Cardiac > Congenital > Triatriatum

Cor Triatriatum:

Clinical:

Cor triatriatum is a rare lesion due to failure of the common pulmonary vein to incorporate normally into the left atrium (cor triatriatum sinister [4]). A fibromuscular septum arising from the interatrial septum and extending toward the atrial free wall separates the embryologic common pulmonary vein from the left atrium. The resultant accessory chamber lies posterior/superiorly (above the level of the left atrial appendage [3]) and receives pulmonary veins. The membrane interposed between the left atrium and pulmonary veins produces obstruction to venous return. The anteroinferior chamber contains the atrial appendage and the mitral valve [2].

 

Patients present with dyspnea, CHF, and failure to thrive (symptoms are similar to mitral valve stenosis). There is good results following surgical correction. However, if left untreated, 75% of patients will die in infancy because of secondary pulmonary hypertension [2]. The lesion is associated with other congenital heart disease such as an ASD (secundum type [2]), PDA, and coronary sinus malformations [2].


More rarely, the condition can also affect the right atrium (cor triatriatum dexter) [4].

X-ray:

The radiographic findings vary with the degree of obstruction. CXR findings include pulmonary venous distention and interstitial edema and left atrial enlargement. The pulmonary trunk/arteries dilated in severe obstruction. The RA and RV also enlarge.

REFERENCES:
(1) Seminars in Roentgenology 1989; Budorick NE, et al. The pulmonary veins. 24 (2) Apr: 127-140

(2) J Cardiovasc Comput Tomogr 2011; Rojas CA, et al. Cardiac CT of non-shunt pathology of the interatrial septum. 5: 93-100

(3) Radiographics 2012; Vyas HV, et al. MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants. 32: 87-98

(4) Radiographics 2019; Rajiah P, et al. Bands in the heart: multimodality imaging review. 39: 1238-1263

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