Giant Left Atrial Myxoma Masquerading as Cough-Syncope Syndrome

Left atrial myxomas are the most common type of benign primary cardiac tumor. Patients can present with generalized symptoms, such as fatigue, symptoms from obstruction of the myxoma, or even embolization of the myxoma causing distal thrombosis. We describe a case with several-month duration of syncopal episodes that occurred after coughing and with exertion. Computed tomography of the chest showed a 6.1 cm by 4.5 cm mass in the left atrium, later evaluated with an echocardiogram. Cardiothoracic surgery removed the mass, and it was determined to be an atrial myxoma. It is important for an internist to be able to diagnose an atrial myxoma because of the risks associated with embolization and even sudden death as myxoma can block blood supply from atrium to ventricle

Autoimmune Pancreatitis Type 2: Case Report

© 2017, © 2017 American Federation for Medical Research. A middle-aged man presents with acute pancreatitis of unknown etiology and is found to have a presentation consistent with the diagnosis of type 2 autoimmune pancreatitis (AIP). AIP is a group of rare heterogeneous diseases that are challenging to diagnose. There are 2 types of AIP. Type 1 disease is the more common worldwide than type 2 AIP. While type 1 AIP is associated with IgG4-positive antibodies, type 2 AIP is IgG4 antibody negative. Both types of AIP are responsive to corticosteroid treatment. Although type 1 AIP has more extrapancreatic manifestations and more commonly relapses, this is a case of a patient with type 2 AIP with inflammatory bowel disease and relapsing course

Case Reports1. A Late Presentation of Loeys-Dietz Syndrome: Beware of TGFβ Receptor Mutations in Benign Joint Hypermobility

Background: Thoracic aortic aneurysms (TAA) and dissections are not uncommon causes of sudden death in young adults. Loeys-Dietz syndrome (LDS) is a rare, recently described, autosomal dominant, connective tissue disease characterized by aggressive arterial aneurysms, resulting from mutations in the transforming growth factor beta (TGFβ) receptor genes TGFBR1 and TGFBR2. Mean age at death is 26.1 years, most often due to aortic dissection. We report an unusually late presentation of LDS, diagnosed following elective surgery in a female with a long history of joint hypermobility. Methods: A 51-year-old Caucasian lady complained of chest pain and headache following a dural leak from spinal anaesthesia for an elective ankle arthroscopy. CT scan and echocardiography demonstrated a dilated aortic root and significant aortic regurgitation. MRA demonstrated aortic tortuosity, an infrarenal aortic aneurysm and aneurysms in the left renal and right internal mammary arteries. She underwent aortic root repair and aortic valve replacement. She had a background of long-standing joint pains secondary to hypermobility, easy bruising, unusual fracture susceptibility and mild bronchiectasis. She had one healthy child age 32, after which she suffered a uterine prolapse. Examination revealed mild Marfanoid features. Uvula, skin and ophthalmological examination was normal. Results: Fibrillin-1 testing for Marfan syndrome (MFS) was negative. Detection of a c.1270G > C (p.Gly424Arg) TGFBR2 mutation confirmed the diagnosis of LDS. Losartan was started for vascular protection. Conclusions: LDS is a severe inherited vasculopathy that usually presents in childhood. It is characterized by aortic root dilatation and ascending aneurysms. There is a higher risk of aortic dissection compared with MFS. Clinical features overlap with MFS and Ehlers Danlos syndrome Type IV, but differentiating dysmorphogenic features include ocular hypertelorism, bifid uvula and cleft palate. Echocardiography and MRA or CT scanning from head to pelvis is recommended to establish the extent of vascular involvement. Management involves early surgical intervention, including early valve-sparing aortic root replacement, genetic counselling and close monitoring in pregnancy. Despite being caused by loss of function mutations in either TGFβ receptor, paradoxical activation of TGFβ signalling is seen, suggesting that TGFβ antagonism may confer disease modifying effects similar to those observed in MFS. TGFβ antagonism can be achieved with angiotensin antagonists, such as Losartan, which is able to delay aortic aneurysm development in preclinical models and in patients with MFS. Our case emphasizes the importance of timely recognition of vasculopathy syndromes in patients with hypermobility and the need for early surgical intervention. It also highlights their heterogeneity and the potential for late presentation. Disclosures: The authors have declared no conflicts of interes

Aortic Dissection in a Healthy Male Athlete: A Unique Case with Comprehensive Literature Review

A young otherwise healthy 27-year-old male who has been using anabolic steroids for a long time developed Type I aortic dissection associated with heavy weightlifting. The patient did not have a recent history of trauma to the chest, no history of hypertension, and no illicit drug use. He presented with severe chest pain radiating to back and syncopal event with exertion. Initial vitals were significant for blood pressure of 80/50 mmHg, pulse of 80 beats per minute, respirations of 24 per minute, and oxygen saturation of 92% on room air. Physical exam was significant for elevated jugular venous pressure, muffled heart sounds, and cold extremities with diminished pulses in upper and absent pulses in lower extremities. Bedside echocardiogram showed aortic root dilatation and cardiac tamponade. STAT computed tomography (CT) scan of chest revealed dissection of ascending aorta. Cardiothoracic surgery was consulted and patient underwent successful repair of ascending aorta. Hemodynamic stress of weightlifting can predispose to aortic dissection. Aortic dissection is a rare but often catastrophic condition if not diagnosed and managed acutely. Although rare, aortic dissection needs to be in the differential when a young weightlifter presents with chest pain as a delay in diagnosis may be fatal

Autoimmune Pancreatitis Type 2: Case Report

A middle-aged man presents with acute pancreatitis of unknown etiology and is found to have a presentation consistent with the diagnosis of type 2 autoimmune pancreatitis (AIP). AIP is a group of rare heterogeneous diseases that are challenging to diagnose. There are 2 types of AIP. Type 1 disease is the more common worldwide than type 2 AIP. While type 1 AIP is associated with IgG4-positive antibodies, type 2 AIP is IgG4 antibody negative. Both types of AIP are responsive to corticosteroid treatment. Although type 1 AIP has more extrapancreatic manifestations and more commonly relapses, this is a case of a patient with type 2 AIP with inflammatory bowel disease and relapsing course

Early Post-Percutaneous Coronary Intervention Chest Pain: A Nationwide Survey on Interventional Cardiologists\u27 Perspective

Background: Early post-percutaneous coronary intervention chest pain (EPPCP) appears to be a common clinical phenomenon. EPPCP has not been fully explained or studied in the literature despite the abundance of clinical trials on percutaneous coronary intervention (PCI). The objective of this questionnaire-based survey is to assess the current perception of EPPCP among practicing interventional cardiologists nationwide. Methods: A survey questionnaire was designed utilizing the Survey Monkey tool to address the perceptions and current practices regarding key aspects of EPPCP among interventional cardiologists. The survey was sent to the interventional cardiologists via email. Results: The survey questionnaire regarding EPPCP was provided to 2615 practicing interventional cardiologists and resulted in 623 total survey responses, with 503 of those respondents completing all eight survey questions. A total of 50.2% of the interventional cardiologists perceive that the incidence of EPPCP is 5–10%, and 57.5% consider that repeat angiography or PCI is rarely needed (1 in 1000 cases). A total of 47.1% of the participants think that EPPCP is due to transient microvascular dysfunction, while 39% perceive it as a different entity requiring a different approach. When asked about developing a standardized labeling for the phenomenon of EPPCP, 34.8% of responders indicated that they believe EPPCP should be labeled as a benign form of chest pain/angina, and 28% preferred to describe EPPCP in non-standardized terms. Among interventional cardiologists, 80% thought that the treatment of this entity is a combination of reassurance and vasodilators and, without ischemic ECG changes, medical management is appropriate. Conclusion: A total of 72% of interventional cardiologists in our survey preferred to label EPPCP as standard nomenclature to facilitate communication between healthcare providers, patients and families in a consistent way. There is a diversity of opinion regarding EPPCP, no standard nomenclature, and no guideline to standardize practice. Further large-scale prospective studies are needed to better understand the pathophysiological mechanisms, optimal management strategies, prognostic implications, and clinical reporting of EPPCP

Comprehensive Review on Methadone-Induced QT Prolongation and Torsades

An alternative analgesic to morphine is methadone, which is used to control chronic pain and is used in opioid rehabilitation treatment programs due to methadone having a long half-life and being relatively inexpensive as compared to extended-release forms of morphine. Despite its benefits, methadone accumulates in adipose tissue due to being lipophilic, binds strongly to plasma proteins, and is metabolized in the liver by the cytochrome P450 system causing methadone levels to be variable and subject to influence according to the individual body compositions and concurrent use of cytochrome P450 inhibitors. In addition to methadone being able to cause both respiratory and central nervous system depression, methadone can also prolong the QT interval and cause potentially life-threatening arrhythmias including torsades de pointes. The susceptibility of unintentional overdosing of methadone due to its varied pharmacologic properties and potentially fatal induction of arrhythmias may cause the risks of methadone use to outweigh its benefits and therefore must be closely monitored