Shiraz A. Maskatia¹, Christopher J. Petit^2,3, Curtis D. Travers², David J. Goldberg⁴, Lindsay S. Rogers⁴, Andrew C. Glatz⁴, Athar M. Qureshi⁶, Bryan H. Goldstein⁵, Jingning Ao^2,3, Ritu Sachdeva^2,3

Congenital Heart Disease, Vol.13, No.6, pp. 892-902, 2018, DOI:10.1111/chd.12671

Abstract Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation).
Results: Eighty‐one… More >

Robin H.S. Chen¹, Adolphus K.T. Chau¹, Pak Cheong Chow¹, Tak Cheung Yung¹, Yiu Fai Cheung^1,2, Kin Shing Lun¹

Congenital Heart Disease, Vol.13, No.6, pp. 884-891, 2018, DOI:10.1111/chd.12658

Abstract Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute… More >

David Aurigemma, John W. Moore, Gabrielle Vaughn, Nasser Moiduddin, Howaida G. El-Said

Congenital Heart Disease, Vol.13, No.2, pp. 226-231, 2018, DOI:10.1111/chd.12552

Abstract Background: Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported.
Objectives: Our aim is to report our experience with using perforation of plate-like pulmonary valve atresia combined with stenting of RVOT as an alternative and equally efficacious intervention for infants with PA/VSD, as compared to a… More >

Rohit S. Loomba^1,2, Andrew N. Pelech^1,2

Congenital Heart Disease, Vol.13, No.1, pp. 92-97, 2018, DOI:10.1111/chd.12510

Abstract Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.
Methods: A retrospective study was conducted. Patients were included if an initial catheterization was done prior to any intervention.… More >

Bruno Lefort^1,2, Christophe Saint‐Etienne¹, Nathalie Soulé¹, Iris Ma¹, Fanny Dion¹, Alain Chantepie^1,2

Congenital Heart Disease, Vol.14, No.5, pp. 814-818, 2019, DOI:10.1111/chd.12812

Abstract Background and objective: Chronic total occlusion (CTO) guidewire have been recently reported as an alternative to radiofrequency for perforating atretic pulmonary valve. Since procedure failures or perforation of the right ventricle still occurred with CTO, we tried to enhance the stability, steering, and pushability of the wire using a microcatheter in order to improve the safety and efficacy of the procedure.
Methods: We performed pulmonary valve perforation with CTO guidewire and microcatheter in five consecutive newborns with pulmonary atresia with intact ventricular septum (PA‐IVS) under fluoroscopic and echocardiographic control.
Results: The valve was easily perforated at the first attempt for… More >