Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease

Ambrusko, Steven J.; Gunawardena, Sriya; Sakara, Allison; Windsor, Beth; Lanford, Lizabeth; Michelson, Peter H.; Krishnamurti, Lakshmanan

doi:10.1002/pbc.20791

Cited by 103 publications

(85 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Suspected obstructive sleep apnea is evaluated via polysomnography and treated accordingly. In a small case series of children with SCD, treatment of obstructive sleep apnea, nocturnal hypoxemia, and asthma reduced the elevated TRV, suggesting that mortality risk can be modified by early intervention (65). Diuretics are used to treat right ventricular volume overload (13), but this must be done carefully to minimize the risk of volume depletion-induced erythrocyte sickling.…”

Section: Associated Conditionsmentioning

confidence: 99%

An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease

Klings¹,

Machado²,

Barst³

et al. 2014

Am J Respir Crit Care Med

Self Cite

215

237

View full text Add to dashboard Cite

Background: In adults with sickle cell disease (SCD), an increased tricuspid regurgitant velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension (PH) diagnosed by right heart catheterization (RHC) are independent risk factors for mortality. Methods: A multidisciplinary committee was formed by clinicianinvestigators experienced in the management of patients with PH and/or SCD. Clinically important questions were posed, related evidence was appraised, and questions were answered with evidencebased recommendations. Target audiences include all clinicians who take care of patients with SCD. Results: Mortality risk stratification guides decision making. An increased risk for mortality is defined as a TRV equal to or greater than 2.5 m/second, an NT-pro-BNP level equal to or greater than 160 pg/ml, or RHC-confirmed PH. For patients identified as having increased mortality risk, we make a strong recommendation for hydroxyurea as first-line therapy and a weak recommendation for chronic transfusions as an alternative therapy. For all patients with SCD with elevated TRV alone or elevated NT-pro-BNP alone, and for patients with SCD with RHC-confirmed PH with elevated pulmonary artery wedge pressure and low pulmonary vascular resistance, we make a strong recommendation against PAH-specific therapy. However, for select patients with SCD with RHC-confirmed PH who have elevated pulmonary vascular resistance and normal pulmonary capillary wedge pressure, we make a weak recommendation for either prostacyclin agonist or endothelin receptor antagonist therapy and a strong recommendation against phosphodiesterase-5 inhibitor therapy.Conclusions: Evidence-based recommendations for the management of patients with SCD with increased mortality risk are provided, but will require frequent reassessment and updating.

show abstract

Section: Associated Conditionsmentioning

confidence: 99%

An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease

Klings¹,

Machado²,

Barst³

et al. 2014

Am J Respir Crit Care Med

Self Cite

215

237

View full text Add to dashboard Cite

show abstract

“…As such, LDH represents a convenient biomarker of intravascular hemolysis and NO bioavailability associated with mortality that Kato and colleagues found helpful in identifying the clinical subphenotypes of hemolysis-associated vasculopathy ( Figure 5). 54 PH is the best characterized clinical complication of acute and chronic hemolysis 55 and occurs in about one third of adults 7,22,56 and children [57][58][59][60][61] with SCD. A Doppler echocardiogram measured tricuspid regurgitant jet velocity (TRV) of 2.5 m/sec or greater, suggesting PH is currently the strongest predictor for early death in SCD, with approximately 10-fold increased risk of early mortality 7,62,63 and a 40% mortality risk within 3 years of diagnosis.…”

Section: Vascular Phenotypes Of Sickle Cell Diseasementioning

confidence: 99%

Mechanisms of Vasculopathy in Sickle Cell Disease and Thalassemia

Morris¹

2008

Hematology

166

214

View full text Add to dashboard Cite

Many mechanisms contribute to the complex pathophysiology of sickle cell disease (SCD), with dysfunction of the vascular endothelium as a unifying theme. Specifically, hemolysis-associated low arginine and nitric oxide (NO) bioavailability, amplified by NO synthase uncoupling, elevated arginase activity, superoxide production, oxidative stress, accumulation of arginine analogs such as asymmetric dimethylarginine, ischemia-reperfusion injury, inflammation, apolipoprotein A-1 depletion, and a hypercoagulable state are significant mechanisms contributing to endothelial dysfunction. Genetic polymorphisms also influence disease severity. Clearly the variable spectrum of disease is the consequence of multiple events and genetic susceptibility that go beyond the occurrence of a single amino acid substitution in the beta globin chain of hemoglobin. Recent studies begin to demonstrate overlap among these seemingly unrelated processes. Impaired NO bioavailability represents the central feature of endothelial dysfunction, and is a common denominator in the pathogenesis of vasculopathy in SCD. The consequences of decreased NO bioavailability include endothelial cell activation, upregulation of the potent vasoconstrictor endothelin-1, vasoconstriction, platelet activation, increased tissue factor, and activation of coagulation, all of which ultimately translate into the clinical manifestations of SCD. Evidence supporting vasculopathy subphenotypes in SCD, including pulmonary hypertension, priapism, cutaneous leg ulceration, and stroke, will be reviewed and relevance to other hemolytic disorders including the thalassemia syndromes will be considered.

show abstract

“…Early retrospective reports on the prevalence of an elevated TRV in children with SCD were similar to adults. 28,[30][31][32][33] The Pulmonary Hypertension and the Hypoxic Response in Sickle Cell Disease (PUSH) study is currently being conducted to evaluate this population prospectively; an interim analysis of the first 399 SCD patients between the ages of 3 and 20 enrolled suggests a slightly lower prevalence (22%) of TRV elevation above 2.5m/sec. 19,34,35 However, based on the mean TRV plus two standard deviations in age-and gender-matched control children, the PUSH study has defined an elevated TRV in children as 2.6 m/sec or above.…”

Section: Doppler-defined Pulmonary Hypertension In Children With Sickmentioning

confidence: 99%

Vascular risk assessment in patients with sickle cell disease

Morris¹

2010

Haematologica

View full text Add to dashboard Cite

Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease

Cited by 103 publications

References 37 publications

An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease

An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease

Mechanisms of Vasculopathy in Sickle Cell Disease and Thalassemia

Vascular risk assessment in patients with sickle cell disease

Contact Info

Product

Resources

About