Refractory Heart Failure With Reversible Left Ventricular Dysfunction and Mitral Regurgitation in Takayasu Arteritis

A 68-year-old woman presented with refractory pulmonary congestion and hypertension (183/55 mmHg). She was afebrile, with laboratory tests showing renal dysfunction and slight inflammation (C-reactive protein [CRP] 0.24 mg/dL). Transthoracic echocardiography (TTE) demonstrated reduced left ventricular ejection fraction (LVEF; 20.7%), left ventricular (LV) eccentric hypertrophy and severe functional mitral regurgitation (FMR; Figure A). Computed tomography (CT) revealed no signs of inflammation in the ascending aorta, but did show progression of isolated stenosis in the descending thoracic aorta, first noted 15 years earlier (Figure B1–B3). Cardiac catheterization demonstrated a 65-mmHg pressure gradient (PG) at the coarctation site (Figure C1). LV dysfunction was attributed to increased LV afterload due to Takayasu arteritis (TAK). After the systemic inflammation was ruled out, the patient underwent ascending-to-abdominal aortic bypass surgery. The pathological specimen of the aorta showed fibrosis and a minor infiltration of non-specific inflammatory cells (Figure D1,D2). Postoperatively, acute inflammation developed (CRP 32.4 mg/dL), and ¹⁸F-fluorodeoxyglucose positron emission tomography/CT confirmed flare up of isolated ascending aortitis (Figure E). Remission was initiated with prednisolone (1.0 mg/kg), resulting in a marked decrease in CRP levels. Postoperative TTE demonstrated improved LV function with LVEF of 40.1% and a reduction in FMR (Figure F). Blood pressure improved to 110/52 mmHg, and N-terminal pro-B-type natriuretic peptide improved from 33,217 to 6,123 pg/mL. Catheterization showed a reduction in the aortic PG to 9 mmHg (Figure C2), and systemic vascular resistance decreased from 2,478 to 1,550 dyne/s/cm⁻⁵.

Figure.

(A) Severe mitral regurgitation (MR). Computed tomography (CT) showing isolated stenosis (arrowheads) 15 years earlier (B1), and at admission (B2). (B3) Preoperative contrast-enhanced CT. (C1,C2) Aortic coarctation pressure gradient. Aortic pathology with minor infiltration of non-specific inflammatory cells with hematoxylin-eosin (D1), and leukocyte common antigen staining (arrowheads; D2). (E) ¹⁸F-fluorodeoxyglucose positron emission/CT demonstrating inflammation flare up (arrowhead). (F) Left ventricle reverse remodeling and decreased MR. LVDd, left ventricular diastolic dysfunction; LVEF, left ventricular ejection fraction.

In TAK, increased PG at coarctation could impose LV afterload leading to LV dysfunction, although severe FMR is rarely reported. Morphological changes in aortic lesions should be closely followed up in cases with refractory heart failure (HF). Aortic bypass surgery for TAK-related atypical aortic coarctation could achieve LV reverse remodeling and FMR resolution, thus improving HF symptoms. However, active aortitis can occur postoperatively. Immunosuppression therapy should be initiated, while the potential benefits must be weighed against the increased risk of periprocedural infections.