CASE 10—2014Eisenmenger Syndrome: Close the Hole?

Lanigan, Megan; Chaney, Mark A.; Tissot, Cécile; Beghetti, Maurice; Dimopoulos, Konstantinos

doi:10.1053/j.jvca.2013.04.023

Cited by 5 publications

(1 citation statement)

References 31 publications

(19 reference statements)

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“…Historically, management options for patients with ES have been limited to palliative measures or heart-lung transplantation. The recent introduction of targeted therapies in PAH has led to a renewed insight in the pathophysiology and treatment of ES [11,12]. Considering ES patients maintain some degree of pulmonary vasoreactivity despite the presence of obstructive pulmonary hypertension [13], ES patients using a diagnostic treatment and repair strategy are amenable to receive surgery or TCC after successful treatment with advanced therapy, but no proof of its efficacy has really been shown in large-scale studies [14][15][16].…”

Section: Discussionmentioning

confidence: 99%

Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

Wang

Shen

et al. 2020

BMC Cardiovasc Disord

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Background Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of benefit from targeted therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether PDA in patients with ES can be treated with transcatheter closure (TCC). Methods Between August 2014 and July 2016, four of fifteen PDA-ES patients whose Qp/Qs improved significantly and Qp/Qs > 1.5 after acute vasodilator testing with 100% oxygen were selected to receive TCC and pulmonary vasodilator therapy. PAH-targeted drugs were prescribed before and after occlusion for all. Trial occlusion was performed before permanent closure. Results The first TCC failed after initiation of PAH-targeted drugs for 6 months in four patients. After the medication was adjusted and extended to 12 months, TCC was performed for all without hemodynamic intolerances during perioperative period. Pulmonary artery systolic pressure (PASP) was significantly decreased (≥ 40%) immediately after TCC. During a mean follow-up of 48 ± 14.70 months, there were a further decrease of PASPs in two patients, the other two showed improved pulmonary vascular resistance, WHO functional class and six-minute walking distance despite deteriorated PASP. Conclusion Some selected PDA-ES patients might benefit from TCC and combined PAH-targeted drugs play a crucial role.

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