Francisco Javier Ruperti-Repilado^1,#,*, Magalie Ladouceur^2,#, Pastora Gallego³, Laura Dos⁴, Joaquin Rueda Soriano⁵, Berto Bouma⁶, Harald Gabriel⁷, Markus Schwerzmann¹, Judith Bouchardy^8,9, Daniel Tobler^10,#, Matthias Greutmann^11,#

Congenital Heart Disease, Vol.15, No.5, pp. 309-338, 2020, DOI:10.32604/CHD.2020.012599

Abstract Background: Long-term outcomes in adults with prior arterial switch operation (ASO) have not yet been well defined. The aim of this study is to elucidate incidence and predictors of adverse cardiac outcomes in a prospectively followed cohort of adults after their ASO. Methods: The comprehensive longterm follow up of adults with ASO is a project within the European collaboration for prospective outcome research in congenital heart disease (EPOCH). It is designed as a prospective, international multicenter cohort study. Consecutive patients (age ≥ 16 years) with prior ASO will be included at 11 European tertiary care centers. Participants will be followed… More >

Ylenia Bartolacelli^*, Giulia Bragantini

Congenital Heart Disease, Vol.15, No.2, pp. 59-68, 2020, DOI:10.32604/CHD.2020.011448

Abstract Objective: The objective of this study was to assess left ventricle (LV) function and remodeling by three-dimensional echocardiography (3DE) in patients who underwent arterial switch procedure (ASO) for transposition of great arteries (TGA) in long-term follow-up. Methods and Results: We studied 54 asymptomatic patients (39 male) who have undergone single-stage ASO for TGA, aged 13.7 ± 4.7 years, with a normal LV ejection fraction (EF), compared to healthy peers. We evaluated LV volume and function in asymptomatic patients with normal ejection fraction by 3DE. All patients had normal EF, measured by modified Simpson’s method (mean 60.9 ± 3.5%) and by… More >

Magalie Ladouceur^1,2,3, Nadjia Kachenoura⁴, Gilles Soulat^1,3, Emilie Bollache⁴, Alban Redheuil⁴, Michel Azizi³, Christophe Delclaux³, Gilles Chatellier³, Pierre Boutouyrie^1,3, Laurence Iserin³, Damien Bonnet^2,3, Elie Mousseaux^1,3

Congenital Heart Disease, Vol.12, No.4, pp. 458-466, 2017, DOI:10.1111/chd.12472

Abstract Objectives: We aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship of these diastolic parameters with exercise performance and BNP, in patients with preserved systolic systemic right ventricle function.
Design: Single-center, cross-sectional, prospective study.
Setting: In patients with D-TGA palliated by atrial switch, diastolic dysfunction of the systemic right ventricle may precede systolic dysfunction.
Methods: Forty-five patients with D-TGA and atrial switch and 45 age and sex-matched healthy… More >

Jana Rubáčková Popelová, MD1,2 | Markéta Tomková1,3 | Jakub Tomek1,4

Congenital Heart Disease, Vol.12, No.4, pp. 448-457, 2017, DOI:10.1111/chd.12466

Abstract Objective: The patients after Mustard and Senning corrections of transposition of the great arteries (TGA) are at an increased risk of unexpected death. The aim of this study was to identify markers allowing risk stratification of patients after atrial switch correction of TGA to provide them with optimum care.
Methods and Results: In this study, 87 patients were retrospectively evaluated after atrial switch correction of TGA followed-up between 2005 and 2015. The mortality during the follow-up was 9% (8 cardiac deaths). Markers significantly predictive of death using univariable Cox proportional hazard ratio survival analysis were: N-terminal pro-B-type natriuretic peptide (NT-proBNP),… More >

Anushree Agarwal, Fatima Samad, Lindsey Kalvin, Michelle Bush, A. Jamil Tajik

Congenital Heart Disease, Vol.12, No.2, pp. 143-152, 2017, DOI:10.1111/chd.12453

Abstract Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital disease that frequently remains undiagnosed until adulthood, especially when there is an absence of other congenital anomalies. Adults with ccTGA may remain asymptomatic and their diagnosis could be missed on initial evaluation, or it could be diagnosed incidentally as an evaluation of murmur. We aim to report the different presentations of ccTGA in eight adult patients and review the key features required to suspect the diagnosis during an initial visit.
Cases: We present some illustrative cases of ccTGA patients who had diverse presentations ranging from being completely… More >

Taner Kasar¹, Pelin Ayyildiz¹, Gulhan Tunca Sahin¹, Erkut Ozturk¹, Selman Gokalp¹, Sertac Haydin², Alper Guzeltas¹, Yakup Ergul¹

Congenital Heart Disease, Vol.13, No.3, pp. 450-457, 2018, DOI:10.1111/chd.12595

Abstract Background: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected transposition of the great arteries (ccTGA) and associated treatment strategies.
Patients and Methods: This retrospective cohort study included 65 pediatric patients with ccTGA who were admitted to the clinic between 2009 and 2017. The patients were divided into two groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electrophysiological data, and device implantation data on the two groups were compared.
Results: Group I (n = 53, 82%) consisted of patients with significant associated lesions, and Group II (n = 12, 18%) consisted of those with minor or… More >

Nelly Sabbaghian¹, Maria C. Digilio², Gillian M. Blue^3,4, Timothée Revil⁵, David S. Winlaw^3,4, William D. Foulkes^1,6

Congenital Heart Disease, Vol.13, No.3, pp. 401-406, 2018, DOI:10.1111/chd.12578

Abstract Objective: We previously identified a pathogenic germline DICER1 variant in a child with transposition of the great arteries who was a member of a family with DICER1 syndrome. In view of a report linking DICER1 knockout in murine cardiomyocytes to cardiac outflow defects, we investigated the involvement of DICER1 in transposition of the great arteries.
Design: We used Fluidigm access array followed by next generation sequencing to screen for variants in the coding exons, their exon/intron boundaries and the 30 untranslated region of DICER1 in patient DNA.
Cases: Germline DNA was collected from 129 patients with either sporadic or familial… More >

Carissa M. Baker-Smith¹, Karina Carlson², Jose Ettedgui³, Takeshi Tsuda⁴, K. Anitha Jayakumar⁵, Matthew Park⁶, Nikola Tede⁷, Karen Uzark⁸, Craig Fleishman⁹, David Connuck¹⁰, Maggie Likes¹¹, Daniel J. Penny¹²

Congenital Heart Disease, Vol.13, No.1, pp. 52-58, 2018, DOI:10.1111/chd.12540

Abstract Objective: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).
Design: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an “open comment period.” Final approval of each… More >

Margaret M. Samyn^1,2, Ke Yan¹, Conor Masterson³, Benjamin H. Goot^1,2, David Saudek^1,2, Julie Lavoie², Aaron Kinney², Mary Krolikowski¹, Kan Hor^4,5, Scott Cohen^1,2

Congenital Heart Disease, Vol.14, No.6, pp. 1138-1148, 2019, DOI:10.1111/chd.12861

Abstract Objective: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are “at-risk” for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch.
Design: This was a retrospective cohort study of patients with dTGA s/p atrial switch operation (February 1966… More >

M. Louise Morrison¹, Brian Grant¹, Brian A. McCrossan^1,2, Andrew J. Sands^1,2, Colum G. Owens², Mark S. Spence², Frank A. Casey¹, Brian G. Craig^1,2, Christopher J. Lockhart²

Congenital Heart Disease, Vol.14, No.5, pp. 846-853, 2019, DOI:10.1111/chd.12822

Abstract Objective: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center.
Design: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic in‐ formation and data regarding the clinical state, NHYA class, cardiopulmonary exer‐ cise testing, NT‐proBNP measurement, and recent cardiac MRI findings.
Results: Forty‐six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty‐two patients (69.6%) had undergone a Senning procedure. The median length… More >