Aortic valve disease. Acknowledgement for slides. Heart Valves 4/28/2018. Adopted from

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1 Aortic valve disease Senthil Kumar, MD th Annual Coming Together in Advanced Practice Conference Acknowledgement for slides Adopted from Dr. Anand Chockalingam Dr. Ajit Tharakan Heart Valves 1

2 Etiology Pathophysiology Physical Exam Natural History Testing Treatment 2

3 Valvular Stenosis the valve opening narrows the valve leaflets may become fused or thickened that the valve cannot open freely obstructs the normal flow of blood EFFECTS: the chamber behind the stenotic valve is subject to greater stress must generate more pressure or work hard to force blood through the narrowed opening initially, the compensates for the additional workload by gradual hypertrophy and dilation of the myocardium heart failure Valvular Insufficiency or Regurgitation scarring and retraction of valve leaflets or weakening of supporting structures incomplete closure of the valve results in leakage or backflow of blood from the previous chamber EFFECTS: causes the to pump the same blood twice (as the blood comes back into the chamber) the dilates to accommodate more blood (the usual blood it needs to pump + regurgitated blood) ventricular dilation and hypertrophy eventually leads to heart failure 33 year old patient has soft murmur noted in the aortic area in early diastole only He is completely asymptomatic Does he need an echo? 3

4 33 year old patient has soft murmur noted in the aortic area in early diastole only He is completely asymptomatic Does he need an echo? Grade 3 or greater for systolic murmur Any diastolic murmur Grading of murmurs In addition to classifying murmurs by the location where they are heard, murmurs are classified by their intensity. Systolic murmurs are classified on a scale of 1 to 6. Grade 1 murmurs are barely audible. Grade 6 murmurs are very loud and can be heard with the stethoscope barely on the chest. Diastolic murmurs are graded on a scale of 1 to 4 ranging again from barely audible to very loud. Numerical gradation of murmur intensity was introduced by Samuel A. Levine, MD, from Boston, MA, in This murmur gradation is used until this day. Murmur Grade Description 1 Faint murmur that can only be heard after a few seconds have elapsed. 2 Faint murmur that is heard immediately. 3 Moderate murmur intensity. 4 Loud murmur, a thrill may be present* 5 6 Loud murmur that can be heard if only the edge of the stethoscope is in contact with the skin, a thrill is present* Loud murmur that can be heard with the chest piece just removed from and not touching the skin, a thrill is present* * A thrill is a palpable vibration over the site of a murmur 4

5 Aortic Stenosis (AS) Aortic Stenosis Overview: Normal Aortic Valve Area: 3 4 cm 2 Symptoms: Occur when valve area is 1/4 th of normal area. Types: Supravalvular Subvalvular Valvular 5

6 Etiology of Aortic Stenosis Congenital Rheumatic Degenerative/Calcific Patients under 70: >50% have a congenital cause Patients over 70: 50% due to degenerative medical-look.com 6

7 Pathophysiology of Aortic Stenosis A pressure gradient develops between the left ventricle and the aorta. (increased afterload) LV function initially maintained by compensatory pressure hypertrophy When compensatory mechanisms exhausted, LV function declines. 7

8 Aortic Stenosis PATHOPHYSIOLOGY Stiffening/Narrowing of Aortic Valve Left ventricular hypertrophy Compression of coronary arteries CO Incomplete emptying of left atrium Pulmonary congestion O2 supply Myocardial O2 needs Right sided heart failure Myocardial ischemia (chest pain) 80 year old man with severe asymptomatic aortic stenosis what symptoms should he watch out for? 8

9 CLINICAL MANIFESTATIONS exertional dyspnea due to CO and pulmonary congestion chest pain (angina) usually occurs during exercise due to inability of the heart to increase coronary blood flow to cardiac muscle exertional syncope, periods of confusion due to CO fatigue & weakness, orthopnea, PND, pulmonary edema (severe cases) signs of right sided heart failure late symptoms Presentation of Aortic Stenosis Syncope: (exertional) Angina: (increased myocardial oxygen demand; demand/supply mismatch) Dyspnea: on exertion due to heart failure (systolic and diastolic) Sudden death Sudden death in AS uncommon event in asymptomatic patients <1% /year 9

10 Physical Findings in Aortic Stenosis Pulse Slow rising carotid pulse (pulsus tardus) & decreased pulse amplitude (pulsus parvus) Heart sounds soft and split second heart sound, S4 gallop due to LVH. Murmur Harsh, rough, mid systolic murmur Systolic ejection murmur diamond shaped cresendo decrescendo character. This peaks later as the severity of the stenosis increases. Loudness is not always correlated to severity Aortic stenosis Play initial 3 minutes of audio from Heartsongs 3 by ACC Mild AS to Severe AS: 8% in 10 years 22% in 22 years 38% in 25 years Natural History The onset of symptoms is a poor prognostic indicator. 10

11 Evaluation of AS Echocardiography is the most valuable test for diagnosis, quantification and follow up of patients with AS. Two measurements obtained are: a) Left ventricular size and function: LV Hypertrophy, Dilatation, and Ejection Fraction (EF) b) Doppler derived gradient and Aortic valve area (AVA) Evaluation of AS Cardiac catheterization: Should only be done for a direct measurement if symptom severity and echo severity don t match OR prior to replacement when replacement is planned. 11

12 Management of AS General IE prophylaxis in dental procedures with a prosthetic aortic valve or history of endocarditis. Medical limited role since AS is a mechanical problem. Vasodilators are relatively contraindicated in severe AS avoid strenous activity Na+ restriction, cautious use of diuretics Echo Surveillance Mild: Every 3 5 years Moderate: Every 1 2 years Severe: Every 6 months to 1 year Plasma levels of BNP (A), NtBNP (B), and NtANP (C) & NYHA class in severe aortic stenosis Bergler Klein, J. et al. Circulation 2004;109: Copyright 2004 American Heart Association 12

13 Kaplan Meier symptom free survival for patients with baseline BNP <130 pg/ml (n=25) vs >=130 pg/ml (n=18) (A), patients with baseline NtBNP <80 pmol/l (n=31) vs >=80 pmol/l (n=12) (B), and patients with NtANP <5000 fmol/ml (n=31) vs >=5000 fmol/ml (n=12) fmol/ml (C) Bergler Klein, J. et al. Circulation 2004;109: Copyright 2004 American Heart Association INTERVENTIONS Aortic Balloon Valvuloplasty: Congenital aortic stenosis. In elderly as bridge to surgery as benefits short lived. Surgical Replacement: Definitive treatment TAVR: exciting new treatment Aortic valve replacement (surgical or percutaneous) if not done poor prognosis 88 year old patient with CHF and severe AS not a candidate for AVR due to ESRD and COPD is he a candidate for TAVR? 13

14 88 year old patient with CHF and severe AS not a candidate for AVR due to ESRD and COPD is he a candidate for TAVR? Initially approved for high risk patients and now also for intermediate risk patients. Not yet for low risk patients. Percutaneous Transcatheter Implantation of an Aortic Valve Prosthesis for Calcific Aortic Stenosis: First Human Case Description Alain Cribier, MD, et al Circulation. 2002;106:3006 TAVR use has grown exponentially since. The Hybrid Operating Room- A special operating room that combines a catheter laboratory with the preconditions necessary to perform surgery an sterile valve preparation before implantation, anesthesiologic equipment, appropriate lighting, and the heart-lung machine. Pasic, M. et al. J Am Coll Cardiol 2010;56: Copyright 2010 American College of Cardiology Foundation. Restrictions may apply. 14

15 Valve Deployment Pasic, M. et al. J Am Coll Cardiol 2010;56: Copyright 2010 American College of Cardiology Foundation. Restrictions may apply. Intraoperative Transesophageal Echocardiography Data Pasic, M. et al. J Am Coll Cardiol 2010;56: Copyright 2010 American College of Cardiology Foundation. Restrictions may apply. 15

16 Simplified Indications for Surgery in Aortic Stenosis Any SYMPTOMATIC patient with severe AS (includes symptoms with exercise) Any patient with decreasing EF Any patient undergoing open heart surgery (eg. CABG) with moderate or severe AS CABG coronary artery bypass grafting AVR mortality rate 0% to 4.0% for isolated AVR 5.5% to 6.8% for AVR plus CABG 8.8% for AVR > 65 years Active Cardiac Conditions for Which the Patient Should Undergo Evaluation and Treatment Before Noncardiac Surgery (Class 1, LOE: B) Condition Examples Unstable coronary syndromes Decompensated HF (NYHA functional class IV; worsening or new-onset HF) Significant arrhythmias Unstable or severe angina* (CCS class III or IV) Recent MI High-grade atrioventricular block Mobitz II atrioventricular block Third-degree atrioventricular heart block Symptomatic ventricular arrhythmias Supraventricular arrhythmias (including atrial fibrillation) with uncontrolled ventricular rate (HR > 100 bpm at rest) Symptomatic bradycardia Newly recognized ventricular tachycardia Severe valvular disease Severe aortic stenosis (mean pressure gradient > 40 mm Hg, aortic valve area < 1.0 cm 2, or symptomatic) Symptomatic mitral stenosis (progressive dyspnea on exertion, exertional presyncope, or HF) or MVA<1.5 cm 2 CCS indicates Canadian Cardiovascular Society; HF, heart failure; HR, heart rate; MI, myocardial infarction; NYHA, New York Heart Association. *According to Campeau. 10 May include stable angina in patients who are unusually sedentary. The ACC National Database Library defines recent MI as more than 7 days but within 30 days) 16

17 CLASS IIb Exercise testing in asymptomatic patients with AS may be considered to elicit exercise induced symptoms and abnormal blood pressure responses. (Level of Evidence: B) CLASS III Exercise testing should not be performed in symptomatic patients with AS. (Level of Evidence: B) Management Strategy for Patients With Severe Aortic Stenosis Bonow, R. O. et al. J Am Coll Cardiol 2008;52:e1-e142 Copyright 2008 American College of Cardiology Foundation. Restrictions may apply. Summary Disease of aging Look for the signs on physical exam Echocardiogram to assess severity Asymptomatic: Medical management and surveillance Symptomatic: Aortic valve replacement (even in elderly and CHF) 17

18 Aortic Regurgitation (AR) Aortic Regurgitation etiology Infections (eg. bacterial endocarditis) Congenital anomaly (eg. bicuspid aortic valve) Aortic dissection Aortic aneurysm Connective tissue disease (eg. Marfan syndrome) Severe hypertension Rheumatic fever 18

19 TEE Dissection flap Aortic regurgitation Flow in true and false lumen Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D TEE long axis view of the ascending aorta visualizes a pear shaped aneurysm of the aortic root with severe AR in Marfan syndrome Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D Aortic Regurgitation 19

20 PATHOPHYSIOLOGY Incomplete closure of the aortic valve Backflow of blood to Left ventricle Left ventricular hypertrophy & dilation Left atrial pressure Left sided heart failure Left atrial enlargement Pulmonary pressure Right sided heart failure Right ventricular pressure Clinical Manifestations pt. may remain asymptomatic for years heart compensates by hypertrophy & dilatation heightened awareness of the heart beat esp. when pt. lies on left lateral position palpitations due to tachycardia, PVC assoc. w/ left ventricular dilation PRESENTATION OF AORTIC REGURGITATION chest pain myocardial ischemia left heart failure exertional dyspnea, fatigue, orthopnea, PND right heart failure peripheral edema PND paroxysmal nocturnal dyspnea 20

21 Physical exam Pulse Bounding pulse marked carotid artery pulsation Collapsing pulse radial artery apical impulse force and volume of contraction of the hypertrophied left ventricle 21

22 Heart sounds The first heart sound is diminished due to premature closure of the mitral valve leaflets. An aortic ejection click follows the first heart sound by about 75 milliseconds. S2 is normal. Murmur Soft, blowing diastolic murmur A high pitched decrescendo murmur occupying the first half of diastole can be heard starting immediately after the second heart sound. The murmur is best heard at Erb's Point (L 3 rd intercostal space) and can be accentuated by having the patient sitting up and leaning forward holding his breath after expiration. Turbulent blood flow from the aorta into the left ventricle creates a murmur during early diastole. May hear Systolic murmur due to increased flow across the valve even without stenosis. Aortic regurgitation Play initial 3 minutes of audio from Heartsongs 3 by ACC AR is never normal - Yoshida K, Yoshikawa J, Shakudo M, et al. Color Doppler evaluation of valvular regurgitation in normal subjects Circulation 1988;78:

23 Echo Surveillance Mild: Every 3 5 years Moderate: Every 1 2 years Severe: Every 6 months to 1 year Dilating LV: More frequently MANAGEMENT avoid physical exertion, competitive sports vasodilators, calcium channel blockers, ACE inhibitors or ARB Aortic valve replacement 23

24 Management Strategy for Patients With Chronic Severe Aortic Regurgitation Bonow, R. O. et al. J Am Coll Cardiol 2008;52:e1-e142 Copyright 2008 American College of Cardiology Foundation. Restrictions may apply. 72 year old man with symptomatic severe AR requires aortic valve replacement Metallic or bioprosthetic? 24

25 72 year old man with symptomatic severe AR requires aortic valve replacement Metallic or bioprosthetic? Age <65 years, metallic Age >65 years, bioprosthetic Structural valve deterioration of Biological Valves at 15 to 20 Years Based on Patient Age at Time of PHV Implantation Rahimtoola, S. H. J Am Coll Cardiol 2010;55: Copyright 2010 American College of Cardiology Foundation. Restrictions may apply. Comparison of Risks of Reoperation and Major Bleeding Between Mechanical and Bioprosthetic Heart Valves Based on Patient Age at Valve Implantation Rahimtoola, S. H. J Am Coll Cardiol 2010;55: Copyright 2010 American College of Cardiology Foundation. Restrictions may apply. 25

26 Valve Prosthesis 26

27 Cardiopulmonary Bypass Machine Bicuspid aortic valve The prevalence of congenital bicuspid aortic valves is 0.5% to 2% of the adult population (2% of men, <0.5% of women). This makes bicuspid valves one of the two most common forms of congenital heart disease recognized in adulthood, the other being atrial septal defects. Notch1 gene. Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D

28 Bicuspid aortic valve A bicuspid aortic valve is suggested by auscultation and can be confirmed by echocardiography in most cases. Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D Bicuspid aortic valve complications and associations Aortic stenosis, aortic insufficiency, or both Infectious endocarditis (potentially with aortic root abscesses) Ascending aorta or aortic root dilation, aneurysm, dissection, or rupture Coarctation and interruption of the aorta (LCC RCC type only) Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D. 2009; ESC

29 Aortic dissection The Great Masquerader Classification 29

30 Surgery vs Medical Rx The International Registry of Acute Aortic Dissection [IRAD]: new insights into an old disease. JAMA 2000 badaorta.com 30

31 Bicuspid aortopathy Some patients with bicuspid aortic valves present with aortic aneurysms, dissection, or rupture as their initial presentation. 7x as many aortic dissections are associated with bicuspid valves as are associated with Marfan syndrome. In all cases in which a bicuspid aortic valve is identified, the ascending aortic dimensions should be determined and coarctation excluded. Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D MRA Coarctation Aortic Diseases: Clinical Diagnostic Imaging Atlas, First Edition. Peterson, Mark D Coarctation 31

32 Figure 3 sign - Aortic Knob 32

33 Thank you! Questions? 33