Eisenmenger syndrome complicated by pulmonary artery dissection

A 63-year-old female patient with Eisenmenger syndrome (NYHA functional class IV) secondary to an uncorrected ostium secundum atrial septal defect was referred to our hospital because of a syncope occurring while she was at rest. She presented with shortness of breath and cyanosis. Blood pressure was 95/55 mmHg and oxygen saturation 75%. The ECG was consistent with right ventricular hypertrophy and overload. A transthoracic echocardiogram confirmed the presence of a large ostium secundum atrial septal defect with bidirectional shunt, right ventricular hypertrophy and dilatation with severe tricuspid regurgitation and an estimated systolic pulmonary pressure of 100 mmHg. An aneurysm of the pulmonary artery trunk was noted, with free-floating membranes originating few millimetres above the valvular plane (Panel A). Pericardial effusion was absent. The MRI study confirmed a giant aneurysm of the pulmonary artery (Panel C), with a diameter of about 95 mm, with intimal flaps consistent with the dissection of the pulmonary trunk. A wide intimal disruption rather than a real false lumen was observed (Panel D: diastolic image with pulmonary regurgitation jet and an intimal flap above the pulmonary valve plane). A 16-slice computed tomography scan ruled out pulmonary embolism and confirmed the dissection (Panel B). Pulmonary artery dissection is a very rare complication of severe pulmonary hypertension, mostly responsible of sudden death and usually diagnosed at post-mortem examination. The patient was treated conservatively, and was alive at 6 months follow-up, without recurrence of syncope.