Cor triatriatum sinister

Orpha code: 99099OMIM code:

Definition

A rare, congenital, non-syndromic, heart malformation characterized by the presence of a thin, fibromuscular membrane subdividing the left atrium into an upper and lower chamber. The upper chamber receives blood from the pulmonary veins and the lower chamber is attached to the left atrial appendage. Therefore, the membrane blocks the orifice of the mitral valve and leads to obstruction of the left ventricular inflow. It may be asymptomatic or present in infancy with tachypnea, dyspnea, hemoptysis, chest pain, syncope, pulmonary edema, pulmonary hypertension, or heart failure, depending on the degree of obstruction. The anomaly may be isolated or associated with other congenital heart anomalies.

Disease data
Classification

Morphological anomaly

Synonyms
Cor triatriatum sinistrum
Podzielony lewy przedsionek
Divided left atrium
ORPHA code
99099
OMIM code
-
ICD10 code
Q24.2
ICD11 code
LA8G.0

No additional description.

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