Porcine Bioprosthetic Aortic Valve Endocarditis with Ring Abscess and Aortic Stenosis

Porcine bioprosthetic valve endocarditis is an infrequent but serious complication of valve replacement surgery. Ring (or annular) abscess is a frequent finding in mechanical valve endocarditis. In contrast, porcine valve endocarditis most often involves the cusps, and annular infection is uncommon. Porcine valvular dysfunction secondary to endocarditis usually takes the form of incompetence, whereas stenosis is less frequent. We report a case of a 76-year-old female who developed endocarditis wilh Staphylococcus epidermidis nine months after placement of a Carpenter-Edwards porcine aortic valve. Her initial presentation included complete heart block and moderate aortic stenosis. Transesophageal echocardiography aided the diagnosis by demonstrating large vegetations, while transthoracic echocardiography showed only slight thickening of the valve leaflets. At operation, there was a circumferential abscess around the sewing ring causing valve dehiscence and virtual discontinuity of the aorta from the left ventricle. Valve degeneration and organisms within the cusps were observed on microscopy. This case illustrates two infrequent complications of porcine aortic valve endocarditis, namely massive annular abscess with invasion of the conducting system and aortic stenosis. It also demonstrates the utility and limitations of transesophageal echocardiography in the diagnosis of this disorder

Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom

Background: The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. Methods and Results: We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67-75% positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2% had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83% with TRV≥3.0 m/sec and 98% with TRV47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death. Conclusions: A TRV≥ 3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable. The study is registered in ClinicalTrials.gov with identifier: NCT00492531

Erythroid-Specific Transcriptional Changes in PBMCs from Pulmonary Hypertension Patients

Gene expression profiling of peripheral blood mononuclear cells (PBMCs) is a powerful tool for the identification of surrogate markers involved in disease processes. The hypothesis tested in this study was that chronic exposure of PBMCs to a hypertensive environment in remodeled pulmonary vessels would be reflected by specific transcriptional changes in these cells.The transcript profiles of PBMCs from 30 idiopathic pulmonary arterial hypertension patients (IPAH), 19 patients with systemic sclerosis without pulmonary hypertension (SSc), 42 scleroderma-associated pulmonary arterial hypertensio patients (SSc-PAH), and 8 patients with SSc complicated by interstitial lung disease and pulmonary hypertension (SSc-PH-ILD) were compared to the gene expression profiles of PBMCs from 41 healthy individuals. Multiple gene expression signatures were identified which could distinguish various disease groups from controls. One of these signatures, specific for erythrocyte maturation, is enriched specifically in patients with PH. This association was validated in multiple published datasets. The erythropoiesis signature was strongly correlated with hemodynamic measures of increasing disease severity in IPAH patients. No significant correlation of the same type was noted for SSc-PAH patients, this despite a clear signature enrichment within this group overall. These findings suggest an association of the erythropoiesis signature in PBMCs from patients with PH with a variable presentation among different subtypes of disease.In PH, the expansion of immature red blood cell precursors may constitute a response to the increasingly hypoxic conditions prevalent in this syndrome. A correlation of this erythrocyte signature with more severe hypertension cases may provide an important biomarker of disease progression

Recurrent Leukemia Cutis in Acute Myeloblastic Leukemia

We report the case of a 64-year-old female with acute myeloblastic leukemia (French-American- British classification: M2) who developed two specific cutaneous manifestations during her illness. She presented with extensive cellulitis involving the face, neck, and upper chest wall. While the cellulitis resolved with antibiotic therapy, a fungating ulcerated nodule remained on the lower lip which proved to be leukemic on biopsy. Concomitant blood and bone marrow findings were diagnostic of acute myeloblastic leukemia. The lip lesion cleared with a course of chemotherapy. An erythematous macular rash subsequently developed over the lower trunk which was thought to be an allergic reaction to the penicillin treatment. However biopsy results were consistent with leukemia cutis. A repeat bone marrow examination revealed excessive blasts. Our observations emphasize the various presentations of leukemia cutis and the need to biopsy any cutaneous lesion of unclear etiology in the setting of acute leukemia