Heart-lung transplantation in Eisenmenger syndrome with pulmonary embolisms

A 37-year-old man with a known history of an atrial septal defect was admitted to the hospital with shortness of breath, hemoptysis, chest pain, and tightness. On admission, the patient had a heart rate of 120 beats/min, respiratory rate of 21 breaths/min, basal resting oxygen saturation of 81%, and blood pressure of 99/60 mmHg. Transthoracic echocardiography showed the presence of a secundum type of atrial septal defect with a diameter of 21 mm, right ventricular (RV) enlargement, interventricular septal flattening, and severely impaired RV systolic function (fractional area change = 27%) (Fig. A). Color Doppler images showed a sizeable bidirectional shunting across the interatrial septum, confirming ongoing interatrial communication and significant pulmonary hypertension(PH) (Fig.B, video 1). In addition, there was severe tricuspid (Video 1) and pulmonary regurgitation. Continuous-wave Doppler imaging showed severe PH with RV systolic pressure of 103 mmHg and mean pulmonary artery (PA) pressure of 61 mmHg. Furthermore, echocardiography showed a mixed echogenicity intravascular mass that expands the PA lumen, accompanied by aneurysmal dilatation of the main PA and the obstruction of the PA branches (Fig. C, video 2). Color Doppler imaging showed no discernable flow of PA branches.