Aortic dissection and multimodality imaging.

Aortic dissection is a life-threatening emergency warranting expeditious diagnosis. Computed tomographic angiography (CTA) is the established gold standard test but is not always fool proof. We report the case of an 18-year-old male patient with traumatic type A aortic dissection which was not evident on the CTA, suggestive on the transthoracic echocardiogram (TTE) and eventually confirmed with a transesophageal echocardiogram (TEE). When the clinical suspicion for dissection is high and in the presence of complications of type A dissection, such as aortic regurgitation, it would be prudent to obtain further imaging with a TTE/TEE to rule in or rule out the diagnosis.

An Overview of Arrhythmias in Pregnancy.

Cardiovascular disease significantly jeopardizes pregnancies in the United States, impacting 1% to 4% of pregnancies annually. Among complications, cardiac arrhythmias are prevalent, posing concerns for maternal and fetal health. The incidence of arrhythmias during pregnancy is rising, partly due to advances in congenital heart surgery and a growing population of women with structural heart disease. While most arrhythmias are benign, the increasing prevalence of more serious arrhythmias warrants a proactive approach. Guidance and reassurance suffice in many cases, but persistent symptoms require cautious use of antiarrhythmic drugs or other therapies for a safe outcome. Managing more serious arrhythmias requires a comprehensive, multidisciplinary approach involving specialists, including maternal-fetal medicine physicians, cardiologists, electrophysiologists, and anesthesiologists.

A Quality Improvement Initiative for Echocardiogram Ordering Patterns in an Academic Hospital.

Background Appropriate Use Criteria (AUC) for echocardiography are a useful tool to deliver quality healthcare. Our quality-based interventional study was designed to assess the trends in appropriate utilization rates for echocardiography in our institution and improve adherence to the AUC criteria for transthoracic echocardiograms (TTE). Methodology A prospective, time series analysis was conducted at the Upstate University Hospital for the months of July 2019 and August 2020. A chart analysis was performed on 620 consecutive inpatients who underwent TTE for the month of July 2019. We assessed the trends of the appropriate ordering of TTEs. We then updated our order form incorporating the 42 most common appropriate indications. A post-intervention chart analysis was performed on all inpatient TTEs ordered for the month of August 2020 (n = 410). The appropriateness of the TTE for the entire group was determined based on the true indication per chart review. The primary outcome was the proportion of appropriate and inappropriate TTEs ordered. Secondary outcomes included assessing for concordance between the indication on the order requisition form and by chart review. A p-value <0.05 was considered significant. Results Using the 2011 AUC for the entire group, 81% of the pre-intervention TTEs and 79.5% of the post-intervention TTEs were appropriate (p = 0.55). There was a statistically significant reduction in the number of discordant TTE orders before and after the intervention (p < 0.01). In addition, we noted increased appropriateness of TTEs in the concordant group both pre and post-intervention. Conclusions Our study demonstrates a significant increase in the concordance between the TTE order sheet and actual indication per chart review with the intervention. This can translate into improved scanning and physician reading quality and time, thereby increasing focus on areas of interest according to the true indication. There was no significant increase in the appropriate TTEs ordered.

Lamin A/C cardiomyopathy presenting as high-grade atrioventricular (AV) block, atrial fibrillation, heart failure and ventricular tachycardia in a single-family cluster.

Mutations in the lamin A/C (LMNA) gene have been associated with both cardiac and skeletal muscle abnormalities. Cardiac manifestations in LMNA cardiomyopathy have a variable age of onset and range from mild to life-threatening. We describe a case series illustrating manifestations of LMNA mutation in a single family with an extensive history of cardiac disease, including sudden cardiac death, and the implications for diagnosis and management. This discussion highlights potential presentations of LMNA mutations and the importance of genetic testing in patients with a family history of conduction abnormalities.

Left Frontal Lobe Cardioembolic Stroke as a Co-presentation of Bicuspid Aortic Valve With Severe Stenosis and a Basal Interventricular Septal Aneurysm in a Young Female Patient.

Bicuspid aortic valve (BAV) and interventricular septum (IVS) aneurysms are common congenital heart defects affecting 1.3% and 0.3% of the population, respectively. The coexistence of membranous types of IVS aneurysm and BAV is even rarer. We report a case of a 48-year-old woman with a history of BAV and severe aortic stenosis who had a seizure in a grocery store and was brought to the emergency department (ED). An MRI of the brain without contrast revealed a left frontal lobe acute lacunar infarct, suggestive of embolic origin. A transesophageal echocardiogram confirmed a basal IVS aneurysm measuring 12.2 mm × 16 mm without intracardiac shunting or thrombi. We diagnosed her with cardioembolic stroke as a complication of BAV and IVS aneurysm and initiated anticoagulation as she did not qualify for surgical intervention. This report emphasizes that IVS aneurysms associated with BAV, although often asymptomatic, may cause adverse outcomes such as cardioembolic stroke. Therefore, timely detection by non-invasive imaging, including echocardiography, CT scans, and MRI, and appropriate intervention are essential to improving health outcomes and survival.

Current Trends in the Management of Epithelial Lacrimal Gland Tumors: A Retrospective National Cancer Database Analysis.

BACKGROUND: Lacrimal gland tumors are rare with data limited to very few large studies. Contemporary strategies like orbit sparing surgeries and neoadjuvant intraarterial chemotherapy remain controversial. METHODS: This is a retrospective cohort analysis of epithelial lacrimal gland tumors from the 2004-2016 National Cancer Database. Patients were stratified based on the type of surgery (limited vs destructive) and various treatment modalities employed. RESULTS: Squamous cell carcinoma (33.48%) and adenoid cystic carcinoma (29.45%) were the commonest histologies (N=669). Comparison of limited (46.33%) vs destructive procedures (53.11%) among 482 patients did not show any survival difference, nor the comparison between surgery vs ± chemotherapy vs ± radiotherapy among 472 patients. CONCLUSION: Squamous cell carcinoma and adenoid cystic carcinoma are the commonest types of lacrimal gland tumors seen in our study. Tumor spread from adjacent sites may have contributed to the higher percentage of squamous cell carcinomas seen. The type of surgery or chemoradiation use did not alter survival.

Association of Marijuana Use and Cardiovascular Disease: A Behavioral Risk Factor Surveillance System Data Analysis of 133,706 US Adults.

BACKGROUND: Marijuana is the most commonly used psychoactive drug, while its effects on cardiovascular health are not well known and remain a subject of interest. METHODS: We used the pooled 2016-2018 data from the Behavioral Risk Factor Surveillance System to perform a cross-sectional analysis evaluating the association of marijuana and cardiovascular disease among US adults who never smoked cigarettes. RESULTS: Among US adults ages 18-74 years, when compared with nonusers, frequent marijuana use was associated with 88% higher odds of myocardial infarction or coronary artery disease (adjusted odds ratio [aOR] 1.88; 95% confidence interval [CI], 1.15-3.08), and 81% higher odds of stroke (aOR 1.81; 95% CI, 1.14-2.89). Among the premature cardiovascular disease group, frequent marijuana users had 2.3 times higher odds of myocardial infarction or coronary artery disease (aOR 2.27; 95% CI, 1.20-4.30), and 1.9 times higher odds of stroke (aOR 1.92; 95% CI, 1.07-3.43). In terms of the modality of marijuana use, frequent marijuana smoking had 2.1 times higher odds of myocardial infarction or coronary artery disease (aOR 2.07; 95% CI, 1.21-3.56), and 1.8 times higher odds of stroke (aOR 1.84; 95% CI, 1.09-3.10). A similar association was observed in the premature cardiovascular disease group who smoked marijuana (aOR [for myocardial infarction or coronary artery disease] 2.64; 95% CI, 1.37-5.09; aOR [for stroke] 2.00; 95% CI, 1.05-3.79). No association was observed between marijuana use in any form other than smoking and cardiovascular disease, across all age groups. CONCLUSION: Frequent marijuana smoking is associated with significantly higher odds of stroke and myocardial infarction or coronary artery disease, with a possible role in premature cardiovascular disease.

Associated factors and outcomes of delaying adjuvant chemotherapy in breast cancer by biologic subtypes: a National Cancer Database study.

PURPOSE: Several studies have evaluated the role of delayed initiation of adjuvant chemotherapy (AC) in breast cancer (BC), but the results have remained controversial and an optimal time has not been defined. Our aim was to determine the effect of time to starting AC from the date of surgery on survival of BC patients, based on estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) status, using data from the National Cancer Database (NCDB). METHODS: A total of 332,927 Stage I-III BC patients who received AC from 2010 to 2016 were analyzed. We included all ER, PR and HER2 statuses and excluded patients with stage 4 and stage 0 (DCIS) disease. The cohort was divided into five groups based on the time of initiating AC from the date of the most definitive surgery i.e., ≤ 30 days, 31-60 days, 61-90 days, 91-120 days and > 120 days. They were further divided into five subgroups based on the receptor status. RESULTS: Hazard ratio (HR) estimates and Kaplan-Meier (KM) analysis shows that starting AC by 31-60 days shows the best survival outcome in all the subtypes, except in hormone positive/HER2 negative BC in which 31-60 days and 61-90 days have similar outcomes. CONCLUSIONS: After surgery for BC, it takes around 4-6 weeks to begin AC and delay in initiating the same leads to poor outcomes. Our results are particularly significant in triple-negative breast cancer (TNBC), similar to prior studies showing a benefit to starting AC as early as possible after surgery.

A Common Medication with an Uncommon Adverse Event: A Case of Doxycycline-induced Pancreatitis.

Drug-induced pancreatitis is a rare entity. The diagnostic criteria for drug-induced pancreatitis include the development of pancreatitis during drug therapy, elimination of all other possible causes, resolution with discontinuation of the offending drug, and reappearance on using the same drug. Several drugs have been implicated in having an association with pancreatitis. Tetracyclines are considered to be a Class I medication (medications implicated in greater than 20 reported cases of acute pancreatitis). However, there are very few reported cases of doxycycline-induced acute pancreatitis. We report the case of a 55-year old male who presented to the emergency department (ED) with three days of progressively severe and constant mid-epigastric abdominal pain. On evaluation, he was found to have elevated lipase levels. Computed tomography (CT) scan of his abdomen revealed findings consistent with pancreatitis without any evidence of gallstones or common bile duct dilation. He denied alcohol use, trauma, and insect bites or stings. His calcium and triglyceride levels were within normal limits. His blood cultures did not show any bacterial growth. He had recently been initiated on doxycycline for concerns of cellulitis and had begun to develop abdominal pain seven days after the initiation of doxycycline. He had completed his antibiotic course on the day of presentation to the ED. He had no other recent medication changes. He had subsequent improvement of symptoms off of the doxycycline and with supportive care. Given that all other causes of pancreatitis had been excluded and that he had been initiated on doxycycline prior to presentation, the etiology was attributed to being likely secondary to doxycycline use. Our case highlights the importance of reviewing outpatient medications by the hospital medicine team and awareness of rare triggers for acute pancreatitis.

Debilitating Darier's Disease and Its Impact on the Quality of Life.

Darier's disease (DD) is a rare, autosomal dominant genodermatosis that occurs due to mutations in the ATP2A2 gene on chromosome 12q23-24 that codes for sarco/endoplasmic reticulum calcium ATPase (SERCA), causing desmosomal breakdown and acantholysis. The disease usually persists for life and is characterized by a relapsing-remitting course. It can be challenging to treat and can cause several complications that may result in frequent hospitalizations. Sepsis, with the damaged skin as the portal of entry, and widespread herpes can occur. Studies have shown genetic links and associations between DD and psychiatric conditions like recurrent depression and bipolar disorder. We report the case of a 70-year-old male with a severe form of the condition and highlight his clinical course. We describe the severe nature of his disease that resulted in multiple complications like recurrent bacterial skin infections, significantly impairing his quality of life and leading to his ultimate demise.

Eyes As the Window to Syphilis: A Rare Case of Ocular Syphilis As the Initial Presentation of Syphilis.

In the pre-antibiotic era, neurosyphilis (NS) was common, occurring in 34% of patients with syphilis. Currently, there has been a rising trend in syphilis with HIV-infected patients being more prone to develop NS. Ocular involvement is very rare in NS and accounts for only 1%-5% of the cases in the United States. We report the case of a 53-year-old male with a past medical history of gastroesophageal reflux disease and hyperlipidemia who presented to his ophthalmologist for blurred vision in both eyes. He had been noticing a black spot in the visual field of his left eye for two weeks. He had also noticed a rash on his forearms. His past and social history was significant for treated Lyme disease, having pet cats. He identified as a heterosexual male, married, and with five children. However, on further history taking, he reported a homosexual exposure about five years prior. He denied any history of genital ulcer or penile discharge. On examination at the ophthalmology clinic, he was found to have a visual acuity of 20/20 right eye and 20/100 left eye. Posterior segment examination of the both eyes showed subtle neuritis and vasculitis. Fundus photography revealed subtle neuroretinitis bilaterally. Work up was initiated for inflammatory and infectious causes. His rapid plasma reagin and fluorescent treponemal antibody absorption showed positive titers for syphilis. His presentation was most consistent with ocular syphilis. A lumbar puncture (LP) was done with Venereal Disease Research Laboratory (VDRL) positivity in the spinal fluid. He was therefore initiated on intravenous (IV) penicillin four million units every four hours for 14 days. His ophthalmology follow-up after one month showed both subjective and objective improvement in his visual symptoms. He also followed with the infectious disease team and a repeat LP done three months later showed nonreactive VDRL in cerebrospinal fluid (CSF). Ocular syphilis is increasing in incidence. Clinical presentation is variable, and a high index of suspicion with a low threshold for serological testing are important as early treatment can reverse retinal changes and restore visual acuity. There is a recommendation for CSF examination in all patients with ocular syphilis including HIV-negative cases. There have been studies showing a high CSF abnormal rate in HIV-negative patients with ocular syphilis. The recommended treatment for NS is aqueous crystalline penicillin G (18 to 24 million units per day, administered as three to four million units IV every four hours, or 24 million units daily as a continuous infusion) for 10 to 14 days. Follow-up is a key component of management with neurological examination and LP for CSF VDRL performed three months after treatment and every six months after, until the CSF is nonreactive for VDRL with normal white blood cell count. It is important to be cognizant of the rising trend of ocular syphilis, even in HIV-negative individuals. Early treatment is time sensitive to preventing permanent vision loss. Our case also emphasizes on thorough history taking, even for patients who appear to be at a low risk for sexually transmitted infections.

Hemodynamic Collapse Following Therapeutic Plasma Exchange in a Patient Receiving an Angiotensin Receptor Blocker.

Therapeutic plasma exchange (TPE) is a procedure for removal of plasma and its components while leaving behind cellular elements via an apheresis device. It is used in multiple conditions one among which is systemic lupus erythematosus (SLE). Adverse reactions from TPE range from mild hypotension and fever to life-threatening cardiovascular compromise. We report the case of sudden hemodynamic collapse following TPE for a neuropsychiatric lupus flare in a patient on losartan. A 62-year-old Caucasian female with a history of drug-induced lupus presented to the hospital with symptoms of a neuropsychiatric lupus flare. She was initiated on TPE with 5% albumin based on recommendations by her rheumatologist. Shortly after TPE, she became hypotensive with poor response to fluid boluses, requiring pressor support and intubation. These symptoms resolved within 24 hours on supportive measures. This was believed to be due to losartan use on the day of TPE. The medication was discontinued and she had further sessions of TPE with no complications. Angiotensin-converting enzyme (ACE) inhibitors have previously been associated with flushing and hypotension in patients undergoing TPE. Patients undergoing TPE have an activation of the prekallikrein and bradykinin system on contact with the extracorporeal membranes. ACE inhibitors potentiate this reaction by inhibiting bradykinin catabolism. Angiotensin receptor blockers (ARBs) have also been postulated to cause elevated bradykinin levels although data pertaining to the use of ARBs in TPE is limited. We hope to highlight this rare interaction in our case and emphasize the need for further data with regard to the same.

Air in the Breast: A Rare Cause of Iatrogenic Pneumomastia.

Air in the breast tissue (pneumomastia) is a rare finding, more so without any history of procedures on the breast. We report the case of an 80-year-old lady who was found to have foci of gas in her right breast on a CT scan. On exclusion of other possible causes for the same, it was concluded that the air in the breast tissue was secondary to a peripheral intravenous line placement. To our knowledge, this is the first reported case of pneumomastia as a complication of peripheral intravenous line placement.

Ductal Adenocarcinoma Ex Pleomorphic Adenoma of the Lacrimal Gland: a Rare and Morbid Malignancy.

Carcinoma ex pleomorphic adenoma (Ca ex PA) is a rare malignant transformation of a benign primary pleomorphic adenoma (PA). We report the case of a 62-year-old male who presented with a swelling over his left temple. Imaging revealed a lytic lesion over the left orbital wall with soft tissue extension suggestive of malignancy. He underwent an en bloc resection of the mass with orbital exenteration, craniotomy, and reconstruction. Pathology demonstrated a lacrimal gland ductal adenocarcinoma arising from a PA which led to the diagnosis of ductal adenoCa ex PA. Postoperatively, he received chemotherapy with 6 cycles of cisplatin and concurrent radiation therapy (RT), but his course was complicated by recurrent bacterial meningitis and abscesses and he ultimately opted for comfort measures. Patients with PA of the lacrimal gland experience an insidiously enlarging painless swelling of the orbit with transformation to Ca ex PA highlighted by a rapid onset of bulbar enlargement, displacement, and often proptosis. Ductal adenoCa ex PA is aggressive with a poor prognosis and has no established standard of care. This case highlights the rarity of this condition and the need for more literature to help direct treatment.

Atypical Cardiac Tamponade Manifesting as Left Ventricular Diastolic Collapse: A Case Report.

Cardiac tamponade is a medical emergency, the diagnosis of which is predominantly clinical with supportive echocardiographic findings. Echocardiographic findings highly suggestive of cardiac tamponade include chamber collapse, inferior vena cava (IVC) plethora, and respiratory volume/flow variations. The right-sided cardiac chambers are a low-pressure system and are the first to show signs of collapse with high specificity for tamponade. We report the case of a 35-year-old woman who demonstrated left ventricular (LV) diastolic collapse on echocardiogram following a tricuspid valve replacement. Although left-sided chamber collapse with tamponade has been reported with localized pericardial effusions postoperatively, our patient had a large circumferential pericardial effusion. Selective chamber compression can be a presenting sign of postoperative tamponade after cardiac surgery. Our case highlights the importance of recognizing atypical forms of cardiac tamponade to help in early identification and emergent management in such patients.

Benign but Terminal: Cardiopulmonary Collapse from a Massive Chest Wall Lipoma.

Lipomas are the most common benign soft tissue tumor. Yet, strikingly simple tumors can become problematic when compounded by odd characteristics such as size and location. We report the case of a 53-year-old male who developed complete right lung collapse secondary to a large right-sided chest wall lipoma with accelerated growth in the past 6 months. Bronchoscopy revealed extrinsic compression of the right mainstem bronchus. Histopathology of the soft tissue mass was suggestive of a lipoma. The mass was not amenable to surgery due to a high risk of mortality from his underlying comorbidities. His hospital stay was complicated by progressive end-stage restrictive lung disease necessitating intubation and eventually a tracheostomy, recurrent pneumonias, multiorgan dysfunction, and his eventual demise. We highlight a rare presentation of an unchecked lipoma, which ultimately led to the death of our patient. Simple lipomas show insidious growth and can remain asymptomatic until they reach a large size. Chest wall tumors should be considered malignant until proven otherwise by excisional biopsy. This reiterates the need to treat all chest wall tumors with wide resection in order to provide the best chance for cure.

Primary Mucinous Adenocarcinoma of the Upper Eyelid in an African American Female: A Rare Clinical Entity. A Case Report and Literature Review.

Primary mucinous adenocarcinoma (PMA) of the eyelid is a rare eccrine gland cancer with an incidence of 0.07 per million person-years. We report a case of a 62-year-old African American female who presented with a tender lesion over her left upper eyelid which was gradually progressive over four years. It was initially presumed to be benign but histopathology after excision was suggestive of a mucinous colloid carcinoma with positive margins. She underwent repeat excision with wide margins and reconstruction and immunohistochemical studies were suggestive of PMA. Workup for metastatic disease and rare possibility of underlying occult malignancy was negative. PMA is uncommon, more so in the African American population and in females. Given the uncommon occurrence of this tumor and similarities in histopathology to colon and breast cancers, underlying occult malignancies need to be ruled out prior to confirming the diagnosis of PMA. The most effective treatment modalities are Mohs micrographic surgery or excision with frozen section control of margins with regular follow up over a prolonged period of time. However, there are no large clinical studies with regard to treatment and follow up. More literature on this tumor would therefore be beneficial to clinicians.