Contributed by Anthony Azakie, MD, with Jillian Lokere
First described 60 years ago1, the heart malformation known as Taussig-Bing heart is characterized by transposed great arteries and a ventricular septal defect. The lesion may be further complicated by obstruction of the aortic arch. Complete, single-stage repair of the condition in the neonatal period via a primary arterial switch operation with septal defect closure is the gold standard for surgical management of the Taussig-Bing heart. Low birth weight in these patients, however, increases the risk of postoperative mortality.2 Here we present the case of a high-risk, lowbirth-weight infant who underwent complete, primary repair of TaussigBing heart and interrupted aortic arch with a very successful long-term outcome.
A newborn male born at term was referred with transposed great arteries and ventricular and atrial septal defects (a Taussig-Bing heart) as well as an interrupted aortic arch. The patient weighed 2.4 kg at birth, placing him into the high-risk, low-birth-weight category (less than 2.5 kg).
The philosophy of primary, complete, early repair was followed in this newborn’s case. The patient underwent an arterial switch operation with closure of the ventricular and arterial septal defects and an aortic arch reconstruction. An intra-operative echocardiogram showed no residual defects. The infant recovered without sequelae and was discharged in good condition. At his 2-year follow-up, the child was doing very well and is developing normally.
A complete, single-stage repair of complex congenital heart disease in neonates avoids the need for multiple surgeries, averts the effects of prolonged cyanosis, and prevents the development of aortic insufficiency, congestive heart failure, ventricular hypertrophy, and pulmonary vascular disease.3 Indeed, among pediatric patients with Taussig-Bing heart, those who undergo complete, singlestage repair have much lower rates of re-intervention, mortality, and cardiac complications than do those who underwent palliative surgical procedures before definitive repair.4
Although low birth weight does increase operative risk in neonates, a delay in complete repair due to low birth weight may not be beneficial and, in most cases, results in lower overall survival rates among these children.5 When placed in the hands of experienced specialists, low-birthweight neonates with Taussig-Bing heart and those with other complex lesions—including tetralogy of Fallot, pulmonary atresia with ventricular septal defect, and aortic arch obstruction with ventricular septal defect—can be considered candidates for complete, single-stage repair.6
About one-quarter of infant deaths in the United States can be attributed to anomalies caused by congenital heart disease. Now new surgical approaches and technology are improving long-term outcomes.Continue reading