Pregnant women with aortic dissection are hemodynamically outmost complex patients. The two major diagnoses that should be considered in pregnant patients with congenital heart disease (CHD) and acute type A aortic dissection presenting with postoperative right ventricular dysfunction are pulmonary thromboembolism and right ventricular infarction. We present a rare case of postoperative right ventricular dysfunction in pregnant women with CHD and acute aortic dissection, which was diagnosed by pulmonary computed tomography angiography and treated by percutaneous coronary intervention.