Exercise‐Induced Abnormal Increase of Systolic Pulmonary Artery Pressure in Adult Patients With Sickle Cell Anemia: An Exercise Stress Echocardiography Study

Lima-Filho, Newton Nunes de; Figueiredo, Maria Stella; Vicari, Perla; Cançado, Rodolfo Delfini; Ac, Carvalho; Bordin, José Orlando; Campos, Orlando

doi:10.1111/echo.12853

Cited by 12 publications

(12 citation statements)

References 55 publications

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“…Furthermore, more than half of hemoglobin SS adults without elevated TRV at rest develop abnormally high PA systolic pressure with exercise. 75 Hemoglobin SS adults with even a modest increase in systolic pulmonary pressure have reduced exercise tolerance 61 and decreased survival. 13,76,77 In addition, 10% to 15% of the pediatric SCD population have elevated systolic pulmonary artery pressure 78 ; although the effects on survival are unclear, there appears to be reduced exercise tolerance with this finding.…”

Section: Postcapillary Pulmonary Hypertension and Left Ventricular Dymentioning

confidence: 99%

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Gordeuk

Castro

Machado

2016

Blood

124

117

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Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.

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Section: Postcapillary Pulmonary Hypertension and Left Ventricular Dymentioning

confidence: 99%

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Gordeuk

Castro

Machado

2016

Blood

124

117

View full text Add to dashboard Cite

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“…Exercise and physical activity are known to induce marked metabolic changes, including lactic acidosis, tissue hypoxia and dehydration, all of which predispose towards HbS polymerization and vaso-occlusion. Studies suggest that even moderate exercise can induce significant hypoxia in children with SCD, 86 impaired heart rate recovery, 87 abnormal increases in pulmonary artery pressure 88 and increased oxidative stress. 89 The clinical significance of these effects is unclear and studies in sickle mice suggested decreased oxidative stress with exercise.…”

Section: Physical Activity and Sickle Cell Diseasementioning

confidence: 99%

Environmental determinants of severity in sickle cell disease

et al. 2015

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“…ACS is one of the most important events in patients with SCA and is a manifestation of the vaso-occlusive phenomenon that can progress to acute respiratory distress syndrome, respiratory failure, and death (3,5). PH has a prevalence of approximately 10%, and its pathophysiology involves vaso-occlusive pulmonary phenomena, chronic hypoxia, pulmonary fibrosis, hemolysis, asplenia, iron overload, and endothelial dysfunction due to resistance to nitric oxide (6,7). …”

Section: Introductionmentioning

confidence: 99%

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

Lopes

Marinho

Alves

et al. 2017

Braz J Med Biol Res

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Sickle cell anemia (SCA) causes dysfunction of multiple organs, with pulmonary involvement as a major cause of mortality. Recently, there has been growing interest in the nitrogen single-breath washout (N2SBW) test, which is able to detect ventilation heterogeneity and small airway disease when the results of other pulmonary function tests (PFTs) are still normal. Thus, the objectives of the present study were to assess the heterogeneity in the ventilation distribution in adults with SCA and to determine the association between the ventilation distribution and the clinical, cardiovascular, and radiological findings. This cross-sectional study included 38 adults with SCA who underwent PFTs, echocardiography, computed tomography (CT), and 6-min walk test. To evaluate the ventilation heterogeneity, the patients were categorized according to the phase III slope of the N2SBW (SIIIN2). Compared with adults with lower SIIIN2 values, adults with higher SIIIN2 values showed lower hemoglobin levels (P=0.048), a history of acute chest syndrome (P=0.001), an elevated tricuspid regurgitation velocity (P=0.039), predominance of a reticular pattern in the CT (P=0.002), a shorter 6-min walking distance (6MWD) (P=0.002), and lower peripheral oxygen saturation (SpO2) after exercise (P=0.03). SIIIN2 values correlated significantly with hemoglobin (rs=-0.344; P=0.034), forced vital capacity (rs=-0.671; P<0.0001), diffusing capacity for carbon monoxide (rs=-0.376; P=0.019), 6MWD (rs=-0.554; P=0.0003), and SpO2 after exercise (P=0.040). Heterogeneity in the ventilation distribution is one of the most common pulmonary dysfunctions in adults with SCA. Moreover, relationships exist between ventilation heterogeneity, worsening of pulmonary structural damage, and reduced tolerance for exercise.

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Exercise‐Induced Abnormal Increase of Systolic Pulmonary Artery Pressure in Adult Patients With Sickle Cell Anemia: An Exercise Stress Echocardiography Study

Abstract: We concluded that adult patients with SCA and normal SPAP at rest may exhibit abnormal exercise-induced increase in SPAP, which was independently related to resting TRV levels, and indices of diastolic impairment and left ventricular filling pressure.

Cited by 12 publications

References 55 publications

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Environmental determinants of severity in sickle cell disease

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

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