Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

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Cardiovascular Manifestations Systemic Sclerosis Jonathan R Lindner, MD M Lowell Edwards Professor of Cardiology Knight Cardiovascular Institute Oregon Health and Sciences University

description

Presentation by Dr. Jonathan R. Lindner MD at the 13th Annual Cheri Woo Scleroderma Education Seminar on March 8, 2014 in Portland, Oregon. The seminar is a free public service hosted by the Oregon Chapter of the Scleroderma Foundation.

Transcript of Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Page 1: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Cardiovascular Manifestations Systemic Sclerosis

Jonathan R Lindner, MD

M Lowell Edwards Professor of Cardiology

Knight Cardiovascular Institute

Oregon Health and Sciences University

Page 2: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Pathophysiology of SSc

Inflammation Thrombosis

Vasoconstriction Vessel wall

hyperplasia

Tissue injury

and fibrosis

Hypoxia

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Cardiovascular Manifestations of SSc

Valve disease?

Pericardial disease

Cor Pulmonale Myocarditis/ Cardiomyopathy

Hypertrophy

Conduction abnormalities

Microvascular disease

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Cardiac Complications:

Scope of the Problem

• Cardiac symptoms often go unnoticed

• Symptoms attributed to lung or musculoskeletal

disease

• Recognized primary cardiac involvement in 20-

25% of those with diffuse SSc (much higher on

autopsy series)

• Presence of cardiac involvement is a poor

prognostic feature and usually occurs in those

with more advanced disease

Page 5: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Pulmonary Hypertension in SSc

• High blood pressure in the lung arterial

circulation.

• Severe pulmonary hypertension affects 10-

12% of patients with SSc

• Mortality 50% within 3 yrs

• Most of the mortality is directly related to

effects on the right ventricle

• In those with long term survival there is

considerable morbidity from the effects on the

right ventricle

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Koch ET, et al. Br J Rheumatol 1996;35:989

PAH and Survival in SSc

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Right Heart Failure in SSc PAH

• For any given increase in pulmonary pressure,

the deleterious effect on the right heart is

greater in SSc than in other diseases of PAH

Page 8: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Right Heart Failure in SSc PAH

Right Heart Failure Normal

Page 9: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Secondary Tricuspid Regurgitation

Page 10: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Right Heart Failure:

Symptoms and Complications

• Fatigue, shortness of breath, exercise

intolerance

• Severe edema (swelling of legs, abdomen)

• Liver dysfunction and cirrhosis

• Gastrointestinal symptoms of bowel edema

• Heart rhythm disorders (atrial fibrillation,

ventricular tachycardia)

Page 11: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Risk Factors for PAH and

Right Heart Failure

• Late age of onset of SSc

• Pre-existing lung disease, smoking

• Raynaud’s

• Certain antibodies (anti-U3RNP)

• More severe SSc

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How to Diagnose PAH

Page 13: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Treatment Options

Pulmonary vasodilators:

• Prostacyclin agonists

• Phosphodiesterase-E5 inhibitors

• Endothelin antagonists

• Calcium channel blockers

Immunosuppressive therapy

Diuretics

Oxygen

Digoxin

Lung transplantation

Experimental: Ivadrabine, Tyrosine kinase inhibitors

Page 14: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Left Ventricular Dysfunction in SSc

Causes:

- Heart inflammation/fibrosis

- Small vessel dysfunction

- “Raynaud’s” of the heart vessels

Occurs in approximately 5% of patients with

SSc

Higher incidence with advanced age,

hypertension, kidney disease, pulmonary

disease, digital ulcers

Page 15: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Left Ventricular Dysfunction

Normal Dysfunction

Page 16: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Myocyte Damage from Microvascular Disease

Page 17: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Myositis and Vasculitis

Histology DE-Gd-MRI

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Symptoms of LV Dysfunction

• Shortness of breath

• Fatigue, weakness

• Cough, frothy sputum

• Inability to sleep flat

Symptoms of Coronary Vasospasm

• Chest pain, acute shortneess of

breath

Symptoms of Myositis

• Chest pain, fever, fatigue

Page 19: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Occult LV Dysfunction:

Common First Manifestations

• Stroke

• Heart rhythm disturbance (atrial

fibrillation, ventricular fibrillation)

• Complications of poor blood flow (kidney

dysfunction, confusion)

Page 20: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Diagnosis

• Clinical suspicion

• Echocardiogram

• Once LV dysfunction is found, there is a

workup for causes not related to SSc

• Evaluation for myocarditis and

microvascular dysfunction

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RNI Detection of Perfusion Defects

Stress Rest

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MCE Evaluation of the Microcirculation

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Treatment

• Diuretics

• ACE-inhibitors; Angiotensin receptor

blocking agents

• Beta blockers???

• If vasospasm suspected: calcium

channel blocking vasodilators or long-

acting nitroglycerine

• ICD

• Cardiac rehabilitation

• If myositis: immunosupppressive therapy

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Diastolic Heart Failure

• No problem with the heart squeeze

• Problem exists with the relaxation of the

heart between squeezes

• Due to fibrosis and enlarged heart cells

that occurs with inflammation, early

microvascular disease, renal disease,

and hypertension

Page 26: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Pericarditis

Sharp chest pain

Positional pain

Respiratory variation

Fevers

Shortness of breath

Palpitations

Symptoms

Page 27: Cardiovascular Manifestations, Systemic Sclerosis by Dr. Jonathan R. Lindner MD

Pericarditis in SSc

Symptomatic pericarditis in 5-12%

Detected by imaging/autopsy in 33-70%

Common in limited scleroderma (CREST)

More common if there is PAH

Treatment with NSAIDs and/or steroids

Complications of disease:

• Effusions (tamponade)

• Constriction

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Pericardial Effusion

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Pericardial Effusion

Symptoms: chest pain, shortness of breath,

dizziness, fatigue, swelling

When severe cardiac collapse (tamponade)

Hemodynamically significant effusion in 10%

of those with pericarditis

Can also be associated with renal disease

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Pericardial Effusion: Detection

1. Clinical suspicion

2. Physical exam

3. Imaging

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Pericardial Constriction

• Encasement of the heart

• Symptoms: fatigue, chest pain, swelling

• Abdominal distention

• Atrial fibrillation

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Pericardial Constriction

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Treatment for Complications

• Drain fluid if it is causing more than mild

symptoms or endangering heart function

• For constriction, diuretics to unload the

heart

• Consider immunosuppressive therapy for

constriction or refractory/recurrent

effusion

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What Does This Mean for You?

1. Awareness that there are cardiac manifestations in

SSc is the first and most important step to

discovering cardiovascular disease

2. Echocardiography is a common diagnostic test – it

is generally part of the routine screening for

pulmonary hypertension

3. More severe disease should lead to more frequent

screening

4. Do not discount symptoms of shortness of breath,

extreme fatigue, dizziness, chest pain

5. Aggressive treatment of hypertension

6. Other risk factor modification (exercise, smoking

cessation, diet)