Prospective Echocardiography Assessment of Pulmonary Hypertension and Its Potential Etiologies in Children With Sickle Cell Disease††Conflicts of interest: Dr. Gordeuk has received grants from BioMarin Pharmaceutical Inc., Novato, California, and Actelion Pharmaceuticals Ltd., Allschwil, Switzerland, and is a consultant for Ikaria Holdings, Clinton, New Jersey.

Abstract

Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle cell disease (SCD) but its importance in children is less clear. We define the incidence and etiologies of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk, and hematologic testing. Echocardiographic measures were compared between SCD and control subjects before and after adjusting for hemoglobin. Correlation of echocardiographic and clinical parameters was performed. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs. 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlled for left ventricular diameter and left atrial pressure. An echocardiography-derived pulmonary resistance was not significantly different between SCD and control patients, although it was elevated in the SCD subgroup with elevated © 2009 Excerpta Medica, Inc. Published by Elsevier Inc. All rights reserved.Correspondence to: Craig Sable, MD, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, Tel: 202-476-2020, Fax: 202-476-5700, Email: csable@cnmc.org. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. TRV. When controlled for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end diastolic velocity, and markers of increased cardiac output correlated with indicators of adverse functional status including history of acute chest syndrome, stroke, transfusions, and 6-minute walk. In conclusion, children with SCD have mildly increased TRV that correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin adjusted analysis also suggests contribution of primary vascular changes. KeywordsPulmonary hypertension; Tricuspid regurgitation; Pulmonary vascular resistance; Sickle cellIn the present report, we compare multiple echocardiographic variables between children with sickle cell disease (SCD) and age and gender matched controls to assess relative contributions of potential determinants of pulmonary artery pressure in this population. We also examine the relations among TRV, other echocardiographic measurements and clinical parameters among children with SCD to better elucidate the physiology and consequences of echo...

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“…As noted previously in pediatric populations 17, 18 , we found that a high TRV in our adult patients with SCD was rarely associated with an elevated PVR. Similarly, Parent, et al 16 found an elevated PVR on right heart catheterization in only a small proportion of patients with high TRV.…”

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“…LAVs were indexed to body surface area. LA emptying fraction (LAEF) to assess LA function was expressed as: LAEF (%) = 100 · [(LAV max − LAV min )/LAV max ] (19–21). …”

Infrared imaging of nitric oxide-mediated blood flow in human sickle cell disease

“…Anemia is known to increase the tricuspid valve regurgitant velocity (TRV) (Dham et al, 2009) and so a term for this significant interaction was included in the model. Diastolic blood pressure and TRV were positively associated with baseline skin temperature, while hemoglobin concentration, serum homocysteine concentration and serum total cholesterol were negatively associated with baseline forearm skin temperature (Table 2, R 2 = 0.84, F = 12.6, p < 0.0001 for the model).…”

“…We also found warm baseline skin temperature to be associated with the degree of anemia, another established marker of disease severity in sickle cell patients. One interpretation of these findings is that anemia leads to a compensatory increase in cardiac output, which contributes to both higher TRV(Dham et al, 2009) and greater perfusion of the skin at baseline.…”