Cor Triatriatum

Question 1

Definition

Answer 1

characterized by a perforate membrane that partitions the kit atrium into a proximal or accessory chamber, which receives the pulmonary veins, and a distal/true left atrial chamber, which usually contains the left atrial appendage and the fossa ovalis

Question 2

Prevalence

Answer 2

Less than 1% of congenital heart disease

male to female ration is 1.5 to 1

Question 3

Embryology

Answer 3

Incomplete absorption of the common pulmonary vein leaving an obstructing membrane of tissue

Question 4

Pathology/ physiology

Answer 4

-Secondary signs similar to valvular MS (RVH, Pulm Vascular changes, PHTN)

-Jet lesions on atrial aspect of MV may be present as well as on mural endocardium of lower atrial walls

• size of perforation(s) determines degree of obstruction

Question 5

Associated conditions

Answer 5

PFO

ASD (communicating with proximal or distal chamber)

Partial anomalous pulmonary venous return

total anomalous pulmonary venous return

Coarctation of aorta

VSD

ToF

Pulmonary stenosis

Question 6

History

Answer 6

-asymptomatic (until 2nd and 3rd week of life)

-dyspnea

-orthopnea

-difficulty feeding

-poor weight gain

-frequent respiratory infections

-pneumonia

-pulmonary edema with exertion

Question 7

Physical Examination

Answer 7

-right ventricular lift

-jugular venous distention

-hepatomegaly

-peripheral edema

-cyanosis (w right to left atrial shunting

Question 8

Cardiac Auscultation

Answer 8

• Loud S2 (suggesting PHTN)
• soft systolic/continuous murmur representing flow across membrane

-apical diastolic murmur (uncommon)

• TR (suggesting PHTN)

-PR (suggesting PHTN)

Question 9

EKG

Answer 9

• RVH

-RAE (P pulmonale)

• Right axis deviation

-LAE

-Afib

Question 10

Chest X ray

Answer 10

-Cardiomegaly (RAE, RVH, MPA)

• venous engorgement of upper pulmonary veins (Staghorn sign)

-Pulmonary edema

-Kerley B lines

Question 11

Cardiac Cath

Answer 11

-pulmonary arterial angiography may demonstrate obstruction

• pulmonary angio shows delayed emptying of left atrium

-evaluates increased pulmonary and RV pressures

-Increased pulmonary wedge pressures

-rules out left to right shunt

-Evaluates presence of anomalous pulmonary venous return (10%)

Question 12

Natural history/ complications

Answer 12

• progressive pulmonary venous htn

-right heart failure

• pulmonary edema

Question 13

medical management

Answer 13

treat CHF (diuretics, digitalis)

Question 14

Surgical management

Answer 14

excision of obstructing fibrous membrane

associated PFO/ASD closure

Question 15

echo views

Answer 15

• parasternal/ apical long axis, parasternal short axis, apical/subcostal four chamber, apical 2ch

Question 17

Mode/ 2D

Answer 17

• identify LA area, fossa ovalis, interatrial septum, LAA
• membrane located in superior portion of the left atrium

-LAA and foramen oval located distal to membrane

-accessory chamber usually receives all four pulmonary veins

-high frequency diastolic oscillations of the mitral valve

dilation of upper pulm vein (staghorn sign)

• LAE

-RVH/RVE

-flax/paradoxical septal motion

-PHTN

-RAE

-determine the presence of associated lesions ( ASD,PFO,ASD,VSD,..)

Question 18

PW/CW/Color doppler

Answer 18

-locate the orifice of the membrane by color flow doppler and measure the diameter (3 to 15 mm)(may be multiple)

-determine peak velocity, peak pressure gradient, mean pressure gradient across membrane

Question 19

Physiologically similar to

Answer 19

tricuspid atresia