Ver el código de «Tricuspid atresia»

==Background==
[[File:Tricuspid atresia.svg|thumb|Anterior view of heart with tricuspid atresia with ASD+VSD. White arrows indicate blood flow. Atresic tricuspid valve labeled at bottom left.]]
*A cyanotic congenital heart defect
*Absence of tricuspid valve resulting in absence of communication between the right atrium and right ventricle
*Due to absence of communication with the right ventricle, RV becomes either under-developed or entirely absent
*Third most common cyanotic [[congenital heart disease]] <ref>Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.</ref>

===Associated cardiac lesions===
*[[ASD]] (100%)
*Right ventricular hypoplasia (100%)
*[[VSD]] (95%)
*Pulmonary outflow obstruction (75%)
*[[Transposition of the great arteries]] (28%)

===Physiology===
*Blood from the RA must exit through the ASD (no tricuspid valve)
*Right to left atrial shunting allows deoxygenated systemic venous blood to enter the LA, then LV
*Cyanosis is present due to mixing of systemic and pulmonary venous blood in the LA

==Clinical Features==
*50% present on the first day of life, additional 30% present by 1 month of age
*Central cyanosis is the most notable feature on physical exam and can be progressive
*May also present with [[failure to thrive (peds)|poor feeding]]
*Holosystolic [[murmur]] at left lower sternal border if VSD is present
*Continuous murmur if there is a [[PDA
*Jugular venous distention and prominent “a” wave and hepatomegaly if there is a restrictive atrial level communication
*[[Shortness of breath (peds)|Tachypnea]] may be present in patients with unrestrictive pulmonary blood flow
*[[EKG]] may show [[left axis deviation]] and [[LVH]]
*Heart size is normal typically

==Differential Diagnosis==
{{Congenital heart disease DDX}}

==Evaluation==
*[[Echocardiography]]
*[[Chest x-ray]]
**May have a paucity of pulmonary markings and normal heart size in patients with normal pulmonary blood flow (no VSD)
**May have cardiomegaly and prominence of pulmonary vascularity in patients with increased pulmonary blood flow (large VSD)
*[[ECG]]
**May demonstrate tall P waves, [[left axis deviation]], [[LVH]], and diminished RV forces

==Management==
*Stabilize cardiopulmonary function prior to surgery
*Supplemental [[oxygen]]
*Mechanical ventilation
*Inotropic agents (eg, [[dopamine]] and [[dobutamine]]) improve myocardial contractility
*[[Prostaglandin E1]]
**Maintain adequate ductal dependent pulmonary flow
**Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension and apnea
**Side Effects: Hypotension, Bradycardia, Seizures and Apnea
*Staged surgical repair

==Disposition==
*Admit

==See Also==
*[[Congenital heart disease]]

==External Links==


==References==
<references/>

[[Category:Cardiology]]
[[Category:Pediatrics]]