TY - CHAP M1 - Book, Section TI - Double-Outlet Right Ventricle A1 - Doherty, Gerard M. PY - 2010 T2 - Quick Answers Surgery AB - • A congenital heart lesion that decreases pulmonary arterial blood flow resulting in a right to left shunt• Cyanosis and decreased oxygen delivery causes compensatory polycythemia (Hct > 70%) and spontaneous thrombosis• Exercise, acidosis, pain worsens cyanosis and can cause hypoxic spells• Squatting increases systemic resistance, causing increased pulmonary flow and blood oxygenation• β-Blockers (decreases spasm), fluid intake, HCO3 administration, norepinephrine (increases systemic resistance) may help decrease hypoxia• Clubbing due to proliferation of capillaries and AV fistulas in extremities• Bronchial and mediastinal arteries enlarge• Ductus arteriosus maintains flow to lungs during fetal development• Alprostadil early can allow time for optimization before definitive treatment• Operative options to increase pulmonary flow: -Fontan procedure: Superior vena cava and inferior vena cava rerouted to pulmonary artery SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/19 UR - accesssurgery.mhmedical.com/content.aspx?aid=58109147 ER -