2018017-01 d-TGA Hypoplast RV Lat Tunnel Fontan

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Male human, aged 25-30. Source: Cardiac CT Scan. Software used: ITK-SNAP, 3D Slicer, Blender, and Meshmixer. Original Diagnosis: The heart's usual situs is present, but a rare condition exists where the great arteries are reversed, known as D-TGA. The atrioventricular (A-V) concordance indicates that the coronary sinus and right superior vena cava connect to the left atrium, while the ventriculoarterial (V-A) discordance means that the pulmonary artery arises from the right ventricle. The heart has a double inlet left ventricle, where both atria drain into the left ventricle. This is accompanied by a hypoplastic right ventricle, which is underdeveloped and unable to pump blood effectively. Two significant obstructions are present: pulmonary stenosis and subvalvular aortic stenosis, both of which restrict blood flow. An atrial septal defect (ASD) also exists, allowing blood to leak between the left and right atria. Procedures: To address these conditions, several surgeries have been performed. In the first year of life, a right Blalock-Taussig shunt was implemented to increase oxygenation of the blood. At age 6, a bidirectional cavopulmonary anastomosis and aortic myomectomy were performed. This involved connecting the superior vena cava directly to the pulmonary artery and removing excess tissue from the aorta. Finally, at age 6, a lateral tunnel Fontan procedure was conducted.