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2014. 09.18
Division of Cardiology
R1 Lee Seong-Kon/Prof. Kim Woo-sik
CASE
20130510 OPD
12343979
유O 환 (M/18)
C.C)
건강검진에서 심잡음
o/s) 2013.5.9
P.I )
상기 18세 남자환자 특이 병력 없는 자로, 운동시 호흡곤란 있었으며,
건강검진에서 심잡음 들려 순환기내과 외래 방문함.
History
PMHx)
HTN/ DM / Tbc / Hepatitis (-/-/-/-)
*Op Hx (-)
PHx)
Alcohol (-), Smoking (-)
FHx.)
Sudden death
Sudden death
Review of system
1. General
Generalized weakness(-) Fatigue(-) Weight loss(-) Fever(-) Chill(-)
2. Skin
Rash(-) Pigmentation(-) Itching(-) Urticaria(-)
3. HEENT
Headache(-) Visual disturbance(-) Otalgia(-) Otorrhea(-) Tinnitus(-)
Nasal obstruction(-) PND(-) Rhinorrhea(-)
Sore throat(-) Swallowing difficulty(-)
4. Respiratory
Cough(-) Sputum(-) Dyspnea(-) Hemoptysis(-) Pleuritic pain(-)
5. Cardiac
Chest pain(-) Palpitation(-) Dyspnea on exertion(+): NYHA II
Orthopnea(-) Paroxysmal nocturnal dyspnea(-)
Review of system
6. GI
A/N/V/D/C (-)/(-)/(-)/(-)/(-) Abdominal pain(-) Bowel habit change(-)
Melena(-) Hematochezia(-)
7. Renal/Urinary
Dysuria(-) Hematuria(-) Incontinence(-) Nocturia(-) Frequenc(-)
Residual urine sense(-) Urgency(-)
8. Musculoskeletal
Pain(-) Tenderness(-) Weakness(-) Cramps(-)
Sprains(-) Fracture(-) Swelling(-) Claudication(-)
9. Nervous
Syncope(-) Seizure(-) Dizziness(-) Tremor(-)
Sensory & Motor disturbance(-)
Physical examination
V/S 130/80 – 80 – 16 – 36.5℃
1. General
Alert consciousness
Not so ill looking appearance
2. Skin
No rash No Pigmentation
3. H & N
Isocoric pupil c PLR(++/++)
Whitish sclera, Pinkish conjunctiva
No LN enlargement, No neck vein engorgement
PI(-), PTH(-/-)
4. Chest
Symmetric chest expansion
Clear breathing sound without rale
Regular heart beat
Ejection systolic murmur Gr. III/VI (Lt. sternal border, apex)
Physical examination
5. Abdomen
Soft & Scaphoid abdomen
Normoactive bowel sound
Td (-), RTd (-)
6. Back/Ext
CVA Td (-/-)
Pretibial pitting edema (-/-)
7. Neurologic
Sensory
Motor
100
100
Ⅴ
Ⅴ
100
100
Ⅴ
Ⅴ
Initial lab finding
1. CBC/DC
wbc 8,300(seg 77.2) Hb 15.8 Hct 46.0
PLT 178K
2. Chemistry
TB/DB -/AST/ALT 25/15
BUN/Cr -/0.8
Ca/p/Mg -/-/-
Prot/Alb 7.3/4.5
ALP/GGT -/Na/K/Cl -/4.4/LD -
3. U/A
RBC 0-1
WBC 0-1
Chest X-ray
EKG
Problem list
#1. Ejection systolic murmur
#2. Dyspnea on exertion
#3. Left ventricular hypertrophy with strain pattern(EKG)
Echocardiography (20130515)
Echocardiography (20130515)
Echocardiography (20130515)
Initial assessment
#1. Hypertrophic cardiomyopathy (obstructive type)
* Additional diagnostic plan?
Symptoms of heart failure - preserved LV function - at any age
 Exertional dyspnea
 Fatigue
 Orthopnea or PND
Chest pain - typical angina pectoris or atypical
- silent myocardial ischemia
Syncope (or near-syncope) and lightheadedness ---- arrhythmias and outflow obstruction.
Symptoms and LV obstruction – No correlation
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
With outflow obstruction
Arterial pulses rapid rise - with bisferiens contour
Double or triple apical impulses may be palpable
 Outward systolic thrust - ventricular contraction
 Presystolic accentuated atrial contraction.
Medium-pitch ESM at the lower left sternal border and apex
Loud murmurs > 3/6 - LV outflow gradients >30 mm Hg
Without subaortic gradients
Subtle - with no or soft systolic murmur
Forceful apical impulse
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
Abnormal - >90% of pts & >75% of asymptomatic relatives
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


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Increased voltages consistent with LV hypertrophy
ST-T changes - T wave inversion in the lateral precordial leads
Left atrial enlargement
Deep and narrow Q waves
Diminished R waves in the lateral precordial leads.
Normal ECG - 5% of pts
 Less severe phenotype and favorable course
 Not predictive of future sudden death
Increased voltages
 Weakly correlated with the magnitude of LV hypertrophy
 Do not distinguish the obstructive and nonobstructive forms
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
 Diffuse hypertrophy of the ventricular septum and
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
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anterolateral free wall (70% to 75%)
Basal septal hypertrophy (10% to 15%)
Concentric hypertrophy (5%)
Apical hypertrophy (<5%)
Hypertrophy of the lateral wall (1% to 2%).
Mitral annulus velocity, Ea - status of myocardial relaxation reduced in most patients with HCM
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
 Normal life expectancy
 Mortality
 Adults – 1% / yr
 Children - 2% / yr
 Subgroups at higher risk for important disease complications
 sudden and unexpected death
 progressive heart failure
 atrial fibrillation (AF)
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
JAMA 281:650, 1999
1980-2006. Circulation 119:1085, 2009
 Primary ventricular tachycardia and ventricular fibrillation
 Adolescents and young adults <30 to 35 years of age
 Most common cause of Athletic field deaths
 Death most commonly occur at rest
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
Secondary prevention
1. Prior cardiac arrest
2. Sustained ventricular tachycardia
Primary prevention
one or more of the following
1. Family history of one or more premature HCM-related deaths,
particularly if sudden and multiple
2. Unexplained syncope, especially if recent and in the young
3. Hypotensive or attenuated blood pressure response to exercise
4. Multiple, repetitive (or prolonged) NSVT on Holter
5. Massive LVH (wall thickness,≥30 mm), particularly in young patients
N Engl J Med 342:1778-85, 2000
Diagnostic plan
#1 Hypertrophic cardiomyopathy (obstructive type)
*Dx.plan: Echocardiography
Stress echocardiography
Cardiac MRI
48hr. Holter monitoring
Clinical Course
Cardiac MRI(20131203)
Holter (20130516)
Treadmill echo. (20131205)
* Test duration: 10:18min
Max. METs: 12.30
Max. HR 187bpm (92% target HR)
Resting BP 131/62, Max. BP 147/73
Reason to stop : Fatigue
Treadmill echo. (20131220)
Treadmill echo. (20131220)
Therapeutic plan
#1 Hypertrophic cardiomyopathy (obstructive type)
*Tx.plan?
Therapeutic plan
#1 Hypertrophic cardiomyopathy (obstructive type)
*Tx.plan: Symptomatic LVOT obstruction
Prevention for SCD
Treatment for HF
Arrhythmia control
Braunwalds Heart Disease A Textbook of Cardiovascular Medicine 9th
Beta blockers (Class I, Level B)
Slowing heart rate
Reducing force of LV contraction
Augmenting ventricular filling and relaxation
Decreasing myocardial oxygen consumption
Long-acting preparations - propranolol, atenolol, metoprolol or nadolol
Blunt LV outflow gradient triggered by physiologic exercise.
Target resting heart rate - 60 beats/min
European Heart Journal doi:10.1093/eurheartj/ehu284
Verapamil (Class I, Level B)
Improves symptoms and exercise capacity
(patients without marked obstruction to LV outflow)
Beneficial effect on ventricular relaxation and filling
Better angina control than BB
Hemodynamic deterioration with CCB agents
- lowering of the afterload in the presence of severe outflow tract gradients
and high diastolic filling pressures
European Heart Journal doi:10.1093/eurheartj/ehu284
1.
2.
3.
Drug-refractory heart failure symptoms
NYHA Classes III and IV
LV outflow obstruction
 Rest - gradient ≥ 30 mm Hg
 Physiologic exercise - gradient ≥ 50 mm Hg
(Class I, Level B)
Transaortic resection of muscle from the proximal to midseptal region
Operative mortality <1 percent
Maintain long-lasting improvement in symptoms and exercise capacity
Mortality may be improved after septal myectomy
European Heart Journal doi:10.1093/eurheartj/ehu284
 Outflow tract gradient is reduced from a mean of 60~70
mmHg often to <20 mmHg
 80–85 % symptomatic improvement
 Complications
 complete heart block < 10 %
 coronary dissections
 large myocardial infarctions
 ventricular septal defects
 myocardial perforations
 ventricular fibrillation
European Heart Journal doi:10.1093/eurheartj/ehu284
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
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(Class IIb, Level C)
Objective measurements of exercise capacity did not differ
significantly
Overall decrease in outflow tract gradient (25 to 40 percent
of baseline)
Role of dual-chamber pacing - patients at high risk for othe
r therapeutic modalities.
Candidates for dual-chamber pacing
 Significant bradycardia in which pacing may allow an inc
reased dosage of medication
 Patients who need ICD as a primary treatment
European Heart Journal doi:10.1093/eurheartj/ehu284
European Heart Journal doi:10.1093/eurheartj/ehu284
Final diagnosis
#1 Hypertrophic cardiomyopathy (obstructive type )