Table 1.

Prevalence, diagnostic tools, and treatment of cardiac complications of systemic sclerosis

Cardiac manifestation	Diagnostic tools	Treatment
Pericardial diseases • Acute/chronic pericarditis • Pericardial effusions • Constrictive pericarditis	EKG typically demonstrates sinus tachycardia; may demonstrate pulsus alternans if effusion is present and large Imaging studies: • 2D echocardiography to evaluate for cardiac tamponade or hemodynamic compromise • CT/CMR may demonstrate pericardial thickening in the setting of chronic pericarditis, constrictive pericarditis Right Heart Catheterization: • Invasive hemodynamics may be indicated in cases of discordant findings and will demonstrate discordance of peak LV and RV pressures at peak inspiration Laboratory Studies: elevated NT-proBNP	Pericarditis: NSAIDs and colchicine; If constriction, then treat right heart failure symptoms with diuretics, sodium and fluid restriction; pericardial stripping surgery is contraindicated in most cases Pericardial effusion: treat only if symptomatic; rule our renal crisis; cautious diuretics in the setting of right heart failure; Pericardiocentesis if severely symptomatic or evidence of cardiac tamponade. Pericardiocentesis is contraindicated in patients with significant pulmonary arterial hypertension or RV dysfunction
Primary left and right ventricular myocardial dysfunction	Imaging Studies: • 2D echocardiography is the mainstay in diagnosis of LV/RV systolic or diastolic dysfunction. Tissue Doppler analysis may demonstrate diastolic dysfunction with elevated LV filling pressures. Noninvasive estimation of RV filling pressures may demonstrate evidence of pulmonary hypertension • Speckle tracking echocardiography can be used to identify subclinical abnormalities in contractility CMR for inflammation or fibrosis Right Heart Catheterization: • Invasive hemodynamics for confirmatory Laboratory Studies: troponin, NT-proBNP	LV systolic dysfunction: GDMT ± CRT, mechanical support devices, cardiac transplantation LV diastolic dysfunction: Symptomatic treatment (i.e. diuretics), adequate blood pressure control RV dysfunction: Symptomatic treatment (i.e., diuretics), Digoxin, invasive hemodynamic testing to rule out pulmonary arterial hypertension
Cardiac conduction disease and arrhythmias	Resting EKG or 24-hour ambulatory Holter monitor. In patients where symptoms are less frequent, and/or are associated with cardiogenic syncope, a 30-day event monitor may be indicated. In cases where a 30-day event monitor is unrevealing, implantable loop recorders may extend the ability to detect arrhythmias over a longer period of time	Bradyarrhythmias: pacemaker according to standard guidelines Tachyarrhythmias: Nondihydropyridine calcium channel blockers, avoid betablockers if Raynaud’s present, cautious use of antiarrhythmics consider ablation or ICD in select patients
	Imaging Studies: • CMR for myocardial fibrosis
Ischemic cardiomyopathy	Imaging Studies: • Myocardial perfusion imaging (SPECT or CMR imaging) • Cardiac CT with coronary artery calcium score may be useful for screening Left heart catheterization/coronary angiography: • gold standard for assessment and management of obstructive epicardial CAD	Microvascular CAD: Calcium channel blockers, ACE-inhibitors/angiotensin receptor antagonists; consider ranolazine if angina is present; statins Macrovascular/epicardial CAD: Coronary stenting or standard medical management of coronary artery disease; statins

Abbreviations: EKG electrocardiogram, NSAIDs non-steroidal anti-inflammatory drugs, CT computed tomography, CMR cardiac magnetic resonance, RV right ventricular, LV left ventricular, GDMT guideline-directed medical therapy, CRT cardiac resynchronization therapy, ICD implantable cardioverter-defibrillator, SPECT single-photon emission computed tomography, CAD coronary artery disease