Cardiomyopathies

Puja ChopraOctober 27, 2011

PGY-2

• Thanks To Dr. Margriet Greidanus!

Objectives

• Hypertrophic Cardiomyopathy– Obstructive– Treatment– EKG

• Dilated Cardiomyopathy– Etiologies– Treatment

• Restrictive Cardiomyopathy– Etiologies– Constrictive pericarditis

Case

56 YO F, post cardiac arrestHx. Waiting surgery for HCM

Hypertrophic Cardiomyopathy

• A refresher

Obstruction: - Ventricle size is small, mitral

valve contacts the ventricle- Venturi effect high flow

through obstruction

• Abnormal Relaxation

• Stiff Ventricle

Hypertrophic Ventricular

Muscle

Increased LA and LV ED pressure

Decreased Diastolic

Filling

- Atrial dilation and back flow into the pulmonary vasculature- Dyspnea with increased oxygen consumption (90%)

- Reduced Cardiac Output - Syncope (30%)

- Thickened ventricular arterioles with reduced lumen- Angina (30%)

P/E: - LVH - Sustained PMI- Irregularly Irregular Pulse- Mid systolic ejection murmur

Back TO the Case:HR 140, RR(intubated and bagged at 12)Sats 100%, BP 95/60

(Case 1) Normal sinus rhythm with large-amplitude QRS complexes consistent with LVH and nonspecific T-wave abnormality. Deep narrow Q waves are also present in the lateral leads I, aVL, V5, and V6.

Normal sinus rhythm with LVH and deep narrow Q waves in the lateral leads I, aVL, V5, and V6.

Normal sinus rhythm with LVH and deep narrow Q waves in the lateral leads I and aVL.

EKG

• LVH: high voltage R waves in the anterolateral leads (V4, 5, 6, I and avL)

• Deep and narrow Q waves can be seen in the inferior leads and in the lateral leads (over the septum) (most specific findings)

Treatment

Beta Blockers! - Prolongs time in diastole- Reduces inotropic demand

Vasopressors: - Increased SVR reduces venturi effect

Avoid Preload Reduction Agents:- Diuretics - Nitroglycerine- Nitropursside

Avoid Inotropic Agents

Surgical Mymectomy: - This is reserved for patients with a high outflow tract obstruction (>50 mmHg) and

those that have failure to medical management- 90% of patients have improvement in their outflow gradient with persistent

symptomatic improvement at 5 years- Reduced amount of SCD in those with surgical mymectomy

Anticoagulation- 6% rate of strokes in these patients

25 yo male syncope with exercise25 yo male light headed post exercise30 yo male chest pain with exercise

23% of patients had an appropriate discharge at follow-up period of 3 years

Take Home• Hypertrophic Crisis:

– Beta blocker– No positive intropic agents

• ECG– LVH– Q waves in lateral and inferior leads

• A. Fib– Stroke Risk: Anticoagulate

63 YO female brought to ED with central crushing chest pain and shortness of breath

• On Exam: Pulmonary edema

• Blood pressure: 70/40

• ?Management

35 YO female, unwellRespiratory DistressUnable to speak full sentencesSitting up

?Asthma Exacerbation

HR: 130, BP 95/67, 88% 15 L non re-breather

But wait…..She had a baby 1 week ago!

DDX of Shock

- Cardiac failure in the last month of pregnancy or within the five months post partum- No determinable cause of the heart failure - No heart disease before the onset of the last month of pregnancy- LV dysfunction seen on echocardiogram

Dilated Cardiomyopathy

• Another Refresher

Etiologies: • Toxins

– Ethanol, – Chemotherapeutic Agents,– Antiretrovial agents, – Cobalt, Lead, Cocaine, Mercury

• Metabolic – Nutritional: Thiamine, selenium, carnitine– Endocrine: Hypothyroid, acromegaly, thyrotoxicosis, cushings, pheochromocytoma, Diabetes

• Inflammatory or Infections: – Collagen Vascular Disesae: Sclerodermia, lupus, Sarcidosis– Peripartum

• Infectious: – Viral Myocarditis: Parvovirus B19, Herpes, coxsackievirus, influenza virus, adenovirus, HIV– Chagas Disease: Protozoa (leading cause in SA and Central America) – Lymes Disease

• Neuromuscular: – Muscular Dystrophies– Freidreich’s ataxia

• Tachycardia• Familial• Stress Induced• Idiopathic

Back to the Case

• Treatment: – A– B– C

• Future Pregnancies

• Complications– ?anti-coagulation

Treatment1. Identify the cause of the cardiomyopathy and determine what can be reversed or prevented

2. Treatment goals include: 1. Prevention of progression 2. Prolonging survival by targeting the poor prognostic indexes3. Symptomatic treatment4. Preventing complications:

1. CHF2. SCD approx 12% of patients will die suddenly3. VTE

Clinical predictors of poor prognosis: - Syncope- S3 gallop- RHF on exam- AV block, BBB (note that an av block in an idependent risk factor for death) - Elevated creatinine- Cardiothoracic ratio- EF less than 35%

Take Home

• Takotsubo Cardiomyopathy– 10 to 15% have LV outflow obstruction

• Treat like CHF

Restrictive Cardiomyopathy

- Amyloidosis- Sarcoidosis- Hemachromatosis- Scleroderma- Neoplastic- Cardiac Infiltration- Radiation Heart Disease- Fabry’s Disease- Gaucher’s Disease- Idiopathic

Restrictive Cardiomyopathy vs Constrictive Pericarditis

Feature Restrictive Cardiomyopathy Constrictive Pericarditis

Physical Exam Prominent Apical Impulse Pericardial knock may be present

ECG Amyloidosis will have lower QRS voltageQ wavesBBBAV conduction A Fib

Repolarization changes

Chest Radiograph Atrial Enlargment Calcific Pericardium

Echocardiography Atrial EnlargementVentricular wall thickness increased

Pericardial Thickening

Take Home

• Constrictive Pericarditis: – Pericardial knock– ECHO

QUESTIONS?