Antiphospholipid Syndrome in a Patient With Autosomal Dominant Polycystic Kidney Disease: The Surface of the Moon.

Antiphospholipid syndrome (APS) is a rare coagulopathic disorder diagnosed with a combination of clinical/imaging findings with specific antibody titer elevations over a period of 12 weeks. The following case report will discuss the unusual and challenging hospital course of a patient with extensive autosomal dominant polycystic kidney disease (ADPKD) being treated for a multi-drug resistant urinary tract infection (UTI). The patient later developed multiple deep vein thrombosis (DVT) and was found to have antiphospholipid syndrome. Warfarin, the anticoagulant of choice for antiphospholipid syndrome, has a higher likelihood of intracerebral hemorrhage than direct oral anticoagulants. This is particularly challenging since patients with autosomal dominant polycystic kidney disease have a higher propensity to develop intracranial aneurysms (ICA).

Ruptured Bullae: A Case of Transthyretin Cardiac Amyloidosis.

Amyloidosis is a rare disease with an incidence of only 16.6 per 100,000 patients per year. A high grade of clinical suspicion is required to suspect an atypical cause of left ventricular hypertrophy or new-onset heart failure. A transthoracic echocardiogram (TTE) is the initial evaluation that may yield clues pointing towards an etiology of cardiac amyloidosis. Due to the subjective nature of TTE interpretations, suspicion for cardiac amyloidosis may be missed. Once suspicion arises, additional tests, such as serum and urine electrophoresis and technetium-99m pyrophosphate myocardial perfusion imaging, can further aid in establishing a diagnosis. The pathophysiology in transthyretin amyloidosis (ATTR) involves the misfolding of the transthyretin/prealbumin protein, which leads to an inherent propensity to aggregate. These proteins can accumulate in the extracellular space between cardiac myocytes, which may thicken sections of the heart, leading to ventricular restriction. Here, we explore the case of an 83-year-old man with chronic, treatment-resistant heart failure with preserved ejection fraction, New York Heart Association class III, who presented with multiple ruptured bullae in the bilateral lower extremity, leading to a new diagnosis of ATTR cardiac amyloidosis.

Perpetually Positive: Post-COVID Interstitial Lung Disease in an Immunocompromised Patient With Diffuse Large B-cell Lymphoma.

As more patients recover from COVID-19 infection, long-term complications are beginning to arise. Our case report will explore a debilitating long-term complication, Post-COVID Interstitial Lung Disease (PC-ILD). We will introduce a patient who developed PC-ILD in the setting of diffuse large B-cell lymphoma, outlining a difficult hospital course, including a positive COVID-19 polymerase chain reaction (PCR) for more than 3 months. We will then discuss the human body's physiological response to the virus and how our patient was not able to adequately mount an immune response. Finally, the pathophysiology of PC-ILD will be explored and correlated with the patient's subsequent computed tomographic images obtained over a 3-month period. The difficult hospital course and complex medical decision-making outlined in this case report serve as a reminder for health care providers to maintain vigilance in protecting our most vulnerable patient population from such a devastating disease process.

Myocardial ischemia in patients with diastolic dysfunction and heart failure.

Coronary artery disease is present in 40-55% of patients with diastolic heart failure, and myocardial ischemia is both a cause and a precipitant of diastolic heart failure. Failure to recognize and treat acute and chronic ischemia in patients with this disorder results in rapid disease progression and poor outcomes. In diastolic heart failure patients without obstructive coronary artery disease, ischemia can be induced by other diseases that diminish perfusion gradient, cause myocardium to outgrow blood supply, or decrease diastolic filling time. In this article, we review the role of ischemia and development of fibrosis in the epidemiology, pathophysiology, and evaluation of patients with diastolic dysfunction and diastolic heart failure.

CHADS(2) score is predictive of left atrial thrombus on precardioversion transesophageal echocardiography in atrial fibrillation.

OBJECTIVE: The goals of this study were to determine: 1) if the CHADS(2) score correlates with left atrial (LA) or left atrial appendage (LAA) thrombus on pre-cardioversion transesophageal echocardiography (TEE) in nonvalvular atrial fibrillation (NVAF); and 2) what, if any, components of the CHADS(2) score are most important in predicting LA/LAA thrombus. BACKGROUND: It is unknown if CHADS(2) score, a marker of thromboembolic risk in NVAF, accurately predicts LA/LAA thrombus on pre-cardioversion TEE. METHODS: We retrospectively studied patients undergoing precardioversion TEE for NVAF at a tertiary hospital. TEE reports were reviewed for presence of LA/LAA thrombus. Using medical records and an ICD-9 coding database, a CHADS(2) score was derived, and the association between CHADS(2) and thrombus was evaluated with Mantel-Haenszel Chi-Square. The relation between the singular components of CHADS(2) and thrombus were analyzed using Pearson's Chi-Square. RESULTS: In 643 consecutive patients undergoing pre-cardioversion TEE, LA/LAA thrombus was identified in 46 (7.2 %). A strong association was present between CHADS(2)score and LA/LAA thrombus (p = 0.0005). No thrombi were identified in patients with CHADS(2) = 0. Among 46 patients with thrombus, all (100%) had CHF. Of the singular components, CHF was the only factor independently associated with thrombus (p < 0.0001). CONCLUSIONS: In non-valvular atrial fibrillation, CHADS(2) is strongly associated with LA thrombus on TEE. Our findings suggest pre-cardioversion TEE may be unnecessary if the CHADS(2) score = 0. Of the components of the CHADS(2) score, CHF was the only independently associated risk factor which correlated with LA/LAA thrombus.

Audiocardiography in the cardiovascular evaluation of the morbidly obese.

Morbid obesity is believed to limit cardiovascular auscultation. We compared audiocardiography to senior attending physicians using conventional stethoscopes in 190 individuals with morbid obesity. Overall, there were 128 (67.4%) women and 62 (32.6%) men with mean ages of 44.9 +/- 12.3 and 51.3 +/- 10.8 , respectively (P = 0.001). The overall body mass index (BMI) was 47.3 +/- 8.5 kg m(-2). Of those with an S(3) by audiocardiography (n = 7), one had a history of coronary artery disease (CAD), none had a history of heart failure, and one had a left ventricular ejection fraction (LVEF) <45%. The mean LVEF was 58.6 +/- 9.9 versus 61.6 +/- 5.3 for those with and without an S(3) by audiocardiography (P = 0.16). By contrast, of those (n = 6) with an S(3) by stethoscope, one had a history of CAD, two had histories of heart failure, and 3 had LVEF < 45%. The mean LVEF of those with and without S(3) by stethoscope was 53.7 +/- 2.3 and 61.6 +/- 5.5%, respectively (P = 0.02). There were 40 (21.1%) patients with an S(4) (S(4) strength >5) identified by acoustic cardiography while there were 42 (22.1%) heard by the stethoscope and it was heard with both methods in nine patients (21.4% concordance). There were no significant correlations between BMI or peak oxygen consumption and S(3) or S(4) strength by audiocardiography. Acoustic cardiography performed with an electronic device was not helpful in assisting the cardiovascular examination of the morbidly obese. These data suggest the careful clinical exam with attention to traditional cardiac auscultation using a stethoscope in a quiet room should remain the gold standard.