms. At the time, his echocardiogram revealed severe concentric LVH and a diagnosis of hypertensive heart disease was made. Diuretic therapy, an angiotensin-converting enzyme inhibitor (ACEI) and a beta-blocker were initiated. He developed mild renal impairment, which had been non-progressive over a period of three years. The index admission was due to worsening symptoms of heart failure. On physical examination, the patient was acutely ill with an irregularly irregular pulse, features of systemic and pulmonary congestion as well as bilateral pleural effusion consistent with acute decompensated heart failure. His ECG showed atrial fibrillation, a pseudo-infarct pattern in the inferior leads and low-voltage QRS complexes in all leads. An echocardiogram done at our facility showed a speckled appearance of the myocardium with diffuse thickening of all walls of the heart (more prominent in the ventricles), non-dilated ventricles and bi-atrial dilatation (Fig. 1). A mild pericardial effusion and bilateral pleural effusion were observed (Fig. 2). There was global hypokinesia with relative sparing of the apex and severe left ventricular (LV) systolic dysfunction (ejection fraction 21%). Echocardiographic speckle-tracking strain analysis showed markedly reduced global longitudinal strain (–1.9%) with relative sparing of the apex (Fig. 3). A chest X-ray showed bilateral pleural effusion. Cardiac magnetic resonance imaging showed an increased LV mass (111 g/m2) and a diffuse pattern of late gadolinium enhancement with a failure to null in look-locker sequences, typical of cardiac amyloidosis (Fig. 4). Multimodality cardiac imaging findings were strongly suggestive of cardiac amyloidosis. Blood work-up (Table 1) revealed mild normochromic normocytic anaemia, a cholestatic pattern of hepatic injury and mild renal impairment, which was unchanged from baseline. His prostate-specific antigen was normal with mild prostamegaly on ultrasonography. Based on the patient’s clinical presentation, investigation results andmultimodality cardiac imaging findings, a presumptive diagnosis of decompensated heart failure secondary to cardiac amyloidosis with atrial fibrillation was made. ATTRwt-CM was considered the most likely cause on account of his age, insidious clinical course and history of carpal tunnel syndrome. His heart failure medications were adjusted. The betablocker and ACEI were withdrawn on account of episodes of hypotension and the possibility of autonomic dysfunction, which can occur with ATTRwt-CM. He was maintained on low doses of a diuretic (furosemide 40 mg daily). Anticoagulation was initiated (rivaroxaban 20 mg daily) on discussion with caregivers based on a CHA2DS2VASc score of 4 and a HASBLED score of 4 (hypertension was well controlled and mild renal impairment was non-progressive). Our patient presented with end-stage disease evidenced by multisystem involvement and hence the prognosis was guarded. His relatives were therefore counselled appropriately. Unfortunately, the patient suffered cardiac arrest in hospital and did not respond to resuscitative efforts. Fig. 1. Apical four-chamber view showing speckled appearance of the myocardium, severe concentric LVH (white stars) with thickening of the right ventricular and atrial walls. Fig. 2. Parasternal long-axis view showing the speckled appearance of the myocardium, mild pericardial effusion (white star) and a large pleural effusion (white cross). Fig. 3. Echocardiographic speckle-tracking strain analysis showed markedly reduced global longitudinal strain (–1.9%) with relative sparing of the apex.
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