As seen in Fig 2, the echocardiogram demonstrated a severely enlarged pulmonary artery of > 6 cm (Fig 2A) and a flap in the main pulmonary trunk separating the vessel into true and false lumens (Fig 2B), communicating through an opening of 10.6 mm. Color Doppler flow imaging showed a maximal velocity of 131 cm/s in the true lumen and 79 cm/s in the false lumen. A large PDA was seen between the descending aorta and the pulmonary artery bifurcation (width of 8.2 mm) with right-to-left shunting. MR pulmonary angiography was obtained, which confirmed the enlargement of the main pulmonary artery trunk, in which the linear dissection was seen to extend from the right pulmonary valve to the origin of the right pulmonary artery (Fig 3). Surgical repair was considered, but the best approach was unclear because a significant Eisenmenger shunt would be a contraindication for surgical intervention. After discussion among teams and with the family, a cardiac catheterization was performed. The pulmonary arterial pressure was 107/51 mm Hg, and pulmonary vascular resistance was 384.32 dynes/s/cm5. The right-to-left shunt was confirmed with a shunt fraction estimated to be > 30%. Three days after the right-sided heart catheterization, although a surgical plan was still being discussed, the patient suddenly went into pulseless electrical activity and could not be resuscitated. The family declined autopsy, and the presumed cause of death was pulmonary arterial rupture.