Clinical Outcomes in Patients With Bicuspid Aortic Valves and Ascending Aorta ≥50 mm Under Surveillance.

Background: Clinical outcomes of bicuspid aortic valve (BAV) patients with ascending aortic diameters ≥50 mm who are under surveillance are poorly defined. Objectives: The purpose of this study was to assess clinical outcomes in BAV patients with ascending aorta ≥50 mm. Methods: Multicenter retrospective cohort study of BAV adults with ascending aorta diameters ≥50 mm by transthoracic echocardiography (TTE). Patients were categorized into 50 to 54 mm and ≥55 mm groups. Clinical outcomes were aortic dissection (AoD), aorta surgery, surgical mortality, and all-cause death. Results: Of 875 consecutive BAV patients (age 60 ± 13 years, 86% men, aortic diameter 51 mm [interquartile range (IQR): 50-53 mm]), 328 (37%) underwent early surgery ≤3 months from index TTE. Of the remaining 547 patients under surveillance, 496 had diameters 50 to 54 mm and 51 had diameters ≥55 mm and were collectively followed for 7.51 (IQR: 3.98-12.20) years. Of 496 patients with diameters 50 to 54 mm under surveillance, 266 (54%) underwent surgery 2.0 (IQR: 0.77-4.16) years from index TTE. AoD occurred in 9/496 (1.8%) patients for an incidence of 0.4 cases per 100 person-years, surgical mortality was 5/266 (1.9%); and ≥moderate aortic stenosis (but not aorta size) was associated with all-cause death, hazard ratio: 2.05 (95% CI: 1.32-3.20), P = 0.001. Conversely, in 547 total patients under surveillance (including 50-54 mm and ≥55 mm), both aorta size and ≥moderate aortic stenosis were associated with all-cause death (both P ≤ 0.027). AoD rate in patients ≥55 mm under surveillance was 5.9%. Conclusions: In BAV patients with ascending aorta 50 to 54 mm under surveillance, AoD incidence is low and the overall rates of AoD and surgical mortality are similar, suggesting clinical equivalence between surgical and surveillance strategies. Conversely, patients with aortas ≥55 mm should undergo surgery. Aortic stenosis is associated with all-cause death in these patients.

Epithelioid angiosarcoma: A rare cause of pericarditis and pleural effusion.

Angiosarcomas are rare cancers accounting for less than 2% of all soft tissue sarcomas. We report the case of an unusual presentation of pleural epithelioid angiosarcoma in a patient with constrictive pericarditis and recurrent pleural effusion. A 62 year old smoker presented with acute chest pain. ECG showed diffuse elevation of ST segments in the precordial leads. After extensive evaluation, he was diagnosed with viral pericarditis and treated with colchicine. Two weeks later the patient presented to the emergency department with a large right pleural effusion. Evaluation of the pleural fluid obtained from a thoracentesis revealed an exudative effusion with negative microbial studies and no evidence of malignant cells. His pleural effusion re-accumulated rapidly, requiring repeated thoracenteses over several weeks. Medical thoracoscopy was performed and pleural biopsy revealed primary pleural epithelioid angiosarcoma. Staging PET scan revealed malignant enhancement of right pleura, pericardium, right iliac bone and right shoulder. He died suddenly within 6 weeks of diagnosis, prior to initiating palliative chemotherapy. Pleural angiosarcoma should be considered in the differential diagnosis of recurrent pleural effusions of unknown etiology. Negative cytology does not rule out the diagnosis; excisional biopsy is required. Reported risk factors include asbestos exposure, prior chest radiation, active smoking and history of complicated pleural tuberculosis. Pleural epithelioid angiosarcomas carry a very poor prognosis, with the majority of patients dying within months of diagnosis.

A rare cause of oligoarthritis with septic presentation.

A 33-year-old man presented with new-onset, asymmetric, migratory oligoarthritis in the setting of several weeks of nausea and vomiting, diarrhoea, fevers and dysuria. He was initially treated in the inpatient setting with broad-spectrum antibiotics due to concern for an evolving sepsis presentation. Arthrocentesis of a large right knee effusion revealed inflammatory synovial fluid without findings suggestive of septic arthritis. Human leucocyte antigen B27 was positive and, taken together with the antecedent history of gastroenteritis, dysuria and inflammatory oligoarthritis, the clinical diagnosis was most consistent with reactive arthritis. Antibiotics were discontinued. His treatment course proved refractory to non-steroidal anti-inflammatory drugs and intra-articular and systemic glucocorticoid therapy with concurrent use of sulfasalazine and ultimately necessitated treatment with a tumour necrosis factor alpha inhibitor.