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Systemic Lupus Erythematosus represents a chronic autoimmune disorder characterized by multiorgan involvement. Lupus myocarditis is a rare presentation of one of the cardiac complications of lupus with an incidence of 3-9%. It usually presents with non-specific symptoms such as dyspnea, orthopnea, chest pain, pedal edema, fever, diaphoresis, paroxysmal nocturnal dyspnea, nausea, vomiting, or palpitations. Even though endomyocardial biopsy is considered the gold standard diagnostic approach, other non-invasive diagnostic alternatives including cardiac magnetic resonance (CMR) have been studied. Therapeutic interventions may range from high-dose steroids, and IVIG, to the most advanced strategies such as mechanical circulatory support including VenoArterial Extracorporeal Membrane Oxygenation (VA-ECMO), and Impella, among others.
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Myocarditis is a rare extraintestinal manifestation of inflammatory bowel disease. Myopericarditis-associated inflammatory bowel disease can be a side effect of the medications used to treat inflammatory bowel disease or the disease process. We present a 25-year-old female with history of ulcerative colitis presented with abdominal pain associated with sharp and central chest pain. She was in a flare of ulcerative colitis with bloody diarrhea. She developed shock and was in intensive care unit. Echocardiogram showed reduced ejection fraction and pericardial effusion. Coronary artery disease, sepsis, thyroid disease were ruled out. She was treated with systemic antibiotics, intravenous steroids, and guideline-directed medical therapy for presumed ulerative colitis associated with myopericarditis and had symptomatic improvement. Treatment of IBD-associated myopericarditis includes the standard induction treatment for IBD with steroids and guideline-directed medical therapy for heart failure.
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BACKGROUND Acute aortic insufficiency can be secondary to multiple conditions, including infective endocarditis, aortic root pathologies (eg, dissection, aortitis), or traumatic injury. Aortitis involves a broad spectrum of disorders characterized by inflammatory changes in the aortic wall. This pathology can be subsequently classified depending on its etiology into inflammatory and infectious causes. Large-vessel vasculitis (giant-cell arteritis, Takayasu arteritis, and IgG4-related vasculitis) is the most common non-infectious causes of aortitis. Giant-cell aortitis usually lacks the classic clinical findings of giant-cell arteritis such as headache, visual symptoms, or jaw claudication, which can be a diagnostic challenge. However, clinicians should have a high index of suspicion, since this pathology can evolve into potentially life-threatening conditions, including aortic aneurysm, aortic wall rupture, and aortic acute dissection. CASE REPORT We present a case of a 76-year-old woman who presented to the Emergency Department (ED) with shortness of breath associated with orthopnea, paroxysmal nocturnal dyspnea, and mild productive cough with white sputum. A transthoracic echocardiogram demonstrated reduced left ventricular ejection fraction, dilated left ventricle, and severe aortic insufficiency. Cardiac catheterization revealed mild non-obstructive coronary arteries and severe aortic regurgitation. The surgical pathology report of the portion of the aorta was consistent with giant-cell aortitis. CONCLUSIONS In this article, we present a case of giant-cell aortitis as an unusual etiology of acute aortic insufficiency, which is most probably under-detected in clinical practice. In addition to describing the case, we aim to highlight the importance of proper ascending aorta evaluation in patients presenting with new-onset aortic regurgitation and heart failure to prevent associated morbidity and mortality.
Assuntos
Ruptura Aórtica , Insuficiência da Valva Aórtica , Aortite , Arterite de Células Gigantes , Arterite de Takayasu , Feminino , Humanos , Idoso , Aortite/complicações , Aortite/diagnóstico , Insuficiência da Valva Aórtica/complicações , Volume Sistólico , Função Ventricular Esquerda , Aorta , Arterite de Takayasu/complicações , Arterite de Takayasu/diagnóstico , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnósticoRESUMO
Despite the lack of direct evidence that hypertension increases the likelihood of new infections, hypertension is known to be the most common comorbid condition in COVID-19 patients and also a major risk factor for severe COVID-19 infection. The literature review suggests that data is heterogeneous in terms of the association of hypertension with mortality. Hence, it remains a topic of interest whether hypertension is associated with COVID-19 disease severity and mortality. Herein, we perform a multicenter retrospective analysis to study hypertension as an independent risk for in-hospital mortality in hospitalized COVID-19 patients. This multicenter retrospective analysis included 515 COVID-19 patients hospitalized from March 1, 2020 to May 31, 2020. Patients were divided into two groups: hypertensive and normotensive. Demographic characteristics and laboratory data were collected, and in-hospital mortality was calculated in both groups. The overall mortality of the study population was 25.3% (130 of 514 patients) with 96 (73.8%) being hypertensive and 34 (26.2%) being normotensive (p-value of 0.01, statistically non-significant association). The mortality rate among the hypertensive was higher as compared to non-hypertensive; however, hypertensive patients were more likely to be old and have underlying comorbidities including obesity, diabetes mellitus, coronary artery disease, congestive heart failure, stroke, chronic kidney disease (CKD), chronic obstructive pulmonary disease (COPD), and cancer. Therefore, multivariable logistic regression failed to show any significant association between hypertension and COVID-19 mortality. To our knowledge, few studies have shown an association between hypertension and COVID-19 mortality after adjusting confounding variables. Our study provides further evidence that hypertension is not an independent risk factor for in-hospital mortality when adjusted for other comorbidities in hospitalized COVID-19 patients.
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The left ventricular outflow tract is a region of the left ventricle that lies between the anterior leaflet of the mitral valve and the ventricular septum. Dynamic left ventricular outflow tract obstruction (LVOTO) has classically been observed in patients with hypertrophic obstructive cardiomyopathy (HOCM) where it occurs secondary to asymmetric septal hypertrophy and systolic anterior motion (SAM) of the mitral valve. However, there are some instances that lead to hypercontractility of the myocardium, and with a combination of other physiological conditions, result in SAM of a mitral valve leaflet, leading to LVOTO in the absence of phenotype. We present such a case of an acute inferolateral wall myocardial infarction that was complicated by cardiogenic shock, requiring an intra-aortic balloon pump (IABP) and inotropic support which paradoxically provoked LVOTO. A reduction in IABP counterpulsation from 1:1 to 1:3 and the addition of intravenous fluids and a beta blocker resulted in significant improvement in blood pressure with rapid tapering of pressors. Inotropes and IABP, although helpful in cardiogenic shock, have the potential to induce or worsen the LVOTO, which can lead to a vicious cycle of worsening hypotension and increasing adrenergic drive that further deteriorates myocardial viability. Timely diagnosis with an echocardiogram and the withdrawal of inotropic and IABP support has the potential to improve haemodynamics and, thereby, outcome. LEARNING POINTS: Dynamic left ventricular outflow tract obstruction (LVOTO) should be one of the differentials in patients with cardiogenic shock, especially if it is refractory in the setting of an intra-aortic balloon pump.The diagnosis of LVOTO by echocardiography should result in immediate implementation of therapy to eliminate the factors that can potentially intensify the obstruction.
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HIV infection is associated with multisystemic manifestations due both to secondary infections caused by a decrease in the CD4+ T-cell count and to the pathogenicity of the HIV virus itself. A common renal manifestation is HIV-associated nephropathy, which is frequently seen in the African population with the APOL1 gene mutation; however, other forms of glomerulopathy such as IgA nephropathy, commonly noted in other ethnicities, are also seen. Vasculitis has rarely been associated with HIV infection and mainly involves small blood vessels, although any size of blood vessel may be involved. The association of Henoch-Schonlein purpura (HSP) with HIV is rare and not well understood. We describe a 53-year-old African American woman with a newly diagnosed HIV infection who presented with a purpuric rash over the bilateral lower extremities with haematuria. Initial work-up revealed renal dysfunction with elevated ESR. Urinalysis was positive for glomerular haematuria and sub-nephrotic range proteinuria. Serum complement level, c-antineutrophil cytoplasmic antibody (ANCA), p-ANCA and anti-nuclear antibody (ANA) were negative. Renal biopsy revealed mesangial IgA deposits with crescent glomerulopathy and fibrinoid necrosis, while skin biopsy revealed leucocytoclastic vasculitis. A diagnosis of HSP was made based on American College of Rheumatology (ACR) criteria. The patient's renal function and purpura improved with a 5-day course of steroid pulse therapy. This case of HSP in a newly diagnosed HIV patient is unusual for the presence of crescentic glomerulopathy. LEARNING POINTS: Henoch-Schonlein purpura (HSP) associated with HIV infection is uncommon but documented; however, all four features of HSP are rarely seen together.Crescent glomerulopathy is rarely seen in HIV-associated HSP.HSP associated with HIV is treated with antiretroviral drugs, while the role of steroid and immunosuppressive therapy remains controversial.
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The heart is a rare site of metastatic lesions of malignancies. Cardiac metastasis may go unrecognized till autopsy, and about half of all newly diagnosed lung cancer patients have metastasis to another organ. Due to the absence of early symptoms, the clinical diagnosis of cardiac metastasis is challenging. Even when they are symptomatic, these symptoms may be masked by the clinical features of primary cancer. Noncardiac neoplasms may spread to the heart through lymphatic or hematogenous dissemination, local extension, or a transvenous route. Here, we report a case of a 56-year-old male with lung mass extending from the right upper lobe to the left atrium was associated with mass effect on superior vena cava and left brachiocephalic vein.
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Left ventricular thrombus (LVT) formation is a serious clinical complication of low-flow states that may be seen in an ischaemic, arrhythmic heart. While LVT formation has a poor prognosis, in the setting of myocardial infarction it is usually a result of post-infarct sequelae such as left ventricle aneurysms, and inflammatory changes from damaged tissue, with the LVT taking several days to form. Arrythmias such as ventricular tachycardia (VT) or ventricular fibrillation (VF) may also lead to thrombus formation, as they contribute to stasis due to decreased cardiac output. Large anterolateral myocardial infarctions can cause electrical or arrhythmic storm, characterized by more than three episodes of VT or VF in a 24-hour period. This prolonged state of dyskinesis further increases the risk of thrombosis, creating a compounding effect. Here, we report the case of a patient who had a VF cardiac arrest with electrical storm secondary to anterolateral myocardial infarction complicated with LVT formation found on echocardiogram after the cardiac arrest, which was absent on presentation. This thrombus formation occurred particularly early during the course of the patient's arrest, possibly due to the compounding factors increasing the risk of thrombosis. Herein, we discuss in detail the risk factors for LVT formation, its mechanism and management options. A review of the literature also shows that LVT formation in the acute phase of arrest, as seen in our patient, is rare. LEARNING POINTS: Left ventricular thrombus (LVT) formation occurs 3-14 days after myocardial infarction, but in the setting of concomitant ventricular fibrillation arrest, may occur within the first 24 hours.Risk factors for LVT formation include a large infarct, anterior/anterior apical infarction, decreased ejection fraction (particularly <30-35%), left ventricular aneurysm, and delayed time to revascularization.Although diagnosis is generally made on transthoracic echocardiography with intravenous contrast, cardiac MRI with contrast has better sensitivity and specificity.Treatment consists of anticoagulation with a vitamin K antagonist or heparin for 3-6 months with a repeat echocardiogram to confirm the thrombus has organized or resolved. Further trials are needed to assess the efficacy of direct oral anticoagulants.
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The eustachian valve (EV) is a vestigial structure found at the junction of the inferior vena cava and the right atrium, a remnant of the embryological sinus venosus that may persist throughout life. Right-sided infective endocarditis of the eustachian valve remains a distinctly rare and under-diagnosed entity. Commonly known risk factors of eustachian valve endocarditis (EVE) are intravenous drug use, in-dwelling intracardiac devices, and central lines, although more recently immunocompromised states, e.g. uncontrolled diabetes mellitus and old age, have been recognized as risk factors for the disease. Although Staphylococcus aureus has been the most commonly implicated organism, cases of infections with gram-negative organisms are emerging. We present a 47-year-old male with uncontrolled type 1 DM who initially presented to the ED with complaints of low back pain and dysuria and was later found to have eustachian valve endocarditis ultimately treated with intravenous antibiotics.
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The emergence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has triggered a global health pandemic that led to substantial morbidity and mortality worldwide. The virus has been known to predominantly cause severe hypoxemic respiratory failure but there have been multiple reports of extra-pulmonary manifestations. Additionally, there has been increasing evidence of COVID-19 hyper-coagulability. Herein, we present a case of a 49-year-old male with a past medical history of diet controlled type II diabetes mellitus and recently diagnosed COVID-19 who presented to the emergency department with a chief complaint of nausea and vomiting. Our patient was found to have a thrombus-like appearing 1.9 cm × 1.2 cm well-circumscribed mass, attached to the greater curvature of the ascending aorta, superior to the right coronary cusp of the aortic valve almost three weeks after his initial diagnosis of COVID-19 virus.
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Wolf-Parkinson-White (WPW) syndrome is a congenital heart condition in which the atrioventricular (AV) node is bypassed by an accessory pathway that connects the atria and ventricle directly. WPW syndrome in patients with a history of peripartum cardiomyopathy (pregnancy-related cardiomyopathy) is associated with a high risk of morbidity and mortality secondary to failure of the pump and the conduction system of the heart. Management of these cases deals with arrhythmia and systolic heart failure, which becomes more challenging in pregnant patients as it requires treatment methods that minimize risks to the fetus. We report a case of a young female patient with WPW syndrome and postpartum cardiomyopathy presenting with symptomatic arrhythmias (tachycardia).
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Syphilis is a sexually transmitted disease spread by spirochete Treponema Pallidum, it has a varied range of symptoms and is divided into stages primary, secondary and tertiary. Central nervous system (CNS) invasion occurs early in the disease in almost all the patients, and does not follow any particular stage. However, clinical manifestation depends on whether inflammatory response occurs. (1)(2) Early neurosyphilis typically affects cerebrospinal fluid (CSF) and meninges presenting like meningitis, while late affects the brain and spinal cord parenchyma, presenting as tabes dorsalis and paresis. Here we present a case of a patient with symptomatic neurosyphilis presenting with CSF findings of bacterial meningitis.
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Infective endocarditis is a multisystem disease. Tricuspid valve endocarditis is frequently seen in patients with intravenous (IV) drug users. Cavitating lung nodules predominantly in a peripheral location in IV drug users indicate the possibility of septic emboli. Large vegetation and persistent bacteremia with septic embolic phenomena are the most common indication for surgery. We present a case of a 62-year-old male with a history of IV drug use who presented with epigastric abdominal pain, pleuritic chest pain, and shortness of breath. CT chest showed cavitating lung nodules suggestive of septic pulmonary emboli. A transesophageal echocardiogram (TEE) showed tricuspid valve vegetation despite a normal transthoracic echocardiogram. The patient was treated with intravenous antibiotics. He was deemed a poor surgical candidate; therefore, he was transferred to a tertiary center for AngioVAC (AngioDynamics, Latham, New York).
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Cocaine is considered a leading non-opioid cause of drug overdose in the US. It acts as a sympathomimetic and increases the amount of catecholamines, thereby increasing the risk of ventricular irritability and resultant arrhythmias. Its sodium (Na) channel blockage is the principal mechanism behind the Brugada pattern on an electrocardiogram (ECG), which is often transient but is indistinguishable from that of Brugada syndrome, the autosomal dominant channelopathy. We are presenting a case of a 32-year-old male with a history of cocaine and nicotine abuse, who sought medical attention for sudden-onset palpitations and pressure-like chest pain after having snorted an impressive amount of cocaine. His ECG depicted a classical Brugada pattern with ST-elevation with T inversion in V1; however, previous ECGs were normal without ST changes, signifying the Brugada pattern unmasked by cocaine use. Other investigations including stress tests and nuclear imaging were equivocal. His symptoms as well as the ECG pattern reverted to baseline signifying the presence of Brugada phenotype in the absence of channelopathy. Hence, as a sodium channel blocker, cocaine may unmask latent Brugada syndrome in asymptomatic patients without a family history. Recognizing Brugada syndrome on ECG is vital to avoid misdiagnosis and mistreatment of the patient with and without a genetic predisposition.
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Cardiac and neurological disorders are the main broad etiologies for loss of consciousness. Ictal bradycardia syndrome refers to epileptic discharges that profoundly disrupt normal cardiac rhythm, resulting in cardiogenic syncope during the ictal event. Convulsive syncope is a well-described phenomenon in both adults and children in which abrupt cerebral hypoperfusion leads to brief extensor stiffening and non-sustained myoclonus. Sick sinus syndrome or tachycardia bradycardia syndrome is a common cause of arrhythmias in the elderly secondary to sinus node dysfunction. We present a case of a 91-year-old male who presented with generalized seizure with associated bradyarrhythmias with telemetry showing sinus rhythm, followed by severe bradycardia, followed by Ventricular tachycardia, followed by an episode of asystole, which likely precipitated seizures as a result of cerebral hypoperfusion. The patient had a permanent dual-chamber pacemaker. He was discharged on antiepileptics as his EEG was abnormal, which might indicate an underlying predisposition.
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Immune thrombocytopenia (ITP) is a bleeding disorder characterized by a decreased number of platelets. It is an immune system-mediated condition, with formation of antibodies against a structural platelet antigen. Although the pathogenesis remains elusive, primary disease is idiopathic and comprises 80% of cases. However, quite a few secondary causes have been established including Helicobacter pylori, varicella-zoster virus and cytomegalovirus. A few cases with an incidental association with herbal medications have been reported, but this causality has not been studied in detail. Here we present the case of 38-year-old African-American woman who presented with symptomatic thrombocytopenia, with a platelet count of 5 K/µl 1 week after she had consumed herbal tea containing Rumex crispus (yellow dock) and Arctium lappa (burdock). The association between unstudied herbs and ITP needs further research, given the widespread use of these substances and ongoing public uncertainty about their benefits. LEARNING POINTS: Over-the-counter herbal products marketed as having beneficial effects are not always harmless and can have clinically significant side effects.When investigating the causes of thrombocytopenia, physicians should be aware of and search for this rare cause.Treatment for herbal tea-induced thrombocytopenia is the same as for immune thrombocytopenia (i.e., steroids and platelet transfusion if there is actively bleeding).
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The growing prevalence of obesity in the USA has resulted in increased consumption of weight loss products that promote fat metabolism. Dietary supplements used for weight loss contain a wide variety of ingredients but the amount of scientific information available on these ingredients varies considerably. Such supplements have documented and undocumented adverse effects. Although the FDA frequently issues health advisories, the health consequences of consuming supplements are often overlooked by the general public. A common supplement used for weight loss is Hydroxycut. The ingredients used in the different forms of Hydroxycut products vary but generally include caffeine and green tea extract, which are responsible for a wide range of adverse effects. We present the case of 41-year-old man with a medical history of polysubstance abuse who developed acute compartment syndrome in the setting of rhabdomyolysis from prolonged immobilisation and the use of Hydroxycut. This case demonstrates the possible adverse effects of consuming weight loss herbal supplements like Hydroxycut. Healthcare professionals and consumers are encouraged to report serious adverse events or product quality problems with the use of these supplements to the FDA's MedWatch adverse event reporting program. LEARNING POINTS: The weight loss supplement Hydroxycut is sold in various formulations whose side effect profiles are not fully known; the latest formulation has a very high concentration of caffeine which increases the risk of rhabdomyolysis.Although uncommon, exercise-induced rhabdomyolysis can cause compartment syndrome, especially in the setting of concurrent use of weight loss supplements.High clinical suspicion, prompt diagnosis and early treatment are key to preventing complications from compartment syndrome.
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Aortic dissection is a relatively uncommon, although catastrophic, disease which requires early and accurate diagnosis and treatment for patient survival. Aortic dissection can be difficult to diagnose due to the diverse symptom presentation, which can lead to later diagnosis, resulting in a higher mortality rate. Here we present a case of type A aortic dissection with a varied symptom presentation, highlighting the importance of early detection and the Bentall procedure for management of such cases. A 50-year-old man with no known medical history presented with bilateral lower extremity swelling and fatigue for 2 weeks. The patient denied any chest pain or dyspnoea. Vital signs showed blood pressure of 160/76 mmHg, pulse of 103 bpm, respiratory rate of 18, and temperature of 36.7°C. Laboratory findings indicated a BNP of 1901 pg/ml and troponin of 0.5 ng/ml. An initial diagnosis of decompensated heart failure was made, and IV Lasix was started. Subsequently, an echocardiogram indicated an EF of 50-55% and ascending dissection of the aorta. A CT angiogram of the chest and abdomen confirmed this diagnosis. This patient presented with unusual symptoms of aortic dissection without the typical presentation of chest pain. It is important to consider aortic dissection in a cardiac-related case as prompt imaging can help confirm the diagnosis. We explore the risks and benefits of the Bentall procedure for the management and early detection of aortic dissection. LEARNING POINTS: The absence of chest pain does not rule out aortic dissection as 50% of patients are pain free.It is critical to diagnose aortic dissection early in the disease course as mortality increases by 1% per hour from symptom onset.The aim of this study is also to raise awareness among healthcare professionals about the Bentall procedure in patients with type A aortic dissection involving the aortic valve.
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The serum osmolar gap, defined as the difference between measured osmolality and calculated osmolarity, is a convenient method to screen for toxins in serum. In normal circumstances, the difference between the two is 6-10 mol/kg. Typical contributors to serum osmolarity are sodium bicarbonate, sodium chloride, glucose and urea. An elevated gap, defined as a difference >10 mol/kg, can occur if a sufficient quantity of an additional solute other than those mentioned above is present in the serum or there are inaccuracies in sodium measurement secondary to hyperlipidaemia and hyperproteinaemia. An elevated serum osmolar gap should thus prompt clinicians to check for toxic alcohol levels. Treatment with fomepizole should not be delayed if suspicion is high. Isolated diabetic ketoacidosis can occasionally present with an elevated osmolar gap in the absence of concomitant alcohol ingestion. This finding is attributed to the production of acetone and glycerol. We describe the case of a 62-year-old man presenting with diabetic ketoacidosis/hyperosmolar hyperglycaemic state and an elevated osmolar gap in the absence of toxic alcohol ingestion. LEARNING POINTS: The osmolar gap is the difference between the measured and the calculated serum osmolarity and should be calculated in all patients presenting with elevated serum osmolarity; if elevated, toxic alcohol ingestion should be considered and prophylactic treatment with fomepizole immediately administered if the index of suspicion is high.Although toxic alcohol ingestion is one of the common causes of an elevated osmolar gap, hyperlipidaemia, hyperproteinaemia and less occasionally lactic acidosis and ketoacidosis have also been implicated.In the setting of ketoacidosis, the osmolar gap can be elevated in the absence of toxic alcohol ingestion, is attributed to increased production of acetone and glycerol, and is responsive to treatment with insulin and intravenous fluids.
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Inherited thrombophilia is an important cause of venous thrombosis. The Factor V Leiden (FVL) is the most commonly encountered mutation, followed by the prothrombin G20210A gene mutation (PTM). The typical venous thrombotic events (VTEs) associated with PTM mutations are deep vein thrombosis (DVT) and pulmonary embolisms (PE). The PTM is inherited in an autosomal dominant pattern with variable penetrance. While heterozygous PTM mutations are more frequent and well documented in the literature, rare cases of homozygous PTM mutations are also reported. In this report, we discuss a 56-year-old male with a past medical history of homozygous prothrombin gene mutation (G20210A) who presented with an unprovoked DVT of the right lower extremity involving both the proximal and distal veins associated with multiple bilateral PEs. This case is unique in terms of the homozygous PTM inheritance, the age at which the patient presented (usually presentation is earlier in life), and the fact that he had a recurrence of both DVT and PE simultaneously.