Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries

Broberg, Craig S.; Dissel, Alexandra van; Minnier, Jessica; Aboulhosn, Jamil; Kauling, Robert M; Ginde, Salil; Krieger, Eric V.; Rodriguez, Fred H.; Gupta, Tripti; Shah, Sangeeta; John, Anitha S.; Cotts, Timothy B.; Kay, W. Aaron; Kuo, Marissa C.; Dwight, Cindy; Woods, Patricia; Nicolarsen, Jeremy; Sarubbi, Berardo; Fusco, Flavia; Antonová, Petra; Fernandes, Susan M.; Grewal, Jasmine; Cramer, Jonathan; Khairy, Paul; Gallego, Pastora; O’Donnell, Clare; Hannah, Jane; Dellborg, Mikael; Rodríguez-Monserrate, Carla P.; Muhll, Isabelle Vonder; Pylypchuk, Stephen; Magalski, Anthony; Han, Frank; Lubert, Adam M.; Kay, Joseph; Yeung, Elizabeth; Roos‐Hesselink, Jolien W.; Baker, David W.; Celermajer, David S.; Burchill, Luke J.; Wilson, William; Wong, Joshua; Kutty, Shelby; Opotowsky, Alexander R.

doi:10.1016/j.jacc.2022.06.020

Cited by 34 publications

(25 citation statements)

References 27 publications

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“…The potential risk factors of clinical events in TGA after AtrS corresponded to those retained in the risk prediction score developed by Woudstra et al 10 and are listed in Table 1. Moreover, additional predictors selected on the basis of a literature review were assessed (in supplemental data Table S1 2,4,6,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] ). Complex TGA was defined as TGA associated with ventricular septal defect (VSD), left ventricular outflow tract (LVOT) obstruction and/or aortic coarctation.…”

Section: Methodsmentioning

confidence: 99%

External validation of a risk score model for predicting major clinical events in adults after atrial switch operation for transposition of the great arteries

Albertini

Santens

Fusco

et al. 2023

Preprint

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Background: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries (D-TGA) repaired by an atrial switch operation. The aim of this study was to externally validate the model. Methods: A retrospective, multicentric, longitudinal cohort of 417 patients with D-TGA (median age 24 years at baseline [interquartile range 18-30], 63% male) independent of the model development and internal validation cohort was studied. Data on risk model predictors (age >30 years, prior ventricular arrhythmia, age >1 year at the atrial switch, moderate or severe right ventricular dysfunction, severe tricuspid regurgitation, and at least mild left ventricular (LV) dysfunction) were collected from the time of baseline clinical evaluation. The performance of the prediction model in predicting risk at 5 years was assessed. Results: Twenty-five patients (5.9%) met the major clinical events endpoint within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell's C-index of 0.73 (95% CI 0.65-0.81)) but tended to overestimate this risk (calibration slope of 0.20 (95% CI 0.03-0.36)). We separately evaluated predictors of major clinical events in our cohort. History of heart failure and at least mildly impaired sub-pulmonary LV function remained the strongest predictors of major clinical events. Conclusions: We reported the first external validation of a major clinical events risk model in a large D-TGA patient population. Although good discrimination, the model tends to overestimate the absolute 5-year risk. Subpulmonary LV dysfunction appears to be a key marker in the prognosis of patients with Senning and Mustard.

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Section: Methodsmentioning

confidence: 99%

External validation of a risk score model for predicting major clinical events in adults after atrial switch operation for transposition of the great arteries

Albertini

Santens

Fusco

et al. 2023

Preprint

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“…Bradyarrhythmias, tachyarrhythmias, and SCD are not uncommon in sRV patients and are potentially fatal. The recent Broberg et al study showed atrial arrhythmias and ventricular arrhythmias to correlate with the primary clinical outcome of death, transplantation, or MCS device requirement in d-TGA patients after the Mustard/Senning procedure ( 60 ). Ventricular arrhythmias were also part of a 5-year risk prediction model of the primary outcome ( 60 ).…”

Section: Mechanisms Of Systemic Right Ventricle Failurementioning

confidence: 99%

“…The recent Broberg et al study showed atrial arrhythmias and ventricular arrhythmias to correlate with the primary clinical outcome of death, transplantation, or MCS device requirement in d-TGA patients after the Mustard/Senning procedure ( 60 ). Ventricular arrhythmias were also part of a 5-year risk prediction model of the primary outcome ( 60 ). Indeed, d-TGA patients after atrial switch surgery are among the CHD patient groups at the highest risk of sudden cardiac death (SCD) ( 4 , 61 ), with numerous potential triggers ( 62 ).…”

Section: Mechanisms Of Systemic Right Ventricle Failurementioning

confidence: 99%

“…Electromechanical dyssynchrony induces sRV dysfunction in d-TGA patients and correlates with exercise intolerance and major adverse cardiac events, including heart failure ( 72 ). Broberg et al recently showed QRS prolongation > 120 ms as an independent 5-year predictor of a composite primary outcome of death, transplantation, or MCS device implantation ( 60 ). Cardiac fibrosis may damage the conduction system, leading to dyssynchrony.…”

Section: Mechanisms Of Systemic Right Ventricle Failurementioning

confidence: 99%

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Heart failure in systemic right ventricle: Mechanisms and therapeutic options

Sabbah

Arabi

Shafqat

et al. 2023

Front. Cardiovasc. Med.

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d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.

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“…Furthermore, baffle leaks are common and are a potential risk for right to left shunting. In addition, in many patients, exercise capacity is diminished [ 64 , 65 ]. Patients with congenital-corrected transposition of the great arteries (ccTGA) also show a severely diminished aerobic exercise capacity and frequently present with AV block, necessitating the implantation of an endovascular pacemaker [ 66 ].…”

Section: Specific Conditionsmentioning

confidence: 99%

SCUBA Diving in Adult Congenital Heart Disease

Kauling

Rienks

Cuypers

et al. 2023

JCDD

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Conventionally, scuba diving has been discouraged for adult patients with congenital heart disease (ACHD). This restrictive sports advice is based on expert opinion in the absence of high-quality diving-specific studies. However, as survival and quality of life in congenital heart disease (CHD) patients have dramatically improved in the last decades, a critical appraisal whether such restrictive sports advice is still applicable is warranted. In this review, the cardiovascular effects of diving are described and a framework for the work-up for ACHD patients wishing to engage in scuba diving is provided. In addition, diving recommendations for specific CHD diagnostic groups are proposed.

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Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries

Cited by 34 publications

References 27 publications

External validation of a risk score model for predicting major clinical events in adults after atrial switch operation for transposition of the great arteries

External validation of a risk score model for predicting major clinical events in adults after atrial switch operation for transposition of the great arteries

Heart failure in systemic right ventricle: Mechanisms and therapeutic options

SCUBA Diving in Adult Congenital Heart Disease

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