Kopecky, Kathleen F.

A 63-year-old female with mixed connective tissue disease on long-term immunosuppression (prednisone, azathioprine, hydroxychloroquine (HCQ)) was referred to a tertiary care center for evaluation of progressive dyspnea and exertional chest discomfort. Cardiac history included transient complete heart block and ventricular tachycardia with dual-chamber AICD, and severe aortic stenosis with bicuspid aortic valve. Laboratory evaluation was notable for NT pro-BNP > 5000 pg/mL and elevated but adynamic troponin (300-500 ng/L). Electrocardiogram revealed atrial pacing with first degree atrioventricular block. Echocardiogram confirmed severe aortic stenosis, elevated filling pressures, abnormal left ventricular longitudinal strain and LV hypertrophy. Cardiac MRI, sarcoid PET, and PYP SPECT/CT were without any evidence of edema, scar, inflammation or amyloid deposition. Catheterization revealed severe pulmonary hypertension and elevated filling pressures, without significant coronary disease. Medical treatment included empagliflozin (discontinued due to urosepsis), bumetanide, spironolactone, and metoprolol succinate. Neither TAVR nor transition to biventricular pacing improved LV function. Ultimately, endomyocardial biopsy was performed, revealing histologic features of HCQ-induced cardiotoxicity, including myocyte hypertrophy and interstitial fibrosis. HCQ was discontinued. She was referred to Rheumatology for alternative treatments. HCQ induced cardiomyopathy represents <2% of adverse events of patients treated with HCQ, presenting as hypertrophic or restrictive cardiomyopathy with conduction abnormalities. Mean treatment duration in HCQ cardiomyopathies is approximately 12.7 years. Confirmation of diagnosis requires endomyocardial biopsy, as imaging findings are often non-specific. Management involves discontinuing the offending agent and optimizing heart failure therapy. Around 45% of patients with HCQ cardiomyopathy exhibit resolution of cardiac function following medication discontinuation. This case emphasizes the importance of a systematic approach to unexplained heart failure, particularly in patients with long-term HCQ use. Infiltrative or toxic cardiomyopathies should be considered when imaging suggests myocardial dysfunction. Multimodal imaging and biopsy are crucial for diagnosis. Early recognition is essential to prevent irreversible myocardial damage and improve long-term outcomes.

Case: A 65-year-old patient presented 4 months after mitral valve repair (annuloplasty band, commissuroplasty, neo-chordae to the anterior mitral leaflet (AL)). At follow up, he developed significant hemolytic anemia (Hemoglobin 10.1g/dL, LDH 650U/L, Bilirubin 1.4mg/dL and haptoglobin<14 mg/dL). TEE revealed the substrate for hemolysis: moderate-severe mitral regurgitation (MR) starting at the coaptation of A2 and P2 scallops due to AL overriding the posterior (PL). The jet was then directed posteriorly to a small (3 x 6 mm confirmed by cardiac CT) area of annuloplasty band dehiscence at the base of the P2 scallop, with the band in direct path of the flow. The unique feature was that AL retained coaptation with the annuloplasty band, thus forming a small tunnel from the A2-P2 coaptation defect to the dehisced area. Given that redo surgery early after initial intervention would be challenging, the Heart Team decided to pursue percutaneous closure at the level of the dehisced annuloplasty to reduce regurgitation and mitigate hemolysis. Under 3D TEE guidance closure with a 12mm AVP plug was attempted but resulted in interference with PL motion with a new jet of regurgitation laterally. Using two 8mm AVP plugs was successful in closing the dehiscence with minimal impact on PL. Overall regurgitation was reduced to mild. Within a week hemolysis labs improved: LDH337 U/L, Bilirubin0.7 mg/dL. Discussion: Annuloplasty band dehiscence is a known cause of recurrent MR after repair. As regurgitation occurs at the leaflet coaptation zone, percutaneous closure of these defects is usually futile. In this patient we took advantage of the maintained coaptation between the AL and annuloplasty band.