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PATIENT STORY

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A 20-year-old man with a single morphologic left ventricle due to tricuspid atresia and ventricular inversion with transposed great arteries, with transposed great arteries presents with stunted growth and maturation. He initially had a pulmonary artery band (Figure 7-1) placed when he was 2 months old with an atrial septectomy at 9 months. At age 3 years he underwent a surgical Fontan palliation, which involved redirection of the superior vena cava (SVC) and inferior vena cava (IVC), by way of a lateral tunnel, to the right pulmonary artery, confluent with the left. The main pulmonary artery (MPA) was excised. Therefore, systemic venous flow was redirected to passively flow to the right and left pulmonary vascular beds, bypassing the heart entirely (Figure 7-2). He had an uneventful recovery in early childhood until he was diagnosed with protein-losing enteropathy (PLE) at the age of 6 years. Subsequently, at age 10, further surgery was done to remove the lateral tunnel and replace this with an extracardiac conduit between the IVC and pulmonary artery. Despite surgical revision and medical therapy, he continued to have evidence of PLE complicated by a small stroke and evidence of mild liver fibrosis. He was started on warfarin. By age 12, he had evidence of delayed maturation and stunted growth (Figure 7-3), as a complication of PLE. He had chronic hypoproteinemia, hypocalcemia, and hyponatremia. Measured testosterone and growth hormone levels were normal. He was considered for heart transplantation, but instead medical therapy was continued, including subcutaneous heparin, testosterone, and spironolactone.

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FIGURE 7-1

Illustration depicting a pulmonary band placed in the patient's anatomy of tricuspid atresia with transposed great arteries. Ao, aorta; LV, left ventricle; PA, pulmonary artery; PV, pulmonary vein; RA, right atrium; RV, right ventricle. (Copyright © McGraw-Hill Education, Photographer: Dr. Laurie Armsby. Modified with permission from Dr. Laurie Armsby.)

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FIGURE 7-2

A. Illustration depicting a lateral tunnel Fontan connecting the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary artery which has been disconnected from the pulmonary valve in the patient's anatomy of tricuspid atresia with transposed great arteries. Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PV, pulmonary vein; RA, right atrium; RV, right ventricle. B. Transthoracic echocardiogram showing a subcostal view of the inferior vena cava (IVC) being directed into the lateral tunnel through the right atrium. SVC, superior vena cava. (Copyright © McGraw-Hill Education, Photographer: Dr. Laurie Armsby. Modified with permission from Dr. Laurie Armsby.)

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FIGURE 7-3

Growth chart depicting the decreased growth velocity in both weight and stature starting at 8 years and 11 years, respectively, and ultimately resulting in short stature.

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