Long-term outcome after percutaneous shunt closure of selected patients with atrial septal defect and severe pulmonary hypertension - 25/12/18
, M. Humbert 2, O. Sitbon 2, G. Simmoneau 2, X. Jais 2, L. Savale 2, J. Lamy 3, A. Boet 1, P. Brenot 4, J. Petit 1, S. Hascoët 1Résumé |
Background |
Pulmonary arterial hypertension (PAH) is a dreaded complication of ostium secundum atrial septal defects (ASD). Shunt closure indication remains controversial in patients with severe PAH.
Purpose |
To investigate late outcome in selected patients with severe PAH who underwent percutaneous ASD closure.
Methods |
We retrospectively included 14 patients (median age 47.5 years old) with mean pulmonary artery pressure (PAPm)>40mmHg and/or pulmonary vascular resistance indexed (PVRi)>8 UW.m2, with persistent left to right shunting, who underwent percutaneous ASD closure.
Results |
Three patients (21.4%) had ASD and coexisting etiology of PAH (chronic thromboembolic pulmonary hypertension, n=1; familial form of PAH, n=1; BMPR2 mutation, n=1). All patients had dyspnea with 6 (42.8%) in WHO functional class (FC) 3–4. Four patients (28.6%) received PAH drugs (monotherapy, n=3; triple therapy including intravenous prostacyclin, n=1). Median [interquartile] PAPm and PVRi were 44mmHg [41–47] and 9.7 UW.m2 [8.0–10.3]. Median delay between symptoms onset and shunt closure was 12 months [6–18]. Median Qp/Qs was 2.0 [1.6–2.2].
During early follow-up, WHO FC 2 was unchanged in 3 patients (21%), while the remaining 11 patients (79%) had clinical improvement. No patient died.
After a median follow-up of 93 months [43–133], all patients were in WHO FC 1 or 2. PAPm at last catheterization (n=10) had decreased in all cases. Median PAPm was 28mmHg [26–32]. PAPm was below 25mmHg in 3 cases. Median PVRi was 8.0 UW.m2 [6.4–9.0]. The 4 patients without catheterization were in WHO-FC 1 and with normal pulmonary pressure on echography. Five patients receiving PAH therapy (monotherapy, n=3; double therapy, n=2). Three patients died (one from lung infection, 6 years after closure and two from cancer, 1 and 2 years after closure).
Conclusion |
Improved WHO FC and decreased pulmonary pressure are observed in carefully selected patients with severe PAH after ASD closure.
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Vol 11 - N° 1
P. 130 - janvier 2019 Retour au numéro
Article précédent
- Usefulness of 3D printed models of congenital heart diseases as educational tools for medical students
- A. Guitarte Vidaurre, K. Hadeed, M. Ratsimandresy, C. Karsenty, Yves Dulac, Philippe Acar
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- S. Hascoët, R.G. Dallapozza, J. Bentham, J. Petit, N. Tahhan, R. Carere, M. Kantzis, P. Ewert, E.K. Biernacka, O. Kretschmar, C. Deutsch, M. Thoenes, P. Bramlage, N. Haas
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