A 22-Month-Old Girl With 3 Weeks of Dyspnea
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A previously healthy 22-month-old girl presented to the ED with a 3-week history of dyspnea on exertion. A chest radiograph showed a right upper-lobe opacity suspicious for pneumonia. The patient was prescribed amoxicillin but she returned to the ED 7 days later with cough and persistent dyspnea and tachypnea. At that time, a repeat chest radiograph was concerning for worsening pneumonia. Treatment with azithromycin and albuterol was initiated, and amoxicillin was discontinued.

Her symptoms briefly improved however, she returned to the ED 10 days later because of worsening cough and tachypnea, and a 2-day history of increased irritability, decreased oral intake, decreased urine output, and intermittent perioral cyanosis.

The patient was tachycardic. Supplemental oxygen was administered by facemask, which increased her oxygen saturation to 100%. However, the patient’s respiratory distress worsened, and the face mask was replaced with CPAP support.
The patient was admitted to the cardiac ICU, and she suffered a cardiac arrest at the time of intubation for respiratory failure. Cannulation was performed for veno-arterial extracorporeal membrane oxygenation (ECMO) support. After ECMO cannulation, a complete transthoracic echocardiogram was performed, revealing the diagnosis of Cor triatriatum sinistrum.

It is seen in 0.0045 of 1,000 live births. It is typically associated with other congenital heart defects. A membrane divides the left atrium, separating the pulmonary veins from the level of the left atrial appendage and mitral valve. The membrane is typically fenestrated with one or more openings, allowing blood to pass through to the distal atrial chamber, across the mitral valve, and into the left ventricle. Definitive treatment requires surgical resection of the membrane using cardiopulmonary bypass.

source: https://journal.chestnet.org/article/S0012-3692(20)31607-X/fulltext?rss=yes