T waves diffuse large and
inverted if cardiac apex
abnormally thick
Large “dagger like” septal
Q-waves (Lome, 2016)
TREATMENTS
Dependent on symptoms
Arrhythmias (Argulian, et al., 2016)
Anticoagulation
Rhythm Control
Cadioversion
Antirhythmic agents
Left Ventricular Outflow Obstruction
Beta blockers: reduced inotropy
and longer ventricular filling times
reduce obstructive symptoms
(Argulian, et al., 2016).
Disopyramide can be used in
combination with beta blockers
and should be considered before
any invasive therapies (Argulian,
et al., 2016).
Surgery and catheter-based
treatment of outflow obstruction,
associated with low complications
and successful relief of obstruction
(Argulian, et al., 2016).
Surgical Myectomy for young patients with low rsk
Catheter-based care for older patient with higher risks
and comorbidities
Non-obstructive (Argulian, et al., 2016)
Beta Blockers
Calcium Channel Blockers
PATHOPHYSIOLOGY
First signs recognized in the pathophysiology of
hypertrophic cardiomyopathy are pathogenic
mutations in cardiac sarcomere
Mitral valve apparatus abnormalities
Diagnosis confirmed via genetic testing
Abnormal loading conditions present causing left
ventricle to require more effort. (HTN, aortic
stenosis)
Left ventricle hypertrophy can lead to obstruction
of the left ventricle outflow which can result in atrial
arrhythmias, embolic phenomena, and HF (Argulian,
Sherrid, & Messerli, 2016
May lead to sudden cardiac death in young
healthy individuals (Jacoby, Depasquale, &
McKenna, 2013).
NURSING IMPLICATIONS
Must be able to interpret ECG reading (Palmer, 2013).
Advocate for other tests if HCM is suspected
Family
History
ECG
Multiple sudden deaths in the family
Review postmortem reports if possible
Advocate for family screening
Assessing Heart Sounds
Harsh systolic crescendo >> decrescendo murmur at the left sternal border
Diminished with squatting and increases when standing
CAUSES
Common inherited type of cardiomyopathy. (Jacoby, Depasquale, & McKenna, 2013)
Described as thickening of left ventricle with no apparent cause. (Argulian, Sherrid, & Messerli,
2016)
General experience is that late development or progression of hypertrophy is
uncommon, with most cases of hypertrophy developing during adolescence and early
adulthood (Argulian, et al., 2016).
Disease is attributed to many sarcomere gene mutations (Argulian, et al., 2016)
"Autosomal dominant disease is predominant, with most sporadic and alternate inheritance patterns
(X-linked, mitochondrial) representing phenocopies. Disease penetrance is incomplete and
expression is variable, making the familial nature of this disease occasionally challenging to
appreciate" Argulian, et al., 2016).
LIFESTYLE CHANGES
Decreased physical activity
Children are forced to quit sports or greatly decrease participation
May hinder future career plans
Social Changes
Some children have fears of informing their peers of their
condition, because they might be treated differently
Children may be separated from social groups
Restricting alcohol
consumption (Bratt,
et al., 2012)