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.

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.

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.

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.

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.

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.

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.

Anomalous Origin of a Right Coronary Artery from Pulmonary Artery.

Congenital defects of the coronary arteries are noted in 0.2-1.4% of the general population. The first case of an anomalous origin of right coronary artery from pulmonary artery (ARCAPA) was described by Brooks in 1885. ARCAPA has an overall incidence of 0.002% in the general population. Most of the cases are asymptomatic; however, it can lead to serious complications such as heart failure, ischemia, and sudden death. A 57-year-old man presented to the cardiologist's office with complaints of shortness of breath and fatigue. The patient also had a previous history of coronary stents and heart failure. Initially, he was evaluated with a stress test which was reported as abnormal. The patient then underwent an invasive coronary angiography that revealed anomalous origin of the right coronary artery (RCA) and multivessel disease. Cardiothoracic surgery evaluated the patient and coronary artery bypass graft was performed. During the surgery, the anomalous origin of RCA from the pulmonary artery was identified and was successfully corrected by reimplanting the RCA into the ascending aorta. The anomalous origin of RCA is a rare yet life-threatening condition. The RCA due to its location of origin from the pulmonary artery tends to be a low-pressure vessel with a very thin and fragile wall. It also serves as a retrograde venous conduit from the left coronary circulation into the pulmonary artery. This connection results in a left-to-right shunt that explains the increase in oxygen saturation in the pulmonary artery and the high cardiac output which is normally seen in these cases. The clinical presentation can vary from coronary ischemia to heart failure or sudden death. Therefore, surgical correction is recommended even in asymptomatic patients. We present a case of an anomalous origin of RCA from the pulmonary artery which, unlike the origin of left coronary from pulmonary artery, is very rare. Patients with this condition should have early correction even if they are asymptomatic in order to prevent long-term complications.