P89Cyanotic adult with atrial septal defect (ASD) is not an equivalent to an Eisenmenger syndrome

Introduction: Atrial Septal Defect (ASD) is the most common CHD in adults. Although pulmonary arterial hypertension is common with ASD, Eisenmenger syndrome (ES) occurs only in 5% of untreated patients. Diagnosis of ES secondary to ASD should be done carefully. We are presenting a cyanotic adult patient with ASD who was deemed as ES highlighting the role of CMR in the diagnosis Case Report: A 33-year-old male patient, known to have CHD since early childhood, complained of dyspnea (NYHA III). Echocardiography showed ASDII with right to left shunt. He was diagnosed as ES and referred to us for 2nd opinion. O2 saturation was 88% dropping to 68% with minimal effort. ECG showed sinus rhythm with left axis deviation and no evidence of right ventricular hypertrophy (Figure 1-A) which raised doubts against ES diagnosis. Echocardiography showed dilated left ventricle with EF of 40%, small right ventricle (RV) (Figure 1-B), ASD with right to left shunt and normal size pulmonary arteries. Notably this patient knew himself as a blue child. CMR was requested for better RV geometry evaluation and assessment of intra-cardiac shunt through the ASD. CMR was performed on 1.5 T scanner (Siemens Magnetom Aera, Siemens Medical Systems, Erlangen, Germany). CMR protocol included cine SSFP long and short axis for biventricular volume and flow evaluation and phase contrast cine images for aorta and pulmonary flows assessment. CMR confirmed being a borderline RV bi-partite RV in shape (having inlet and outlet parts, apical part is absent) (Figure 1-C and D) with good systolic function EF 50%. RV volumes were small, RV-EDVI 42 ml/m2, RV-ESVI 21 ml/m2 and RV-SV 21 ml/m2. Left ventricle was dilated with impaired systolic function EF 40%, LV-EDVI 126 ml/m2, LV-ESVI 86 ml/m2, LV-SV 41 ml/m2. There was an ASD was obligatory shunting right to left, Qp:Qs was 0.9. Cardiac catheterization revealed mean pulmonary artery pressure of 8 mmHg and pulmonary vascular resistance of 1.4 WU. Patient was eligible for 1.5 ventricle repair, Glenn shunt leaving the azygous open with closure of ASD with a fenestrated patch. O2 saturation became 94% on 6 minutes-walk test with marked improvement in functional capacity. Conclusion: We concluded that not all cyanosed adults with ASD have ES. Meticulous history taking, systematic clinical approach, and comprehensive CMR examination are the clues not to miss such complex patients.