2011
DOI: 10.1183/09059180.00006211
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The management of Eisenmenger syndrome in the modern treatment era: a case report

Abstract: A 41-yr-old female with a history of Eisenmenger syndrome (ES) was referred to the Royal Brompton Hospital, London, UK in 2002 for tertiary care.

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Cited by 8 publications
(3 citation statements)
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“…The response to O 2 administration can be a reliable guide to assess the pulmonary vasculature, a fall in PVR with an increase in saturations being a positive sign. There are limited data[8] on which to base a definition of a safe upper limit for PVR when considering surgical repair, although <6 Wood units after vasoreactivity testing is generally accepted. Single lung transplantation or Heart-lung transplantation is definitive treatment in later stages.…”
Section: Discussionmentioning
confidence: 99%
“…The response to O 2 administration can be a reliable guide to assess the pulmonary vasculature, a fall in PVR with an increase in saturations being a positive sign. There are limited data[8] on which to base a definition of a safe upper limit for PVR when considering surgical repair, although <6 Wood units after vasoreactivity testing is generally accepted. Single lung transplantation or Heart-lung transplantation is definitive treatment in later stages.…”
Section: Discussionmentioning
confidence: 99%
“…Even if the prevalence of ES has significantly reduced in the Western world, CHD-associated PAH remains a problem in patients undiagnosed till adulthood or in those living in developing countries (such in this case). Among different alternatives (eg, “treat-and-repair,” “repair-and-treat” approach), all lacking of long-term results, 3 -5 our case proposes a cautious multistep strategy in which PVR reduction is obtained and maintained by mean of a “combined” approach. In fact, associating two different pathophysiological mechanisms of PVR reduction (vasodilation and pulmonary blood flow reduction) might prepare for a safer correction by intracardiac septation later.…”
Section: Commentmentioning
confidence: 99%
“…At present, treatment steps for goal-oriented therapy include escalating from monotherapy to sequential combination therapy [7,8]. The use of combination therapy and the evolution of patient management in recent years are nicely demonstrated in a case report by GATZOULIS [23]. In addition, preliminary evidence described by SITBON and SIMONNEAU [24] in this issue of the European Respiratory Review indicates that a future treatment trend may be upfront double or triple combination therapy, especially for patients in the severe stage of the disease who represent a particularly challenging population.…”
mentioning
confidence: 98%