A systematic search of the medical literature was performed on
April 1, 2008. The search, limited to human subjects and English
language journals, included the National Guideline Clearinghouse, the
Cochrane database, PubMed, UpToDate®, and PIER.
The current American College of Cardiology/European Society
of Cardiology Clinical Expert Consensus Document for Hypertrophic
Cardiomyopathy can be found at www.acc.org
Hypertrophic cardiomyopathy (HCM) is a complex cardiac disease
that can manifest in multiple ways and can occur in patients of
all ages.1 The prevalence of HCM is much higher than what
was previously thought and it occurs in one
out of 500 people. It is now recognized that a gene mutation
is responsible for HCM and that it is the most common genetic cardiovascular
disease. Many people with HCM are asymptomatic and their lives are
not affected by HCM. Others may experience severe limitations in
their daily lives and some will have sudden cardiac death (SCD)
at an early age. Therefore, it is important for primary care providers
to recognize this common cardiac condition and to provide proper
Symptoms of HCM can manifest at any phase of a person’s
life; many of these people have relatives with known HCM. Symptoms
do not always correlate to the severity of the left ventricular (LV)
outflow tract obstruction. Dyspnea on exertion occurs in almost
90% of the symptomatic patients, usually in the presence
of preserved systolic function.2 This is due to the increased
stiffness of the LV wall leading to elevated LV diastolic and left
atrial pressures. Other symptoms range from fatigue to near-syncope
or syncope. Typical or atypical chest pain can occur in patients
with a normal coronary arteriogram. This may reflect microvascular
angina. Unfortunately, the first manifestation of HCM can be sudden
death occurring in children or young adults after strenuous physical
Atrial fibrillation (AF) occurs late in the disease process and
is generally poorly tolerated. Patients with a stiff, noncompliant
left ventricle need the atrial contribution for adequate ventricular
filling during diastole. AF in patients with HCM increases the risk
for systemic embolization.
In asymptomatic patients with HCM, the physical examination may
be normal or may provide nonspecific findings such as a fourth heart
sound (some specific recommendations are mentioned in Table 5-1).
However, as the outflow obstruction increases, several characteristic
abnormalities can be elicited (Fig. 5-1). High-grade obstruction
creates a harsh crescendo–decrescendo systolic murmur at
the lower left sternal border and apex. The murmur may radiate to
the axilla and base, but usually not to the neck. It begins just
after S1 and usually represents aortic outflow obstruction often
with mitral regurgitation. A prominent S4 is usually present unless
the patient is in AF.
Table 5-1. Specific Recommendations3
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