Alexander C. Egbe, William R. Miranda, Patricia A. Pellikka, Sorin V. Pislaru, Barry A. Borlaug, Srikanth Kothapalli, Sindhura Ananthaneni, Harigopal Sandhyavenu, Maria Najam, Mohamed Farouk Abdelsamid, Heidi M. Connolly

Congenital Heart Disease, Vol.14, No.4, pp. 657-664, 2019, DOI:10.1111/chd.12768

Abstract Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived RV volumetric indi‐… More >

An Van Berendoncks^1,2, Roderick Van Grootel¹, Jackie McGhie¹, Matthijs van Kranenburg¹, Myrthe Menting¹, Judith A.A.E. Cuypers¹, Ad J.J.C. Bogers³, Maarten Witsenburg¹, Jolien W. Roos‐Hesselink¹, Annemien E. van den Bosch¹

Congenital Heart Disease, Vol.14, No.4, pp. 628-637, 2019, DOI:10.1111/chd.12762

Abstract Aims: Reliable evaluation of the severity and consequences of pulmonary regurgita‐ tion (PR) in patients with repaired tetralogy of Fallot (TOF) is crucial to timely identify the need for pulmonary valve intervention. We aimed to identify the accuracy of echocardiographic parameters to differentiate between moderate and severe PR, using phase contrast cardiac magnetic resonance imaging (CMR) as gold standard.
Methods and results: In this cross‐sectional study, 45 TOF patients with both echo‐ cardiographic and CMR measurements of PR were enrolled. All quantitative and semiquantitative echocardiographic measurements such as pressure half time (PHT), Color flow jet width (CFJW), ratio CFJW/right ventricle… More >

Juan Villafane¹, Thomas C. Edwards², Karim A. Diab³, Gary M. Satou⁴, Elizabeth Saarel⁵, Wyman W. Lai⁶, Gerald A. Serwer⁷, Peter P. Karpawich⁸, Russell Cross⁹, Russell Schiff¹⁰, Devyani Chowdhury¹¹, Thomas J. Hougen¹²

Congenital Heart Disease, Vol.12, No.6, pp. 762-767, 2017, DOI:10.1111/chd.12523

Abstract Objective: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF).
Design: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years. A set of quality metrics (QMs) was proposed by the WT. The metrics… More >

Takaya Hoashi¹, Toru Iwasa², Koji Kagisaki¹, Masatoshi Shimada¹, Kenichi Kurosaki², Isao Shiraishi², Hajime Ichikawa¹

Congenital Heart Disease, Vol.12, No.4, pp. 441-447, 2017, DOI:10.1111/chd.12477

Abstract Objective: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations.
Methods: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support. During operation, dilated central pulmonary arteries (cPAs) were plicated and retracted anteriorly in all patients, except for the first patient of the study cohort. VSD was completely closed, and the right ventricular outflow… More >

Katie E. Cohen¹, Matthew W. Buelow¹, Jennifer Dixon¹, Ruta Brazauskas², Scott B. Cohen³, Michael G. Earing^1,3, Salil Ginde¹

Congenital Heart Disease, Vol.12, No.4, pp. 435-440, 2017, DOI:10.1111/chd.12470

Abstract Objective: Abnormal lung function characterized by a reduced forced vital capacity (FVC) is common in adults with repaired tetralogy of Fallot (TOF) and is associated with previous thoracotomies and sternotomies. The impact of abnormal lung function on clinical outcomes in adult patients with repaired TOF is unclear. The aim of this study was to determine the impact of abnormal lung function on the outcome of hospitalization and death in adults with repaired TOF when analyzed with other traditional cardiac risk factors.
Design: Retrospective study of adults with repaired TOF, who underwent spirometry between 2000 and 2014. FVC < 60% of… More >

Maryanne Caruana¹, Victor Grech²

Congenital Heart Disease, Vol.12, No.3, pp. 301-308, 2017, DOI:10.1111/chd.12439

Abstract Objectives: To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.
Design: All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan–Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95–51.21) years).
Results: Patients born after 1985 were… More >

Shelby C. White¹, Jennifer Sedler², Trahern W. Jones³, Michael Seckeler¹

Congenital Heart Disease, Vol.13, No.6, pp. 1045-1049, 2018, DOI:10.1111/chd.12673

Abstract Objective: Applications of three‐dimensional (3D) printed models in medicine in‐ clude preprocedure planning, patient education, and clinical training. Reproducing complex anatomy as a 3D printed model can be useful for understanding congenital heart defects (CHD). We hypothesized that using 3D printed models during didactic sessions with resident physicians will improve trainees’ understanding of CHD.
Design and intervention: We performed a prospective, randomized educational in‐ tervention for teaching pediatric and pediatric/emergency medicine residents about simple (ventricular septal defect [VSD]) and moderately complex (tetralogy of Fallot [ToF]) CHD. Residents were divided into two groups: intervention and control. Each group completed a subjective… More >

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 >

Keri M. Shafer^1,2, Alexander R. Opotowsky^1,2, Jonathan Rhodes¹

Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661

Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
Results: Of the 42 cases, 27 had a Fontan… 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 >