Outcomes of treatment pathways in 240 patients with congenitally corrected transposition of great arteries

Barrios, Paola; Zia, Aisha; Pettersson, Gösta; Najm, Hani K.; Rajeswaran, Jeevanantham; Bhimani, Salima; Karamlou, Tara; Klein, Jared; Saarel, Elizabeth V.; Komarlu, Rukmini; Shaheen, Naseeb; Blackstone, Eugene H.; Lee, Leah J.

doi:10.1016/j.jtcvs.2020.11.164

Cited by 23 publications

(19 citation statements)

References 20 publications

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“…In patients with TV regurgitation, such as ours, the physiologic repair is associated with low freedom from reintervention, whereas anatomic repair provides improved results. 1 Heart block is a complication of repair, and in this case, occurred during the LVOT resection. As described in literature, the conduction system is abnormal in ccTGA in that the AV node is likely displaced antero-superiorly, leading to a bundle superiorly to the PV annulus and sweeps down to the ventricular septum.…”

Section: Commentsmentioning

confidence: 85%

“…Congenitally corrected transposition of the great arteries (ccTGA) represents around 0.5% of congenital heart disease. 1 A majority of these patients have additional congenital defects such as abnormal conduction system, variable coronary anatomy, ventricular septal defects (VSD), left ventricular outflow tract obstruction (LVOTO), tricuspid, and mitral valve abnormalities. 2,3 Our aim is to describe the surgical management of a complex ccTGA patient with multiple complicating lesions and emphasize the importance of optimal preoperative imaging and intraoperative decision making.…”

Section: Introductionmentioning

confidence: 99%

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Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs

Najm

Tucker

Patel

et al. 2022

World J Pediatr Congenit Heart Surg

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We describe the management of a 2 month-old male (BSA 0.27 m2) with congenitally corrected transposition of the great arteries (CCTGA), left and right ventricular outflow tract obstruction, incomplete atrioventricular (AV) canal defect, Ebsteinoid left AV valve, and multiple VSDs. In short, the patient presented with respiratory failure, metabolic acidosis with worsening AV valve regurgitation, and acute decompensation from pulmonary overcirculation. We performed a complex double switch operation described below. Despite the unique anatomical complexities, the patient’s successful course demonstrates the importance of multidisciplinary discourse, preoperative imaging and planning, and intraoperative decision making from patient selection to anatomic repair.

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Section: Commentsmentioning

confidence: 85%

Section: Introductionmentioning

confidence: 99%

Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs

Najm

Tucker

Patel

et al. 2022

World J Pediatr Congenit Heart Surg

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“…Due to diverse surgical procedures among the patients, we classified them into 3 types and were approximately consistent with a recent study (21).…”

Section: Surgical Classificationmentioning

confidence: 92%

Cardiovascular Phenotypes Profiling for L-Transposition of the Great Arteries and Prognosis Analysis

Shen

Shao

et al. 2022

Front. Cardiovasc. Med.

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ObjectivesCongenitally corrected transposition of the great arteries (ccTGA) is a rare and complex congenital heart disease with the characteristics of double discordance. Enormous co-existed anomalies are the culprit of prognosis evaluation and clinical decision. We aim at delineating a novel ccTGA clustering modality under human phenotype ontology (HPO) instruction and elucidating the relationship between phenotypes and prognosis in patients with ccTGA.MethodsA retrospective review of 270 patients diagnosed with ccTGA in Fuwai hospital from 2009 to 2020 and cross-sectional follow-up were performed. HPO-instructed clustering method was administered in ccTGA risk stratification. Kaplan-Meier survival, Landmark analysis, and cox regression analysis were used to investigate the difference of outcomes among clusters.ResultsThe median follow-up time was 4.29 (2.07–7.37) years. A total of three distinct phenotypic clusters were obtained after HPO-instructed clustering with 21 in cluster 1, 136 in cluster 2, and 113 in cluster 3. Landmark analysis revealed significantly worse mid-term outcomes in all-cause mortality (p = 0.021) and composite endpoints (p = 0.004) of cluster 3 in comparison with cluster 1 and cluster 2. Multivariate analysis indicated that pulmonary arterial hypertension (PAH), atrioventricular septal defect (AVSD), and arrhythmia were risk factors for composite endpoints. Moreover, the surgical treatment was significantly different among the three groups (p < 0.001) and surgical strategies had different effects on the prognosis of the different phenotypic clusters.ConclusionsHuman phenotype ontology-instructed clustering can be a potentially powerful tool for phenotypic risk stratification in patients with complex congenital heart diseases, which may improve prognosis prediction and clinical decision.

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“…[68][69][70][71][72][73][74][75][76][77][78][79][80] Radical repair is also used for congenitally corrected transposition of the great arteries. [81][82][83][84][85][86][87][88][89][90][91][92][93][94][95][96][97][98][99][100] In these malformations, conventional repair in which the circulation was functionally repaired placing the morphologically right ventricle as a systemic chamber used to be the only choice.…”

Section: ▋ 221 Biventricular Repairmentioning

confidence: 99%

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

Ohuchi

Kawata²,

Uemura

et al. 2022

Circ J

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9JCS 2022 Guideline on Management/Re-Intervention in ACHD comorbidity, 172 and cardiac catheterization is fundamental for its evaluation. 173 ▋ 3.4.4 Left Heart Catheterization and Coronary Angiography Echocardiography, CT, and MRI are often substituted for left ventriculography, aortography, or coronary angiography. CT precisely illustrates abnormal origins and courses of the coronary arteries in many cases, which is why coronary angiography is not recommended as a routine examination (Class III). In some patients, nonetheless, coronary angiography is quite informative beyond CT resolution; for example, anastomotic stenosis at the coronary arterial origin would be better demonstrated by catheter angiography after the arterial switch procedure. 174 ▋ 3.4.5 Endomyocardial Biopsy Endomyocardial biopsy is rarely required in patients with postoperative CHD. ▋ 3.4.6 Catheter InterventionTranscatheter closure of atrial septal defect or patent ductus arteriosus is carried out routinely in the cardiac catheterization room, as is percutaneous pulmonary angioplasty. Interventional closure of ventricular septal defect needs more evidence before becoming established in Japan.Transcatheter pulmonary valve replacement will be introduced in the near future.Electrophysiological study and catheter ablation are common practices for arrhythmias in the CHD field. Recent advances in technology and devices, such as 3D mapping systems, have contributed to notable results in anti-arrythmic treatments in patients with repaired CHD. 175

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Outcomes of treatment pathways in 240 patients with congenitally corrected transposition of great arteries

Cited by 23 publications

References 20 publications

Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs

Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs

Cardiovascular Phenotypes Profiling for L-Transposition of the Great Arteries and Prognosis Analysis

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

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