CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 10, November 2012
560
AFRICA
obstruction with aortic and pulmonary regurgitation. Sometimes
the aneurysms are annular, extending in a circular direction
around the heart and may extend laterally, superiorly and
anteriorly. Left atrial aneurysms may be found in some patients.
Atrio-ventricular conduction abnormalities, including
complete heart block are common. The cause of the aneurysms is
unknown, although the pathological findings and the presence of
fibrinous pericarditis support the theory that the out-pouchings
are initiated by myocarditis.
Proposed new classification
Earlier, we posed this question ‘which of the several classifications
of myocardial disorders is suitable for Africa?’ The problem
is that there is no agreement at present on the definition of
cardiomyopathy. While some regard it as ‘any disorder of the
myocardium’, others believe that it should be defined as heart
muscle disease of unknown cause. Yet others believe that it
should be defined as ‘any disease of the myocardium with
cardiac dysfunction’. This last definition implies that patients
with hypertensive heart disease/failure will be described as
hypertensive cardiomyopathy and those with aortic valve disease
for example, aortic valve cardiomyopathy.
While waiting for another consensus meeting to resolve
this difficulty, we wish to propose the following classification
(
Table 2). It is our view that this could stimulate the continental
society (PASCAR) to set up an expert committee to look into
this subject.
TABLE 2. PROPOSED CLASSIFICATION OF MYOCARDIAL DISORDERS FORAFRICA
1.
DISEASES THATARISEWITHIN THE CARDIOVASCULAR
SYSTEM
2.
DISEASES THATARISE OUTSIDE THE CARDIOVASCULAR
SYSTEM
GENETIC
A. DISORDERS OF THE MYOCYTES
(
i) Non-dilated types:
•
Hypertrophic cardiomyopathy
•
LV non-compaction
(
ii) Dilated types:
•
Arrhythmogenic right ventricular cardiomyopathy
•
Familial dilated cardiomyopathy
B. DISORDERS OFTHE ELECTRICAL STRUCTURES
(
i) Conduction system disease
Structural types: Lenegre disease
Non-structural types: ion channelopathies
•
Long-QT syndrome
•
Brugada syndrome
•
Catecholaminergic polymorphic ventricular tachycardia
•
Short-QT syndrome
•
Idiopathic ventricular fibrillation
NON-GENETIC
(
i) Hypertrophic
•
Hypertensive heart disease (concentric, asymmetric)
•
Chronic rheumatic heart diseases (obstructive forms such as aortic/
pulmonary stenosis
•
Chronic lung disease, pulmonary embolism, primary pulmonary
hypertension. These affect only the right ventricle and can dilate in
untreated cases
•
Congenital heart diseases – obstructive types
(
ii) Dilated
•
Hypertensive heart disease/failure (of different grades and severity)
•
Alcohol heart disease
•
Myocarditis e.g. viral, bacterial, protozoal, rickettsial
•
Chronic rheumatic heart diseases (regurgitant forms such as mitral/
aortic/tricuspid/pulmonary regurgitation
•
Ischaemic cardiomyopathy
(
iii) Associated with pregnancy
•
Peripartum heart disease
(
iv) Fibrotic/obliterative
•
Löeffler’s endomyocardial disease
•
Endomyocardial fibrosis
(
v) Unknown cause
•
Dilated cardiomyopathy
•
Left ventricular non-ischaemic ventricular aneurysms
INFILTRATIVE
•
Amyloidosis (primary, familial autosomal dominant, senile,
secondary forms)
•
Gaucher disease
•
Hurler’s disease
•
Hunter’s disease
STORAGE
•
Haemochromatosis
•
Fabry’s disease
•
Glycogen storage disease (type II, Pompe)
•
Niemann-Pick disease
TOXICITY
•
Drugs
•
Heavy metals
•
Chemical agents
GRANULOMA
•
Sarcoidosis
ENDOCRINE
•
Diabetes mellitus
•
Hyperthyroidism
•
Hypothyroidism
•
Hyperparathyroidism
•
Phaeochromocytoma
•
Acromegaly
CARDIOFACIAL
•
Noonan syndrome
•
Lentiginosis
NEUROMUSCULAR/NEUROLOGICAL
•
Friedreich’s ataxia
•
Duchenne-Becker muscular dystrophy
•
Emery-Dreifuss muscular dystrophy
•
Myotonic dystrophy
•
Neurofibromatosis
•
Tuberous sclerosis
NUTRITIONAL DEFICIENCIES
•
Beri beri (thiamine)
•
Pellagra
•
Scurvy
•
Selenium
•
Carnitine
•
Kwashiorkor
AUTOIMMUNE/COLLAGEN
•
Systemic lupus erythematosus
•
Dermatomyositis
•
Rheumatoid arthritis
•
Scleroderma
•
Polyarteritis nodosa
ELECTROLYTE IMBALANCE
CONSEQUENCE OF CANCER THERAPY
•
Anthracyclines: doxorubicin (adriamycin), daunorubicin
•
Cyclophosphamide
•
Radiation