Kounis Syndrome: A Review of an Uncommon Cause of Acute Coronary Syndrome.

Kounis syndrome is a hypersensitivity disorder secondary to allergy or anaphylaxis that can result in acute coronary syndrome. Kounis syndrome has an increasing prevalence since its first identification in 1950. Divided into 3 subtypes, each with diagnostic criteria, the management of Kounis syndrome presents a clinical challenge. We aim to identify the pathophysiological mechanisms and review the diagnosis, epidemiology, management strategies, and future directions of Kounis syndrome. As Kounis syndrome becomes more widely recognized in the medical community, the role of diagnosis, treatment, and future immunomodulatory prevention strategies will continue to unfold.

Intracardiac leadless versus transvenous permanent pacemaker implantation: Impact on clinical outcomes and healthcare utilization.

BACKGROUND: Transvenous permanent pacemakers are used frequently to treat cardiac rhythm disorders. Recently, intracardiac leadless pacemakers offer potential treatment using an alternative insertion procedure due to their novel design. Literature comparing outcomes between the two devices is scarce. We aim to assess the impact of intracardiac leadless pacemakers on readmissions and hospitalization trends. METHODS: We analyzed the National Readmissions Database from 2016 to 2019, seeking patients admitted for sick sinus syndrome, second-degree-, or third-degree atrioventricular block who received either a transvenous permanent pacemaker or an intracardiac leadless pacemaker. Patients were stratified by device type and assessed for 30-day readmissions, inpatient mortality, and healthcare utilization. Descriptive statistics, Cox proportional hazards, and multivariate regressions were used to compare the groups. RESULTS: Between 2016 and 2019, 21,782 patients met the inclusion criteria. The mean age was 81.07 years, and 45.52 % were female. No statistical difference was noted for 30-day readmissions (HR 1.14, 95 % CI 0.92-1.41, p = 0.225) and inpatient mortality (HR 1.36, 95 % CI 0.71-2.62, p = 0.352) between the transvenous and intracardiac groups. Multivariate linear regression revealed that length of stay was 0.54 (95 % CI 0.26-0.83, p < 0.001) days longer for the intracardiac group. CONCLUSION: Hospitalization outcomes associated with intracardiac leadless pacemakers are comparable to traditional transvenous permanent pacemakers. Patients may benefit from using this new device without incurring additional resource utilization. Further studies are needed to compare long-term outcomes between transvenous and intracardiac pacemakers.

Atypical Stress Cardiomyopathy: A Case Report.

Stress cardiomyopathy is a reversible cause of cardiomyopathy characterized by a transient dysfunction in left ventricular systolic function. It is most common in postmenopausal women and usually occurs following an emotional and/or physical stressor. The classical imaging finding is described as left ventricular apical ballooning. However, several rare variants have been reported with a strikingly different regional distribution of wall motion abnormalities. We describe a case of a 65-year-old female who was found to have stress cardiomyopathy with variant wall motion abnormality on the left ventriculogram without a preceding stressor event. We postulate that there may be a link between stress-induced cardiomyopathy without a preceding stressor event and variant wall motion abnormality patterns.

Anti-MDA5 Associated Clinically Amyopathic Dermatomyositis With Rapidly Progressive Interstitial Lung Disease.

Anti-melanoma differentiation-associated protein 5 (anti-MDA5) associated clinically amyopathic dermatomyositis (CADM) is a rare entity that is frequently associated with rapidly progressive interstitial lung disease. The disease is characterized by its association with a distinct myositis specific antibody, the lack of muscle involvement seen with other inflammatory myopathies, and a strong correlation with the development of rapidly progressive interstitial lung disease. Diagnosis is based on clinical findings and the presence of autoantibodies. Management generally involves combination immunosuppression therapy. However, the disease course is often aggressive and lends a poor prognosis. We report a case of a healthy 55-year-old male who presented with dyspnea, dry cough, and joint pain for 1 month. The patient was diagnosed with anti-MDA5 associated CADM with interstitial lung disease after a complete rheumatological workup found elevated titers of MDA5 antibodies and computed tomography of the chest without contrast revealed radiographic evidence of interstitial lung involvement. Disease course was complicated by the development of Pneumocystis pneumonia as a result of profound immunosuppression from combination immunosuppressant therapy. Our patient eventually succumbed to his illness approximately 10 weeks following initial symptom onset. This case highlights the aggressive nature of the disease and the challenges in management. Further research is warranted to establish more effective therapeutic options.

Arrhythmogenic Right Ventricular Cardiomyopathy: The Role of Genetics in Diagnosis, Management, and Screening.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a predominantly autosomal dominant genetic condition in which fibrous and fatty tissue infiltrate and replace healthy myocardial tissue. This uncommon yet debilitating condition can cause ventricular arrhythmias, cardiac failure, and sudden cardiac death. Management focuses primarily on prevention of syndrome sequelae in order to prevent morbidity and mortality. Genetic testing and screening in affected families, although utilized clinically, has not yet been incorporated in guidelines due to lack of larger studies and data. We aim herein to identify causative gene mutations, present advancements in diagnosis and management, and describe the role of genetic screening and counseling in patients with ARVC. With the advancement of genetic testing and therapy, diseases such as ARVC may become more accurately diagnosed and more effectively managed, ultimately significantly reducing morbidity and mortality.

Methotrexate Failure in the Treatment of Adult-Onset Still's Disease: A Case Report.

Adult-onset Still's disease (AOSD) is a rheumatological condition associated with significant morbidity and mortality. Typically a diagnosis of exclusion, the therapeutic management has relied mainly on symptom control and immune suppression. Methotrexate (MTX), a disease-modifying anti-rheumatoid drug (DMARDs), has become a drug of choice in treating several autoimmune conditions, including AOSD. Unfortunately, despite being largely effective, this medication can result in treatment failure, exacerbation, and a flare of symptoms. We present the case of a 31-year-old male who presented to us with weakness and palpitations, who was ultimately found to have a flare of his Still's disease, despite being on MTX therapy. Our hope is to encourage a suspicion for treatment failure in patients with similar symptoms, in order to encourage a faster initiation of alternative therapies to alleviate their discomfort.

Cardiac Sarcoidosis: A Unique Presentation.

Isolated cardiac sarcoidosis is a rare subset of sarcoidosis, a systemic autoimmune condition primarily found in African American females. The manifestations of cardiac sarcoidosis include atrioventricular and bundle branch blocks, arrhythmias, heart failure, and pericardial effusions, although these complications occur at varying prevalence. The diagnosis of cardiac sarcoidosis requires several different criteria; however, recent literature has focused heavily on imaging modalities such as cardiac magnetic resonance imaging. We present a case of a 42-year-old Caucasian male who was found to have unexplained cardiac arrhythmias and ultimately diagnosed with cardiac sarcoidosis by imaging modalities.

A Rare Case of Superior Vena Cava Syndrome in a Patient With Rheumatoid Arthritis and IgA Nephropathy.

Superior vena cava syndrome (SVCS) is a vascular condition resulting from an impaired venous return to the right atrium. The majority of SVCS cases are caused by mass effect in which extrinsic compression of the vessel leads to obstruction of blood flow. In less common cases of SVCS, thrombus formation and luminal narrowing can result in poor return through the SVC. Inflammatory causes of SVCS are even rarer and poorly documented. IgA nephropathy and rheumatoid arthritis (RA) are two autoimmune diseases with the potential to cause vasculitis, thus increasing the likelihood of intraluminal vessel occlusion. We report a rare case of SVCS in a 65-year-old female with a past medical history significant for atrial fibrillation, IgA nephropathy, chronic kidney disease stage IIIA, and RA who presented with headache, dizziness, and neck pain and swelling extending down the left upper extremity for three days. Inflammatory SVCS is uncommon and cases of SVCS secondary to RA and IgA nephropathy are underreported in the literature thus far. Our hope in presenting this case is to encourage a greater degree of suspicion for vascular complications, such as SVCS, in patients with autoimmune and inflammatory conditions.

Acute Liver Failure due to Altered Fontan Circulation in a Patient With Tricuspid Atresia: A Case Report.

Tricuspid atresia is a congenital malformation of the tricuspid valve resulting in a lack of blood flow between the right atrium and the right ventricle. Management generally involves staged surgical intervention enabling affected individuals to survive into adulthood. Although surgical intervention greatly improves morbidity and mortality in this patient population, there are many long-term complications associated with the creation of a surgical shunt. We report a case of a 33-year-old male with tricuspid atresia who underwent Fontan surgery as a child and presented to our hospital with acute liver failure.

Supraventricular Tachycardia: An Atypical Presentation of Thyroid Storm.

Thyroid storm (or thyrotoxic crisis) is commonly defined as a life-threatening condition caused by the exaggeration of the clinical manifestations of thyrotoxicosis. Supraventricular tachycardia (SVT) is an atypical precipitating symptom of thyrotoxicosis that clinicians should be aware of. An empirically derived scoring system known as the Burch-Wartofsky Point Scale (BWPS) has been used by clinicians since the early 1990s. The BWPS considers an array of precipitating factors and the severity of symptoms of multiple organ decompensation. In recent years, there has been an increasing correlation between SARS-CoV-2 and thyroid pathologies. We present a case of an unresponsive elderly male with a recent coronavirus disease 2019 (COVID-19) infection presenting with SVT and a BWPS score of 45, highly indicative of a thyroid storm.

Idiopathic Pleuroparenchymal Fibroelastosis.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia. The disease is characterized by fibrosis of the pleura and subpleural lung parenchyma predominantly affecting the upper lobes. Various triggers have been proposed as inciting factors in the development of the disease. Diagnosis is made clinically in conjunction with radiographic findings and histopathology when available. There are no known effective treatment options and several cases of lung transplantation have been reported. We report a case of an 86-year-old female who presented to the emergency department with worsening dyspnea and hypoxia. She had a history of unexplained pneumomediastinum and a 20 - 25 pounds unintentional weight loss over 10 months. Computed tomography (CT) of the chest without contrast revealed radiographic evidence of IPPFE. Despite symptomatic management with antibiotics, diuretics, and steroids, her condition continued to deteriorate. Unfortunately, our patient was not a candidate for a lung transplant. She was transitioned to hospice care and succumbed to her disease. IPPFE is a rare disease with an unknown prevalence. It has a median survival rate of 2 years. Usually, there is an overlap with interstitial lung diseases, making it challenging to diagnose. There are only a few cases reported in the literature, and there are currently no guidelines available on the appropriate management of this debilitating disease. We recommend more cases be reported, and further research is done to establish better criteria for diagnosis and management.

Neuromyelitis Optica Spectrum Disorder: A Rare Cause of Spinal Cord and Optic Nerve Involvement.

Neuromyelitis optica spectrum disorder (NMOSD) is defined as a rare central nervous system, demyelinating, autoimmune disorder with autoantibodies against aquaporin-4. Commonly affecting females, NMOSD is known to also be a relapsing disease and can increase in severity during each episode. Diagnostic criteria include ruling out multiple sclerosis, spinal magnetic resonance imaging, and autoantibody detection. While management focuses on relapse treatment and prevention, high-dose steroids and plasma exchange have proven to be useful options. We present a case of a young female presenting with NMOSD relapse appropriately managed with plasma exchange.

Iatrogenic Infective Endocarditis With Septic Emboli: An Unusual Complication of Intracardiac Manipulation.

Radiofrequency catheter ablation (RFA) is an effective treatment for recurrent symptomatic atrial fibrillation (AF) despite medical therapy. Complications include cardiac tamponade, pulmonary vein stenosis, and rarely atrial-esophageal fistula (AEF). We present a case of a 77-year-old male patient with a history of persistent AF status post posterior left atrial isolation 50 days prior, who presented to the emergency department with bilateral lower extremity weakness ongoing for an hour. In addition, he reported a non-productive cough, chest discomfort, and dysphagia. He was hypertensive, febrile, tachycardic, and with tachypnea on initial assessment. Initial labs revealed leukocytosis with elevated troponin level. Brain imaging confirmed subacute infarcts suggestive of embolic disease. Tissue plasminogen activator was contraindicated. He was admitted for management of altered mental status and sepsis of an unknown origin. Due to associated chest discomfort and dysphagia, computed tomography (CT) scan of the chest with contrast obtained revealed a small focus of air between the posterior wall of the left atrium and the esophagus. A few hours later, he deteriorated with worsened hypoxic respiratory failure, altered mental status, requiring intubation and mechanical ventilation. Cardiothoracic surgery was consulted, and he was taken to the operating room for open-heart surgery due to concern for AEF. Peri-operatively, a left atrial fistula measuring 0.5 cm was identified and closed. Also, a fibrinous material (3 × 4 cm) was identified and removed in the left inferior pulmonary vein. On post-operative day 1, he underwent an upper endoscopy, and a 1 cm esophageal defect was closed. Biopsy from fibrinous material was positive for polymicrobial organisms (lactobacillus, candida, and prevotella), while blood cultures grew Streptococcus anginosus. Despite treatment with aggressive intravenous antibiotics, the patient deteriorated with poor neurological function and ventilator dependency. A multidisciplinary team reached a consensus agreement for palliative measures. To our knowledge, this case represents one of the few cases documenting life-threatening infective endocarditis with septic emboli as a complication of AEF from RFA. Therefore, clinicians should have a high index of suspicion due to associated grave prognosis.

Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease.

BACKGROUND: Patients with sickle cell disease can experience various crises including sequestration crisis, haemolytic crisis and aplastic crisis. Due to alloantibody formation, transfusion alloantibodies can cause a haemolytic crisis. Treatment involves avoiding packed red blood cell transfusions, as well as intravenous immunoglobulin, steroids and eculizumab to decrease the chances of haemolysis. CASE DESCRIPTION: We report the case of a 42-year-old man who was found to have worsening anaemia after packed red blood cell transfusion with evidence suggestive of haemolytic crisis. Due to reticulocytopenia, aplastic crisis was also suspected and later confirmed via parvovirus IgG and IgM titres. The patient did not improve with steroid and intravenous immunoglobulin therapy and was treated with eculizumab as a salvage therapy. CONCLUSION: Concurrent hyper-haemolytic crisis and aplastic crisis should be suspected in patients with features of haemolysis and reticulocytopenia. Prompt recognition and treatment with eculizumab are paramount in those who fail steroid and intravenous immunoglobulin treatment. LEARNING POINTS: Treatment of hyper-haemolytic and aplastic crisis in sickle cell disease with eculizumab offers therapeutic benefit.A high index of suspicion for hyper-haemolytic crisis and aplastic crisis should be maintained in those with haemolytic features as well as reticulocytopenia in the setting of sickle cell disease.

Anomalous Coronary Arteries: A Case of Rare and Incidental Findings.

Anomalous coronary artery from the opposite sinus (ACAOS) is an uncommon, yet highly variable anatomical abnormality. These coronary anatomy variants are often discovered incidentally during cardiac catheterization. These variants can be challenging intraoperatively and require adjustment by the operator. We present the case of a 93-year-old female who presented for shortness of breath due to severe mitral regurgitation (MR), who was found to have an anomalous left main coronary artery (LMCA) from the right sinus of Valsalva (RSOV). This asymptomatic finding was managed conservatively and patient underwent successful MitraClip treatment.

Rare Case of Small Bowel Obstruction Secondary to Cryptosporidium in a Young Patient With Uncontrolled AIDS.

Small bowel obstruction is a common cause of abdominal pain and accounts for approximately 20% of surgical admissions related to abdominal pain. In the United States alone, there are over 300,000 admissions annually for small bowel obstruction and account for every 15 out of 100 admissions for abdominal pain. If treated appropriately with medical management, over 80% of cases resolve without life-threatening, long-term complications or the need for surgical intervention. The three most common causes including adhesions, tumors, and hernias account for the majority of cases. Less frequently reported causes include infections. We present the case of a 26-year-old male with a history of AIDS who was found to have a small bowel obstruction in the setting of active Cryptosporidium infection. Cryptosporidium is an opportunistic infection that more commonly affects immunocompromised hosts, especially those noncompliant with antiretroviral therapy. Our patient had an uncomplicated hospital course and made a full recovery due to early diagnosis and immediate intervention. We hope to make the medical community more aware of this rare and potentially life-threatening association given the rarity of such a presentation. Early diagnosis and intervention are critical to preventing morbidity and mortality.

Atypical Presentation of Plasma Cell Neoplasm of the Sternum.

Multiple myeloma (MM) is a rare plasma cell neoplasm characterized by monoclonal cell infiltration in the bone marrow, which can cause anemia, bone pain, and recurrent infections. Extramedullary myeloma (EM) is a rare clinical presentation with a poor prognosis. It involves the accumulation of clonal plasma cells in soft tissues with a tumor-like appearance, either presenting as a primary (initial) or secondary (relapse) malignancy. We present a case of a 65-year-old male who experienced an abrupt onset of chest pain associated with a localized sternal mass while exercising the day prior to arrival. Chest computed tomography (CT) scan with contrast revealed an expansile lytic lesion around the sternal area. Due to high suspicion for malignancy, a CT-guided core needle biopsy was done, which showed plasma cells with rare Dutcher bodies consistent with MM. Bone marrow smear showed the presence of 70% plasma cells confirming a diagnosis of MM. Early detection of this devastating disease may help improve survival. Therefore, physicians should have a high index of suspicion for MM in older patients with similar clinical presentations.

Sprue-Like Enteropathy and Liver Injury: A Rare Emerging Association with Olmesartan.

Olmesartan-induced enteropathy is an underreported phenomenon, first described in 2012. While olmesartan's antihypertensive properties were confirmed early on, its association with a sprue-like enteropathy was subsequently noted. Although this association has been reported with olmesartan, there have been few reports of this association with other angiotensin-receptor blockers. We present a case of a 79-year-old male who presented with diarrhea, weight loss, jaundice, and transaminitis. Further history revealed that he had been taking olmesartan 40 mg daily for hypertension. Workup of his diarrhea and jaundice included duodenal and liver biopsies revealed findings consistent with a sprue-like enteropathy and an autoimmune hepatitis-like pattern. On discontinuation of olmesartan, his 1-month follow-up revealed significant improvement in his clinical status as well as his liver function tests. Olmesartan is an effective antihypertensive medication; however, physicians must be mindful of its side effect of causing a sprue-like enteropathy and liver injury. Patients should be counseled on discontinuing olmesartan, and they should be started on an alternative therapy for hypertension.

Staphylococcus capitis Endocarditis of a Native Valve.

Described as primarily an opportunistic pathogen, Staphylococcus capitis is primarily found as normal flora of the human skin but has been defined in literature as being a rare cause of infective endocarditis. We present a case of an otherwise healthy 65-year-old male who presented to our institution with symptoms similar to septic emboli. Blood cultures were obtained and ultimately grew Staphylococcus capitis in both bottles with repeat cultures one day later confirming the growth. A transthoracic echocardiogram was performed showing an ejection fraction of 60-65% and a thickened mitral value with mild-to-moderate mitral regurgitation. He was ultimately treated with IV cefazolin and improved with symptom resolution in outpatient follow-up. Staphylococcus capitis pathogenesis continues to be poorly understood, yet aggressive management with surgery and antibiotics has proven to decrease morbidity and mortality.

Trimethoprim-Sulfamethoxazole-Induced Aseptic Meningitis: A New Approach.

Inflammation of the meningeal linings of the central nervous system (CNS), also known as meningitis, is one of the serious presentations in the emergency because it carries high morbidity and mortality. The most common cause is pus-producing organisms. However, non-suppurative meningitis, termed aseptic meningitis, is another cause of meningeal inflammation. Many etiologies stand behind aseptic meningitis. Those etiologies include viral and non-viral, drug-induced, malignancy, and systemic inflammation. Drug-induced aseptic meningitis is a rare type of meningitis. Although it is easily treated, it can be a challenging disease if not present in the differential diagnosis. It is commonly associated with nonsteroidal anti-inflammatory drugs (NSAIDs). Nonetheless, other medications have been also reported to cause aseptic meningitis, including antibiotics. Trimethoprim-sulfamethoxazole (TMP-SMX) is one of the most prescribed antibiotics as a prophylactic and therapeutic drug due to its effectiveness and low cost. Although immunocompromised patients are at a higher risk to develop aseptic meningitis, immunocompetent patients are also at risk. Unrelated to the source of the infection, TMP-SMX carries a risk of aseptic meningitis and should be considered as an etiology in patients presenting with meningeal signs and symptoms. Hereby, we report a young immunocompetent patient who developed aseptic meningitis eight days after being prescribed TMP-SMX. Like all drug-induced aseptic meningitis, all his symptoms resolved two days after stopping the medication.