cardiac valves sylvia dennis fellow pathology/anatomy nycom friday, october 07, 2005 1

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Cardiac Valves Sylvia Dennis Fellow Pathology/Anatomy NYCOM Friday, October 07, 2005 1

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Cardiac Valves

Sylvia Dennis

Fellow Pathology/Anatomy

NYCOM

Friday, October 07, 2005

1

Objectives

Concepts

To understand Cardiac Valve Gross & Micro Anatomy

Review of normal valve function

To learn the Pathophysiology of Stenosis and Regurgitation

Heart Murmurs/Physical exam findings

Cases

2

Concepts

StenosisStenosis: Failure of the valve to open & forward flow is impeded

InsufficiencyInsufficiency (incompetence, regurgitation): Failure of the valve to close & reverse flow of blood

Damaged valves:Damaged valves: Nidus for infection & thrombosis

Dilated cardiac chambersDilated cardiac chambers: stasis of blood, conduction defects

Normal blood flow is laminar --- turbulence causes murmurs

Rate of dysfunctional development determines symptoms and severityChronic vs Acute

3

Murmur Rules

Intra-thoracic Pressure & PreloadIntra-thoracic Pressure & Preload

Right sided Murmurs with InhalationLeft sided Murmurs with ExhalationStanding/Valsalva: all Murmurs except HCM and MVPSquatting: all Murmurs except HCM and MVP

4

Cardiac Valves

Four valvesTricuspid & Mitral (between atria and ventricles) Aortic & Pulmonic (semilunar)

• Derived from truncus arteriosus

Maintain unidirectional flowFibrous skeleton supports valves

Four fibrous rings (annuli fibrosi) – each encircling a valve , two trigonesElectrical insulator (R trigone provides passage of AV bundle)Provides attachments for the leaflets and cusps

5

Cardiac Valves

Semilunar ValvesSemilunar ValvesPulmonic valve on the right

Aortic valve on the left

Closure produces S2 (A2/P2)

Open in systole

Function depends on integrity of fibrous annulusDilatation of aortic root distorts annulus

Competency depends on ability to stretch/moldCusp/leaflet surface area increases up to 50% during diastole

Overlap of cusps at the lunula

Nodules of Arantii facilitate closure

6

AV valve AnatomyFeatures:Features:Cordae TendineaePapillary musclesBicuspid Mitral valve (L)

with 2 cuspsTricuspid valve ® with 3

cuspsFibrous annuli anchoring

the cusps

7

Clinical Evaluation of Valvular Anatomy

DopplerDoppler

EchocardiographyEchocardiographyEvaluation of LV function

Detection of abnormal wall motion

Measurement of LV volume, EJF and CO

EchocardiogramEchocardiogramTrans-Thoracic

Noninvasive 2 DSuperior spacial resolution

Trans – EsophagealTransducer placed in the esophagus Excellent evaluation of posterior cardiac chambers and aorta2D

8

Valve - Histology

Lined with endothelium (simple squamous epithelium)

Three major layers Ventricularis: rich in elastin – contracts during systole

Spongiosa: loose CT – shock absorber

Fibrosa: dense collagenous CT – provides strength, continuous with supporting structures

9

Cardiac Cycle

Cardiac Cycle & Heart Cardiac Cycle & Heart SoundsSounds

S1 (closure of AV valves) at the same time as QRS

Systole between S1& S2

S2 (closure of Semilunar valves) just after T wave

Diastole between S2 & S1

S3 & S4 are diastolic sounds

10

Cardiac Physiology

                                           

                          

Review on your own!

Jugular Venous PulseJugular Venous Pulse

11

Classification of Murmurs

Systolic Systolic Stenosis of Semilunar valves (systolic ejection Murmur)

Regurgitation of AV-valves

MVP (click/murmur)

VSD (children) • Left to Right• Right to Left with

Eisenmenger Syndrome

DiastolicDiastolicStenosis of AV-ValvesRegurgitation of Semilunar valves

• S3 early diastole• S4 late diastole

12

Murmurs

Sounds due to turbulent blood flowSystolic vs DiastolicFeatures

• Radiation • Maneuvers (Inhalation/Exhalation, valsalva & squatting, positioning)

Mechanisms• Structural valve disease• Anemia • Innocent (children)

Types• Continuous (venous hum, PDA)• Stenosis vs Regurgitation

13

Auscultation

AS: Right 2AS: Right 2ndnd ICS ICSSystolic murmur/crescendo-decrescendo

Radiation to the neck

Carotid Pulse: pulsus parvus et tardus

Decreases in Intensity w/ valsalva

Clinical : exertional Syncope,

Angina and Dyspnea

LVH --- sustained apical impulse (late)

A2 – soft, absent, or late

A2 in aortic sclerosis: normal intensity and timing

MR: 5MR: 5thth ICS/ Left ICS/ Left midclavicular line

Holosystolic murmur

Radiation to the left axilla

Varies little with respiration ( with valsalva)

Acute MR:

sudden volume overload

no time for compensation

Rapid increase in pulmonary venous Pressure acute pulmonary edema

Murmur: loud/blowing apical systolic

15

Regurgitation vs. Stenosis

Acute vs. ChronicLeaky valve/ backflow of bloodCompensatory Mechanism: eccentric hypertrophy

Intrinsic valvular pathologyPost-inflammatory scarringEndocarditisMyxomatous changes (MVP)

Functional regurgitationVentricular Dilation Aortic/Pulmonic Root dilation

Chronic process

Stiff valve/increase in afterload

Compensatory mechanism: concentric hypertrophy

MCC - primary valve pathology

Calcific stenosis *Dystrophic calcification

Congenital

Post-inflammatory scarring

16

HypertrophyEccentric Concentric

Volume overload

Increase in myocardial cell length and cross sectional area

Dilation with increased ventricular diameter

Dilation of ventricle leads to stretching of papillary muscles

Pressure overload Sarcomere deposition parallel to long axis of cell Increased wall thickness (increase in wall stress)

Increase in metabolic requirements

Structural/molecular alterations (decreased capillary to myocyte ratio

Adaptive changes

17

Dystrophic Calcification

Normal serum Calcium & Metabolism

Micro: fine white granule/clumps with gritty consistency

Pathogenesis: injury or microtrauma leading to early inflammatory lesion resulting in final deposition of calcium phosphate crystals

18

Case 1

A 26-year old women with no significant past medical history, presents with a history of chest palpitations particularly when anxious. Physical exam reveals a mid-systolic ejection click followed by a mid-systolic ejection click followed by a murmurmurmur. The click murmur moves closer to S1 when the patient is standing and closer to S2 when lying down. The murmur is heard loudest at the apex.

Diagnosis: MVP

Mechanism of valvular disorder: myxomatous degeneration

Which compound in the extracellular matrix would be increased? Dermatan Sulfate

Explain why the murmur varies with position of the patient: standing decreases

Venous return --- decreased Left ventricular volume – systole occurs faster

19

Case 2

A 60-year-old man is evaluated for a heart murmur. He jogs 3 miles per day and is asymptomatic. Physical examination reveals a delayed carotid upstroke, a 3/6 late-peaking systolic ejection murmur that radiates to the neck, and a single S2.

An echocardiogram shows normal systolic

function and a heavily calcified aortic valve.

Diagnosis: AS

Features that identify the murmur?

Possible etiologies for this patients AS?

Which symptoms can be anticipated with progressive stenosis?

20

Case 3

59 year old man is brought to the ED for syncopal episodesyncopal episode while running in the park. He reports no similar preceding events. He denies shortness of breath and dyspnea. He has a childhood history of pharyngitis x3pharyngitis x3. BP is 110/90 mmHg. He has a late and weak carotid upstrokelate and weak carotid upstroke. On cardiac exam there is a midsystolic murmurmidsystolic murmur 3/6, the apical impulse is sustained and and displaced laterally and the murmur is found to radiate to the neck.radiate to the neck. An S4 heart sound is audible. Lungs are clear. There is no lower extremity edema.

Diagnosis: Aortic StenosisDiagnosis: Aortic StenosisFeatures of the Murmur of ASMost likely etiology of AS in this case ?

21

Aortic stenosis

EtiologyEtiologycongenital

senile calcific AS

rheumatic AS

PathologyPathologyPartially fused leaflets

fibrosis and calcification

Leaflets are less flexible, calcium deposits at valve base

leaflet tips relatively normal

Fibrosis and calcification of valve leaflets, associated with AR

Most common valvular pathology

22

Case 4

A 45 year old women who recently immigrated from Mexico complaints of exertional dyspnea, fatigue, palpitations and edema. She mentions coughing up pink, frothy looking sputum several times over the past weeks. Further questioning reveals some difficulty swallowing solids as well as hoarseness. Her past medical history is significant for a several severe throat infection in childhood. She is otherwise healthy. PE reveals a soft mid-diastolic murmur that increases with expiration. There is an accentuated P2. EKG shows

atrial fibrillation. Diagnosis: Mitral Stenosis with Ortner’s Syndrome

What would you see on TEE? Enlarged Left atrium

Explain the accentuated P2: Pulmonary HTN/RVH

23

Mitral Stenosis

LV inflow tract obstruction Diastolic Murmur loudest at the apex/Opening snap Accentuated P2 with Pulmonary Hypertension (PH)RVH secondary to PHDysphagia for solids --- left atrial enlargement

Hoarseness: compression of left recurrent laryngeal nerve Etiology:

MC- chronicchronic rheumatic valvulitis leading to fusion of commissure, and shortened Chordae tendineae

Intermittent or acute LV inflow tract obstruction --- atrial myxoma or

Atrial thrombus can mimic MS

24

Case 5

Your patient is a 55 year old man on his 4th day after an MI involving the RCA. The EKG shows NSR and PE was within normal limits at 7am. At 4 pm the nurse calls you because the patient complains of severe sudden SOB and diaphoresis. On evaluation he has dyspnea and rales are heard at the lung bases. His BP is 80/50 mmHg. Cardiac exam reveals a new holosystolic murmur loudest at the apex.

Diagnosis: Acute MR due to rupture of papillary muscle

Sudden left atrial volume overload

Rales and dyspnea indicate pulmonary edema25

Case 6

A 53-year old man presents complaining of diarrhea and “hot flashes” for 4 days. He also noticed his skin becoming unusually dark after having spend a few days at the beach. His son noted that dad was somewhat forgetful lately and sometimes confused. On physical exam there is hyperpigmentation of sun exposed areas. The patient is having some trouble when ask to inhale deeply. On cardiac exam there is a pansystolic murmur at the apex with radiation to the axilla. The murmur had not been noted on previous exams. The patients urine revealed high levels of serotonin metabolites. After careful examination and appropriate ancillary studies the patient was diagnosed with carcinoid tumor of the ileum/Carcinoid Syndrome.

Knowing that right sided valvular pathology is often associated with

Carcinoid syndrome, how do you explain the findings in the patients

Cardiac exam?26

Case 7

66-year-old man with a medical history notable only for locally treated prostate cancer presented with a two-month history of dyspnea on moderate exertion and repeated episodes of angina with mild effort or at rest. He came to the emergency room with exertional dyspnea that had worsened during the preceding week. There were no features of Marfan's syndrome. The blood pressure was 164/49 mm Hg (with a pulse pressure of 120 mm Hg), the radial pulses were bounding (Corrigan's pulse), and the carotid pulses were prominent. Cardiac examination revealed decreased S1 and increased S2 intensity, with a grade 2/6 systolic murmur and a grade 3/6 diastolic murmur along the left sternal border. Echocardiography showed a dilation of the aortic root, annuloaortic ectasia, severe aortic regurgitation, and mild tricuspid and mitral regurgitation. The left ventricle was enlarged, with an end-diastolic diameter of 90 mm, an end-systolic diameter of 61 mm, and an ejection fraction of 51 percent. NEJM

:Volume 353:e12

    

September 29, 2005

    

27

Aortic Regurgitation

Etiology• CHRONIC vs ACUTE

• Intrinsic valve disease vs Aortic pathology

Typical Murmur: early diastolic/decrescendo • Austin flint murmur (apical/diastolic)

• Systolic flow murmur

Volume overload

Wide Pulse Pressure (large SV)

28

Case 8

A 9-year-old child in a developing country is brought to a clinic by his parents because he has trouble keeping up with his classmates on the playground. Physical examination is remarkable for pulmonary rales. Cardiac Echo shows biventricular dilation of the heart. There is a soft holosystolic murmur appreciated at the apex with radiation to the axilla. Furthermore, the murmur increases on inhalation or squatting. EKG showed bilateral atrial enlargement.

Diagnosis: Wet Beriberi secondary to Thiamine (B1) deficiency Diagnosis: Wet Beriberi secondary to Thiamine (B1) deficiency

Name the two valvular dysfunctions and the supporting characteristics

Pathophysiology of valvular dysfunction

Other causes leading to TR 29

Referenceshttp://info.med.yale.edu/intmed/cardio/imaging/contents.htmlhttp://www-medlib.med.utah.edu/WebPath/webpath.htmlhttp://www.med.ucla.edu/wilkes/intro.htmlwww.blaufuss.orgRobbins and Cotran; Pathologic Basis of Disease, 7 th Ed.Ganong, Lingappa, Mc Phee; Pathophysiology of Disease, 4 th Ed.Kochar; Kochar’s Concise Textbook of Medicine, 4 th Ed.Harrison’s Principles of IM, 16th Ed.NEJM, Volume 351;15: 1539-1545, October 7, 2004

Aortic Regurgitation

Circulation 1994;90:844-853Otto CM, Kuusisto J, Reichenbach DD, Gown AM, O'Brien KD. Characterization of the early lesion of `degenerative' valvular aortic stenosis: histological and immunohistochemical studies

NEJM, Volume 352:2389-2397, June 9, 2005 Number 23“A Randomized Trial of Intensive Lipid-Lowering Therapy in Calcific Aortic Stenosis”