Sildenafil in the management of the failing Fontan circulation

Reinhardt, Zdenka; Uzun, Orhan; Bhole, Vinay; Ofoe, Victor; Wilson, Dirk G.; Onuzo, Obed; Wright, John; Stümper, Oliver

doi:10.1017/s1047951110000648

Cited by 64 publications

(40 citation statements)

References 16 publications

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“…A prospective, randomized exercise study reported that sildenafil increases maximum O 2 consumption in Fontan patients at sea level. 365 Several case reports indicate beneficial effects of sildenafil in Fontan patients at low altitude, 410,587,588 but most of the patients underwent other interventions during the study period. A double-blind, placebo-controlled, crossover RCT of sildenafil for 6 weeks on exercise performance at sea level found small improvements in respiratory rate and minute ventilation at peak exercise.…”

Section: High Altitude and Chdmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

921

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: High Altitude and Chdmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

921

405

View full text Add to dashboard Cite

show abstract

“…Experience with phosphodiesterase-5 inhibitors has shown clinical improvement in failing Fontan patients, 5 which, along with our finding of elevated PVRi in the PVE group, suggest a possible role for pulmonary vasodilators to optimize hemodynamics in failing Fontan patients before OHT. Our findings also support thorough as- sessment and aggressive embolization of APCs in failing Fontan patients before OHT to possibly limit intraoperative bleeding and subsequent graft failure after OHT.…”

mentioning

confidence: 52%

Failed Fontan heart transplant candidates with preserved vs impaired ventricular ejection: 2 distinct patient populations

Simpson¹,

Cibulka²,

Lee³

et al. 2012

The Journal of Heart and Lung Transplantation

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“…It is well established that endothelin receptor antagonists such as bosentan, sitaxentan, and ambrisentan improve the clinical symptoms and haemodynamic status of various types of pulmonary arterial hypertension [16][17][18][19]. Recently, the beneficial effects of pulmonary vasodilators administered to Fontan candidate patients or failing Fontan patients have also been reported [20][21][22][23][24]. For the Fontan candidate patients, low pulmonary vascular resistance is the most crucial factor in predicting the success of the Fontan operation; therefore, high pulmonary vascular resistance (N3 Wu m 2 ) is considered a contraindication to the Fontan surgery.…”

Section: Discussionmentioning

confidence: 99%