Successful surgical repair of Taussig Bing anomaly with pulmonary artery and pulmonary valve stenosis in a neonate patient: A case report

Abstract

Introduction and importance: Taussig Bing anomaly (TBA), a subtype of double outlet right ventricle, is a rare congenital cardiac malformation. TBA consists of double outlet right ventricle, sub-pulmonary ventricular septal defect, and side-by-side localization of the great arteries. The association of pulmonary artery and pulmonary valve stenosis with TBA is an uncommon phenomenon.

Case presentation: A 2-day-old male neonate presented with central cyanosis and poor breastfeeding following birth. His mother had no history of drug use or medical issues during pregnancy. The neonate was diagnosed TBA with pulmonary artery and pulmonary valve stenosis based on investigations including electrocardiogram, chest X-rays, and echocardiography. At three months of age, the child underwent palliative Blalock-Taussig shunt surgery (BTS) before undergoing Réparation à l'Ètage Ventriculaire (REV) repair surgery at the age of two. The follow-up showed that the child is currently in good health without any complications.

Clinical discussion: Patients with TBA should be diagnosed early to plan the correct surgical technique and timely intervention, which can significantly improve the quality of life for these patients.

Conclusion: TBA is a surgical challenge, which becomes even more challenging when accompanied by other malformations such as pulmonary artery and pulmonary valve stenosis. Medical team should work together to improve the patient's outcome.

Keywords: Blalock-Taussig shunt; Case report; Double outlet right ventricle; Pulmonary arteries stenosis; Pulmonary valve stenosis; REV procedure; Taussig Bing anomaly.

Fig. 1

Electrocardiogram reveals right axis deviation, indicating hypertrophy of right ventricle.

Fig. 2

Chest X-rays shows situs solitus and boot-shaped heart.

Fig. 3

Echocardiography reveals side-by-side localization of the great arteries, sub pulmonary ventricular septal defect, severe pulmonary valve stenosis, and relatively small pulmonary arteries A: Continuous wave Doppler showing gradients across the pulmonary valve (severe valve stenosis); B: Parasternal long axis view showing the aorta anteriorly and the pulmonary arteries posteriorly, with relatively small pulmonary arteries; C: Parasternal long axis view displaying the anterior aorta and posterior pulmonary artery with pulmonary valve stenosis; D: Subcostal long axis view indicating pulmonary valve stenosis; E: Subcostal short axis view at the level of the ventricles revealing a VSD with a left-to-right shunt. Star indicates the presence of pulmonary stenosis; RA: Right Atrium; RV: Right Ventricle; LA: Left Atrium; LV: Left Ventricular; VSD: Ventricular Septal Defect; PA: Pulmonary Artery; AO: Aortic.