Abstract 17062: Reversible Golimumab Induced Cardiomyopathy - First Reported Case Report

Case Presentation: An 80 year old female with medical history of hypertension, diabetes mellitus, chronic atrial fibrillation presented with four weeks of lower extremity edema and dyspnea. Notably, she had also been taking Golimumab for 6 months for Rheumatoid Arthritis (RA). Vital signs on presentations were: Temp:99 F, HR: 140bpm, BP: 105/64, oxygen saturation of 88% on room air. Physical exam revealed crackles at the mid lower lung fields bilaterally and 2+ lower extremity edema. EKG showed new ST inversions in lead 1, avF and V2. Troponin T was elevated to 0.11 ng/ml and proBNP was 21,246 pg/ml. Chest X Ray showed cardiomegaly with diffuse alveolar opacities. Transthoracic echocardiogram (TTE) revealed severely reduced left ventricular systolic function with LVEF of 25-29%, left ventricular regional wall hypokinesis and mildly reduced right ventricular systolic function. All findings were new compared to her last TTE 3 months prior, which showed preserved biventricular systolic function. Coronary angiography revealed no coronary artery disease. The patient was started on intravenous furosemide, and her home beta blocker and ARB were resumed. The patient’s Golimumab was discontinued given prior reports of TNF alpha inhibitor induced cardiomyopathy. Over the ensuing days, she was aggressively diuresed with improvement in oxygenation and ultimately discharged home. Three months after discontinuation of Golimumab, repeat TTE showed normalization of biventricular systolic function. Discussion: TNF alpha inhibitors have revolutionized the treatment of chronic immune mediated inflammatory diseases. Several TNF alpha inhibitors have been associated with cardiomyopathy, however there remains a paucity of evidence regarding cardiotoxicity with Golimumab. We now present, to our knowledge, the first reported case of reversible heart failure due to Golimumab in an 80 year old woman with RA. Golimumab, like other TNF alpha inhibitors, represents a historic advancement in the treatment of immune mediated inflammatory diseases. However, our case implicates this innovative drug in cardiotoxicity similar to other TNF alpha inhibitors. Further prospective studies are needed to establish a stronger correlation between Golimumab and cardiotoxicity.

Abstract 16848: The Effect Of Rhabdomyomas On Underlying Cardiomyopathy: A Case Report

Case Presentation: Term male infant born to SARS-CoV-2 positive mother with infant testing negative. ECG for perinatal bradycardia revealed ventricular pre-excitation. Echocardiogram showed asymmetric LV hypertrophy with prominent trabeculations, subaortic narrowing with no pressure gradient, and normal biventricular systolic function. Rapid increase in RV pressure estimates and NT-proBNP in first week if life concerning for diastolic dysfunction. Anti-arrhythmic therapy initiated for SVT with subsequent resolution. Later, developed progressive LV dilation and systolic dysfunction. Myocardium showed regions resembling non-compaction and others concerning for infiltrative process. Cardiac MRI showed no obvious tumors, but rhabdomyomas could not be ruled out given similar appearance to myocardium. Due to worsening heart failure, everolimus therapy initiated to target potential rhabdomyomas while awaiting genetic testing for tuberous sclerosis. Subaortic narrowing and LV hypertrophy improved within days, and LV appearance became more consistent with non-compaction. Genetic testing revealed a TSC2 gene variant consistent with tuberous sclerosis. Systolic function improved, and patient discharged on afterload reduction. Echocardiogram 6 months post-discharge shows continued LV dilation and mild systolic dysfunction. Discussion: Although outflow obstruction and arrhythmias are common with cardiac rhabdomyomas and can cause dysfunction, our patient developed progressive dysfunction in the absence of outflow tract gradient or prolonged arrhythmia. As rhabdomyomas subsided, it became clearer that he had an underlying cardiomyopathy. We suspect that rhabdomyomas in the setting of abnormal myocardium led to abnormalities in myocardial contractility and compliance causing combined systolic and diastolic dysfunction. After complete resolution of rhabdomyomas, cardiac function has improved. However, he continues to have ventricular dilation and mild dysfunction attributable to cardiomyopathy. It is unlikely that mother’s SARS-CoV-2 infection played a role as infant tested negative and clinical picture was not consistent with myocarditis.

Myocarditis with Biventricular Dysfunction Caused by Parvovirus B19 – A case report

ABSTRACT Introduction: Myocarditis is often a diagnostic challenge given the heterogeneity of the clinical presentation. Cardiac magnetic resonance imaging is an important diagnostic tool required for the diagnosis as well as detecting the prognosis of the patient. Although rarely used, an endomyocardial biopsy is the gold standard diagnostic method. Clinical case: We present the case of a 29-year old man diagnosed with myocarditis who later developed severe depression of biventricular systolic function. An endomyocardial biopsy was performed, which revealed a parvovirus B19 infection. After treatment of the left ventricular dysfunction, the global systolic function was recovered.

P724 The mysteries of sinus of Valsalva

Sinus of Valsalva aneurysms are a rare pathology that can be clinically silent during years and/or become suddenly symptomatic. We described 3 cases with different clinical presentation, complications and surgical treatment. A 26-year-old male, competitive cyclist was admitted with a 1.5 month history of fatigue, palpitations, and nocturnal cough and sweating. At physical examination he was apyretic, blood pressure 157/49mmHg and radial pulse 96 bpm; jugular vein engorgement and continuous heart murmur, were noticed. Blood samples only revealed a mild raised BNP. EKG showed sinus tachycardia and right intraventricular conduction delay and the transthoracic echocardiography (TTE) revealed high velocity systolic-diastolic shunt (image) between a non-dilated right coronary sinus (RCS) and the right atrium (RA); moderate left ventricle dilation, severe right atrium and mild right ventricle enlargement with mild pulmonary hypertension and normal biventricular systolic function. He was referred to angioCT that showed a large communication between a non-dilated RCS and RA with signs of right ventricular overload. He was submitted to surgery, where ruptured aneurysm sac was found. Fistula closure, aneurysm sac suture and tricuspid annuloplasty were performed. A 45-year-old male asymptomatic, with no relevant medical background went to a Cardiology consultation after detection of a holossistolic murmur in the physical exam. EKG showed sinus rhythm arrhythmia with a nonspecific intraventricular conduction delay and the TTE revealed a communication between a dilated noncoronary sinus (NCS) and RA; the biventricular systolic function was normal. He was referred to angioCT which confirmed the diagnosis. He was submitted to surgery where an exeresis and closure of the fistula was performed. A 38-year-old male with no relevant medical background was admitted to emergence room for an intermittent anterior thoracic pain radiating to the neck (2 days of evolution). The EKG showed sinus tachycardia, incomplete right bundle branch block and the high-sensitivity troponin assays were negative. The TTE revealed severe sinus of Valsalva aneurysm, particularly of the NCS with mild aortic regurgitation. The patient performed transesophageal echocardiography and angioCT which excluded an acute aortic syndrome. He was oriented to surgery where a contained rupture of the RCS and a non-visualization of the ostium right coronary artery were detected, suggesting an intimal dissection. The patient was submitted to Bentall procedure and a bypass with internal mammary artery to right coronary artery. In spite of the unclear etiology, all the cases had a good clinical and echocardiographic evolution at follow-up. The difficulty in making a timely diagnosis is related to the variability of clinical presentation and the need of high clinical suspicion. Echocardiography and angioTC have demonstrated its value, in providing a prompt diagnosis and appropriate management guidance. Abstract P724 Figure. Ruptured aneurysm sac and fistula

Biventricular systolic function in young lambs subject to chronic systemic right ventricular pressure overload

In various clinical situations of congenital heart disease, the right ventricle (RV) is subject to a chronic systemic pressure overload which affects biventricular function and may progress to the development of RV failure. Young lambs (2–3 wk old) underwent adjustable pulmonary artery banding (PAB) at systemic (aortic) level for 8 wk. Biventricular function was determined by using load-independent indexes of global ventricular contractile performance by the end-systolic pressure-volume relationship (ESPVR) using the conductance catheter at baseline and during dobutamine infusion. PAB resulted in a significant fivefold increase in RV end-systolic pressure (12–64 mmHg) and a doubling of the RV-to-left ventricular (LV) wall thickness ratio ( P < 0.01). RV global contractile performance increased significantly, as indicated by an increased slope of the ESPVR. Compared with age-matched control lambs, cardiac output decreased from 2.6 to 1.6 l/min ( P < 0.05) whereas heart rates were equal. In contrast with RV volume, LV volume decreased significantly after PAB ( P < 0.01), whereas the LV-ESPVR slope was unchanged. In the PAB group, the RV, but not the LV, showed a reduced response to dobutamine. We concluded that chronic RV pressure overload for 8 wk results in diminished pump function despite compensatory increased RV global contractile performance.