Eisenmenger Syndrome and Pericardial Effusion: The Challenge of Diagnosing “Tamponade” In Complex Congenital Heart Disease

A 31year-old female with Trisomy21, uncorrected complete atrioventricular septal defect (AVSD) and consequent Eisenmenger syndrome presented to our emergency department with dyspnea, hypoxia, and worsening cyanosis. She was found to be hemodynamically stable with a heart rate of 70bpm and blood pressure of 100/60. There was no pulsusparadoxus. She was notably cyanotic, platypneic, and hypoxic. Her oxygen saturation was between 80-85% despite O 2 supplementation. Her recorded saturation at a clinic visit a year ago was 93% on room air. An arterial blood gas showed a PaO2 was 51mmHg on a 100% O 2 . Her pH, PCO2 and HCO 3 were normal. She underwent an urgent CT pulmonary angiogram (CTPA), which confirmed features of Eisenmenger syndrome (i.e., complete AVSD, right ventricular hypertrophy, right atrial dilatation and an enlarged pulmonary artery) but showed no pulmonary embolus. The CTPA was negative for signs of pulmonary congestion or edema. Incidentally, a very large pericardial effusion was found. Additional history revealed that her functional capacity had steadily declined over the course of two weeks with marked dyspnea on exertion of less than a meter of walking.