1. Background Methods Results Conclusion
The High prevalence of Primary Amyloidosis among Afro Caribbean Heart Disease
SK. Randhawa1, G. Dwivedi2, D. Neal3, RP. Steeds4, RJ. Macfadyen5 - (1) Sandwell and West Birmingham Hospitals NHS Trust, Department of Cardiology , Birmingham, United Kingdom (2) University of Ottawa Heart Institute, Ottawa, Canada (3) University of Birmingham, Birmingham, United Kingdom (4) Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom (5) University of Melbourne, Melbourne, Australia
Background
Methods
Results
LV internal diameters (d and s) were lower in A than C. Renal function was poorer in A cases (Urea A,13.5, mM; C 8.5, mM p=0.02; Creat A,188, ; C,119, µM; p=0.004).
Notably in our series neither atrial volume nor inter atrial septal wall thickness were recorded routinely despite their known sensitivity and specificity in the TT echo diagnosis of A.
Primary amyloid (A) is a rare infiltrative disease in late adult life affecting the heart and other tissues diagnosed by tissue analysis of the affected organ.
We reviewed our echocardiographic database of hypertensive Afro Caribbean (AC) patients seen with heart failure symptoms to define prevalence of this disease using accepted biopsy and/or MRI criteria.
Imaging
Echocardiogram
Cardiac MRI
Figure 2: Pie chart demonstrating demographics
From ethnicity specific heart failure service records (from n=106 total subjects) we identified 86 AC subjects with a complete trans-thoracic (TT) echo dataset.
A was defined by typical TT echo criteria and confirmed by positive RV septal tissue histology at biopsy and or typical features on late gadolinium MRI (reported independently).
Cases positive for A (28) were compared to negative (58) controls (C) by multivariate regression (MVr).
We found a high prevalence (33%) of A (n=29;18male;68±17yr;Y±Ykg) compared to C (n=58;39male;71±16yr;Y±Ykg) in this sample.
Conclusion
Both demographic criteria (age;gender;body mass;blood pressure) and a range of standard TT echo measures of morphology (LVDDd LVDDs) or function (ePAP; Simpsons LVEF) were similar on MVr.
Only septal (IVSs;A,1.86±0.77;C,1.45±0.39; IVSd A,1.7 ( cm);C,1.1 ( cm)p=0.033) and PW thickness (LVPWs A,1.89±0.69;C,1.44±0.61;LVPWd A 1.63±0.38;C,1.23±0.55 p=0.002 ) were significantly higher in A.
We have recorded an unusually high retrospective prevalence of A in elderly AC heart disease patients with HF symptoms.
Given the development of specific treatments for this condition all clinicians seeing AC patients with abnormal TT echo should consider biopsy and/or MRI in suspicious cases.
Figure 1: Cardiac MRI of a 47 yr old man with cardiac amyloidosis.
FISP (fast imaging with steady-state precession) sequences in four-chamber view.
Myocardial hypertrophy of the left ventricle (septal thickness, 15 mm) and the interatrial septum (thickness, 5 mm) are visible.
Declaration of Interest
No conflicts of interest declared
Tissue biopsy
Typically stains light pink with hematoxylin and eosin and turquoise green with sulphated alcian blue.
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