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Table 7 Possible causes of cardiac symptoms with a suggestion for further exploration and therapeutic management

From: French recommendations for the management of systemic sclerosis

Disease type/symptom

Order of prevalence

Examinations to be discussed on a case-by-case basis

Treatment proposals

to be discussed on a case-by-case basis

Coronary

microcirculatory disorder

 > 60%

EKG

Troponin

Myocardial scan*

Cardiac MRI

Calcium channel blocker

ACE/ARB II

Control of risk factors

Coronary macrocirculatory disorder

 < 10%

EKG

Troponin

Myocardial scan*

Cardiac stress MRI

Stress echocardiography

Coronary scan

Coronary angiography

Control of risk factors

Treatment based on recommendations

Revascularization

Systolic heart failure

If LVEF ≤ 40%

5%

EKG

Echocardiogram

Natriuretic peptides

Troponin

Myocardial MRI

Based on recommendations: ACEi or ARB II (ANRI, sacubitril, valsartan in second line)/cardioselective beta-blocker to be discussed according to ILD and Raynaud/digital ulcers

Mineralocorticoid receptor antagonists

Defibrillator…

Preserved LVEF heart failure

30–35%

EKG

Echocardiogram

Natriuretic peptides

Troponin

Cardiac MRI

Diuretics

Isolated VD dysfunction

38%

EKG

Echocardiogram

Natriuretic peptides

Troponin

Cardiac MRI

Right heart catheterization

Diuretics

Pericardial effusion

Frequent

Echocardiogram

Cardiac MRI

Usually monitoring

Colchicine+++

Constriction

Rare

Echocardiogram

Cardiac MRI

Right heart catheterization

Diuretics, lower doses if bradycardia medication

Pericardiectomy

Pulmonary hypertension Precapillary

 < 15%

EKG

Echocardiogram (VTI)

Natriuretic peptides

Troponin

Right heart catheterization

Specific treatment of PAH if group 1

Treatment of ILD if group 3

Pulmonary arterial hypertension

With veno-occlusive component

Rare

Right heart catheterization

Chest scan

Diuretics

Specific treatment of PAH

Pulmonary hypertension Post-capillary

 < 12%

EKG

Echocardiogram

Natriuretic peptides

Troponin

Cardiac MRI

Right heart catheterization

Treatment of heart failure

Tachycardia

Supraventricular

15%

EKG

Holter monitor

Anti-arrhythmic treatment based on recommendations

Anticoagulant treatment

Tachycardia Ventricular

Rare

EKG

Holter monitor

Echocardiogram

Look for myocardial ischemia

Antiarrhythmic treatment based on recommendations

Defibrillator

Treatment of ischemia

Bradycardia and conduction disorders

Rare

EKG

Holter

Stop bradycardia medication

Stimulator

  1. *Myocardial MRI is still being evaluated for the study of microcirculation; myocardial scintigraphy is still more suitable now
  2. NSAIDs nonsteroidal antiinflammatory drugs, ANRI angiotensin II receptor neprilysin inhibitor, ARB II angiotensin II receptor blockers, LVEF left ventricular ejection fraction, PAH pulmonary arterial hypertension, ACE angiotensin-converting enzyme inhibitor, MRI magnetic resonance imaging, ILD diffuse infiltrating lung disease, VTI rate of tricuspid insufficiency