146 Diseases of heart muscle

Acute myocarditis This is inflammation of myocardium. Causes: Idiopathic (~50%),

viral (flu, hepatitis, mumps, rubeola, Coxsackie, polio, HIV); bacterial (Clostridia,
diphtheria, TB, meningococcus, mycoplasma, brucellosis, psittacosis); spirochaetes
(leptospirosis, syphilis, Lyme); protozoa (Chagas’, p426); drugs (cyclophosphamide,
Cardiovascular medicine

herceptin, penicillin, chloramphenicol, sulfonamides, methyldopa, spironolactone,

phenytoin, carbamazepine); toxins; vasculitis, p558. Symptoms & signs: Fatigue, dysp-
noea, chest pain, fever, palpitations, tachycardia, soft S1, S4 gallop (p42).Tests: ECG:
ST elevation or depression, T wave inversion, atrial arrhythmias, transient AV block.
In proper clinical setting (and absence of MI) +ve troponin I or T confirms the di-
agnosis. Negative antimyosin scintigraphy also excludes active myocarditis. : Sup-
portive. Treat the underlying cause. Patients may recover or get intractable heart
failure (p130).
Dilated cardiomyopathy A dilated, flabby heart of unknown cause. Associations:
alcohol, BP, haemochromatosis, viral infection, autoimmune, peri- or postpartum,
thyrotoxicosis, congenital (X-linked). Prevalence: 0.2%. Presentation: Fatigue,
dyspnoea, pulmonary oedema, RVF, emboli, AF, VT. Signs: Pulse, BP, JVP, displaced,
diffuse apex, S3 gallop, mitral or tricuspid regurgitation (MR/TR), pleural effusion,
oedema, jaundice, hepatomegaly, ascites. Tests: Blood: plasma BNP is sensitive and
specific in diagnosing heart failure. Na+ indicates a poor prognosis. CXR: cardiomeg-
aly, pulmonary oedema. ECG: tachycardia, non-specific T wave changes, poor R wave
progression. Echo: globally dilated hypokinetic heart and low ejection fraction. Look
for MR, TR, LV mural thrombus. : Bed rest, diuretics, digoxin, ACE-i, anticoagulation, bi-
ventricular pacing, ICDs, cardiac transplantation. Mortality: Variable, eg 40% in 2yrs.
Hypertrophic cardiomyopathy HCM ≈ LV outflow tract (LVOT) obstruction from
asymmetric septal hypertrophy. HCM is the leading cause of sudden cardiac death
in the young. Prevalence: 0.2%. Autosomal dominant inheritance, but 50% are spo-
radic. 70% have mutations in genes encoding -myosin, -tropomyosin, and troponin
T. May present at any age. Ask about family history of sudden death. Symptoms &
signs: Sudden death may be the first manifestation of HCM in many patients (VF is
amenable to implantable defibrillators), angina, dyspnoea, palpitation, syncope, CCF.
Jerky pulse; a wave in JVP; double-apex beat; systolic thrill at lower left sternal edge;
harsh ejection systolic murmur. Tests: ECG: LVH; progressive T wave inversion; deep
Q waves (inferior + lateral leads); AF; WPW syndrome (p120); ventricular ectopics; VT.
Echo: asymmetrical septal hypertrophy; small LV cavity with hypercontractile pos-
terior wall; midsystolic closure of aortic valve; systolic anterior movement of mitral
valve. Cardiac catheterization may provoke VT. It helps assess: severity of gradient;
coronary artery disease or mitral regurgitation. Electrophysiological studies may
be needed (eg if WPW, p120). Exercise test ± Holter monitor (p102) to risk stratify. :
-blockers or verapamil for symptoms (the aim is reducing ventricular contractil-
ity). Amiodarone (p124) for arrhythmias (AF, VT). Anticoagulate for paroxysmal AF
or systemic emboli. Dual-chamber pacing (p126) is rarely used. Septal myomectomy
(surgical, or chemical, with alcohol, to LV outflow tract gradient) is reserved for
those with severe symptoms. Consider implantable defibrillator. Mortality: 5.9%/
yr if <14yrs; 2.5%/yr if >14yrs. Poor prognostic factors: age <14yrs or syncope at
presentation; family history of HCM/sudden death.
Restrictive cardiomyopathy Causes: Idiopathic; amyloidosis; haemochromatosis;
sarcoidosis; scleroderma; Löffler’s eosinophilic endocarditis; endomyocardial fibrosis.
Presentation is like constrictive pericarditis (p148). Features of RVF predominate:
JVP, with prominent x and y descents; hepatomegaly; oedema; ascites.
Diagnosis: Cardiac catheterization. : Treat the cause.
Cardiac myxoma (fig 1) Rare benign cardiac tumour. Prevalence ≤5/10,000, :≈2:1.
Usually sporadic, but may be familial (Carney complex: cardiac and cutaneous myxo-
mas, skin pigmentation, endocrinopathy, etc, p215). It may mimic infective endocar-
ditis (fever, weight loss, clubbing, ESR), or mitral stenosis (left atrial obstruction,
systemic emboli, AF). A ‘tumour plop’ may be heard, and signs may vary according to
posture. Tests: Echo. : Excision.
 147

Cardiovascular medicine
Fig 1a. Echocardiogram of a 35yr-old patient who presented with se-
vere exertional dyspnoea and several episodes of syncope. Look at the
large mass (cardiac myxoma) in left atrium. Abbreviations: RV: right
ventricle; LV: left ventricle; AV: aortic valve; AO: aorta; MV: mitral valve.

Fig 1b. Echocardiogram of the same patient during diastole. Notice

how the large mass of myxoma protrudes into the left ventricle during
diastole, and obstructs the mitral valve almost completely. Abbrevia-
tions: RV: right ventricle; LV: left ventricle; AO: aorta.
Figures reproduced with permission from Hamid Reza Taghipour.