An inferior sinus venosus interatrial communication associated with a secundum atrial septal defect, clinically presenting in an adult patient: autopsy report
Keywords:Congenital heart defects, Atrial septal defect sinus venosus, Heart septal defects, Atrial, Pulmonary hypertension, Pneumonia, Autopsy
AbstractAtrial septal defects (ASD) are the most common congenital cardiac lesion
in adults, representing up to 40% of acyanotic shunt lesions in patients older
than 40 years. Secundum ASD comprises defects in the area of the oval
fossa, and may be associated with other types of interatrial communications.
We present a case of a 25 year-old female patient who was asymptomatic
until the age 22 when she started presenting exertion dyspnea and lower
limbs edema, during her third pregnancy. The patient was admitted in the
emergency department because of a febrile respiratory distress syndrome
dying in less than 36 hours after her arrival. The autopsy examination revealed
pneumonia and a secundum ASD, associated with a rare form of interatrial
communication, the inferior sinus venosus defect, which is characterized by
an anomalous connection of the right pulmonary veins to the inferior caval vein
at its opening in the right atrial cavity, while retaining partially their connection
to the left atrium. A dysplastic and thickened mitral valve was also present.
Signs of pulmonary hypertension confirm the Eisenmenger syndrome in this
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