Estimated pulmonary artery systolic pressure and sickle cell disease: a meta‐analysis and systematic review

  title={Estimated pulmonary artery systolic pressure and sickle cell disease: a meta‐analysis and systematic review},
  author={Melissa C. Caughey and Charles L Poole and Kenneth I. Ataga and Alan L Hinderliter},
  journal={British Journal of Haematology},
Many studies report estimated pulmonary artery systolic pressure (ePASP) in patients with sickle cell disease (SCD) screened by echocardiography. To better understand the prevalence and outcomes of elevated ePASP in clinically stable SCD patients, we conducted a random‐effects meta‐analysis. A total of 45 studies, representing 15 countries and including 6109 individuals, met our inclusion criteria. In most (70%) studies, elevated ePASP was defined by a tricuspid regurgitant velocity of 2·5 m/s… 
Prevalence of pulmonary hypertension in children with sickle cell disease
High pulmonary artery pressures do occur in children with sickle cell disease and Screening by echocardiography can lead to early detection and intervention.
Exercise‐induced changes of vital signs in adults with sickle cell disease
Any increase in SBP during the 6MWT was associated with improved survival and may be indicative of a patient's ability to increase stroke volume, and VSC is associated with markers of anemia and TRV and can be used for risk stratification.
Cardiac manifestations in sickle cell disease varies with patient genotype
Cardiac involvement in SCA was related to anaemia and haemolysis, while LV diastolic dysfunction and TRV in HbSC disease patients were related to arterial hypertension and overweight comorbidities.
Doppler-Defined Pulmonary Hypertension in Sickle Cell Anemia in Kurdistan, Iraq
It is revealed that pulmonary hypertension is rather frequent among Iraqi Kurds with sickle cell anemia, and reticulocyte count is identified as an independently associated parameter with PH in this population.
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An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity on echocardiography, consistent with a calculated pulmonary artery systolic pressure of >140 mm Hg.
Cardiovascular complications in patients with sickle cell disease.
  • M. Gladwin
  • Medicine
    Hematology. American Society of Hematology. Education Program
  • 2017
A number of tests have been validated in multiple replicate cohort studies that identify patients with SCD at the highest risk of experiencing pulmonary and systemic vasculopathy and death, providing for screening strategies tied to targeted, more aggressive diagnostic and therapeutic interventions.
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Screening for hip and eye disorder is recommended; however, different countries adopt different screening strategies and Hydroxyurea is probably of potential benefit in preventing chronic organ damage but this requires further study in order to be fully demonstrated.
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PH can develop in children with SCD and, while the exact clinical implications are still being elucidated, current guidelines and research are aimed at early identification and treatment to improve outcomes.
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This review aims to assemble what has been learnt from clinical and animal studies about the manifestation of PH-HFpEF in SCD, specifically the contributions of LV diastolic dysfunction and myocardial fibrosis, in an attempt to gain an understanding of its evolution.
Pulmonary Hypertension in Adults with Sickle Cell Anaemia: A Prevalence Study in the Niger Delta Region of Nigeria
The prevalence of pulmonary hypertension in adults living with Sickle Cell Anaemia in this study is 5.7% and it is significantly influenced by the age of the study cohort and the method used in assessing this parameter.


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Pulmonary hypertension is common among Nigerian sickle cell disease patients and the public health implication is significant considering the potential number of individuals at risk for this complication.
A Prospective Appraisal of Pulmonary Hypertension in Children With Sickle Cell Disease
Elevation of TRV was relatively uncommon in pediatric patients as compared with prevalence reported in adults and may be associated with increased hemolysis, and there was no association with obstructive sleep apnea or abnormal pulmonary function.
Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease
This study reinforced the use of echocardiography as a screening tool for PH in SCD and the mandatory role of RHC for proper diagnosis and confirmed the prognostic significance of PH inSCD as its association to pronounced haemolytic profile.
Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom
Age >47 years, male gender, chronic transfusions, WHO class III–IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death.
Pulmonary hypertension in sickle cell disease children under 10 years of age
Significant correlation was found between precocious TRV elevation and high platelet and reticulocyte counts and frequent acute chest syndromes (ACS), and ACS was the only variable predicting TRV ≥2·5 m/s.
Prevalence and Risk Factors of Elevated Pulmonary Artery Pressures in Children With Sickle Cell Disease
Screening by echocardiography can lead to early detection and intervention that may potentially reverse this disease process, and high pulmonary artery pressures do occur in children with sickle cell disease.
Abnormalities in cardiac structure and function in adults with sickle cell disease are not associated with pulmonary hypertension.
Paediatric sickle cell disease: pulmonary hypertension but normal vascular resistance
PVR should be measured before therapy with agents such as sildenafil or bosentan is contemplated, and pulmonary hypertension due to raised cardiac output is common in HbSS SCD children and is associated with normal PVR.
Pulmonary hypertension in sickle cell disease.
Recommendations include intensification of sickle cell-directed therapies, treatment of causal factors or associated diseases, general supportive measures, and use of PH-specific pharmacologic agents to improve survival.
Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia
Higher baseline hemolytic component and E/Etdi predicted elevated tricuspid regurgitation velocity at both baseline and follow up, and these elevations in turn predicted decline in six-minute-walk distance.