Nikki M. Singh¹, Rohit S. Loomba², Todd M. Gudausky¹, Michael E. Mitchell³

Congenital Heart Disease, Vol.13, No.6, pp. 935-943, 2018, DOI:10.1111/chd.12670

Abstract Introduction: In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients.
Design: Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36.1%) had a monocusp valve placed. Immediate outcomes were assessed… 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 >

Brian Robichaud¹, Garick Hill², Scott Cohen^1,3, Ronald Woods⁴, Michael Earing^1,3, Peter Frommelt¹, Salil Ginde^1,3

Congenital Heart Disease, Vol.13, No.5, pp. 734-739, 2018, DOI:10.1111/chd.12639

Abstract Background: Pulmonary valve replacement (PVR) is a common operation in patients with congenital heart disease (CHD). As survival with CHD improves, infective endo‐ carditis (IE) is a growing complication after PVR. The aim of this study was to assess the incidence, risk factors, and clinical outcomes of IE after surgical PVR in patients with CHD at our institution.
Methods: Retrospective analysis of all cases of surgical PVR performed at Children’s Hospital of Wisconsin between 1975 and 2016 was performed. All cases of IE after PVR were identified and clinical and imaging data were obtained by review of medical records.
Results:… More >

Clotilde Kingsley¹, Saad Ahmad², John Pappachan¹, Sujata Khambekar¹, Thomas Smith¹, Diane Gardiner¹, James Shambrook¹, Shankar Baskar³, Ryan Moore³, Gruschen Veldtman³

Congenital Heart Disease, Vol.13, No.2, pp. 288-294, 2018, DOI:10.1111/chd.12569

Abstract Background: The right ventricular (RV) contractile reserve is a measure of the dynamic function of the RV and is a sensitive indicator of volume load. This can be measured noninvasively using the tricuspid annular plane systolic excursion (TAPSE) during exercise. We studied the RV contractile reserve of patients after tetralogy of Fallot (TOF) repair with varying degree of RV dilation and pulmonary regurgitation (PR), and compared them to a control group.
Methods: Twenty-six patients who had undergone TOF repair (mean age 29 ± 10 years) were identified and stratified into three group based on the presence and severity of RV… More >

Wendy F. Li¹, Heidi Pollard², Mohsen Karimi³, Jeremy D. Asnes¹, William E. Hellenbrand¹, Veronika Shabanova⁴, Constance G. Weismann^1,5

Congenital Heart Disease, Vol.13, No.1, pp. 140-146, 2018, DOI:10.1111/chd.12544

Abstract Objective: Trans-catheter (TC) pulmonary valve replacement (PVR) has become common practice for patients with right ventricular outflow tract obstruction (RVOTO) and/or pulmonic insufficiency (PI). Our aim was to compare PVR and right ventricular (RV) function of patients who received TC vs surgical PVR.
Design: Retrospective review of echocardiograms obtained at three time points: before, immediately after PVR, and most recent.
Patients: Sixty-two patients (median age 19 years, median follow-up 25 months) following TC (N = 32) or surgical (N = 30) PVR at Yale-New Haven Hospital were included.
Outcome Measures: Pulmonary valve and right ventricular function before, immediately after, and… More >

Rachel L. Hansen^1,2, Iman Naimi³, Hongyue Wang¹, Nader Atallah^3,4, Frank Smith^3,4, Craig Byrum^3,4, Daniel Kveselis^3,4, Glenn Leonard^1,5, Rajiv Devanagondi¹, Matthew Egan^3,4

Congenital Heart Disease, Vol.14, No.6, pp. 1037-1045, 2019, DOI:10.1111/chd.12788

Abstract Objective: Evaluate long‐term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS).
Background: Long‐term data following BPV is limited to small, single center studies.
Methods: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis.
Results: Among 254 patients, mean age at BPV was 3.8 years (range 1 day‐67 years), initial PS catheter gradient was 56 mm Hg (IQR 40‐70), 19% had… More >

Subeer K. Wadia¹, Gentian Lluri¹, Jamil A. Aboulhosn¹, Hillel Laks², Reshma M. Biniwale², Glen S. Van Arsdell², Daniel S. Levi³, Morris M. Salem³, Kevin M. Shannon^1,3, Jeremy P. Moore^1,3

Congenital Heart Disease, Vol.14, No.5, pp. 838-845, 2019, DOI:10.1111/chd.12818

Abstract Objective: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC‐) or surgical (S‐) pulmo‐ nary valve replacement (PVR).
Design/Setting: This was a retrospective observational study of patients who under‐ went PVR from 2010 to 2016 at UCLA Medical Center.
Patients: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded.
Outcome Measures: The primary outcome was a time‐to‐event analysis of… 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 >

Brian A. Boe, Sharon L. Cheatham, Aimee K. Armstrong, Darren P. Berman, Joanne L. Chisolm, John P. Cheatham

Congenital Heart Disease, Vol.14, No.2, pp. 297-304, 2019, DOI:10.1111/chd.12728

Abstract Objective: We sought to describe the leaflet morphology variation in the Melody Transcatheter Pulmonary Valve (TPV) and evaluate associated outcomes. The Melody TPV is constructed from harvested bovine jugular venous valves which have been rigorously tested. Natural anatomic leaflet variations are seen in the Melody TPV but have not been evaluated.
Design: A Melody TPV leaflet morphology classification system was devised after reviewing a subset of photographed and implanted TPVs. All images were blindly re‐ viewed by implanters and classified. Midterm hemodynamic outcomes and complica‐ tions of the Melody TPVs were compared by leaflet morphology.
Results: Photographed Melody TPVs implanted… More >

Kailyn Anderson¹, James Cnota^2,3, Jeanne James^4,5, Erin M. Miller^2,3, Ashley Parrott³, Valentina Pilipenko^1,2, Kathryn Nicole Weaver^1,2, Amy Shikany³

Congenital Heart Disease, Vol.14, No.2, pp. 264-273, 2019, DOI:10.1111/chd.12721

Abstract Objective: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD.
Design: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected. Chi‐square test, Fisher’s exact test, t test, Wilcoxon rank‐sum test, and ANOVA were used for comparisons among the groups. Logistic regression was used to test for the association between… More >