LAUSR

A 49-year-old African American female with lymph node biopsy-proven pulmonary sarcoidosis complicated by skin and eye involvement presented with dyspnea on exertion. The patient was in her usual state of health until 2 years prior to presentation at which time she reported decreasing exercise tolerance. She otherwise denied chest pain, palpitations, paroxysmal nocturnal dyspnea, orthopnea, lower extremity edema or syncope. She was found on transthoracic echocardiogram (TTE) to have severe mitral regurgitation due to bileaflet mitral valve prolapse and subsequently underwent mitral valve repair with relief of symptoms. She continued to do well until 1 year later, she noted recurrent symptoms with worsening edema. Repeat TTE revealed failure of the prior repair with recurrent severe mitral regurgitation and decline in left ventricular (LV) function. She underwent a mitral valve replacement with bioprosthesis (27 mm Edwards Lifesciences pericardial tissue valve, Magna type). Despite successful replacement and guideline-directed medical therapy (GDMT), patient had repeat hospitalizations for heart failure. Of note, cardiac tissue surrounding the excised mitral valve apparatus at time of MV replacement was notable for non-necrotizing granulomatous inflammation without clear identifiable pathogen. The patient was initiated on prednisone 40 mg daily given histological diagnosis consistent with cardiac sarcoidosis. GDMT was further optimized. She was discharged with a life vest as there was evidence of non-sustained ventricular tachycardia (VT) on telemetry monitoring. She presented for a routine follow-up visit. The patient was on maximally tolerated doses of GDMT including beta-blocker, angiotensin receptorneprilysin inhibitor, mineralocorticoid antagonist and diuretic. She was also taking prednisone 40 mg daily, Vitamin D/Calcium supplementation, proton-pump inhibitor and single-dose trimethoprim sulfa daily. She had no known drug allergies. Family history was unknown as the patient was adopted. She was a five pack-year former smoker. She denied alcohol or illicit drug use. The patient’s vital signs were in normal range with blood pressure 118/83, heart rate 64 beats per minute (bpm), and 98% oxygen saturation on room air. Her physical examination was notable for mildly elevated jugular venous pressure with prominent v-wave and II/ VI holosystolic murmur at right sternal border. On review of laboratory data, patient had mild leukocytosis with white blood cell count 13.8 9 10 cells per liter with a normocytic anemia with a hemoglobin of 10.3 g/dL and normal platelet count. Her creatinine was 1.0 mg/dL. NT-proBNP was elevated at 1300 pg/mL and ACE (angiotensin converting enzyme) level was elevated at 138 U/L (normal 9 to 67 U/L). Liver tests and pulmonary function tests were normal. She had normal inflammatory markers, calcium level and 1,25-OH vitamin D levels. Electrocardiogram (ECG) showed normal sinus rhythm at a heart rate 75 bpm, first-degree See related editorial, pp. 2005–2006