Acute cardiovascular complications of immune-mediated systemic inflammatory diseases

Abstract

Immune-mediated systemic inflammatory conditions (IMIDs) are associated with an increased risk of atherosclerosis and adverse cardiovascular (CV) events secondary to pathogenic inflammation and derangements in the innate and adaptive immune responses inherent to the underlying rheumatic diseases. As the intersection of cardio-rheumatology continues to expand, a multi-disciplinary approach must be considered to optimize clinical outcomes and long-term survival. This review will highlight acute cardiac manifestations of systemic inflammatory diseases and propose a clinically relevant framework for diagnosis, management, and the role of integrated multimodality imaging.

Keywords: Acute coronary syndrome; Autoimmunity; Myocarditis; Pericardial disease; Pulmonary hypertension; Right ventricular failure.

Graphical Abstract

Conceptual model of the role of multi-disciplinary care in patients with IMIDs who require ICU-level care. *If available, consider cardio-rheumatology consultation. **For pulmonary hypertension management, consider pulmonary vascular specialists as available. ^Advanced heart failure involvement for mechanical support and/or heart transplant evaluation.

Figure 1

Fifty-five-year-old female with long-standing SLE presenting with acute shortness of breath. (A) Parasternal 2D echocardiographic long-axis image shows increased echogenicity and thickening of the anterior pericardium. (B) LV global longitudinal strain demonstrates reduced strain of the basal anterior and lateral wall segments, a pattern seen in constrictive pericarditis. (C) Systolic flow reversal is noted in the hepatic vein Doppler signal consistent with elevated right ventricular end-diastolic pressure. (D) The transaxial four-chamber image and (E). transaxial longitudinal view demonstrated dense calcification and thickening of the anterior pericardium extending to the left ventricular apex. Paradoxical septal motion is noted from the short-axis CMR images in systole (F) and diastole (G), suggestive of interventricular dependency and constrictive physiology.

Figure 2

Acute heart failure in cardiac sarcoidosis. (A) Cardiac magnetic resonance imaging: three-chamber view demonstrating dense, transmural late gadolinium enhancement (red arrowheads) in the left ventricle and septum. (B) Photomicrograph showing a large non-necrotizing granuloma with associated lymphocytic inflammation (blue arrowheads) within the explanted myocardium adjacent to epicardial adipose tissue. H&E stained section, 100 × original magnification. Photomicrograph courtesy of Robert F. Padera MD Ph.D., Brigham and Women’s Hospital, Boston, MA.

Figure 3

Pulmonary hypertension and right heart failure (RHF) in SSc. Pulmonary hypertension and RHF in SSc. (A) Apical four-chamber echocardiogram demonstrates severe right atrial and ventricular enlargement with moderate-severe tricuspid regurgitation. (B) Continuous wave Doppler across the tricuspid jet demonstrates right ventricular systolic pressure 76 mmHg, which is consistent with severe pulmonary hypertension when added to the right atrial pressure. Cardiac magnetic resonance image in systole (C) and diastole (D) dilated right ventricle with flattening of the interventricular septum and impaired function (right ventricular ejection fraction 30%).

Figure 4

Twenty-eight-year-old female presenting with shortness of breath and asymmetric radial pulses. (A) Computed tomography angiogram with coronal imaging at the level of the aortic arch demonstrating significant vessel thickening. (B) Computed tomography angiogram with coronal and (C) sagittal MIP reconstructions displaying significant bilateral common carotid artery thickening with concomitant narrowing.