Acute Decompensated Heart Failure and Cardiogenic Shock Following Propofol Infusion: A Report and Review of Pathophysiology.

Propofol is a widely used general anesthetic agent with a generally familiar and predictable adverse effect profile. Severe left ventricular dysfunction to an ejection fraction of < 35% is a rare adverse effect of propofol, with a scarcity of data in the literature. In this case, we report a 36-year-old female at 36 weeks gestation with a prior remote history of peripartum cardiomyopathy, who, while receiving propofol for general anesthesia during a C-section, developed severe left ventricular dysfunction with an ejection fraction of 20-25%, flash pulmonary edema, and cardiogenic shock. She required initiation of inotropic support and, following weaning of propofol, gradually recovered her ejection fraction over the next 24 hours to 40-45% and to 50-55% at follow-up two weeks after discharge. This case highlights a unique adverse effect of propofol with scarce pre-existing literature and no guidelines on appropriate management. It is essential for clinicians to be familiar with this uncommon complication, particularly as propofol use continues to rise worldwide.

Screening Echocardiography in Adults with Down Syndrome.

Down syndrome is the most common chromosomal disorder in the United States, occurring in about 14.14/10,000 births. It is associated with multiple medical anomalies, including cardiac, gastrointestinal, musculoskeletal, and genitourinary abnormalities, which increases the burden of morbidity for this patient population. Management is typically directed toward optimizing health and function throughout childhood and into adulthood; however, consensus regarding their management in adulthood is controversial. The burden of congenital cardiac diseases in children with trisomy 21 is well established, seen in more than 40% of cases. Although screening echocardiography is performed routinely within 1 month of birth, current consensus advocates for diagnostic echocardiography only in symptomatic adults with Down syndrome. Here, we advocate that screening echocardiography should be performed routinely in this patient population at all ages, particularly in late adolescence and early adulthood, because of a high percentage of residual cardiac defects and an increased risk of developing valvular and structural cardiac disease.

Paraneoplastic Scleroderma: A Case Report of Systemic Sclerosis in the Setting of Pulmonary Adenocarcinoma.

A 42-year-old female with a past medical history significant for scleroderma and extensive tobacco use presented with a dry cough and pleuritic chest pain. Further workup was significant for leukocytosis, macrocytic anemia, left lower lung mass, bilateral supraclavicular, hilar, and mediastinal lymphadenopathy. After a comprehensive rheumatologic workup was completed, the patient was found to have strongly positive antinuclear antibody (ANA) and negative scleroderma-specific antibodies with fluorescent ANA indicating a nucleolar pattern. We present a case of paraneoplastic scleroderma in the setting of lung adenocarcinoma which emphasizes the bidirectional relationship that exists between malignancy and rheumatic diseases.

The Stressed Heart: A Case Report of Takotsubo Cardiomyopathy in a Patient With Known Coronary Artery Disease.

Takotsubo cardiomyopathy and acute coronary syndrome are often clinically indistinguishable, making their differentiation challenging for physicians. We present a case of a 65-year-old female who presented with acute chest pain, shortness of breath, and a recent psychosocial stressor. This is a unique case in which our patient, with known history of coronary artery disease and recent percutaneous intervention, favored a misleading initial diagnosis of non-ST elevation myocardial infarction.

Thrombotic Storm Induced by Rituximab in a Patient With Pemphigus Vulgaris.

Thrombotic storm is a rare hypercoagulable condition characterized by a clinical trigger causing extensive thrombotic events affecting multiple vessels over a short period of time. We present a case of thrombotic storm that developed in a patient who received rituximab therapy. The patient presented to the hospital with dyspnea and shortness of breath and was subsequently diagnosed with extensive thrombotic burden including multiple deep vein thrombi and pulmonary emboli. Hypercoagulable workup for the thrombotic storm was unrevealing with the only identifiable trigger being the rituximab infusion. The patient was treated successfully with anticoagulation and discontinuation of rituximab. There are very few reports highlighting thrombotic events as a complication of rituximab therapy. We aim to increase recognition of thrombotic storm as a potential complication of receiving rituximab therapy.

Unusual Instance of Primary Diffuse Large B-cell Lymphoma of the Colon.

Diffuse large B-cell lymphoma (DLBCL) commonly affects the gastrointestinal (GI) tract, although primary DLBCL rarely occurs in the colon. Primary colorectal lymphoma is a surprisingly rare diagnosis, accounting for a minute percentage of GI lymphomas and colorectal malignancies. We present an interesting case of an immunocompromised young adult female who was diagnosed with DLBCL confined to a cecum polyp after she underwent a colonoscopy for a GI bleed. The lymphoma presented endoscopically as a semi-sessile polyp in the cecum that was successfully removed. The patient was treated with appropriate therapy of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP).

Graves' Disease-Associated Dilated Cardiomyopathy Unmasked by Blunt Chest Trauma.

Graves' disease can manifest with numerous cardiovascular complications, although very few cases result in cardiomyopathy. Our patient presented following a motor vehicle accident where she suffered blunt chest trauma. Her initial presentation was concerning for acute heart failure due to blunt cardiac injury, with diffuse pulmonary edema, hemodynamic instability, and an acutely reduced ejection fraction with global hypokinesis on transthoracic echocardiography. On further evaluation with thyroid function testing and autoimmune studies, she was found to have uncontrolled Graves' disease. She was subsequently started on methimazole for her Graves' cardiomyopathy. Following discharge, an outpatient cardiac MRI revealed no late gadolinium enhancement and complete recovery of her ejection fraction to normal values. This case serves to highlight the importance of a complete evaluation of cardiomyopathy and highlights an interesting case of a patient with blunt chest injury with a background of undetected Graves' cardiomyopathy.

Hypercalcaemia secondary to acute rhinovirus infection.

Hypercalcaemia-induced rhinovirus has only been reported in a single study in children. Here, we report a case of hypercalcaemia in an adult who tested positive for rhinovirus. This patient underwent an extensive evaluation of hypercalcaemia, and it was found to be mediated by an increase in 1,25 hydroxy-vitamin D that could not be attributed to a cause. Their hypercalcaemia responded to standard treatment with intravascular expansion, bisphosphonates and calcitonin. Serum 1,25 OH vitamin D levels returned to normal with recovery from rhinovirus infection.

A Stroke of Luck: Central Diabetes Insipidus Unmasked by a Heat Stroke.

Diabetes insipidus is a rarely encountered cause of hypernatremia, often presenting a diagnostic and therapeutic dilemma for the encountering physician. Patients are often asymptomatic for a number of years due to compensation of their polyuria with polydipsia, but may have dramatic presentations in situations where they lose access to hydration. Our case is of a 62-year-old woman who was found unconscious with signs and symptoms of a heat stroke, and later was found to have resistant hypernatremia that persisted despite extensive free water supplementation. She had dilute polyuria throughout her hospital course, eventually warranting testing for diabetes insipidus with a vasopressin challenge test. She responded well to therapy with intranasal desmopressin and currently remains asymptomatic. Because our patient was reported to have polyuria and polydipsia for a number of years presumed to be due to underlying diabetes mellitus, it is possible that she had pre-existing central diabetes insipidus that was exacerbated by the lack of access to free water while she was in her intubated and sedated state. Alternatively, she may have also developed new-onset diabetes insipidus due to severe hyperthermia. This case serves to highlight a dramatic presentation of diabetes insipidus, and the importance of careful consideration of its diagnosis in patients with persistent dilute polyuria despite signs of intravascular volume depletion.

Two PerPLEXing Cases of Hashimoto's Encephalopathy Unresponsive to Steroid and Intravenous Immunoglobulin Therapy.

Hashimoto's encephalopathy is a nebulous entity over which much controversy exists. Often referred to as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), it describes a myriad of neurological sequelae that typically are observed to occur in patients with the presence of thyroid antibodies. We aim to raise clinical awareness of this seldom diagnosed entity as a potential etiology for altered mental status in patients who present with supporting clinical features and elevated thyroid antibodies. While steroid responsiveness is deemed a typical presenting feature of this medical condition, our cases aim to describe two cases that required escalation of therapy to intravenous immunoglobulins, and ultimately, plasmapheresis therapy for improvement in their clinical status. Our patients had a dramatic improvement in their mentation within three to four sessions of plasmapheresis, improving rapidly toward their baseline. Such a dramatic improvement, coupled with the corresponding reduction in their thyroid antibody titer supports the diagnosis of Hashimoto's encephalopathy and highlights the importance of having a low clinical threshold for the diagnosis of this entity in patients who, despite extensive evaluation, reveal no apparent cause for their altered mental status.

Pancreatic herniation: a large pancreatic mass concealed within the intrathoracic cavity.

Cancer of unknown primary is a challenging entity. We present an elderly woman with metastatic cancer of unknown primary despite comprehensive imaging and immunohistochemical analysis. Based on a thorough history, a gastrointestinal source was suspected and a diagnosis of pancreatic cancer concealed within a type IV hiatal hernia was made using multimodal imaging. On review of prior imaging, due to the highly complex anatomy within our patient's hiatal hernia, the pancreatic mass was retroactively noted. While initial imaging may detect metastatic disease, identifying the primary malignancy requires a thorough history and physical examination, multimodal imaging where malignancy is suspected, and immunohistochemical analysis of metastatic deposits. Herniation of pancreatic cancer has not been previously described in the literature and serves as an important reminder of the importance of multimodal imaging in patients with significantly complex anatomy.

The Lupus Attack: A Case Report and Literature Review of Myocardial Infarction and Antiphospholipid Syndrome.

Acute myocardial infarction in a young patient is a nebulous entity, but in the absence of traditional cardiovascular risk factors, particular attention must be paid to thrombotic disorders and hypercoagulable states. A 28-year-old male presented with worsening substernal chest pain for 36 hours. He was recently diagnosed with systemic lupus erythematosus (SLE) with active class II lupus nephritis. With an initial electrocardiogram revealing ischemic changes, and an elevated troponin I, a concern was raised for myocardial infarction. Transthoracic echocardiography revealed a severely reduced ejection fraction of 25%, and a subsequent emergent left heart catheterization revealed a complete, massive thrombotic occlusion of the proximal left anterior descending artery, requiring aspiration thrombectomy. After extensive workup for hypercoagulable states, he was found to have elevated anticardiolipin IgG and IgM antibodies on two occasions, twelve weeks apart. The patient was managed with triple anticoagulation with aspirin, clopidogrel, and warfarin for one month, followed by dual anticoagulation with clopidogrel and warfarin with a targetted international normalized ratio (INR) of 2.0 - 3.0. The management of acute coronary syndrome caused by antiphospholipid syndrome (APS) is highly individualized and driven by clinician gestalt owing to the lack of a standardized consensus. While systemic thrombolysis, primary percutaneous coronary intervention (PCI), and coronary artery bypass grafting all have their utility, only a very small handful of case reports exist on the benefits of each. This particular case serves to showcase an instance where a patient was successfully managed with PCI with dual antiplatelet therapy. Further prospective randomized controlled trials are necessary to determine the optimal management of this rarely encountered patient population.

A Breathtaking Hernia: A Giant Hiatal Hernia Masquerading as Poorly Controlled Asthma.

A 93-year-old female presented with persistent shortness of breath and wheezing since the consumption of a meal. Her past medical history is significant for a clinical diagnosis of asthma at the age of 88 years, without pulmonary function testing, complicated by several prior visits to the emergency department (ED) for recurrent exacerbations. Multiple bronchodilators in the ED provided only minimal improvement in her symptoms. Chest imaging eventually revealed a giant, fluid-filled hiatal hernia exhibiting a compressive effect on the posterior aspect of the left atrium. The etiology of the patient's airway bronchoconstriction was likely multifactorial. We hypothesize that the extrinsic, dynamic compression of the bronchial tree by the peristaltic motion of the hiatal hernia, microaspiration from gastroesophageal reflux, and peribronchial edema from left atrial compression accounted for our patient's unique presentation. An outpatient methacholine challenge test eventually excluded bronchial asthma. Although she was considered a poor surgical candidate, she has had no further recurrences of her symptoms with counseling on conservative lifestyle changes. This case serves to highlight the heterogeneity in presentations of hiatal hernias, particularly in elderly females. Furthermore, it remains prudent to maintain a broad differential for wheezing, as evidenced by our patient who was previously managed for a number of years as poorly controlled asthma.

A Case of Multiple Calcific Embolic Strokes in a Patient With a Porcelain Left Atrium.

Mitral annular calcification (MAC) commonly manifests as an incidental, asymptomatic finding that is associated with several cardiovascular risk factors, atherosclerosis, cardiovascular death, and all-cause mortality. Very rarely, patients with severe MAC can have extensive dystrophic calcification extending into the left atrial wall, termed porcelain left atrium. In this case report, we describe a patient who experienced multiple calcific acute embolic strokes in the setting of severe mitral annular calcification and porcelain left atrium. Our patient presented with multiple, small bilateral acute infarcts scattered throughout the cerebrum and cerebellum confirmed on magnetic resonance imaging (MRI). He was placed on continuous telemetry and underwent multimodal imaging with transthoracic and transesophageal echocardiography, carotid neck ultrasound (US), head and neck computed tomography angiogram (CTA), and cardiac MRI. There were no arrhythmic events detected on telemetry, and all imaging excluded left ventricular thrombi, aortic atheroma, carotid artery stenosis, intracardiac shunting, or large vessel stenosis. Noted on imaging, however, was severe mitral annular calcification with numerous, highly mobile calcific extensions and densely calcified plaque along the posterior left atrial wall, presumed to be the source of this patient's embolic stroke. Cardiac catheterization was significant for severe three-vessel disease requiring coronary artery bypass grafting, and our patient was subsequently discharged to outpatient follow-up on event monitoring and aspirin monotherapy. This case serves to highlight a previously unreported complication of calcific embolic stroke in severe MAC and porcelain left atrium, and highlight the need for further randomized controlled trials to determine the optimum management of these cases.