test 5 cor triatriatum Flashcards
Cor Triatriatum characteristics
- characterized by 3 atria in the heart
- first case in 1868
- affects 0.1 - 0.4% of population
- symptoms are breathing difficulty, easily fatigablility, and bloody sputum
- surgically treated
- 75% death rate if untreated
- complications: cardiac arrhythmia, ascites, and liver dysfunction
- if treated 10 year survival rate is 83%
Cor Triatriatum
- Cor triatriatum is a heart with 3 apparent atria (tri-atrial heart)
- congenital anomaly in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into 2 parts by a fold of tissue, a membrane, or a fibromuscular band
Membrane that causes Cor Triatriatum
• varies significantly in size and shape
▫ a diaphragm
▫ funnel-shaped, band like, entirely intact (imperforate)
▫ contain 1 or more openings (fenestrations) ranging from small, restrictive-type to large and widely open.
Cor Triatriatum Dextrum
- Right Atrial
* extremely rare
Cor Triatriatum Sinistrum
- Left Atrial
* Misdiagnosed frequently as asthma, mitral stenosis or obstructed pulmonary venous return
Cor Triatriatum Sinistrum pathophys
- Current theory holds that cor triatriatum sinistrum occurs when the common pulmonary vein fails to incorporate the pulmonary circulation into the left atrium.
- The result is a septum-like structure that divides the left atrium into 2 compartments.
- Cases have been reported in which 1 or 2 pulmonary veins drain into the proximal (accessory) chamber and the others drain directly into the true LA
- Others believe that the membrane dividing the left atrium is an abnormal growth of the septum primum
Cor triatriatum dextrum Pathophysiology
• During embryogenesis:
▫ the original embryologic RA forms the trabeculated anterior portion of the RA
▫ The right horn of the sinus venosus gradually grows to form the smooth
posterior portion of the RA
▫ Connection between right horn and embryonic RA forms a sinus valve
• Complete persistence of the right sinus valve of embryonic life results in separation of the smooth and trabeculated portions of the right atrium and constitutes cor triatriatum dextrum.
• This forms a sheet that serves to direct the oxygenated venous return from the IVC across the foramen ovale to the left side of the heart.
• If this membrane is fenestrated and web like, then it is referred to as the Chiari network.
▫ Usually not
symptomatic because little to no obstruction from upper to lower chamber
Mortality/Morbidity for cor triatriatum sinistrum
• high in those who are symptomatic in infancy.
▫ Due to the severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congenital heart lesions.
• Mortality may exceed 75% in untreated symptomatic infants.
• Severe obstruction = poor prognosis
Surgery for cor triatriatum
- just go in and cut out the membrane • Performed soon after diagnosis • Median sternotomy • CPB with XC • XC time is short - surgical correction can be done through the foramen ovale
cor triatriatum cannulation
- Aortic Arterial cannulation
- Bicaval venous cannulation: (open procedure)
- Procedure will be quick if the Pulmonary Veins are not involved
- Mild to “drift” cooling
- Circulatory arrest if a small child or Pulmonary veins involved