LAUSR

A 32-year old patient, with a longstanding history of pulmonary hypertension (PH) due to a congenital ventricular septal defect (VSD) with shunt reversal and Eisenmenger syndrome was initially admitted to the emergency department of a secondary health center due to dyspnea and hemoptysis. During his childhood, he underwent an operative reconstruction of an associated aortic isthmus stenosis. A subsequent VSD closure was not performed for unknown reasons. The last transthoracic echocardiography before hospitalization showed normal left ventricular ejection fraction (LVEF) of 55–60%, good right ventricular ejection fraction, a large ventricular septal defect of approximately 13 mm with significant right to left shunt (Fig. 1b). Systolic pulmonary arterial pressure was approximately 130 mmHg and a main pulmonary artery aneurysm of 49 mm. At admission, severe pulmonary bleeding and respiratory hypoxic failure were evident, with a peripheral oxygen saturation of 70% with 10 l/min oxygen insufflation via reservoir mask. Noninvasive ventilation (NIV) was initiated. Pulmonary bleeding however progressed until massive hemoptysis made endotracheal intubation inevitable. Computed tomography revealed alveolar hemorrhage associated with pathologic major aortopulmonary collateral arteries (MAPCAs) from the proximal aorta descendent and the right subclavian artery plus an obstruction of the intermediate bronchus with a coagulum and a resulting subtotal atelectasis of the middle and lower pulmonary lobe. Bronchoscopic hemostasis in the sixth lung segment was performed and primarily successful. Therapy strategy: vvECMO and angiographic embolization Severe complication: Pulmonary bleeding from main aorto-pulmonary collateral arteries (MAPCAs) Ventricular septal defect (VSD) and pulmonary hypertension (PH)