<p>A patient presented with recurrent syncope due to transient severe hypotension. The patient's history, physical examination, and initial baseline investigation did not suggest a cardiovascular cause. After fluid resuscitation, a raised jugular venous pulse was noted. Bedside transthoracic echocardiogram showed a pericardial effusion and a proximally dilated aorta. Computed tomography of the thorax confirmed these findings and also demonstrated an intramural hematoma of the proximal aortic wall.</p><p>The patient was transferred to a cardiothoracic center, where he was at first treated medically. He then developed sudden cardiogenic shock due to pericardial tamponade and was successfully operated on.</p><p>It is important to recognize an acute intramural hematoma of the proximal aortic wall as a cardiothoracic emergency. This condition can present atypically, but nevertheless warrants urgent surgical intervention, equal to type A aortic dissection. Echocardiography can help in making the diagnosis.</p>
IgG4-Related Periaortitis Initially Suspected of Being an Aortic Intramural Hematoma in the Ascending Aorta