A Case of Cryoglobulinemia With Central Nervous System Involvement.

Cryoglobulinemia may result in small-to-medium vessel vasculitis. Central nervous system (CNS) involvement is rare, and presentation may range from stroke/transient ischemic attack, reversible ischemic neurological deficits, to encephalopathic syndromes. This is a rare case discussing cryoglobulinemia with CNS involvement. A 56-year-old female with a history of cryoglobulinemia was found unresponsive to verbal and physical stimuli. She was admitted to the intensive care unit. CT head without contrast showed diffuse cerebral edema and mass effect in the right cerebral hemisphere causing right to left midline shift, brainstem infarct, hemorrhage in the right lateral ventricle, and obstruction of the fourth ventricle. The patient was managed with hypertonic saline, external ventricular drain (EVD) placement, and high-dose steroids, which led to an improvement in her condition. In conclusion, testing for cryoglobulins and serologic tests for hepatitis C should be considered in syndromes of cerebral ischemia or infarction without an obvious cause, especially in young individuals since encephalopathy may be reversible. Cryoglobulinemia with CNS manifestations may be associated with purpura, high RF, and low C4. The treatment can be a combination of steroids, immunosuppressants, plasmapheresis, and rituximab. Cyclophosphamide may also be considered as adjunctive therapy to corticosteroids in rapidly progressive severe neurological complications. Further research for treatment standards in nonviral cryoglobulinemia is needed.

Linear IgA Bullous Dermatosis Attributable to the Use of Spironolactone: A Case Report.

Linear IgA bullous dermatosis (LABD) is a rare, idiopathic, or drug-induced vesiculobullous disease caused by IgA autoantibodies in the basement membrane zone. An 84-year-old man was started on spironolactone two weeks before presentation for the management of hypertension and heart failure with preserved ejection fraction. He presented to our hospital for evaluation of worsening lower extremity swelling and a painful pruritic rash that started on the day preceding his presentation. On examination, he had 3+ lower extremity edema and an erythematous, painful, pruritic, bullous rash on all his extremities. He had a significantly elevated IgA level (1033 mg/dL). A lesional skin biopsy demonstrated epidermal ulceration with degenerated collagen fibers. Direct immunofluorescence of the perilesional skin showed linear IgA at the dermal-epidermal junction. The rash resolved following steroid therapy and discontinuation of spironolactone. There have been previous reports of bullous pemphigoid induced by spironolactone. To our knowledge, LABD associated with spironolactone has not previously been reported.

Takotsubo Cardiomyopathy as a Cardiovascular Manifestation of COVID-19: A Case Report and Literature Review.

Coronavirus disease 2019 (COVID-19) has a wide range of clinical manifestations, affecting multiple organ systems. Cardiovascular manifestations of COVID-19 that have been reported include arrhythmias, myocarditis, and an increased predisposition to acute myocardial infarction. Takotsubo cardiomyopathy (TCM), which is characterized by apical ballooning of the heart leading to acute left ventricular dysfunction, is scarcely seen in COVID-19 patients. We present a case of COVID-19-associated TCM in a 68-year-old man.  A 68-year-old man with no significant past medical history presented with sudden-onset midsternal pressure-like chest pain at rest, associated with diaphoresis and shortness of breath. This occurred ten days after diagnosis of COVID-19 with mild symptoms, with no other recent physical or emotional stressors. At presentation, he was afebrile (98.5 °F), hypertensive (177/108 mmHg), tachycardic (HR 118 bpm), and saturating 100% on room air. Labs were significant for leukocytosis with 15.1 × 103 WBCs/mcL, elevated creatinine (1.46 g/dL), brain natriuretic peptide (BNP) of 156, troponin of 4 ng/mL that peaked at 16.28 ng/mL. The rapid COVID-19 test was positive. EKG showed anterolateral ST elevation and QTc interval of 446 ms. Echo showed severe hypokinesis of mid and apical segments and severely decreased left ventricular ejection fraction (LVEF)of <30%. Emergent left heart catheterization showed 75% mid left anterior descending coronary artery (LAD) stenosis and moderate right coronary artery (RCA) disease, while the ventriculogram showed a left ventricular ejection fraction of 35% with anteroapical and inferoapical akinesia suggestive of Takotsubo cardiomyopathy. The patient was placed on aspirin, ticagrelor, and atorvastatin, carvedilol, and lisinopril. EKG the next day showed a prolonged QTc of 526 ms with T-wave inversion and no ST elevations. The patient had no findings consistent with myocarditis or pheochromocytoma. He was discharged two days later. Within the next few weeks, his symptoms improved, and a follow-up echo confirmed normalization of left ventricular function.  There has been an increased incidence of Takotsubo cardiomyopathy during the COVID-19 pandemic compared to the pre-pandemic period. There is only a slight female preponderance in COVID-19-induced TCM, possibly because males are predominantly affected by COVID-19. Our patient satisfied all four Mayo Clinic criteria required for the diagnosis of TCM. Pathophysiology of TCM in COVID-19 is linked with cytokine storm and consequent catecholamine surge. Most patients improve within succeeding weeks or months. Nonetheless, the case fatality rate is high 36.5%, which is significantly higher compared to TCM patients without COVID-19. COVID-19 has a multisystem involvement with various clinical presentations. New cardiomyopathy in COVID-19 patients should raise suspicion among clinicians regarding stress-induced cardiomyopathy.

Co-occurrence of Anomalous Right Coronary Artery Origin and Subaortic Membrane in an Adult Male.

Anomalous origin of the right coronary artery (ARCA) represents <3% of congenital coronary anomalies, while the subaortic membrane represents 6.5% of congenital heart anomalies. Symptomatic co-occurrence of ARCA and subaortic membrane in an adult is rare. A 68-year-old man developed a non-ST-elevation myocardial infarction necessitating percutaneous coronary intervention (PCI) four years prior to presentation at our hospital. In the years after his PCI, he developed progressive exertional breathlessness. Following a positive treadmill EKG, he underwent coronary CT angiography that indicated RCA dominance with ARCA arising from the left coronary sinus and coursing between the ascending aorta and pulmonary artery, causing 50-60% intraluminal narrowing at rest without atherosclerotic plaque. Echo showed normal left ventricular ejection fraction (LVEF) and a surprise finding of the subaortic membrane, with a modest gradient. He underwent successful resection of the subaortic membrane and unroofing of the anomalous RCA tunnel with tract marsupialization. The post-operative period was complicated by arrhythmias necessitating electrical cardioversion. At discharge, he was sent home on apixaban, bisoprolol, aspirin, atorvastatin, and an amiodarone taper. The subaortic membrane would not have required intervention independently because it was not associated with a severe gradient. However, surgery is recommended for symptomatic ARCA or subaortic membrane; hence, our patient underwent surgical management. Atrial fibrillation and flutter are the most common arrhythmias following cardiac surgery. Due to the patient's increased risk of complications, cardioversion and anticoagulation were pursued. Although ARCA is congenital, our patient had been asymptomatic for most of his life, suggesting that the development of the subaortic membrane might have triggered symptom onset, combining a modest subaortic gradient with previously asymptomatic exercise-induced right coronary ischemia. Clinicians should consider evaluating for secondary structural heart conditions in newly symptomatic adult patients with ARCA due to the risk of sudden cardiac death, to provide the most complete treatment.

A Rare Presentation of Systolic Anterior Motion Occurring Eight Years After Mitral Valve Replacement.

Systolic anterior motion (SAM) is the dynamic displacement of mitral valve leaflets anteriorly toward the left ventricular outflow tract (LVOT) during systole. SAM-like physiology has been reported to occur shortly after mitral valve replacement (MVR) surgery; occurrence beyond two years after surgery is very rare. A 55-year-old woman who had bioprosthetic MVR eight years earlier for non-rheumatic mitral stenosis presented to the emergency room with progressive dyspnea and sudden-onset chest pressure. Physical examination noted a grade 3/6 systolic murmur at the cardiac apex, a soft diastolic murmur at the left sternal border, and diffuse expiratory wheezing. B-type natriuretic peptide (BNP) was elevated (286 pg/mL). Transthoracic echocardiography (TTE) showed mitral regurgitation and severe aortic insufficiency; the mitral prosthesis was protruding into the LVOT, causing LVOT obstruction with a peak gradient of 16.3 mmHg and peak velocity of 2.0 m/s. Transesophageal echocardiography (TEE) confirmed severe bioprosthetic MV dysfunction, severe aortic regurgitation, and SAM-like physiology. Left cardiac catheterization showed normal coronaries. She underwent repeat MVR and aortic valve replacement. She was started on daily aspirin and warfarin post-operatively, then discharged home on post-operative day 10. During post-operative office visits, she reported no complications. SAM-like physiology was absent in a two-month follow-up TTE, with reduced LVOT peak gradient of 6.5 mmHg and peak velocity of 1.3 m/s. Dynamic SAM-induced LVOT obstruction could be asymptomatic or result in severe heart failure with 20% risk of sudden cardiac death. SAM may occur within days following MVR or may have a delayed presentation. Medical management, including beta-blockade, is the cornerstone of initial management, while structural damage to the prosthetic valve mandates repeating mitral valve replacement surgery. This case highlights the importance of long-term follow-up of patients after MVR to assess for SAM, which could occur with or without degenerative changes of the prosthetic valve.

Listeria Bacteremia Presenting With Cerebral Abscess and Endocarditis in an Elderly Patient With Chronic Immune Thrombocytopenia.

Central nervous system involvement by Listeria monocytogenes usually presents as meningitis, meningoencephalitis or, less frequently, rhombencephalitis. Listerial brain abscesses are rare. Moreover, only 5-8% of listerial bacteremia is complicated by infective endocarditis (IE). A 70-year-old man with chronic immune thrombocytopenia (ITP) presented to our emergency department with acute onset of altered mental status and right-sided weakness. He was afebrile, with no heart murmurs or peripheral IE stigmata. Neurologic examination showed disorientation, expressive aphasia, and right-sided hemiparesis. Laboratory findings were unremarkable except for leukocytosis and hyponatremia. Brain MRI showed an irregular rim-enhancing lesion in the left frontal lobe, suspicious for a high-grade glial neoplasm. The lesion was excised, and he was started empirically on vancomycin, ceftriaxone, and metronidazole. After blood cultures grew Listeria monocytogenes, antibiotics were de-escalated to ampicillin and gentamicin. Echocardiography showed mitral valve vegetation. By Day 6, his mental status had improved. On Day 9, he was discharged to our inpatient rehabilitation center to complete six weeks on IV ampicillin and IV gentamicin. Pathology of the brain mass was subsequently reported as a listerial brain abscess. Chronic treatment with high-dose oral glucocorticoids and pre-existing ITP have been independently implicated as predisposing factors in listerial brain abscess. There is a propensity to misdiagnose listerial brain abscess as an intracranial neoplasm due to similar clinical/imaging findings. In addition, Listeria monocytogenes is an atypical cause of IE. Therefore, a high index of suspicion is necessary for early recognition and successful treatment of listerial brain abscess and listerial endocarditis in high-risk patients.