Taussig–Bing syndrome

Taussig–Bing Syndrome

Taussig–Bing syndrome is a rare congenital heart defect characterized by a combination of transposition of the great arteries (TGA) and a ventricular septal defect (VSD). This condition is named after the cardiologists Helen B. Taussig and Richard J. Bing, who first described it.

Pathophysiology

In Taussig–Bing syndrome, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, which is the opposite of the normal anatomical configuration. This is similar to the condition known as dextro-Transposition of the great arteries (d-TGA). However, in Taussig–Bing syndrome, there is also a large ventricular septal defect that allows for mixing of oxygenated and deoxygenated blood between the ventricles.

The presence of the VSD is crucial because it allows some oxygenated blood from the left ventricle to enter the aorta, which is necessary for survival. However, this mixing is inefficient, and the body receives a lower than normal amount of oxygenated blood, leading to cyanosis and other complications.

Clinical Presentation

Patients with Taussig–Bing syndrome typically present with symptoms of cyanosis shortly after birth. Other symptoms may include difficulty breathing, poor feeding, and failure to thrive. The degree of cyanosis and other symptoms can vary depending on the size of the VSD and the degree of mixing of blood.

Diagnosis

Diagnosis of Taussig–Bing syndrome is typically made using echocardiography, which can visualize the abnormal connections of the great arteries and the presence of a VSD. Additional imaging studies, such as cardiac MRI or cardiac catheterization, may be used to further assess the anatomy and plan for surgical intervention.

Treatment

The primary treatment for Taussig–Bing syndrome is surgical correction. The most common surgical approach is the arterial switch operation, which involves switching the aorta and pulmonary artery to their correct positions. This is often combined with closure of the VSD. In some cases, additional procedures may be necessary to address other associated cardiac anomalies.

Postoperative care is critical, and patients require close monitoring and follow-up to manage any complications and ensure proper cardiac function.

Prognosis

With advances in surgical techniques and postoperative care, the prognosis for patients with Taussig–Bing syndrome has improved significantly. Many patients go on to lead healthy lives, although they may require ongoing cardiac care and monitoring.

Also see

• Congenital heart defect
• Transposition of the great arteries
• Ventricular septal defect
• Cyanotic heart defect