Published on March 11, 2014
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 FailureNormal
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 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
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)
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