Texas Heart Institute journal
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Review Case Reports
Platypnea-Orthodeoxia Syndrome: To Shunt or Not to Shunt, That is the Question.
Platypnea-orthodeoxia syndrome is a rare disease defined by dyspnea and deoxygenation, induced by an upright position, and relieved by recumbency. Causes include shunting through a patent foramen ovale and pulmonary arteriovenous malformations. A 79-year-old woman experienced 2 syncopal episodes at rest and presented at another hospital. ⋯ In patients with unexplained or transient hypoxemia in which a cardiac cause is suspected, it is important to evaluate shunting in both the recumbent and upright positions. In this syndrome, elevated right atrial pressure is not necessary for significant right-to-left shunting. Percutaneous closure, if feasible, is first-line therapy in these patients.
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Tetralogy of Fallot often requires reconstruction of the right ventricular outflow tract with a transannular patch (TAP), but this renders the pulmonary valve incompetent and eventually leads to right ventricular dysfunction. We retrospectively evaluated the efficacy of a reconstructed pulmonary valve and annulus in 70 patients who underwent, from December 2006 through December 2010, complete correction of tetralogy of Fallot. We divided the 70 patients into 2 groups in accordance with whether they required (n=50) or did not require (n=20) a TAP. ⋯ There was no significant difference in outflow gradient or in the occurrence of pulmonary insufficiency between the TAP group (none, 31; mild, 12; moderate, 6; and severe, 1) and the No-TAP group (none, 16; moderate, 2; and severe, 2) (P=0.59). Nor was there any thickening or calcification in the constructed valves. We conclude that pulmonary valves constructed of untreated autologous pericardium performed as well as native valves after total tetralogy of Fallot correction at midterm.