Critical Care Medicine-Neurologic Disorders>>>>>Congenital Heart Disease in Adults
Question 1#

A 36-year-old female with known Eisenmenger syndrome (ES) is admitted to the ICU after 2-day hospital course for worsening hypoxia. She is followed as an outpatient in the cardiology clinic, and it is noted that her oxygen levels are usually low. Her vital signs show a heart rate of 95 bpm, oxygen saturation of 86% on noninvasive positive pressure ventilation, blood pressure of 116/80 mm Hg, and a respiratory rate of 18. On examination, you note the female in mild respiratory distress with signs of cyanosis. Chest X-ray showed mild pleural effusions bilaterally but no concern for pulmonary edema. Transthoracic echocardiography done the prior day showed concern for biventricular dysfunction.

What is the next best step to confirm this patient’s diagnosis?

a. Transesophageal echocardiography
b. CTA of the chest
c. Cardiac catheterization
d. Repeat CXR

Correct Answer is B


Correct Answer: B

This patient is exhibiting signs of acute respiratory distress leading to respiratory failure with a known history of ES. Given this history, she likely is suffering from pulmonary arterial thrombosis and requires a CTA of her chest to confirm the diagnosis. ES is a consequence of severe PAH, more commonly as a result of congenital heart disease. It is most commonly associated with atrial and/or ventricular septal defects. The primary pathophysiology results in left to right shunting. This leads to shearing forces and stress on the pulmonary vasculature, resulting in endothelial dysfunction, release of inflammatory mediators, and vascular remodeling. The increase in pulmonary vascular resistance causes a right-to-left shunt that leads to cyanosis, which completes the picture of ES. TEE will only show the shunting and might not reveal the thrombosis (A). Cardiac catheterization will reveal the elevated pulmonary pressures but will not be diagnostic for a pulmonary venous thrombosis (C). Because the patient has only mild pleural effusions, a chest radiograph will not be very useful either (D). 

Patients with ES are at risk for multiple comorbidities, including pulmonary arterial thrombosis (∼20%). Risk factors that further increase this risk include biventricular dysfunction, female sex, dilation of the pulmonary arteries, and decreased pulmonary blood flow. 


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