Valvular Heart Disease and the Cardiac Exam 2009

Overview Clinical syndromes Overview of cardiac murmurs and maneuvers Left sided valvular lesions Aortic stenosis and sclerosis Mitral stenosis Rheumatic fever prophylaxis Acute and chronic aortic regurgitation Acute and chronic mitral regurgitation Right sided valvular lesions Tricuspid valve disease Prosthetic valves Endocarditis prophylaxis Questions

General Appearance Marfan Syndrome Acromegaly Turner Syndrome Tall, long extremities Associated with: aortic root dilitation, MV prolapse Acromegaly Large stature, coarse facial features, “spade” hands Associated with: Cardiac hypertrophy Turner Syndrome Web neck, hypertelorism, short stature Associated with: Aortic coarctation, pulmonary stenosis Pickwickian Syndrome Severe obesity, somnolence Associated with: Pulmonary hypertension Fredreich ataxia Lurching gait, hammertoe, pes cavus Associated with: hypertrophic cardiomyopathy Duchenne type muscular dystrophy Pseudohypertrophy of the calves Cardiomyopathy Ankylosing spondylitis Straight back syndrome, stiff (“poker”) spine Associated with: AI, CHB (rare) Lentigines (LEOPARD syndrome) Brown skin macules that do not increase with sunlight Associated with: HOCM, PS

“Spade” hands in acromegaly

General Appearance- 2 Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) Small capillary hemangiomas on the face or mouth Associated with: Pulmonary arteriovenous fistula Lupus Butterfly rash on face, Raynaud phenomenon- hands, Livedo reticularis Associated with: Verrucous endocarditis, Myocarditis, Pericarditis Pheochromocytoma Pale diaphoretic skin, neurofibromatosis- café-au-lait spots Associated with: Catecholamine-induced secondary dilated CM Sarcoidosis Cutaneous nodules, erythema nodosum Associated with: Secondary cardiomyopathy, heart block Tuberous Sclerosis Angiofibromas (face; adenoma sebaceum) Associated with: Rhabdomyoma Myxedema Coarse, dry skin, thinning of lateral eyebrows, hoarseness of voice Associated with: Pericardial effusion, LV dysfunction

Grading the Intensity of Cardiac Murmurs Grade 1 Murmur heard with stethoscope, but not at first Grade 2 Faint murmur heard with stethoscope on chest wall Grade 3 Murmur hears with stethoscope on chest wall, louder than grade 2 but without a thrill Grade 4 Murmur associated with a thrill Grade 5 Murmur heard with just the rim held against the chest Grade 6 Murmur heard with the stethoscope held away and in from the chest wall

Cardiac Murmurs Most mid systolic murmurs of grade 2/6 intensity or less are benign Associated with physiologic increases in blood velocity: Pregnancy Elderly In contrast, the following murmurs are usually pathologic: Systolic murmurs grade 3/6 or greater in intensity Continuous murmurs Any diastolic murmur

Maneuver Hemodynamic Effect Murmur Effect Normal respiration Transient ↑ in venous filling during inspiration ↑ right-sided murmurs Passive leg elevation ↑ venous return (transient ↑ in LV size and preload) ↑ right-sided murmurs, ↓murmur of HOCM and MVP Stand to squat ↑ right-sided murmurs, ↓murmur of HOCM and MVP Squat to stand ↓ venous return (transient ↓ in LV size and preload) ↑ murmur of HOCM, moves midsystolic click of MVP closer to S1 and ↑ MVP murmur, ↓ AS murmur Valsalva ↓ venous return (transient ↓ in LV size, preload, and relative systemic hypotension) ↑ murmur of HOCM, moves midsystolic click of MVP closer to S1, and ↓ murmur of MVP Isometric handgrip exercise ↑ afterload ↑ murmur of MR and VSD, ↓the murmur of HOCM, ↓AS murmur Inhaled amyl nitrate ↓ afterload ↓ murmur of MR and VSD, no change in AS murmur

Diagnostic Testing ECHOCARDIOGRAM Exercise testing To assess the clinical severity of valvular heart disease Those with inconsistent resting hemodynamics Equivocal history of symptoms Exercise testing in AS patients Should be ended promptly if: Cardiac symptoms provoked Decrease or minimal increase (<20 mmHg) in blood pressure Prior history of angina, congestive heart failure, or exertional syncope absolute contraindications to exercise testing Cardiac catheterization Usually not needed for primary evaluation

Aortic Stenosis Most common cause is calcific degeneration Active disease process with risk factors of male sex, smoking, HTN, DM, older age, hypercholesterolemia 2% of the general population have bicuspid aortic valves Symptomatic or severe AS occurs earlier (age 40-60 years) AS less commonly from rheumatic heart disease valvulitis Invariably MV involved first Associated AV involvement in <1/2 patients AV sclerosis Valve thickening without obstruction Present in >20% of people >65 years Associated with 50% increased risk of MI and CV death

Progression of Aortic Sclerosis Hemodynamic progression usually slow Average rate of increase in aortic jet velocity of 0.3 m/s per year Increase in mean transaortic gradient of 7 mmHg Decrease in AVA of 0.1 cm2 per year Severe AS Aortic jet velocity > 4 m/s Mean transvalvular pressure gradient > 50 mmHg AVA < 1.0 cm2

Pathophysiology of Aortic Stenosis Obstruction of LV outflow increases intracavitary systolic pressures and leads to LV pressure overload Initial compensatory mechanism is myocardial hypertrophy with preservation of systolic function Diastolic function impaired as a consequence of increased wall thickness and abnormal myocardial relaxation Increased wall stress and afterload causes eventual decrease in ejection fraction

Pseudostenosis Occurs in patients with impaired systolic function and aortic stenosis Unable to generate transvalvular gradient Careful diagnostic testing with dobutamine infusion protocols can aid in differentiating between true AS and pseudostenosis If the calculated AVA increases with augmentation of cardiac output, then pseudostenosis present If AVA does not increase with dobutamine, then obstruction fixed and true AS present

Clinical Presentation of Aortic Stenosis Cardinal symptoms: Angina Occurs in >50% of patients, not sensitive due to prevalence of CAD Syncope CHF Sudden cardiac death rare, <1% per year In earlier stages, AS presentation more subtle Dyspnea Decreased exercise tolerance Rarely, AS diagnosed in the setting of GI bleeding Heyde’s syndrome Bleeding caused by AVM Concurrent AS occurs at prevalence rate of 15-25% Associated with an acquired von Willebrand syndrome due to disruption of vW multimers through a diseased AV

Management of Aortic Stenosis Prognosis in asymptomatic disease excellent Conservative approach with monitoring for symptoms recommended When severe stenosis present- 38% of asymptomatic patients develop symptoms within 2 years 79% are symptomatic within 3 years Once symptoms occur, AVR needed LV dysfunction and severe AS have increased perioperative mortality with AVR But outcomes still favorable with surgery Nitroprusside may transiently improve cardiac function as a bridge to valve replacement Does not supplant AVR in symptomatic patients

Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51

Aortic Valve Replacement Prophylatic AVR in asymptomatic patients not routinely performed due to surgical risks Thromboembolism, bleeding associated with anticoagulation, prosthetic valve dysfunction, and endocarditis Occurs at a rate of 2-3% annually Only should be considered: If other cardiac surgery (such as CABG) planned Severe LVH or systolic dysfunction Women contemplating pregnancy Patients remote from health care Surgical valve replacement with operative morbidity and mortality of 10% Percutaneous balloon aortic valvotomy rarely used

Mitral Stenosis Usually associated with history of rheumatic fever >40% of cases of RHD result in mitral stenosis Women affected more than men (2:1) Presentation 20-40 years after the initial episode of rheumatic fever If infected at a young age, latent period is a few years

Clinical Presentation of Mitral Stenosis Significant MS leads to ↑LA pressure and pulm HTN Symptoms include dyspnea with ↑ cardiac demand Exercise Pregnancy Survival excellent with asymptomatic or minimally symptomatic patients >80% survival at 10 years Survival in symptomatic patients much worse 10 year survival drops to 15% or lower (if pulm HTN present) Findings consistent with severe MS: Transvalvular diastolic pressure gradient >10 mmHg MVA <1.0 cm2 Severe pulmonary hypertension (>60 mmHg)

Management of Mitral Stenosis Atrial fibrillation Prevalence >30% in symptomatic patients and associated with poorer long term outcome Warfarin indicated: In patients with AF and MS Patients without history of AF but with MS and embolic CVA In patients with prior history of AF who have mitral valve surgery, decreased postoperative AF observed if MAZE performed concominantly

Mitral Valve Repair Percutaneous valvotomy Therapeutic intervention of choice if: LAA thrombus excluded MR less than moderate Valvular characteristics favorable Pliable leaflets, minimal commisural fusion, minimal valvular or subvalvular calcification Pulmonary HTN not contraindication to valvotomy Major complications include: severe MR (1-8%), systemic embolization (1-3%), and tamponade (1-2%) Periprocedural mortality- 1% Surgical commissurotomy or MVR can be performed in unfavorable anatomy

Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51

Rheumatic Fever Prophylaxis Primary prophylaxis If living in an endemic area, with pharyngitis and a +test for group A strep or positive throat culture Given once, may be repeated as needed: PCN G 1.2 million U IM or PCN V 500 mg TID x 10d Azithromycin 500 mg on day 1, 250 mg daily for 4d Secondary prophylaxis PCN G 1.2 million units IM every 4 weeks or PCN V 250 mg PO BID or erythromycin 250 mg BID RHD without carditis- At least 5 years or until >21 y of age RHD with carditis, no valvular HD- At least 10 y or well into adulthood RHD with carditis and valvular HD- At least 10 years from last episode or until patient is older than 40 years

Acute Aortic Regurgitation Causes of acute aortic regurgitation: Aortic dissection Valve distruction from endocarditis Traumatic rupture Classic physical exam findings may be absent in the acute presentation Diastolic murmur may not be present due to sudden increase of LVEDP TTE, along with TEE, cath, CT or MRI may be used for diagnosis Surgical AV repair or replacement should be performed emergently Afterload reducing medications and inotropes may help to acutely stabilize the patient IABP contraindicated

Acute Mitral Regurgitation Most often occurs in: Chordae tendineae rupture due to myxomatous valve disease or endocarditis Myocardial infarction with papillary muscle dysfunction or rupture Symptoms almost always occur Dyspnea and pulmonary edema Systolic function may occur normal or hyperdynamic IABP or afterload reducing drugs to temporize Surgical intervention for treatment

Chronic Valvular Regurgitation Cardiac chamber size and function have time to compensate for dysfunction May allow patients to remain asymptomatic for a long time Both preload and afterload increases Once increase in cardiac output insufficient→ systolic function declines → pulmonary HTN may develop and symptoms develop LV enlargement and progressive systolic dysfunction are associated with significant morbidity and mortality Serial echocardiography and evaluation by a cardiologist is indicated

Chronic Aortic Regurgitation Occurs most often in bicuspid AV Other causes include ascending aortic aneurysm and Marfan’s Disease Risk factors for poorer outcome: Age Cardiac symptoms Atrial fibrillation LV enlargement Lower LVEF Asymptomatic patients with normal LV size and function do not require prophylatic surgery Surgery should be considered if: LVESD > 55 mm Ejection fraction <60% Symptoms develop Oral afterload reduction (nifedipine or ACE-I) may slow rate of LV dilation

Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51

Chronic Mitral Regurgitation Often caused by myxomatous disease or MVP Myxomatous mitral valve disease with progressive MR associated with poor long term outcome Higher risk of arrhythmias and sudden cardiac death Mitral valve prolapse occurs in ~2% of the general population Consists of the buckling of the mid portion of the valve leaflets into the LA Usually asymptomatic, but may be associated with palpitations or chest discomfort Prognosis usually benign Antibiotic prophylaxis now not indicated

Chronic Mitral Regurgitation Other causes include secondary or acquired leaflet dysfunction Endocarditis Rheumatic heart disease Annular tethering from LV dilation Tethering of the chordal apparatus from ischemic heart disease Rare cause: Fenfluramine and phentermine, also associated with AI Compensatory increase in LV chamber size initially allows for increase in total stroke volume and restoration or total forward cardiac output

Treatment of Chronic Mitral Regurgitation Mitral valve repair preferred over mitral valve replacement Avoids risk of anticoagulation Preservation of subvalvular apparatus Better postoperative LV function and long term survival When MR occurs in volume overloaded states, afterload reduction can be beneficial Dilated CM CAD Revascularization may improve dysfunction of the papillary muscle Biventricular pacing may improve LV geometry

Timing of Intervention for Left-Sided Valvular Conditions Aortic Stenosis Mitral Stenosis Chronic Severe AR Chronic Severe MR Intervention: AVR Percutaneous valvotomy if anatomy amenable and <moderate MR, and no LAA clot. Otherwise, open commissurotomy or MVR Surgical AVR with aortic root replacement if needed Surgical mitral valve repair if anatomy amenable. Otherwise, MVR IF: Patient is symptomatic (NYHA class II or greater, angina due to AS, or syncope) OR Patient has symptomatic severe AS and needs other cardiothoracic surgery (i.e. CABG) Patient has moderate or more severe MS (MVA < 1.5 cm2) Pulmonary hypertrension at rest (PAP > 60 mmHg) Abnormal hemodynamic response to exercise: PAP > 60 mmHg Mean gradient > 15 mmHg Patient is symptomatic (NYHA class II or greater) EF <60% ESD > 55 mm Abnormal hemodynamic response to exercise PAP increase by 25 mmHg ESD > 45 mm Pulmonary hypertension or atrial fibrillation OTHERWISE Depending on the severity of AS, at least annual clinical evaluation with TTE to monitor for symptom onset Clinical evaluation at least annually, depending on the severity of the mitral stenosis Repeat TTE at least yearly, repeat clinical evaluation at least biannually depending on the severity of the LV dilitiation Repeat TTE yearly, repeat clinical evaluation biannually

Tricuspid Valve Disease Tricuspid stenosis is rare Associated with rheumatic heart disease TR usually occurs secondary to: Pulmonary hypertension RV chamber enlargement with annular dilatation Endocarditis (associated with IV drug use) Injury following pacer lead placement Other secondary causes: carcinoid, radiation therapy, anorectic drug use, and trauma Primary causes: Marfan’s syndrome and congenital disorders such as Ebstein’s anomaly and AV canal malformation Echo is diagnostic in most cases

Tricuspid Regurgitation Severe tricuspid regurgitation is difficult to treat and carries a poor overall clinical outcome Symptoms are manifestations of systemic venous congestion Ascites Pedal edema Surgical intervention usually considered if other cardiac surgery planned Surgical options include valvular annuloplasty or replacement If replacement planned, bioprosthetic valve preferred

Prosthetic Valves- Mechanical Three types: Ball-cage valve Single tilting disk valve Bileaflet valve Durable but require life long anticoagulation For operative procedures, warfarin typically is discontinued for 48-72 hours and restarted postop as soon as possible, except for: Mechanical mitral prosthesis Atrial fibrillation Prior thromboembolic events

Ball-cage valve Single tilting disk valve Bileaflet valve

Prosthetic Valves- Biological Biological Valves Composed of autologous or xenograft biological material mounted on stents and a sewing ring Warfarin therapy not required due to lower thromboembolic potential Valve durability less when compared to mechanical valves Newer stentless valves with increased longevity

Anticoagulation Guidelines for Mechanical Valves Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51

Prosthetic Valve Complications Common complications include: Structural valve deterioration Valve thrombosis Embolism Bleeding Endocarditis Endocarditis prophylaxis required for patients with all types of prosthetic valves Suspect valve dehiscence or dysfunction in: Acute CHF in the immediate postop period New cardiac symptoms Embolic phenomena Hemolytic anemia New murmurs TEE is the diagnostic procedure of choice Postop TTE should be done 2-3 months after surgery

Valve Thrombosis Incidence with mechanical prosthesis of 2-4 % per year Suspect in patients with new murmur, change in cardiopulmonary symptoms, or an embolic event Diagnosis based on clinical presentation, TTE/TEE, and fluroscopy In small thrombus, treatment with heparin may be adequate Optimal treatment for left sided thrombosis is emergency surgery Consider thrombolytic therapy for right sided thrombosis or if surgery cannot be performed with left sided disease

Endocarditis Prophylaxis 2007 AHA Prevention of Infective Endocarditis Guidelines