Daniel Spevack

An Elevated Estimated Pulmonary Arterial Systolic Pressure, Whenever Measured, Is Associated with Excess Mortality in Adults with Sickle Cell Disease

amined the impact of an elevated TR jet on mortality in 215 inpatients or outpatients with SCD who were followed in an urban community-based practice. In multivariate analyses, we found that a mildly elevated ePASP was highly predictive for mortality in this unselected SCD cohort. Although our data could not discriminate whether an elevated ePASP was causal for mortality or epiphenomenological to severe underlying SCD, additional evidence for vasculopathy, i.e. depressed renal function, was also associated with an increased risk for mortality. The Clinical Looking Glass (CLG) is a searchable electronic database developed at Montefiore Medical Center (Bronx, N.Y., USA) that retains clinical and follow-up information on annual inpatient and outpatient visits. All subjects preliminarily identified as having SCD (including homozygous HbSS or compound heterozygous HbS 0-thalassemia and HbSC) were confirmed by review of clinically obtained hemoglobin electrophoreses. Charts for subjects with SCD who had undergone echogardiographic examinations between 2000 and 2008 (identified via ICD9 diagnostic codes) were reviewed for comorbid conditions or complications. Use of hydroxyurea, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, x02 -blockers, and calcium chanSickle cell disease (SCD) has a complex clinical phenotype that belies the simple underlying molecular defect, in which a single mutation in the x02 -globin chain causes abnormal hemoglobin polymerization. The pleiotropic clinical features of SCD include cardiac, cerebral, and renal vasculopathy, osteonecrosis, and recurrent painful vaso-occlusive crises. An elevated estimated pulmonary arterial systolic pressure (ePASP) has emerged as a major predictor of morbidity and mortality in adults with SCD [1–3] at levels, estimated by tricuspid regurgitant (TR) jet velocity, that are not clinically significant in nonanemic patients. A TR jet velocity of 6 2.5 m/s is present at steady state in approximately 1/3 of SCD outpatients [2] , and is prospectively associated with increased mortality. In addition, mortality during long-term follow-up is increased in SCD patients who have short-term elevations in TR jetestimated PASP during an episode of acute chest syndrome [4] ; transient elevations in TR jet velocity have also been described during vaso-occlusive crises [5] , although the impact of these on long-term mortality is not known. We reasoned that an elevated TR jet, whenever measured (whether during disease activity or during clinical steady state), would be associated with a long-term risk of excess mortality in patients with SCD. Therefore, we exReceived: June 30, 2010 Accepted after revision: December 9, 2010 Published online: February 1, 2011

Disparities in emptying velocity within the left atrial appendage

AIMSnPulsed Doppler measurement of left atrial appendage (LAA) emptying velocity, a marker of left atrium contractile function, has been shown to predict success of cardioversion, thrombo-embolic risk, and maintenance of sinus rhythm after cardioversion and pulmonary vein isolation. However, in the published literature, emptying velocity measurement location is not uniform, and no standard currently exists. We assessed the hypothesis that emptying velocity when acquired near the LAA orifice differs from that at the LAA apex.nnnMETHODS AND RESULTSnThe study group comprised 44 patients (32 in sinus rhythm and 12 in atrial fibrillation) who were able to complete a non-emergent transoesophageal echocardiography. Pulsed Doppler recordings were obtained with the sample volume first positioned 1 cm from the LAA orifice, and then positioned within 1 cm of the LAA apex. At each location, we calculated the average of the peak end-diastolic LAA emptying velocity from five consecutive cardiac cycles. LAA orifice emptying velocity was higher than the apex emptying velocity in all patients. The median velocity at the orifice was 72 cm/s, which was 45% higher than the median velocity at the apex (43 cm/s, P < 0.001). Lower LAA emptying velocity at the orifice was associated with a larger discrepancy between orifice and apex velocities. The ratio of orifice to apex velocity did not vary with orifice velocity. Multivariate analysis demonstrated that clinical patient characteristics were not significant predictors of the discrepancy between orifice and apex velocities.nnnCONCLUSIONnLAA emptying velocity is greater at the LAA orifice compared with the LAA apex. Higher, more easily measured velocity and greater variability observed with orifice measurements make it the location of choice for research and clinical applications.

HIGH PREVALENCE OF APICAL HYPERTROPHIC CARDIOMYOPATHY NOTED IN NON-WHITE PATIENTS REFERRED FOR CLINICAL ECHOCARDIOGRAPHY

Background: Apical hypertrophic cardiomyopathy (ApHCM) is considered to be a relatively rare variant of hypertrophic cardiomyopathy (HCM) in non-Asian populations, with most studies suggesting a prevalence of 1 to 3 percent. We noted a high prevalence of ApHCM encountered on echocardiography in our

AT WHAT FLOW RATE DOES AORTIC VALVE GRADIENT BECOME SEVERELY ELEVATED? IMPLICATIONS FOR GUIDELINE RECOMMENDATIONS ON VALVE AREA CUTOFFS

Current guidelines state: Aortic stenosis (AS) is severe when valve area (AVA) is < 1.0 cm2 and mean aortic valve gradient (mAVG) exceeds 40 mmHg. We sought to determine AVA at which mAVG tends to exceed 40 mmHg in subjects with varied transvalvular flow rates.nnOur echocardiography database was

HOW ACCURATE ARE 3D-ECHO MEASUREMENTS? COMPARISON OF IN-VIVO MEASUREMENT OF PROSTHETIC VALVE SEWING RING DIAMETER TO KNOWN PROSTHESIS SIZE

We evaluated the accuracy of real-time 3D-TEE (RT3DTEE) by measuring mitral (MV) and aortic (AV) prosthetic sewing ring diameter and comparing to known prosthesis size. We also compared RT3DTEE accuracy to standard 2DTEE.nnRT3DTEE and 2DTEE were performed simultaneously in 30 consecutive patients