Hypertrophic Cardiomyopathy (HCM) in a Middle-Aged Female with Heart Failure

 
55 years old female presented to us with SOB at rest, chest pain on minimal exertion sheis
known case of HTN  was diagnosed with heart failure with reduced ejection fraction for 1
year ago and recurrent hospital admission  And on this admission she was admitted as a
case of pulmonary oedema
On examination
Patient was middle age lying flat
Mildly dyspnic
Pulse rate 70 bpm
Blood pressure 150/90 mmhg
Cardiac examination heaving apex beat
Ejection systolic murmer at LLSB
Positive  family history of cardiac disease
ECG voltage LVH and non specific ST T changes
 
undefined
 
Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease most often
caused by mutations in one of several sarcomere genes that encode components of the contractile
apparatus.
 
HCM is characterized by left ventricular hypertrophy (LVH) of various
morphologies, with a wide array of clinical manifestations and
hemodynamic abnormalities Depending in part upon the site and extent
of cardiac hypertrophy, patients with HCM can develop one or more of
the following abnormalities:
LV outflow obstruction
Diastolic dysfunction
Myocardial ischemia
Mitral regurgitation
 
In broad terms, the symptoms related to HCM can be categorized as those related to heart failure (HF),
chest pain, or arrhythmias. For the majority of patients with HCM,
 
 
 
Pathophysiological changes in HOCM:
Increase intraventricular pressure
Prolong ventricular relaxation
Increase myocardial wall stress
Increase oxygen demand
Decrease LVOT
 
Clinical presentation:
Dyspnoea (90%), orthopnoea and PND
Palpitation
CHF
Angina (70-80%)
Syncope and presyncpe
Sudden cardiac can be the first clinical manifestation
 
Physical examination:
Carotid pulse (bifid )
JVP (prominent a wave )
Apical beat ( double or triple)
Heart sound:
S1 normal
S2 usually split (reverse )
S3 indicate late stage
S4 usually due hypertrophy
Murmer:
Medium pitch crescendo decrescendo LLSB and apex radiate to suprasternal notch.
SAM with MR
 
Dynamic maneuverer:
Murmur intensity increase with decrease preload (Valsalva, standing )
Murmur intensity decrease with increase preload (squatting , hand grip)
 
Diagnosis:
ECG
Echocardiography
Cathetarization
Cardiac imaging
 
ECG features of HCM
Left ventricular hypertrophy
 with increased precordial voltages and non-specific ST segment and T-
wave abnormalities
Deep, narrow (“dagger-like”) Q waves in lateral 
(I, aVL, V5-6) +/- 
inferior 
(II, III, aVF) leads
Other 
associated features
 may include:
Left atrial enlargement(“P mitrale”) — left ventricular diastolic dysfunction may lead to
compensatory left atrial hyertrophy
Signs of WPW (short PR, delta wave) — ECG features of Wolff-Parkinson-White (WPW)
were seen in 33% of patients with HCM in one study, and at least one genetic mutation has
been identified that is associated with both conditions
Dysrhythmias: atrial fibrillation and supraventricular tachycardias are common; PACs,
PVCs, VT
Giant precordial T-wave inversions in apical HCM
 
ECHO FEATURES:
 
Abnormal systolic motion of anterior leaflet of the mitral valve
LVH
Left atrial enlargement
Diastolic dysfunction
Small ventricular chamber size
Septal wall hypertropthy
Partial closure of the aortic valve in midsystole
 
Endocarditis
only 3-4% develop
endocarditis
Usually mitral valve
effected
 
Heart failure
Only 15% develop
NYHA3 systolic failure
Only 3% develop end
stage systolic heart
failure
 
Autonomic dysfunction
develop in 25% of
patients
Poor prognosis
 
Atrial fibrillation
Develop in 30% in
elder patient
 
Natural history
 
Family screen
Holter monitor
Exercise test
 
Risk factor
Risk factor
undefined
 
THANK YOU
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A 55-year-old female with a history of hypertension and heart failure presented with symptoms of dyspnea, chest pain, and a positive family history of cardiac disease. Exam findings included LVH on ECG and a systolic murmur at LLSB. This case highlights the importance of recognizing and managing hypertrophic cardiomyopathy (HCM) in patients with heart failure, describing its pathophysiological changes, clinical presentations, and physical examination findings.

  • Hypertrophic Cardiomyopathy
  • Heart Failure
  • LVH
  • Dyspnea
  • Chest Pain

Uploaded on Sep 18, 2024 | 0 Views


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  1. 55 years old female presented to us with SOB at rest, chest pain on minimal exertion sheis known case of HTN was diagnosed with heart failure with reduced ejection fraction for 1 year ago and recurrent hospital admission And on this admission she was admitted as a case of pulmonary oedema On examination Patient was middle age lying flat Mildly dyspnic Pulse rate 70 bpm Blood pressure 150/90 mmhg Cardiac examination heaving apex beat Ejection systolic murmer at LLSB Positive family history of cardiac disease ECG voltage LVH and non specific ST T changes

  2. Hypertrophic cardiomyopathies Dr. Mohammed adel F.I.B.M.C M.B.Ch.C University of Basra- Al zahraa teaching hospital

  3. Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease most often caused by mutations in one of several sarcomere genes that encode components of the contractile apparatus. HCM is characterized by left ventricular hypertrophy (LVH) of various morphologies, with a wide array of clinical manifestations and hemodynamic abnormalities Depending in part upon the site and extent of cardiac hypertrophy, patients with HCM can develop one or more of the following abnormalities: LV outflow obstruction Diastolic dysfunction Myocardial ischemia Mitral regurgitation In broad terms, the symptoms related to HCM can be categorized as those related to heart failure (HF), chest pain, or arrhythmias. For the majority of patients with HCM,

  4. Pathophysiological changes in HOCM: Increase intraventricular pressure Prolong ventricular relaxation Increase myocardial wall stress Increase oxygen demand Decrease LVOT

  5. Clinical presentation: Dyspnoea (90%), orthopnoea and PND Palpitation CHF Angina (70-80%) Syncope and presyncpe Sudden cardiac can be the first clinical manifestation

  6. Physical examination: Carotid pulse (bifid ) JVP (prominent a wave ) Apical beat ( double or triple) Heart sound: S1 normal S2 usually split (reverse ) S3 indicate late stage S4 usually due hypertrophy Murmer: Medium pitch crescendo decrescendo LLSB and apex radiate to suprasternal notch. SAM with MR

  7. Dynamic maneuverer: Murmur intensity increase with decrease preload (Valsalva, standing ) Murmur intensity decrease with increase preload (squatting , hand grip)

  8. Diagnosis: ECG Echocardiography Cathetarization Cardiac imaging

  9. ECG features of HCM Left ventricular hypertrophy with increased precordial voltages and non-specific ST segment and T- wave abnormalities Deep, narrow ( dagger-like ) Q waves in lateral (I, aVL, V5-6) +/- inferior (II, III, aVF) leads Other associated features may include: Left atrial enlargement( P mitrale ) left ventricular diastolic dysfunction may lead to compensatory left atrial hyertrophy Signs of WPW (short PR, delta wave) ECG features ofWolff-Parkinson-White (WPW) were seen in 33% of patients with HCM in one study, and at least one genetic mutation has been identified that is associated with both conditions Dysrhythmias:atrial fibrillation and supraventricular tachycardias are common; PACs, PVCs, VT Giant precordial T-wave inversions in apical HCM

  10. ECHO FEATURES: Abnormal systolic motion of anterior leaflet of the mitral valve LVH Left atrial enlargement Diastolic dysfunction Small ventricular chamber size Septal wall hypertropthy Partial closure of the aortic valve in midsystole

  11. Natural history Heart failure Only 15% develop NYHA3 systolic failure Only 3% develop end stage systolic heart failure Atrial fibrillation Develop in 30% in elder patient Endocarditis only 3-4% develop endocarditis Usually mitral valve effected Autonomic dysfunction develop in 25% of patients Poor prognosis

  12. EVALUATION: Family screen Holter monitor Exercise test

  13. Risk factor

  14. THANK YOU

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