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Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, [9] is a congenital heart defect characterized by four specific cardiac defects. [4] Classically, the four defects are: [ 4 ] pulmonary stenosis , which is narrowing of the exit from the right ventricle;
The Blalock–Thomas–Taussig shunt (BTT shunt), [1] previously known as the Blalock–Taussig Shunt (BT shunt), [2] is a surgical procedure used to increase blood flow to the lungs in some forms of congenital heart disease [3] such as pulmonary atresia and tetralogy of Fallot, which are common causes of blue baby syndrome. [3]
In 1944, Alfred Blalock at Johns Hopkins Hospital began successfully performing surgery on the great vessels around the heart to relieve the symptoms of tetralogy of Fallot, demonstrating that heart surgery could be possible. Lillehei participated in the first successful surgical repair of the heart on 2 September 1952.
The surgery had been designed and first performed on laboratory dogs by Thomas, who taught the technique to Blalock. Although Thomas perfected the technique, he could not perform the surgery because he was not a doctor. The surgery was not completely successful, since Eileen Saxon became cyanotic again a few months later. Another shunt was ...
Mutations in the gene for one of the notch ligands, Jagged1, are identified in the majority of examined cases of arteriohepatic dysplasia (Alagille syndrome), characterized by defects of the great vessels (pulmonary artery stenosis), heart (tetralogy of Fallot in 13% of cases), liver, eyes, face, and
The new operation not only directly saved thousands of lives, it marked the start of the modern era of cardiac surgery, as it was the first successful surgery on the human heart of the modern medical era. [1] [9] During his later years at Hopkins, Blalock continued his research on the heart and vascular surgery.
A diagnosis of TOF is usually made with echocardiography, which can even be done prenatally. Most patients with tetralogy of Fallot will have cardiac repair surgery in the first year of life, where the ventricular septal defect is closed with a patch, and the right ventricular outflow tract is enlarged.
The LeCompte maneuver is a technique used in open heart surgery, primarily on infants and children. The maneuver entails cutting the main pulmonary artery and moving it anterior to the aorta before reattaching the pulmonary artery during the following reconstruction of the great vessels.