Diseases of Aorta: Marfan, Dissection, Atheroma
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1 31 st Annual State of the Art Echocardiography San Diego, CA February 20, :00 9:20 AM 20 min Diseases of Aorta: Marfan, Dissection, Atheroma Muhamed Sarić MD, PhD, MPA Director of Noninvasive Cardiology Echo Lab Associate Professor of Medicine 1
2 Disclosures Speakers Bureau (Philips, Medtronic) Advisory Board (Siemens) ARNOLD SCHWARZENEGGER SAYS: IT S NOT A TUMOR! KID: What s the matter? ARNOLD: I have a headache KID: It might be a tumor. ARNOLD: It s not a tumor. Not a tumor. At all! NYU Division of Cardiology 4 2
3 CASE PRESENTATION 80-year-old man presents with severe headache and nonexertional chest pain in the setting of severe hypertension (BP 210/90 mm Hg) PAST MEDICAL HISTORY Hypertension Coronary artery disease (s/p CABG & PCIs) WORKING DIAGNOSES Hypertensive urgency Acute coronary syndrome Abdominal aortic aneurysm Bradycardia (s/p Permanent pacemaker placement) Transthoracic echocardiogram was ordered NYU Leon H. Charney Division of Cardiology 5 TRANSTHORACIC ECHOCARDIOGRAM Hypertensive heart disease with paced rhythm. Otherwise, no wall motion abnormalities; no valvular disease. NYU Leon H. Charney Division of Cardiology 6 3
4 CLINICAL COURSE Headache resolved after normalization of blood pressure He was ruled out for acute coronary syndrome YET Severe nonexertional chest pain continued NYU Leon H. Charney Division of Cardiology 7 MAYBE IT S AORTIC DISSECTION Let s order a chest CT NYU Leon H. Charney Division of Cardiology 8 4
5 CHEST CT The good news is there is no aortic dissection but, there is something very concerning on noncontrast portion of CT. NYU Leon H. Charney Division of Cardiology 9 NONCONTRAST CHEST CT Crescentic hyperdense lesion involving distal arch & descending aorta NYU Leon H. Charney Division of Cardiology 10 5
6 CT DIAGNOSIS Acute intramural hematoma (IMH) of distal aortic arch and descending thoracic aorta NYU Leon H. Charney Division of Cardiology 11 First described on autopsy in 1760 of king George II of England CLASSIC AORTIC DISSECTION INTRAMURAL HEMATOMA (IMH) First described in 1920 in Germany on autopsy. ACUTE AORTIC SYNDROME King George II of England ( ) PENETRATING ATHEROSCLEROTIC ULCER (PAU) First described in 1986 on aortography & CT at Mayo Clinic. NYU Leon H. Charney Division of Cardiology 12 6
7 INTRAMURAL HEMATOMA FIRST DESCRIPTION Krukenberg E. Beiträge zur Frage des Aneurysma dissecans [Contributions to the question of dissecting aneurysm]. Beitr Pathol Anat Allg Pathol. 1920;67: Krukenberg correctly deduced that IMH results from rupture of vasa vasorum. NYU Leon H. Charney Division of Cardiology 13 INTRAMURAL HEMATOMA FIRST DESCRIPTION Although he was first to describe intramural hematoma (IMH) of the aorta. he is actually better known for describing Krukenberg tumors transperitoneal ovarian metastases from stomach and colon cancers. ERNST KRUKENBERG ( ) German pathologist NYU Leon H. Charney Division of Cardiology 14 7
8 BACK TO OUR PATIENT Six weeks later, severe chest pain recurred and repeat chest CT was ordered NYU Leon H. Charney Division of Cardiology 15 CHEST CT #2 6 WEEKS AFTER INITIAL CT Increase in the thickness of IMH with appearance of contrast inside IMH. NYU Leon H. Charney Division of Cardiology 16 8
9 CHEST CT #2 6 WEEKS AFTER INITIAL CT Increase in the thickness of IMH with appearance of contrast inside IMH. NYU Leon H. Charney Division of Cardiology 17 CHEST CT #3 Done 1 day after chest CT #2 NYU Leon H. Charney Division of Cardiology 18 9
10 CHEST CT #3 ONE DAY AFTER CT #2 Conversion of IMH into aortic dissection NYU Leon H. Charney Division of Cardiology 19 CHEST CT #3 ONE DAY AFTER CT #2 Conversion of IMH into aortic dissection NYU Leon H. Charney Division of Cardiology 20 10
11 CHEST CT #3 ONE DAY AFTER CT #2 Conversion of IMH into aortic dissection NYU Leon H. Charney Division of Cardiology 21 TEACHING POINTS Intramural hematoma (IMH) was first described by Ernst Krukenberg of Germany in 1920 IMH is likely the result of vasa vasorum rupture in the aortic media IMH is visualized as crescentic thickening of the aortic wall IMH may be a precursor to aortic dissection 22 NYU Leon H. Charney Division of Cardiology 11
12 INTRAMURAL HEMATOMA ONE OF CAUSES OF ACUTE AORTIC SYNDROME The term ACUTE AORTIC SYNDROME was first proposed in 2001 by Vilacosta & San Román of Spain ISIDRE VILACOSTA Spanish Physician JOSÉ ALBERTO SAN ROMÁN Spanish Physician NYU Leon H. Charney Division of Cardiology 23 ACUTE AORTIC SYNDROME INTRAMURAL HEMATOMA Crescentic thickening of the aortic arch wall NYU Leon H. Charney Division of Cardiology 24 12
13 ACUTE AORTIC SYNDROME PENETRATING ATHEROSCLEROTICULCER (PAU) * PAU = Erosion of atherosclerotic plaque into the media Note the intramural hematoma underneath the PAU NYU Leon H. Charney Division of Cardiology 25 ACUTE AORTIC SYNDROME PAU LEADING TO PSEUDOANEURYSM PAU >> Pseudoaneurysm (Focal aortic rupture) NYU Leon H. Charney Division of Cardiology 26 13
14 ACUTE AORTIC SYNDROME ON TEE CLASSIC AORTIC DISSECTION True True Flap Flap False False Noncontrast TEE TEE with Microbubble Contrast NYU Leon H. Charney Division of Cardiology 27 Michael Ellis DeBakey (originally دبغي : Dabaghi) Lebanese-American surgeon J. Thorac. Cardiovasc. Surg. 1965;49: Ann Thorac Surg Sep;10(3): Stanford Type A & B classification is now used for IMH, PAU & dissection. NYU Leon H. Charney Division of Cardiology 28 14
15 FURTHER READING Chapter 159: Acute aortic syndrome Pages ASE's Comprehensive Echocardiography 2nd Edition Elsevier (April 29, 2015) NYU Leon H. Charney Division of Cardiology 29 Thank You! New York University Langone Medical Center 15
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