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

Pathophysiology of SSc

Inflammation Thrombosis

Vasoconstriction Vessel wall

hyperplasia

Tissue injury

and fibrosis

Hypoxia

Cardiovascular Manifestations of SSc

Valve disease?

Pericardial disease

Cor Pulmonale Myocarditis/ Cardiomyopathy

Hypertrophy

Conduction abnormalities

Microvascular disease

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

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

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

PAH and Survival in SSc

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

Right Heart Failure in SSc PAH

Right Heart Failure Normal

Secondary Tricuspid Regurgitation

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)

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

How to Diagnose PAH

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

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

Left Ventricular Dysfunction

Normal Dysfunction

Myocyte Damage from Microvascular Disease

Myositis and Vasculitis

Histology DE-Gd-MRI

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

Occult LV Dysfunction:

Common First Manifestations

• Stroke

• Heart rhythm disturbance (atrial

fibrillation, ventricular fibrillation)

• Complications of poor blood flow (kidney

dysfunction, confusion)

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

RNI Detection of Perfusion Defects

Stress Rest

MCE Evaluation of the Microcirculation

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

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

Diastolic Heart Failure

Pericarditis

Sharp chest pain

Positional pain

Respiratory variation

Fevers

Shortness of breath

Palpitations

Symptoms

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

Pericardial Effusion

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

Pericardial Effusion: Detection

1. Clinical suspicion

2. Physical exam

3. Imaging

Pericardial Constriction

• Encasement of the heart

• Symptoms: fatigue, chest pain, swelling

• Abdominal distention

• Atrial fibrillation

Pericardial Constriction

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

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)