An Atypical Presentation of Extrapulmonary Sarcoidosis.

Sarcoidosis is an idiopathic, chronic, multisystem, granulomatous, inflammatory disease involving almost all organs. Sarcoidosis can occur with an atypical presentation of hepatosplenic involvement, like in the case of our patient. In this case report, we present a rare case of extrapulmonary sarcoidosis with isolated involvement of the liver and spleen in a 39-year-old Caucasian female. There is a possibility of this isolated involvement of an organ in the complete absence of pulmonary disease, which makes the diagnosis of sarcoidosis very difficult as it is usually not suspected. Ultrasound and CT are important in ruling out other differential diagnoses, but a definitive diagnosis is possible only on histological examination, differentiating sarcoid lesions from tuberculosis, primary biliary cirrhosis, metastasis, malignancy, and other granulomatous infections or diseases. Hence, the most credible criterion for diagnosis remains histology. After diagnosis, regular follow-up for systemic manifestations is recommended. Asymptomatic patients with hepatosplenic sarcoidosis have a good prognosis without any medical intervention, while patients with abnormal labs or symptoms must commence treatment.

An Atypical Presentation of Median Arcuate Ligament Syndrome.

Median arcuate ligament syndrome (MALS) is a rare condition where the celiac artery is compressed by the ligament, uniting the diaphragmatic crura of the aortic hiatus. Patients mostly present with abdominal symptoms. We present a case of a 51-year-old male who presented with abdominal pain. The patient was evaluated with a computed tomography (CT) scan of the abdomen and found to have celiac artery stenosis secondary to the median arcuate ligament (MAL). The patient was assessed by surgery and interventional radiology, but no intervention was offered. He was started on anticoagulation with spontaneous resolution of abdominal pain.

Cardiac Dysrhythmia Associated With Opioid Toxicity.

Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder involving abnormal motility. The patients are commonly started on symptom control management for IBS - diarrhea subtype by prescribing antimotility agents, usually having opioid agonist activity, and newer medications have been emerging for this as well. Patients sometimes self-medicate themselves to exceedingly high doses of these medications to achieve symptoms control. There are only a few cases of opioid-induced arrhythmia in the literature, primarily loperamide being used as a drug substitute by substance abusers. Still, it has been rarely reported to cause arrhythmia in a patient with IBS. We present a case of a 33-year-old female with a past medical history of hypertension and depression who presented to the emergency department for evaluation of syncope. She had wide complex tachycardia on electrocardiogram (EKG) with prolonged rate-corrected QT interval (QTc). Her medications, including eluxadoline, Lomotil, and loperamide, were held and she was discharged on mexiletine with normal QTc. She did not have any more incidences of arrhythmia. This case highlights the importance of not overdosing on opioid agonist medications prescribed to treat IBS as these can lead to potentially fatal complications. Physicians have to be judicious in promptly determining that the cause of arrhythmia can also be over-the-counter (OTC) medications.

Tirofiban Induced Thrombocytopenia: A Rare but Severe Adverse Effect.

With the advent of medical technology, coronary angiography is a common practice to evaluate patient for coronary artery disease. Normally, patients undergoing angiogram receive antiplatelets, anticoagulants, and platelet aggregation inhibitor agents. Glycoprotein IIb/IIIa receptor inhibitors are a type of platelets antiaggregant agents that can cause severe thrombocytopenia in very few cases. We present a case of a 69-year-old female who presented with chest pain, underwent an angiography and had two stents placed. She was administered tirofiban during angiogram that caused acute severe thrombocytopenia decreasing her platelets count from 224 to 2 k/mm3 within 1 day. Patients platelets gradually recovered after trial of steroid and platelets transfusion. Antiplatelets (Aspirin and Clopidogrel) were resumed; however, patient's platelets remained stable.

A case of transcatheter prosthetic aortic valve endocarditis.

Transcatheter aortic valve implantation (TAVR) constitutes an established treatment in inoperable or high perioperative risk patients with severe aortic stenosis. Prosthetic valve endocarditis after ΤΑVR occurs with an incidence of 0.3-1% per patient-year. Infective endocarditis may stem from hematogenous dissemination or contact with infected adherent tissue. Few cases of infective endocarditis after TAVR have been reported. We present an interesting case of a 79-year-old male with a history of severe aortic stenosis status post TAVR greater than one year ago, and pulmonary vein isolation for atrial fibrillation six weeks ago was found to have infective endocarditis with a vegetation on the prosthetic valve leading to multiple embolic strokes as a result of Enterococcus faecalis bacteremia. The patient was not a surgical candidate with his Society of Thoracic Surgery (STS) risk score being 18%; therefore, he was managed conservatively on intravenous antibiotics. Our case had endocarditis from enterococcus bacteremia; however, the patient never had any gastrointestinal or genitourinary procedure.

Left anterior descending artery hyper dominance giving rise to the posterior descending artery: an extremely rare coronary anomaly and its clinical implications.

The prevalence of congenital coronary artery anomalies is approximately 1% in the general population. They are a common cause of sudden death in younger persons. The origination of the posterior descending artery (PDA) from left anterior descending (LAD) artery is an extremely rare anomaly. We present a case of a 54-year-old female who presented with diabetic ketoacidosis with co-existing non-ST elevation myocardial infarction, therefore, had an invasive angiogram that identified the anomalous origin of PDA from LAD. It is vital to define coronary anatomy as anomalies dictate which cardiac intervention should be attempted in cases of ischemia.

Comparison between blood pressure readings using a mercury versus an aneroid sphygmomanometer.

OBJECTIVE: For more than a century since its introduction, mercury sphygmomanometer (HgS) had been the mainstay for office measurement of blood pressure (BP). In light of the environmental and health hazards associated with mercury, there is a need to replace it with mercury-free alternatives all over the world. We aimed to validate the widely used aneroid sphygmomanometer (AnS) by comparing its BP readings against BP readings taken with an HgS. METHODS: We compared the BP readings using AnS vs. HgS on a sample of 300 patients of 18 years or older age admitted to a tertiary care hospital in Karachi, Pakistan. RESULTS: The differences between mean HgS and AnS BP readings were found to be statistically significant (P-value <0.01). The mean systolic blood pressure (SBP) readings of the two devices were still significantly correlated (r = 0.989; P < 0.01). Similarly, the mean diastolic blood pressure (DBP) readings were also significantly correlated (r = 0.988; P < 0.01). The aneroid device identified a higher proportion of hypertensive participants compared to the mercury device. CONCLUSION: The difference in the two devices used was found to be significant; however, the readings were correlated with each other. The AnS significantly overestimated BP readings, thereby identifying a higher proportion of hypertensives as compared to the HgS. There is a considerable room for improvement in the accuracy of the AnS, only then an accurate and a well-calibrated AnS could provide an acceptable alternative to the use of the HgS.

Is Physiologic Stress Test with Imaging Comparable to Anatomic Examination of Coronary Arteries by Coronary Computed Tomography Angiography to Investigate Coronary Artery Disease? - A Systematic Review and Meta-Analysis.

Objective Coronary computed tomography angiography (CCTA) is a noninvasive diagnostic modality that remains underutilized compared to functional stress testing (ST) for investigating coronary artery disease (CAD). Several patients are misdiagnosed with noncardiac chest pain (CP) that eventually die from a cardiovascular event in subsequent years. We compared CCTA to ST to investigate CP. Methods We searched MEDLINE, PubMed, Cochrane Library, and Embase from January 1, 2007 to July 1, 2018 for randomized controlled trials (RCTs) comparing CCTA to ST in patients who presented with acute or stable CP. We used Review Manager (RevMan) [Computer program] Version 5.3 (Copenhagen: The Nordic Cochrane Centre, The Cochrane Collaboration, 2014) for review and analysis. Results We included 16 RCTs enrolling 21,210 patients; there were more patients with hyperlipidemia and older patients in the ST arm compared to the CCTA arm. There was no difference in mortality: 103 in the CCTA arm vs. 110 in the ST arm (risk ratio [RR] = 0.93, 95% confidence interval [CI] = 0.71-1.21, P = .58, and I2 = 0%). A significant reduction was seen in myocardial infarctions (MIs) after CCTA compared to ST: 115 vs. 156 (RR = 0.71, CI = 0.56-0.91, P < .006, I2=0%). On subgroup analysis, the CCTA arm had fewer MIs vs. the ST with imaging subgroup (RR = 0.70, CI = 0.54-0.89, P = .004, I2 = 0%) and stable CP subgroup (RR = 0.66, CI = 0.50-0.88, P = .004, I2 = 0%). The CCTA arm showed significantly higher invasive coronary angiograms and revascularizations and significantly reduced follow-up testing and recurrent hospital visits. A trend towards increased unstable anginas was seen in the CCTA arm. Conclusions Our analysis showed a significant reduction in downstream MIs, hospital visits, and follow-up testing when CCTA is used to investigate CAD with no difference in mortality.

A rare case of adult congenital heart disease: single ventricular chamber with anomalous right coronary artery in an octogenarian.

Patients with single-ventricle physiology encompass a wide array of anatomic subtypes, including but not limited to: tricuspid atresia, hypoplastic left heart syndrome, double-outlet or double-inlet ventricles. The outcomes for patients with single ventricle born before 1990 are relatively poor. An 81-year-old female presented to the hospital as non-ST elevation myocardial infarction. She was started on antiplatelet and anticoagulation. Echocardiogram revealed a single ventricle which was thought to be left ventricle with possible transposition of great vessels. Angiography was performed that identified the single ventricle and anomalous origin of the right coronary artery (RCA). She was also found to have double vessel coronary artery disease with diffuse stenosis of mid-RCA at 80% and proximal circumflex at 95%. She was managed conservatively as was high risk for CABG given her rare congenital condition. Patients with single ventricle are at risk of long-term morbidity, including heart failure, neurological injury, and early death. The mortality risk of these patients is high as most of the patients without corrective surgery do not proceed to adulthood. Our case had multivessel coronary artery stenosis along with a rare presentation of congenital heart disease in adulthood. The patient was offered percutaneous coronary intervention, but she declined and chose to be treated conservatively with only medical management. We present a rare case of an elderly female surviving with a single ventricular chamber. The patient is an exception to the usual process of the pathology as most patients without corrective surgery seldom survive into adulthood.

An entire coronary system arising from right coronary cusp: a rare anomaly.

The prevalence of coronary artery anomalies is approximately 0.6% in individuals undergoing angiography. Most of the anomalies are benign, but some can lead to myocardial infarction, cardiomyopathy, and sudden cardiac death. It is very rare to have an entire coronary circulation that arises from the right coronary cusp. We present a case of a 57-year-old male who presented with complaints of chest pain and dyspnea on exertion. An invasive angiogram revealed all the three coronary arteries originating from the right coronary cusp. It is crucial to define coronary anatomy as anomalies dictate which cardiac intervention should be attempted in cases of ischemia.

A Unique and Rare Presentation of Adult Congenital Heart Disease: Common Atrium Associated with Coronary Aneurysms and Fistula.

An atrial septal defect is the second most common congenital heart disease found in adults with a female to male ratio of 4 : 1. However, it is rare to have a complete absence of the interatrial septum (IAS) to be diagnosed in an elderly patient associated with other coexisting anomalies. We present a case of a 60-year-old female presenting with common atrium, coronary arteriovenous fistula, and coronary artery aneurysms. This case highlights rare adult congenital cardiac anomalies and the importance of thorough workup to evaluate for the intracardiac shunt in a patient who has right heart enlargement and development of pulmonary disease in adulthood without a significant history of chronic smoking. A 60-year-old female patient presented with substernal chest pain. The nuclear stress test showed no reversible ischemia; however, right ventricle (RV) dilation was present. The patient underwent further evaluation for RV dilation with a transthoracic echocardiogram that demonstrated a complete absence of IAS and was confirmed by a positive bubble study. The patient had an invasive angiography that showed severely elevated RV pressure. Oxygen saturation in the right atrium was higher than in the inferior vena cava. Hence, an intracardiac shunt with a 10% increase in oxygen saturation was identified. It also identified aneurysmal coronary arteries (measuring 0.8 to 1.0 cm). Cardiac computed tomography angiogram was performed that identified all coronary arteries to be ectatic/aneurysmal measuring up to 8-10 mm, an absence of IAS, and a possible fistula between the distal left anterior descending and a coronary vein. To our knowledge, this is the first-ever presentation of a complete congenital absence of IAS in a patient who has survived into adulthood with the development of severe pulmonary hypertension without Eisenmenger syndrome. It is unclear at this point if surgical treatment to correct the anatomical defect (which would be probably palliative) would be superior to conservative medical therapy. Besides, the presence of coronary arteriovenous fistula would make the decision-making process more complex regarding surgical versus conservative management.

Tolvaptan-induced Liver Injury: Who is at Risk? A Case Report and Literature Review.

Hyponatremia is a common clinical condition encountered in the hospital setting. Syndrome of inappropriate antidiuretic hormone (SIADH) is an important and one of the most common causes of hyponatremia. SIADH accounts for approximately one-third of all cases of hyponatremia. Tolvaptan is a vasopressin receptor antagonist used to treat SIADH. Hepatoxicity is a rare yet dangerous side effect from Tolvaptan use. We present a case of cholestatic liver injury in an elderly female who presented with hyponatremia. She received two doses of tolvaptan 15mg and developed worsening in her total bilirubin (T Bili) and alkaline phosphatase (Alk Phos) levels. Tolvaptan is known to cause elevated transaminase levels and the mechanism of action is thought to be idiosyncratic. Fortunately, the patient responded with an improvement in T Bili and Alk Phos levels after stopping tolvaptan. This case highlights the cautious use of tolvaptan in elderly patients with SIADH as even small doses can potentiate hepatotoxicity.

Membranous Nephropathy in a Patient with Human Immunodeficiency Virus Shortly After Initiation of HAART with Atripla.

A human immunodeficiency virus (HIV) infection has long been associated with kidney disease. The pathogenesis of renal complications may be directly related to the presence of HIV viral particles or may occur secondary to an immune response against the virus. A number of HIV medications have been associated with the development of acute and chronic kidney disease. It has been estimated that approximately 60 percent of patients suffering from HIV/acquired immunodeficiency syndrome (AIDS) will, at some point, manifest clinically significant renal sequelae.The most common kidney disease affecting HIV patients is HIV-associated nephropathy (HIVAN) or focal segmental glomerulonephritis (FSGS). A very small subset of patients suffering from HIV/AIDS does go on to develop membranous glomerulonephritis. We present a case of a 55-year old Caucasian male who presented to the hospital after two weeks of weakness and falling when attempting to stand. The patient had a history of HIV, diagnosed in 1996. The latest cluster differentiation 4 (CD4) count was 245 cells/uL and the HIV-ribonucleic acid (RNA) viral load was reported as less than 75 copies/ml. The physical exam at presentation was insignificant. The laboratory examination revealed elevated creatinine. Potential nephrotoxic home medications, including Atripla and lisinopril, were held. After a brief inpatient stay, he was discharged but was ultimately readmitted for worsening renal function and nephrotic syndrome was diagnosed. Renal biopsy was performed, and membranous glomerulonephritis was confirmed. To this point, there are no associated cases reported of membranous glomerulonephritis after initiation of therapy with Atripla. We present a case of a rare etiology of membranous nephropathy in an HIV patient. Physicians should be judicious in detecting the etiology of renal dysfunction in HIV patients.

Supratherapeutic International Normalized Ratio causing Nephropathy: A Rare Adverse Effect of Warfarin.

There are many common causes of nephropathy (abnormal pathology of kidneys) such as diabetes, hypertension, autoimmune, and drugs. Amongst the drugs, warfarin has recently been recognized to cause nephropathy in rare cases. Warfarin-related nephropathy (WRN) is clinically defined as an increase in serum creatinine of 0.3 mg/dl within one week of international normalized ratio (INR) being greater than 3. A 61-year-old male was referred by his primary care physician (PCP) for having complaints of elevated creatinine associated with hematuria for one month. On evaluation with computed tomography (CT) of the abdomen/pelvis, it was revealed that he had small non-obstructing stones. The creatinine had increased from a baseline of 2.03 mg/dl to 6.8 mg/dl. Hemoglobin (Hb) had decreased from a baseline of 12.8 gm/dl to 8.1 gm/dl, the INR was 3.52. On subsequent days, the patient's renal function did not improve with fluids and supportive measures. Workup was unremarkable; therefore, a kidney biopsy was done. The biopsy specimen concluded the diagnosis of WRN. The patient was started on prednisone without any effect and then intermittent hemodialysis. Our case highlights the rare instance in which the cause of nephropathy is warfarin. If an early diagnosis had been made, the patient might have had a better prognosis; therefore, it is essential to have a high index of clinical suspicion when a patient presents with supratherapeutic INR and acute kidney injury (AKI) not getting better.

Spontaneous Coronary Artery Dissection Masquerading as Coronary Artery Stenosis in a Young Patient.

BACKGROUND Spontaneous coronary artery dissection (SCAD) is primarily found in females. SCAD can have many precipitating factors such as exercise, trauma, pregnancy, drugs, and connective tissue disease. Prognosis is poor for left main stem, left anterior descending (LAD) artery, and multivessel involvement, especially for females. CASE REPORT We present a case of young African American male with sickle cell disease who presented with chest pain associated with shortness of breath. He was found to have non-ST elevation myocardial infarction (NSTEMI). He was diagnosed with SCAD during catheterization with the help of intravascular ultrasound imaging. Three drug-eluting stents were placed to cover the proximal LAD vessel along its whole length until resolution of the lesion. The patients' hospital course was complicated by an additional finding of left ventricular thrombus, possibly a complication of NSTEMI, which was treated with anticoagulation to complete resolution. CONCLUSIONS SCAD is fatal, it can proceed to cause myocardial infarction as in this particular patient's case, and sudden death if not recognized early. It can be missed on angiography alone; further intracoronary imaging such as intravascular ultrasound and optical computed tomography should be used to confirm the diagnosis of SCAD so that early and appropriate treatment can ensue.

Elevated Factor VIII Level Associated with Transverse Cerebral Venous Sinus Thrombosis.

BACKGROUND Cerebral venous sinus thrombosis (CVST) is an uncommon cause of stroke. CVST can be caused by systemic conditions as well as mechanical factors that reduce blood flow to promote thrombosis. These can include hormonal therapies, pregnancy, malignancy, genetic conditions. trauma, neurosurgical procedures, and adjacent infections (mostly mastoiditis). This case report describes a patient with right transverse sinus thrombosis with no prior risk factors. CASE REPORT A 50-year-old female with no risk factors presented with complaints of headache associated with diplopia for 2 weeks. She did not have any other neurologic signs or symptoms. The patient initially underwent a cerebral magnetic resonance imaging that revealed right transverse sinus thrombosis. She underwent an extensive procoagulant workup and was found to have an increased factor VIII level. All other workups were negative. The patient was started on heparin infusion and bridged to coumadin to achieve a therapeutic international normalized ratio. The patient had improvement in her headache symptoms and was discharged to be followed as an outpatient. CONCLUSIONS We report a case of right transverse sinus thrombosis in a patient with increased factor VIII levels. It is prudent to promptly diagnose cerebral sinus venous thrombosis and start antithrombotic treatment for complete resolution of symptoms.

A Case of an Anomalous Right Coronary Artery Arising from the Left Coronary Cusp.

An anomalous origin of the right coronary artery is usually asymptomatic. It is mostly found incidentally on an invasive diagnostic angiogram. It does lead to an increased risk of sudden cardiac death, especially in younger patients. We present a case of a 41-year-old who had presented to the hospital with complaints of chest pain. The patient was evaluated by cardiology who performed an angiography that identified an anomalous origin of the right coronary artery arising from the left coronary cusp but no evidence of coronary artery disease. Once identified, these anomalous vessels should be corrected surgically, as these conditions increase the risk of sudden cardiac death arrhythmia and ischemic events.

Congenital anomaly of coronary artery: absence of left circumflex artery.

The prevalence of congenital coronary artery anomalies is approximately 1% in the general population. They are a common cause of sudden death in younger persons. Congenital absence of the left circumflex artery is usually a benign condition but can cause symptoms of exertional angina. We present a case of a 59-year-old female who presented with complaints of chest pain. She was evaluated by the cardiology service. An invasive angiogram identified the absence of the circumflex artery, a large right coronary artery, and large septal and diagonal branches of the left main coronary artery possibly as a compensatory mechanism to supply blood to the LCx territories. It is important to define coronary anatomy as anomalies dictate which cardiac intervention should be attempted in cases of ischemia.

Anti-thrombotic therapy strategies with long-term anticoagulation after percutaneous coronary intervention - a systematic review and meta-analysis.

Background: Long-term oral anticoagulants (OAC) increases bleeding risk after the percutaneous coronary intervention (PCI) with dual antiplatelet therapy (DAPT) with Aspirin and P2Y12 inhibitors. We hypothesize that dual anti-thrombotic therapy (DATT) reduces bleeding without increased cardiovascular events. Objectives: DATT does not increase adverse cardiovascular events compared to triple anti-thrombotic therapy (TATT). Method: We searched MEDLINE, PUBMED, Google Scholar, Cochrane and EMBASE from inception to 6 April 2019 for randomized control trials (RCTs) comparing DATT to TATT after PCI. Results: We identified 641 citations (411 after excluding duplicates). Four RCTs with 5,317 patients (3,039 on DATT vs 2,278 on TATT) were included. DATT arm showed significantly reduced [total bleeding, 731 vs. 784, odds ratio [OR] = 0.51, Confidence Interval [CI] = 0.39-0.67, p < 0.00001, I2 = 71% (I2 = 0% without WOEST study)], [TIIMI major bleeding 60 vs. 80, OR = 0.56, CI = 0.4-0.79, p = 0.0009, I2 = 0%], and [TIIMI minor bleeding, 70 vs 126, OR = 0.43, CI = 0.32-0.59, p < 0.00001, I2 = 0%]. There was no difference in subsequent strokes, myocardial infarction, stent thrombosis, and mortality. A trend towards decreased non-cardiac deaths with DATT was observed, 14 vs 26, OR = 0.55, CI = 0.27-1.10, p = 0.09, I2 = 6%. Conclusions: DATT is associated with significantly reduced bleeding and a trend towards reduced non-cardiac death with no difference in adverse cardiovascular outcomes.

Aortoesophageal Fistula: A Fatal Complication of Thoracic Endovascular Aortic Stent-Graft Placement.

BACKGROUND Hemetemesis is rarely caused by an aorta-esophageal fistula with thoracic aorta aneurysm in patients. This uncommon etiology, AEF/TAA, can potentially rupture and cause death if left untreated. Thoracic endovascular aorta repair places a stent-graft to seal the aneurysm and cover the fistulous track. Open surgical repair is associated with high risk of morbidity and mortality; therefore, TEVAR is a much safer alternative to it. However, recurrent or persistent infection remains a major concern with TEVAR for AEF. CASE REPORT We present a rare case of an 80-year-old woman who presented with complaints of hemetemesis and epigastric pain. The patient underwent a computerized tomography scan, highlighting a TAA and AEF. A stent was placed in the descending thoracic aorta via endovascular approach and a subsequent EGD was negative for any residual bleeding. Methicillin-resistant Staphylococcus aureus was isolated from the patient's sputum cultures and she was treated with a prolonged course of antibiotics. She presented to the hospital a few weeks later with new-onset hematemesis. Workup identified an AEF. The patient was high risk for open surgical repair due to her comorbid conditions; therefore, an esophageal stent was placed. She was diagnosed with AEF secondary to an infected endovascular thoracic aorta stent. CONCLUSIONS Patients who are high risk for open surgical repair from immediate rupture of TAA with AEF can benefit from use of the TEVAR approach. The stent itself is a foreign body; therefore, the risk of infection persists. AEF is a rare but potentially fatal complication of the infected thoracic aortic stent itself.