Tricuspid Regurgitation in Hypoplastic Left Heart Syndrome: Three-Dimensional Echocardiography Provides Additional Information in Describing Jet Location.

BACKGROUND: Twenty-five percent of patients with hypoplastic left heart syndrome (HLHS) require tricuspid valve (TV) repair. The location of tricuspid regurgitation (TR) is important in determining the type of repair performed. Studies using three-dimensional echocardiography (3DE) have reported a high incidence of error on two-dimensional echocardiography (2DE) for the identification of TV leaflets. The aim of this study was to compare assessment of TR on 3DE and 2DE in patients with HLHS (jet location, TR grade, and reproducibility). METHODS: A retrospective, single-center review was performed. Fifty-six patients with HLHS with available two-dimensional and three-dimensional echocardiograms, and mild or greater TR, were included. TR location, grade, vena contracta area, and TV annular diameter were measured on 2DE and 3DE. Reproducibility was assessed by blinded reviewers. RESULTS: Three-dimensional echocardiography identified the primary jet location as central (57%) followed by anteroseptal (36%). There was poor agreement between findings on 3DE and 2DE for jet location (κ = 0.05; 95 CI, -0.08 to 0.19). Interobserver reproducibility for location on 3DE was excellent (κ = 0.8), whereas reproducibility for 2DE was poor (κ = 0.32). The most common jet location pre-Norwood and pre-Glenn was central (70%), whereas pre-Fontan and post-Fontan, jet location was central (45%) and anteroseptal (48%). Vena contracta area on 2DE correlated moderately with vena contracta area on 3DE (r = 0.60, P < .0001). TV annular diameters on 2DE and 3DE for lateral (r = 0.85, P < .0001) and anteroposterior (r = 0.74, P = .001) dimensions were strongly correlated. CONCLUSIONS: In children with HLHS, assessment of TR location on 2DE had poor agreement with assessment on 3DE and was poorly reproducible. In contrast, TR jet location on 3DE was highly reproducible. Pre-Glenn, a central TR jet was the most common, while post-Glenn, central and anteroseptal locations were equal, highlighting the importance of preoperative identification of TR jet location in patients with HLHS.

Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula.

OBJECTIVE: To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA). METHODS: All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively. RESULTS: Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%. CONCLUSION: ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.