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… 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… More >

Zhedian Zhou¹, Tal Geva², Rahul H. Rathod², Alexander Tang², Chun Yang³, Kristen L. Billiar⁴, Dalin Tang^1,*,3, Pedro del Nido⁵

Molecular & Cellular Biomechanics, Vol.15, No.2, pp. 99-115, 2018, DOI:10.3970/mcb.2018.00558

Abstract Patients with repaired Tetralogy of Fallot (ToF), a congenital heart defect which includes a ventricular septal defect and severe right ventricular outflow obstruction, account for the majority of cases with late onset right ventricle (RV) failure. The current surgical approach, which includes pulmonary valve replacement/insertion (PVR), has yielded mixed results. A computational parametric study using 7 patient-specific RV/LV models based on cardiac magnetic resonance (CMR) data as "virtual surgery" was performed to investigate the impact of patch size, RV remodeling and tissue regeneration in PVR surgery design on RV cardiac functions. Two patch sizes, three… 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… 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… 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… 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… More >

Richard J. Dobson¹, Ify Mordi², Mark H. Danton¹, Niki L. Walker¹, Hamish A. Walker¹, Nikolaos Tzemos²

Congenital Heart Disease, Vol.12, No.1, pp. 58-66, 2017

Abstract Objective: Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes.
Design: Single centre cohort study
Setting: National tertiary referral center
Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD… More >

Dalin Tang^1,*,2, Heng Zuo^2,*, Chun Yang², Zheyang Wu², Xueying Huang³, Rahul H. Rathod⁴, Alexander Tang⁴, Kristen L. Billiar⁵, Tal Geva⁴

Molecular & Cellular Biomechanics, Vol.14, No.3, pp. 137-151, 2017, DOI:10.3970/mcb.2017.014.137

Abstract Patients with repaired tetralogy of Fallot (TOF) account for the majority of cases with late onset right ventricle failure. Comparing TOF patients with healthy people may provide information to address this challenge. Cardiac magnetic resonance (CMR) data were obtained from 16 TOF patients (patient group, PG) and 6 healthy volunteers (healthy group, HG). At begin-of-ejection, better patient group (n=5, BPG) stress was very close to HG stress (54.7±38.4 kPa vs. 51.2±55.7 kPa, p=0.6889) while worse patient group (n=11, WPG) stress was 84% higher than HG stress (p=0.0418). Stress may be used as an indicator to differentiate More >

Ashish Das^∗, William M. Gottliebson^†,‡, Madhura Karve^∗, Rupak Banerjee^∗,§,¶

Molecular & Cellular Biomechanics, Vol.8, No.1, pp. 21-42, 2011, DOI:10.3970/mcb.2011.008.021

Abstract Right ventricular (RV) enlargement and pulmonary valve insufficiency (PI) are well-known, unavoidable long term sequelae encountered by patients who undergo tetralogy of Fallot (TOF) surgery. Despite their lifelong need for cardiac surveillance and occasional re-intervention, there is a paucity of numerical data characterizing blood flows in their pulmonary arteries (PA). Specifically, although PA regurgitation is well-known to be ubiquitously present in adult repaired TOF (rTOF) patients yet, there have been only limited numerical studies to fully characterize this process. The few studies available have utilized idealized, simplistic geometric models or overly simplistic boundary conditions that… More >