|
Sudden Cardiac Death -
- Sudden cardiac death has the highest incidence in
adolescent children but not excluding younger adults, and is typically associated with sports or
vigorous exertion.
- The arrhythmia that causes sudden death is usually but
not exclusive alone to, ventricular fibrillation.
- A pathophysiology and epidemiology report have shown in
more than 80% of patients with HCM and with known congenital heart disease brings a higher risk for
sudden cardiac death. 19
- For many patients with HCM develop over time,
ventricular fibrillation (VFib) following atrial fibrillation (AFib), also atrial flutter (AF), and
supraventricular tachycardia (SVT’s) associated with Wolff-Parkinson-White (WPW) syndrome. Commonly
found in documented findings, there ventricular tachycardia (VT) with low-cardiac-output, associated
with hemodynamic collapse. 20
- Early diagnosis is of prime importance, if death is to
be prevented by prescription of an appropriate levels of safe activity, medications, surgery (e.g.,
electrophysiology invasive procedures), and/or an implantable cardioverter defibrillator may be in
order.
- Remember, that because this is an autosomal (i.e.,
genetic disorder) dominantly inherited disease, an investigation with screening of first-degree
relatives, which should include a physical examination, and an electrocardiography (ECG) screening,
with a transthoracic 2D- echocardiography, with color flow mapping, which will be useful to identify
additional family members with possible cardiomyopathy (CM) before the onset of significant symptoms
or sudden death occur.
Physical Examination Findings
–
Most patients with HCM or
cardiomyopathy (CM), excluding the pediatric patients with known congenital heart disease, do not have
outflow tract obstruction and may show completely normal during the physical examination findings.
Therefore, it is important that the nurse investigates further, using her skills in evidence-based practice
towards listening to heart sounds, cardiac impulse, murmur, and other findings:
- The first heart sound (S₁) is influenced by the position
of the mitral leaflets at the onset of ventricular systole. The amount of tissue, air, or fluid
between the heart and the stethoscope may be louder if diastole is shortened because of
tachycardia. Also, in a prolonged P-R interval, or with an imperfect closure due to reduced
valve substance in the mitral regurgitation S₁ sound may appear soft.
- The second heart sound will appear to sound
“split”. Splitting in S₂ that persists with expiration from the pulmonic area or left sternal
border may be due to many causes, including patients with chronic pulmonary hypertension. The
oxygen partial pressure (P₀₂) may appear loud and also, patients with HCM/CM will split
paradoxically.
- The third heard sound (S₃) is a low-pitched sound
produced in the ventricle after the closing sound of the aortic valve (A₂), at the termination of
rapid filling. In younger patients with CM a sound or “gallop” may appear but does not have the
same ominous significance as in patients with valvular aortic stenosis (VAS) or in older
adults. 21 Place the stethoscope at the
left ventricular apex during expiration and with the patient in the left lateral
position.
- The fourth heart sound (S₄) is frequently heard in
patients with HCM, and is due to atrial systole against a highly non-compliant LV wall. It is
usually loudest at the left ventricular apex, when the patient is in the left lateral position.
The right-sided (S₄) sound is present in patients with right ventricular hypertrophy, secondary to
either pulmonic stenosis or pulmonary hypertension. 22
Cardiac Impulse –
- The apical precordial impulse is frequently laterally
displaced and is usually abnormally forceful and enlarged in patients with
HCM.
- Double apical impulse resulting from a forceful left
atrial contraction against a highly non-compliant LV and occurs in young children plus, a variance
within younger adults with HCM. 23
- A triple apical impulse are rarely but does accrue from
a belated systolic bulge (e.g., sudden increase sound) that appears when the heart is almost empty
and is performing near-isometric contraction. 24ʹ25
Murmurs -
- Systolic ejection murmurs are often
“crescendo-decrescendo” in shape when blood is
ejected across the aortic or pulmonic outflow tracts. 26 Place your stethoscope
between the apex and the left sternal border; it radiates to the suprasternal notch but not to the
carotid arteries or neck. The murmur may vary with the subaortic gradient across the LV outflow
tract.
- The systolic or holosystolic (pansystolic) murmurs are
generated when there is flow between two chambers that have widely different pressures throughout
systole. The holosystolic murmur of the mitral regurgitation is heard at the apex and left
axilla in patients with systolic anterior motion of the mitral valve and significant LV outflow
tract. 27
|