Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension

Anthi, Anastasia; Machado, Roberto F.; Jison, Maria; Taveira-DaSilva, Angelo M.; Rubin, Lewis J.; Hunter, Lori A.; Hunter, Christian J.; Coles, Wynona; Nichols, James S.; Avila, Nilo A.; Sachdev, Vandana; Chen, Clara C.; Gladwin, Mark T.

doi:10.1164/rccm.200610-1498oc

Cited by 229 publications

(257 citation statements)

References 41 publications

(43 reference statements)

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“…Obstructive disease, either alone or mixed with restrictive disease, was relatively uncommon in adult patients, occurring in 3% of the patients. Similar findings of mild restrictive abnormalities have been observed in more recent cohort studies of adult patients (57).…”

Section: Restrictive Lung Diseasesupporting

confidence: 89%

Pulmonary Complications of Sickle Cell Disease

Firth

2009

N Engl J Med

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Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes.

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Section: Restrictive Lung Diseasesupporting

confidence: 89%

Pulmonary Complications of Sickle Cell Disease

Firth

2009

N Engl J Med

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“…Several studies have documented the cardiopulmonary changes of this cohort. [58][59][60] However, there is an absence of cellular-and tissue-specific data from SCD-associated PAH lungs. Our team has reported bilateral lung transplantation for the treatment of end-stage SCD-associated PAH.…”

Section: Discussionmentioning

confidence: 99%

Cellular, Pharmacological, and Biophysical Evaluation of Explanted Lungs from a Patient with Sickle Cell Disease and Severe Pulmonary Arterial Hypertension

et al. 2013

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Pulmonary hypertension is recognized as a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). We now report benchtop phenotyping from the explanted lungs of the first successful lung transplant in SCD. Pulmonary artery smooth muscle cells (PASMCs) cultured from the explanted lungs were analyzed for proliferate capacity, superoxide (O 2 • − ) production, and changes in key pulmonary arterial hypertension (PAH)-associated molecules and compared with non-PAH PASMCs. Upregulation of several pathologic processes persisted in culture in SCD lung PASMCs in spite of cell passage. SCD lung PASMCs showed growth factor-and serum-independent proliferation, upregulation of matrix genes, and increased O 2• − production compared with control cells. Histologic analysis of SCDassociated PAH arteries demonstrated increased and ectopically located extracellular matrix deposition and degradation of elastin fibers. Biomechanical analysis of these vessels confirmed increased arterial stiffening and loss of elasticity. Functional analysis of distal fifth-order pulmonary arteries from these lungs demonstrated increased vasoconstriction to an α1-adrenergic receptor agonist and concurrent loss of both endothelial-dependent and endothelial-independent vasodilation compared with normal pulmonary arteries. This is the first study to evaluate the molecular, cellular, functional, and mechanical changes in endstage SCD-associated PAH.

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“…All studies were recorded and later reviewed by cardiologists. We defined PAH as PASP C30 mm Hg that was based on TRV C2.5 m/s [13] as marker of PAH in SCD. TTE performed while patients undergoing acute sickle crisis or ACS were excluded.…”

Section: Transthoracic Echocardiographymentioning

confidence: 99%

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

Joshi

Anjum

Gowda

et al. 2011

Indian J Hematol Blood Transfus

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Serum uric acid (UA) is emerging as a strong and independent marker for pulmonary arterial hypertension (PAH). PAH is well recognized as a life threatening complication of sickle cell disease (SCD). However, the association between UA and PAH in SCD is unknown. We reviewed electronic medical records (EMR) of 559 consecutive adult SCD patients from Kings County Hospital Center (KCHC) between January 2005 and February 2010. Patients (n = 96) with measurement of UA in close temporal proximity to the transthoracic echocardiography (TTE) were identified. PAH was defined as pulmonary artery systolic pressure (PASP) C30 mm Hg. Patients (n = 16) with other risk factors which may cause PAH and chronic renal insufficiency were excluded. In 18 patients, TTE could not measure PASP. Finally, 62 patients were selected. Statistical analysis was performed using Student t tests, Pearson correlation coefficient and multivariate regression analysis. Out of 62 patients, 30 had PAH. Patients with PAH had a higher UA level (8.67 ± 4.8 vs. 5.35 ± 2.1, P = 0.001). We found strong positive correlation between the UA level and PASP (r = 0.71; P \ 0.0001). This correlation was independent of diuretic use. UA could be a potential marker for PAH in SCD. However, its' prognostic and pathophysiologic role in SCD patients with PAH needs to be further investigated.

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Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension

Cited by 229 publications

References 41 publications

Pulmonary Complications of Sickle Cell Disease

Pulmonary Complications of Sickle Cell Disease

Cellular, Pharmacological, and Biophysical Evaluation of Explanted Lungs from a Patient with Sickle Cell Disease and Severe Pulmonary Arterial Hypertension

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

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