Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas

Goldring, Roberta M.; Fishman, Alfred P.; Turino, Gerard M.; Cohen, Herbert I.; Denning, Carolyn R.; Andersen, Dorothy H.

doi:10.1016/s0022-3476(64)80286-9

Cited by 103 publications

(32 citation statements)

References 29 publications

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“…541 Past reports suggest that the degree of PH is usually not severe and that PH has been most commonly reported in adults with end-stage lung disease. 542 However, PH may still contribute to decreased exercise tolerance and quality of life in the setting of advanced cystic fibrosis.…”

Section: Lung Diseasesmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

916

405

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Abstract-Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension. Key Words: AHA Scientific Statements ◼ bronchopulmonary dysplasia ◼ congenital diaphragmatic hernia ◼ congenital heart disease ◼ genetics ◼ persistent pulmonary hypertension of the newborn ◼ sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.This document was approved by the American Heart Association Science Advisory and Coordinating Committee on May 12, 2015, the American Heart Association Executive Committee on July 22, 2015, and the American Thoracic Society on July 24, 2015.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIR.0000000000000329/-/DC1. The American Heart Association requests that this document be cited as follows: Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Cardiovascular Surgery and Anesthesia, and the American Thoracic Society. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132:2037-2099 Copies: This document is available on the World Wide Web site of the American Heart Associat...

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Section: Lung Diseasesmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

916

405

View full text Add to dashboard Cite

show abstract

“…This prevalence of PH is higher than previously reported by other investigators (between 21% and 59%) who mainly used echocardiography to estimate systolic PAP. [12][13][14][15][16][17][18] This discrepancy is most likely due to different degrees of disease severity and methods for PAP measurement among the studies. 12,15,17,19 The occurrence of PH in CF has been linked to development of hypoxemia and progressive destruction of the lung parenchyma and the corresponding capillary bed.…”

Section: Discussionmentioning

confidence: 99%

“…[12][13][14][15][16][17][18] This discrepancy is most likely due to different degrees of disease severity and methods for PAP measurement among the studies. 12,15,17,19 The occurrence of PH in CF has been linked to development of hypoxemia and progressive destruction of the lung parenchyma and the corresponding capillary bed. 13,15,17,[19][20][21][22][23][24][25][26][27][28] In support of the former, we observed lower PaO 2 on room air, increased need to use oxygen at rest, and higher oxygen flow in patients with PH.…”

Section: Discussionmentioning

confidence: 99%

“…12,15,17,19 The occurrence of PH in CF has been linked to development of hypoxemia and progressive destruction of the lung parenchyma and the corresponding capillary bed. 13,15,17,[19][20][21][22][23][24][25][26][27][28] In support of the former, we observed lower PaO 2 on room air, increased need to use oxygen at rest, and higher oxygen flow in patients with PH. We were not able to demonstrate a correlation between PH and severity of lung involvement by pulmonary function tests, probably because all patients had severe obstructive lung disease, thus cancelling out the pulmonary function parameters as predictors for PH.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival

Tonelli

Fernández-Bussy

Lodhi

et al. 2010

The Journal of Heart and Lung Transplantation

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“…Pulmonary hypertension is seen in humans with cystic fibrosis; however, this condition has most often been attributed to the destructive lung disease and the subsequent loss of pulmonary capillaries and/or hypoxic pulmonary vasoconstriction (11,34). The results presented here suggest that a defect in ATP release from erythrocytes of cystic fibrosis patients as they traverse the pulmonary circulation could be an additional factor leading to the development of pulmonary hypertension in these individuals.…”

Section: Discussionmentioning

confidence: 99%

Erythrocytes of humans with cystic fibrosis fail to stimulate nitric oxide synthesis in isolated rabbit lungs

Liang¹,

Stephenson²,

Lonigro³

et al. 2005

American Journal of Physiology-Heart and Circulatory Physiology

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Erythrocytes (red blood cells) of either rabbits or healthy humans are required to demonstrate the participation of nitric oxide (NO) in the regulation of pulmonary vascular resistance in the isolated rabbit lung. The property of the erythrocyte that is responsible for the stimulation of NO synthesis was reported to be the ability to release ATP in response to physiological stimuli, including deformation. Moreover, a signal transduction pathway that relates mechanical deformation of erythrocytes to ATP release has been described, and the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is a component, i.e., erythrocytes of individuals with CF do not release ATP in response to deformation. Here, we investigated the hypothesis that, in contrast to those of healthy humans, erythrocytes of humans with CF fail to stimulate endogenous NO synthesis in the isolated rabbit lung. We report that CFTR is a component of the membranes of both rabbit and human erythrocytes. The addition of the NO synthase inhibitor Nω-nitro-l-arginine methyl ester (l-NAME, 100 μM) produced increases in vascular resistance in isolated rabbit lungs perfused with physiological salt solution (PSS) containing erythrocytes of healthy humans, but l-NAME was without effect when the lungs were perfused with PSS alone or PSS containing erythrocytes of CF patients. These results provide support for the hypothesis that, in CF, a defect in ATP release from erythrocytes could lead to decreased endogenous pulmonary NO synthesis and contribute to pulmonary hypertension.

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Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas

Cited by 103 publications

References 29 publications

Pediatric Pulmonary Hypertension

Pediatric Pulmonary Hypertension

Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival

Erythrocytes of humans with cystic fibrosis fail to stimulate nitric oxide synthesis in isolated rabbit lungs

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