CLINICAL
CHARACTERISTICS OF INFANTILE HYPERTROPHIC CARDIOMYOPATHY
Yao Weiqing,
Chen Shubao, Wang Rongfa, Sun Kuan, Huang Meirong
Xinhua
Children's Hospital, Shanghai Second Medical University,
Shanghai
200092, China
Purposes: To investigate clinical
characteristics of infantile hypertrophic cardiomyopathy (IHCM), so as to
improve the diagnosis and treatment.
Methods: Retrospectively
analysis the characteristics of clinical presentation, EKG, chest X-ray
(CXR), 2-dimensional echocardiography (2DE), cardiac pathology, and outcome
of 7 cases of IHCM (all meets WHO/ISFC diagnosis criteria) hospitalized
between 1984 and 1996.
Results: With 6 boys and 1 girl
in total. Presented with cough and dyspnea in 6/7 cases, cyanosis in 3/7.
One case with edema.4/7 cases had systolic murmur in 3-4 LCSs, in which 2
with thrill. 5/7 patients were considered as congenital heart defects (CHD)
at initial diagnosis. EKG revealed right ventricular hypertrophy (LVH) in
1/6, right ventricular hypertrophy (RVH) in 4/6. Abnormal Q wave in only 1
case.2DE found that all cases (7/7) had LVH, with symmetrical hypertrophy
in 6/7 cases and asymmetrical hypertrophy in only one case. 2/7 cases had
whole heart enlargement and low contractility. Only one case with left
ventricular turbulent flow in Doppler examination. 5/7 treated with
dopamine or dobutamine, digitalis and diuretics. Only one case improved
after added with propranolol. 2/7 cases died 2 and 18 days respectively
after admission, while others withdrew therapy.
Conclusions: The clinical
characteristics of HCM of infants are much different from that of children
and adults. Apart from heart murmur there may be marked symptoms of heart
failure, even cyanosis. RVH is common while abnormal Q wave is rare in EKG,
usually with heart enlargement in CXR. In time 2DE is helpful in early
diagnosis. It suggests poor outcome if symptoms and heart failure appear in
the first year of life. β-blocker should be given
if there is septal ventricular hypertrophy and outflow obstruction, while
digitalis and diuretics as well as angiotensin converting enzyme inhibitors
used if with ventricular enlargement and poor contractility. The actual
incidence of IHCM may be higher than suggested before, since more asymptom
cases are being reported as a result of widely and frequently using of 2DE
examination.