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Atypical twin atrioventricular nodal re-entrant tachycardia in a congenitally corrected transposition of the great arteries patient with a surgical repair of a ventricular septal defect: a case report.

ABSTRACT:

Background

Twin atrioventricular (AV) nodal tachycardia is a rare mechanism of supraventricular arrhythmia, only seen in some specific congenital heart defects (CHD). It consists of a re-entrant circuit between two distinct AV nodes (anterior and inferior). Since both nodes have antegrade and retrograde conduction, there is usually two QRS morphologies in sinus rhythm.

Case summary

This case is about an atypical twin AV nodal tachycardia in a 15 years old patient with congenitally corrected transposition of the great arteries and previous history of a ventricular septal defect repair. The surgical closure was probably responsible of a poor antegrade conduction over the inferior AV node, which was responsible for a unique QRS morphology. He finally received a catheter ablation of the inferior AV node. He remained asymptomatic without anti-arrhythmic drugs at 8-months post-ablation.

Discussion

Twin AV nodal re-entrant tachycardia is a rare phenomenon. The presentation and electrophysiological study can be both atypical due to previous surgical repair. The operator should be aware of specific CHD where twin AV nodal re-entrant tachycardias are expected.

SUBMITTER: Gardey K

PROVIDER: S-EPMC8350359 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundAdults with unoperated congenitally corrected transposition of the great arteries are rare but form a distinct group among adults with congenital heart disease. Patients with congenitally corrected transposition of the great arteries often have one or more associated cardiac anomalies that dictate the need for, and timing of, surgical intervention in childhood. However, in a proportion of patients, the hemodynamics does not require surgical attention during childhood, and, in some patients, a correct diagnosis is not established until adulthood. Here we report an adult case of unoperated congenitally corrected transposition of the great arteries with a large ventricular septal defect and probable pulmonary arterial hypertension.Case presentationOur patient was a 46-year-old Korean man. Transthoracic echocardiography and cardiac catheterization demonstrated hemodynamically balanced ventricles with a non-regurgitant systemic atrioventricular valve, normal pulmonary arterial pressure, and a reasonable difference between the oxygen saturation values of the aorta and pulmonary trunk, even with the presence of a large ventricular septal defect. Further morphological assessments using cardiac computed tomography and three-dimensional modeling/printing of his heart revealed that the mitral valve was straddling over the posteriorly positioned ventricular septal defect, which could explain the functional and anatomical subvalvular pulmonary stenosis and a small amount of shunt flow through the large ventricular septal defect. We interpreted this combination of cardiac defects as able to sustain his stable cardiac function. Thus, we decided to maintain his unoperated status.ConclusionA detailed anatomical understanding based on transthoracic echocardiography, cardiac computed tomography, and three-dimensional printing can justify a decision to not operate in cases of congenitally corrected transposition of the great arteries with hemodynamically balanced pulmonary stenosis and a ventricular septal defect, as observed in the present case.