Extranodal Rosai-Dorfman Disease Presenting as a Pericardial Mass and Constrictive Pericarditis.

Rosai-Dorfman disease is a rare, idiopathic disorder of histiocyte proliferation. We describe a case of a 59-year-old woman who presented with heart failure symptoms from a large pericardial mass causing constrictive pericarditis. Pathologic findings including immunohistochemistry suggested the diagnosis. She was treated with corticosteroids and improved clinically. (Level of Difficulty: Advanced.).

Recurrent Ischemic Stroke: A Manifestation of a Sinus of Valsalva Pseudoaneurysm.

An 83-year-old female presented to the emergency department with bilateral eye pain, dizziness, and acute shortness of breath. Her blood pressure (BP) at presentation was 184/93 mmHg. She was admitted for hypertensive emergency and her symptoms improved with BP control. However, during hospitalization, she developed left-sided paresthesia which on investigation was found to be secondary to ischemic stroke. She had two previous aortic mechanical valve replacements for aortic stenosis. Transthoracic echocardiogram revealed a large pseudoaneurysm of the non-coronary sinus of Valsalva that we believe was the cause of her recurrent stroke. A sinus of Valsalva pseudoaneurysm is an uncommon complication of aortic valve replacement surgery which in turn has been associated with aortic rupture, myocardial infarction, and stroke. Our case identifies a situation where sinus of Valsalva pseudoaneurysm predisposed our patient to recurrent ischemic strokes and this should be kept in mind when evaluating a patient with ischemic stroke.

M. chimaera: A Multi-Headed Pathogen.

We present the case of a 64-year-old man with Mycobacterium chimaera prosthetic aortic valve endocarditis likely acquired through exposure to a contaminated heater-cooler device used for cardiopulmonary bypass during valve replacement. This case demonstrates multi-system involvement with a non-tuberculous mycobacterial species that was previously not considered a significant human pathogen. We review the key features of the case and review published literature regarding an international outbreak of M. chimaera arising from point-source contamination during the manufacturing process. This report highlights the importance of clinician awareness of this international outbreak.

Amiodarone Refractory Electrical Storm Post-Revascularization for Anterior ST Elevation Myocardial Infarction: A Case Report and Brief Review of the Literature.

"Electrical storm" is a life-threatening condition defined as the occurrence of at least two episodes of malignant ventricular arrhythmia in a 24-hour period despite anti-arrhythmic therapy. It leads to unpredictable degrees of clinical instability - in the most severe cases mechanical support for circulation and oxygenation and for definitive therapy radiofrequency ablation or cardiac sympathetic denervation. We describe a case of medication refractory electrical storm complicating severe left ventricular dysfunction after an anterior ST-elevation myocardial infarction.

Mechanical Valve Thrombosis on Rivaroxaban: Are Novel Anticoagulants Really an Option?

This is a case of a 54-year-old female with a history of mechanical aortic valve replacement who presented in cardiogenic shock. Her primary care provider started her on rivaroxaban for anticoagulation therapy. An urgent transesophageal echocardiogram revealed a significant gradient and thrombosis on one leaflet of the valve that was immobile. Given that she was not a surgical candidate, she underwent thrombolysis. However, she later died due to complications from the thrombotic valve. The utility of target-specific oral anticoagulants has yet to be established in clinical practice.

Mitral Stenosis Presenting as Asthma.

Although wheezing is one of the most common symptoms and physical findings in asthma, other causes of wheezing should be kept in mind: vocal cord dysfunction, postnasal drip syndrome, chronic obstructive pulmonary disease, bronchiectasis, and non-pulmonary diseases, like heart failure and pulmonary edema. Here, we present a case of severe mitral stenosis with pulmonary edema treated for resistant asthma. If asthma is difficult to control, other etiologies of wheezing, including cardiac disease, should be taken into consideration during diagnosis.

Cardiac Presentation of Multiple Myeloma with Amyloidosis.

Amyloidosis is a rare disease encompassing the accumulation of abnormal and insoluble amyloids systemically or in specific organs. This is a case of a previously healthy 60-year-old male complaining of fatigue and chest pain who proceeded to rapidly decline in functional status within six months from the onset of these symptoms. Cardiac evaluation demonstrated characteristic changes on EKG and echocardiogram was consistent with cardiac amyloidosis. Muscle and gastrointestinal biopsies confirmed multisystem amyloidosis. Monoclonal kappa light chain was identified by serum electrophoresis and the diagnosis of multiple myeloma was confirmed by bone marrow biopsy. The process of amyloid deposition secondary to multiple myeloma was managed with chemotherapy after the patient was referred to a center specialized in amyloidosis treatment. This case highlights the diagnostic challenges, non-specific signs and symptoms, and treatment dilemmas involved in managing amyloid light-chain cardiac amyloidosis.

A Case of Silent Q Fever Endocarditis.

Q fever endocarditis is a rare, culture negative endocarditis caused by Coxiella burnetii, a spore-forming gram negative coccobacillus. Presenting symptoms can be very non-specific; thus, diagnosis may be delayed. We present a case of a 65-year-old male patient with history of aortic aneurysm who complained of chronic fatigue. He was found to have aortic valve vegetation on routine echocardiography. Q fever endocarditis was diagnosed based on elevated Q fever serology; there was absence of fever. This case illustrated a rare, under-recognized and atypical manifestation of Q fever endocarditis. We would like to encourage physicians of rural states like South Dakota to remain vigilant when it comes to screening for the suspected cases of Q fever, specifically in cases of unexplained fatigue and valvulopathy.

Peripartum Cardiomyopathy: Literature Review.

Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure that affects women during the last month of pregnancy to the first five months after delivery. The disease occurs in about one in 1,000 births in the U.S. Risk factors include advanced age, multiparity, twin pregnancy, African origin, preeclampsia or preexisting hypertension, and severe anemia. Heart failure in PPCM is treated similarly to heart failure from other causes, bearing in mind the pregnancy and lactation status. In this review article, we discuss the background, etiology, clinical evaluation, treatment, and natural history of peripartum cardiomyopathy, with a major emphasis on treatment.