Aortic Systemic Disease Classification 65 10

Aortic & Systemic Disease

Classification 65% 10% 20% 5%

Symptoms v Pain l 96% of patients l Sudden severe onset l Tearing, ripping, sharp, stabbing l Migrating pain (17%) l Location • Anterior only: ascending aorta in 90% • Interscapular only: descending thoracic aorta in 90% • Neck, throat, jaw, face: ascending aorta • Back, abdomen, lower extremities: descending aorta l Pleuritic pain • Hemopericardium

Laboratory Findings v Chest X-ray l Widening of the aortic silhouette • 81 – 90% l Calcium sign • Intimal calcification from the outer aortic border > 1 cm • Suggestive l Pleural effusion • Left side l Normal chest • Up to 12% X-ray

Calcium Sign

Diagnostic Techniques v Contrast-enhanced CT l Diagnostic clues • 2 distinct aortic lumen and intimal flap • 3 -dimensional display by spiral CT – Aorta and branches

Diagnostic Techniques v Contrast-enhanced CT l Advantages • • • Noninvasive High sensitivity and specificity (96 -100%) Readily available Thrombus in the false lumen Pericardial effusion Branch vessel compromise l Disadvantages • Contrast materials

Diagnostic Techniques v Transthoracic echocardiography (TTE)

Diagnostic Techniques v Echocardiography l Advantages • Readily available • Noninvasive • Quick to perform at the bedside l Transthoracic (TTE) echocardiography • Sensitivity: 59 – 85% • Specificity: 63 – 96% • Limited by image quality

Diagnostic Techniques v Transesophageal echocardiography (TEE) l Advantages • • • High imaging quality with high frequency ultrasound Not require contrast materials Aortic regurgitation Pericardial effusion High sensitivity and specificity – Sensitivity: 98 – 99% – Specificity: 94 – 97% l Disadvantages • Not visualize distal ascending aorta and proximal arch

Diagnostic Techniques v Transesophageal Echocardiography (TEE)

Immediate Medical Management v Blood pressure reduction l Goal • Systolic BP: 100 – 120 mm. Hg • Heart rate: 60 – 80 / m • Reducing d. P/dt (beta blocker) l Pain control • Morphine

Immediate Medical Management v Blood pressure reduction l Anticipating aorta surgery • Sodium nitroprusside • Esmolol l Labetalol • Alpha and beta blocking l Contraindicated to beta blocker • Severe sinus bradycardia, AV block, bronchospasm, congestive heart failure • Diltiazem, verapamil • ACE inhibitors

Definitive Therapy v Surgery l Acute proximal dissection l Complicated acute distal dissection • • Progression with vital organ compromise Rupture or impending rupture (saccular aneurysm) Retrograde extension into the ascending aorta Dissection in Marfan syndrome v Medical l Uncomplicated acute distal dissection l Stable isolated arch dissection l Stable chronic dissection

Aortic Intramural Hematoma v Pathology l Hemorrhage contained within the medial layer l Without an evident intimal tear v Pathogenesis l Rupture of the vasa vasorum l Small or micro tears in the intima

Aortic Intramural Hematoma v Computed tomography

Aortic Intramural Hematoma v TEE

Abdominal Aortic Aneurysm

Behcet’s Disease

Behcet’s Disease TEE

Takayasu’s Arteritis

Takayasu’s Arteritis TEE

Severe Aortic Atherosclerosis

End-Stage Renal Disease l LV hypertrophy l Pericardial l Early effusion stage: diastolic dysfunction Late stage: systolic dysfunction

End-Stage Renal Disease

Cardiac Amyloidosis l LV hypertrophy l Atrial enlargement l Sparkling texture of myocardium l Restrictive physiology l Pericardial effusion l Valvular thickening & regurgitation l Low QRS voltage

Cardiac Amyloidosis

Carcinoid Syndrome l Common in hepatic involvement l Caused by secretion of serotonin, bradykinin l Fibrosis & thickening of TV & PV stenosis or regurgitation l Lt side valve: 10%

Carcinoid Syndrome

Cardiac Sarcoidosis l Cardiac involvement: 20% l Cardiac fibrosis & RWMA • Basal & lateral LV • Wall thinning & aneurysm formation • restrictive morphology

Cardiac Sarcoidosis