test 5 cor triatriatum

Question 1

Cor Triatriatum characteristics

Answer 1

• 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%

Question 2

Cor Triatriatum

Answer 2

• 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

Question 3

Membrane that causes Cor Triatriatum

Answer 3

• 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.

Question 4

Cor Triatriatum Dextrum

Answer 4

• Right Atrial

* extremely rare

Question 5

Cor Triatriatum Sinistrum

Answer 5

• Left Atrial

* Misdiagnosed frequently as asthma, mitral stenosis or obstructed pulmonary venous return

Question 6

Cor Triatriatum Sinistrum pathophys

Answer 6

• 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

Question 7

Cor triatriatum dextrum Pathophysiology

Answer 7

• 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

Question 8

Mortality/Morbidity for cor triatriatum sinistrum

Answer 8

• 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

Question 9

Surgery for cor triatriatum

Answer 9

- 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

Question 10

cor triatriatum cannulation

Answer 10

• 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