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

Abstract

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.

High haemoglobin levels at the time of occlusion predict worse outcome for patients with patent ductus arteriosus and pulmonary hypertension

“…In another study, patent ductus arteriosus closures were performed in patients with Eisenmenger after pre-treatment with pulmonary hypertension targeted therapy. 14 Of the four patients, two had regression of pulmonary hypertension while another two did not. The initial good response after closure does not translate to excellent long-term outcomes as some of these patients experienced continual progression of pulmonary hypertension over time.…”

“…14.35 ± 1.66 WU x m 2 versus Poor Outcome: 20.07 ± 2.44 WU x m 2 ; p = 0.033), and higher Rp: Rs (Good Outcome: 0.44 ± 0.16 versus Poor Outcome: 1.08 ± 1.21; p = 0.042) suggestive a worse degree of PAH (Fig2). The mean Qp/Qs was similar between the Good (2.07 ± 0.83) and Poor (1.88 ± 1.48) Outcome groups (p = 0.701).…”

“…The long-term outcomes of patent ductus arteriosus occlusion in these patients with severe pulmonary arterial hypertension non-conclusive. [10][11][12][13][14] Moreover, predictive factors for a favourable long-term outcome remain elusive.…”

Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management

“…Expert centers may choose to initiate and escalate PAH therapies (as single or combination therapy) in symptomatic patients, monitoring hemodynamics for a significant drop in PVR and/or significant increase in the left-right shunt and ventricular overload. There are several case reports and series describing patients with post-tricuspid shunts and established PVD who responded to PAH therapy and underwent repair of the defect ( 38 , 39 ). However, patients with post-tricuspid shunts often present with more severe PVD, with a higher baseline PVR than their counterparts with pre-tricuspid shunts, making a treat-and-repair strategy less attractive.…”

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options

“…Over time, L‐R shunting can result a higher pulmonary fluid volume and promotes pulmonary edema, bronchopulmonary dysplasia, chronic lung disease, and PAH, especially in premature infants 165–167 . Pulmonary vascular remodeling and accelerated PAH induced by persistently PDA ultimately lead to R‐L shunting and Eisenmenger syndrome 168 . In addition, abnormal shunting also induces left heart volume overload and systemic hypoperfusion, which aggravate left ventricular remodeling and predispose the development of necrotizing enterocolitis, renal failure, and death 167 …”