Successful treatment of cardiac sarcoidosis based on clinical suspicion and advanced cardiac imaging: A case report.

INTRODUCTION: Confirming the diagnosis of cardiac sarcoidosis (CS) is a challenging task as we often do not count with histopathologic evidence. However, prompt initiation of treatment is sometimes necessary, and advanced cardiac imaging along with key clinical findings can play a crucial role in the diagnostic workup. PATIENT CONCERNS: A 77-year-old male with a history of heart failure presented with chest pain and shortness of breath. He was found to have an acute drop in left ventricular ejection fraction associated with frequent premature ventricular contractions and nonsustained ventricular tachycardia. Coronary angiogram was negative for acute coronary syndrome. Advanced cardiac imaging with cardiac magnetic resonance raised suspicion of CS, and steroids were started empirically. Endomyocardial biopsy was attempted but was not successful. DIAGNOSIS: The patient's presentation was highly suggestive of cardiac sarcoidosis. INTERVENTIONS: Corticosteroids, diuresis, guideline-directed medical therapy for heart failure. OUTCOMES: The patient's symptoms and ventricular arrhythmias improved on steroids. Subsequent FDG-PET revealed increased uptake in a pattern consistent with CS. CONCLUSION: This clinical scenario highlights the importance of advanced cardiac imaging and clinical findings for the diagnosis of CS and exposes the practical need for a standardized, noninvasive strategy to the diagnosis of CS.

Acute Severe Mitral Regurgitation Secondary to Ischemic Papillary Muscle Rupture: A Case Report.

Mitral valve rupture secondary to ischemic papillary muscle necrosis is rare in the contemporary era due to improved revascularization techniques. However, when it does occur, prompt diagnosis and urgent surgical intervention can be lifesaving. A 69-year-old male with morbid obesity, hypothyroidism, and a family history of coronary artery disease presented to the hospital with chest pain and dyspnea that began five hours prior. He had an acute infero-postero-lateral myocardial infarction due to total occlusion of the left circumflex artery that was revascularized with the deployment of a drug-eluting stent. Two days after the myocardial infarction, the patient had an episode of ventricular tachycardia. He subsequently went into respiratory distress from flash pulmonary edema and developed cardiogenic shock due to acute mitral valve rupture. The patient underwent surgical mitral valve replacement, extracorporeal membranous oxygenation (ECMO), and hemodialysis. His course was complicated by an acute lower gastrointestinal bleed that progressed into multiorgan failure and eventually his demise. This case highlights the need to include papillary muscle rupture high on the differential when evaluating a hemodynamically unstable patient in the setting of an acute myocardial infarction (MI). Rapid diagnosis by urgent bedside echocardiogram and surgical intervention is crucial.

Pericardial effusion with tamponade - an uncommon presentation leading to the diagnosis of eosinophilic granulomatosis polyangiitis: A case report.

BACKGROUND: Eosinophilic granulomatosis polyangiitis (EGPA) is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma; however, it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade. Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare. Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes. CASE SUMMARY: 52-year-old woman with no past medical history presented with progressive dyspnea and dry cough. On physical exam she had a pericardial friction rub and bilateral rales. Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89% oxygen saturation. On laboratory exam, she had 45% peripheral eosinophilia, troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL. TTE confirmed a large pericardial effusion and tamponade physiology. She underwent urgent pericardial window procedure. Pericardial and lung biopsy demonstrated eosinophilic infiltration. Based on the American College of Radiology guidelines, the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade. She was treated with steroid taper and mepolizumab. CONCLUSION: This case highlights that when isolated pericardial involvement occurs in EGPA, diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.

Achilles Tendon Xanthoma Thickness and Carotid Intima-Media Thickness in a Patient With Heterozygous Familial Hypercholesterolemia on PCSK9 Inhibition: A Case Report and Literature Review.

Ultrasound-guided measurement of carotid intima-media thickness can be used as a surrogate marker to predict future risk of atherosclerotic cardiovascular disease, and to understand the efficacy of lipid-lowering drugs. Aggressive lipid-lowering drugs such as proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors have been shown to reduce carotid artery plaque burden, total cholesterol, and low-density lipoprotein-c in patients with heterozygous familial hypercholesterolemia (FH). We describe a patient with heterozygous FH treated with PCSK9 inhibitor over the course of two years, and the drug's impact on carotid intima-media thickness, Achilles tendon thickness, and cardiovascular disease risk reduction.

Concordance and Discordance of Echocardiographic Parameters Recommended for Assessing the Severity of Mitral Regurgitation.

BACKGROUND: The American College of Cardiology/American Heart Association and American Society of Echocardiography guidelines recommend assessing several echocardiographic parameters when evaluating mitral regurgitation (MR) severity. These parameters can be discordant, making the assessment of MR challenging. The degree to which echocardiographic parameters of MR severity are concordant is not well studied. METHODS: We enrolled 159 patients in a prospective multicenter study. Eight parameters were included in this analysis: proximal isovelocity surface area (PISA)-derived regurgitant volume, PISA-derived effective regurgitant orifice area, vena contracta, color Doppler jet/left atrial area, left atrial volume index, left ventricular end-diastolic volume index, peak E wave, and the presence of pulmonary vein systolic reversal. Each echocardiographic parameter was determined to represent severe or nonsevere MR according to the American Society of Echocardiography guidelines. A concordance score was calculated as [Formula: see text] so that a higher score reflects greater concordance. There was no discordance when all the echocardiographic parameters agreed and high discordance when 3 or 4 parameters were discordant. RESULTS: The mean concordance score was 75±14% for the entire cohort. There were 9 (6%) patients with complete agreement of all parameters and 61 (38%) with high discordance. There was greater discordance in patients with severe MR but no difference between primary versus secondary or central versus eccentric jets. There was an improvement in concordance when only considering PISA-based regurgitant volume, PISA-based effective regurgitant orifice area, and vena contracta with agreement in 68% of patients. CONCLUSIONS: There was limited concordance between the echocardiographic parameters of MR severity, and the discordance was worse with more severe MR. Concordance improved when considering only 3 quantitative measures of vena contracta and PISA-based effective regurgitant orifice area and regurgitant volume. These findings highlight the challenges facing echocardiographers when assessing the severity of MR and emphasize the difficulty of using an integrated approach that incorporates multiple components. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04038879.

Usefulness of Mitral Regurgitant Volume Quantified Using Magnetic Resonance Imaging to Predict Left Ventricular Remodeling After Mitral Valve "Correction".

MRI studies have shown a tight correlation between mitral regurgitant volume and left ventricular end-diastolic volume (LV EDV) in patients with primary chronic mitral regurgitation (MR). They have also shown a tight correlation between regurgitant volume and the decrease in LVEDV following mitral valve surgery. The purpose of this study is to validate an empiric calculation that can be used preoperatively to predict the amount of left ventricular remodeling following mitral valve correction. This is a prospective multicenter study of 63 (61 ± 13 years, male 65%) patients who underwent an MRI before and after mitral valve correction. Pre and postmitral valve correction ventricular volumes and ejection fractions were quantified. The predicted change in LV EDV was empirically calculated as mitral regurgitant volume/left ventricular ejection fraction. The observed change in LV EDV was compared to the predicted change in LV EDV. The LVEDV decreased in 61 (97%) patients following mitral valve correction (237 ± 66 ml vs 164 ± 46 ml, p <0.0001). Correlation between the observed and predicted change in LVEDV was good for the entire cohort (r = 0.77, p <0.0001) and excellent in patients with <10 ml of residual MR (r = 0.87, p <0.0001). This tight correlation was seen in both patients with primary (0.86, p <0.0001) and secondary MR (0.97, p <0.0001) and <10 ml of residual MR. Multivariate predictors of LV remodeling were MR volume, primary MR, and LVESV. In conclusion cardiac MRI volumetric measurements accurately predict LV remodeling following mitral valve correction. This finding supports the notion that MRI accurately quantifies the severity of chronic mitral regurgitation and a cardiac MRI should be strongly considered before mitral valve correction.

A Rare Desmoglein-2 Gene Mutation in Arrhythmogenic Right Ventricular Cardiomyopathy Inciting Incessant Ventricular Fibrillation.

A case of a 51-year-old female with history of hypertension and a significant family history of premature coronary artery disease presented to the hospital after cardiac arrest. She successfully completed a targeted temperature management therapy with full neurologic recovery. Her hospital course was complicated by several bouts of ventricular fibrillation (VF) arrest which was rescued by timely defibrillation, high quality cardiorespiratory resuscitation, and administration of antiarrhythmic medications and inotropic agents. An automatic implantable cardioverter defibrillator (AICD) was inserted for secondary prevention of sudden cardiac death (SCD). A targeted genetic testing for idiopathic ventricular fibrillation revealed a mutation in the desmoglein-2 (DSG2) gene involved in arrhythmogenic right ventricular cardiomyopathy (ARVC). Eventually, a ventricular fibrillation radiofrequency ablation prevented recurrence of fatal arrhythmia and its associated symptoms.

Utilization of Echocardiogram, Carotid Ultrasound, and Cranial Imaging in the Inpatient Investigation of Syncope: Its Impact on the Diagnosis and the Patient's Length of Hospitalization.

BACKGROUND: Although guidelines suggest that the best strategy for evaluating syncope is clinical history and physical examination, the inappropriate utilization of diagnostic imaging is common. METHODS: A single center retrospective analysis conducted in adult patients admitted for evaluation and management of syncope for a period of 12 months. Charts were reviewed to abstract demographic data, admitting and discharge diagnosis, diagnostic investigatory tests including imaging modalities (echocardiogram, carotid ultrasound, and cranial computed tomography (CT)) ordered, subspecialty consultation requested, treatment rendered and hospital length of stay (LOS). RESULTS: A total of 109 patients were admitted for syncope, mean age was 68.74 ± 21.04 years and 39.44% were men. Echocardiogram, carotid ultrasound, and cranial CT were ordered in 69.72%, 33.02%, and 76.14% respectively. The mean hospital LOS was 2.6 days. Patients with no imaging test, one imaging test, two imaging tests, and three imaging tests ordered have an average hospital LOS of 2.22 days, 2.44 days, 2.58 days, and 3.07 days respectively. The number of imaging test and its relation to the admitting (Chi-square (chi-sq) P = 0.4165, nominal logistic regression (LR) P = 0.939) and discharge (chi-sq P = 0.1507, nominal LR P = 0.782) diagnosis as well as the LOS in relation to the number of imaging test ordered (analysis of variance (ANOVA) P = 0.368, Kruskal Wallis (KW) P = 0.352) were not statistically significant although there was a trend of prolonged hospital LOS the more imaging diagnostic test had been ordered. Syncope was the admitting and discharge diagnosis in 89.9% and 91.74% respectively. CONCLUSIONS: Choosing the appropriate diagnostic tests as dictated by the patient's clinical manifestation and utilizing less expensive test would be appropriate and cost-effective approach in appraising patients with syncope.

A Rare Case of Native Mitral Valve Bacillus Cereus Endocarditis Culminating Into a Cerebrovascular Infarction.

We report a case of a 56-year-old man who presented initially with a sudden onset of right-sided facial droop and weakness, aphasia, and confusion with no associated fever, chills, syncope, fatigue, weight loss, night sweats, nausea, vomiting, diarrhea, odontalgia, palpitations, cough, or dyspnea. Code stroke was called and the patient received tissue plasminogen activator (tPA) with subsequent resolution of his symptoms. Cranial magnetic resonance imaging showed left frontal punctate cortical restricted diffusion consistent with subacute to acute infarction. Transesophageal echocardiogram showed a severely thickened anterior mitral valve leaflet with a shaggy echodensity consistent with a vegetation. Blood cultures grew Bacillus cereus sensitive to clindamycin, trimethoprim sulfamethoxazole, and vancomycin. He was initially treated with ampicillin, clindamycin, and vancomycin and was eventually maintained solely on vancomycin. He had complete return of his neurological function and was discharged on intravenous antibiotic to complete a 6-week course.

High Grade Atrioventricular Block in Von Recklinghausen Disease: A Rare Phenomenon.

A case of a 73-year-old woman with a history of von Recklinghausen disease (neurofibromatosis type 1) who presented initially with a gradual onset of shortness of breath and lightheadedness with no associated fever, chills, angina, palpitations, cough, weight loss, night sweats, nausea, vomiting, or constipation. She was found to be severely bradycardic and in third degree atrioventricular block by her primary care physician. She was admitted in the hospital because of intermittent bouts of lightheadedness and progression of the shortness of breath. Twelve-lead electrocardiogram documented the high grade atrioventricular block. Chest radiograph showed subtle mild pulmonary congestion. Transthoracic echocardiogram revealed mild concentric hypertrophy and normal systolic function with no regional wall motion abnormalities or evidence of significant valvular disease. Pacemaker was inserted and her symptoms improved significantly.

Brockenbrough-Braunwald-Morrow Sign: An Evaluative Hemodynamic Maneuver for Left Ventricular Outflow Tract Obstruction.

A case of a 77-year-old woman with a history of hypertrophic cardiomyopathy (HCM) presented with intermittent episodes of exertional dyspnea and chest discomfort. Her coronary angiogram revealed normal coronary arteries but with hypertrophic obstructive cardiomyopathy with an increasing left ventricular-aortic gradient on isoproterenol provocation. Likewise, an intensified gradient was observed after a premature ventricular contraction (PVC) that is distinguished as the Brockenbrough-Braunwald-Morrow sign substantiating confirmation of left ventricular outflow tract (LVOT) obstruction.

Angio-Seal™ Embolization: A Rare Etiology of an Acute Distal Limb Ischemia.

We herein report a serious vascular complication of diagnostic cardiac catheterization due to an embolization of an Angio-Seal closure device causing acute lower limb ischemia. The Angio-Seal was deployed via the right femoral artery following the catheterization which embolized several hours later to the right popliteal artery. Fogarty embolectomy restored perfusion to the right lower limb; however, compartment syndrome subsequently developed which required evacuation of a hematoma and repair of right popliteal artery.

Heterozygous Familial Hypercholesterolemia With APOE Haplotype: A Prospective Harbinger of a Catastrophic Cardiovascular Event.

We report a very young man with heterozygous familial hypercholesterolemia (FH) with APOE haplotype and a significant cardiac family history who underwent cardiac catheterization for intermittent episodes of exertional dyspnea and was noted to have a severe triple vessel coronary artery disease (CAD). He underwent coronary artery bypass graft (CABG) surgery which was uneventful. He was discharged on antiplatelet, beta blocker, nitrate, and statin. On routine health maintenance evaluation, he had no cardiac complaints and had been tolerating well his activities of daily living.

A Very Late Presentation of a Right Coronary Artery Occlusion After Transcatheter Aortic Valve Replacement.

Involvement of the coronary ostia is a rare complication after transcatheter aortic valve replacement (TAVR). This complication has been more commonly described immediately after valve deployment. This is the first reported case of delayed coronary obstruction caused by TAVR 6 months after the procedure.

Cough induced syncope: A hint to cardiac tamponade diagnosis.

We report a case of a 75-year-old male with history of lung adenocarcinoma who presented with shortness of breath and frequent episodes of cough-induced syncope. A large pericardial effusion was found on echocardiogram suggestive of cardiac tamponade. Pericardiocentesis was done which improved the dyspnea and eventually resolved the syncope. There are only two other cases reported in the literature with cough-induced syncope in the setting of pericardial effusion or cardiac tamponade. Our clinical vignette also highlights the importance of pulsus paradoxus identification in patients with cough induced syncope to rule out cardiac tamponade since this is the most sensitive physical finding for its diagnosis.

Concomitant Left Atrial Myxoma and Patent Foramen Ovale: Is It an Evolutional Synergy for a Cerebrovascular Event?

We report a case of a 48-year-old female who presented initially with an abrupt onset of left facial and hand numbness after her routine yoga with no associated syncope, palpitation, chest pain or dyspnea. She consulted her primary care physician and recommended hospital care for possible stroke. On the day of admission, she complained of left facial and hand hemiparesthesia. Cranial imaging and angiography were unremarkable but echocardiography and cardiac computed tomography revealed left atrial mass. She underwent resection of the left atrial mass with an incidental finding of patent foramen ovale intraoperatively. The left atrial mass was confirmed to be an atrial myxoma. Patient's neurologic complaints resolved towards the end of her hospital course. She was discharged stable with no recurrence of neurologic symptoms on health maintenance evaluation.

Mediterranean Spotted Fever: A Rare Non-Endemic Disease in the USA.

We report a case of a 43-year-old Israeli male who presented with an intermittent fever associated with a gradual appearance of diffusely scattered erythematous non-pruritic maculopapular lesions, generalized body malaise, muscle aches, and distal extremity weakness. He works in the Israeli military and has been exposed to dogs that are used to search for people in tunnels and claimed that he had removed ticks from the dogs. In the hospital, he presented with fever, a diffuse maculopapular rash, and an isolated round black eschar. He was started on doxycycline based on suspected Mediterranean spotted fever (MSF) in which he improved significantly with resolution of his clinical complaints. His immunoglobulin G (IgG) MSF antibody came back positive.