Cardiology Core Curriculum A problem-based approach - part 1 potx

example, a short PR interval and δ waves suggest pre-excitation andsupraventricular tachycardia; left ventricular hypertrophy, atrialpremature beats, or P-wave abnormalities of mitral va

Cardiology Core Curriculum

A problem-based approach

© BMJ Publishing Group 2003 BMJ Books is an imprint of the BMJ Publishing Group

All rights reserved No part of this publication may be reproduced, stored in

a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording and/or otherwise, without the prior

written permission of the publishers.

First published in 2003

by BMJ Books, BMA House, Tavistock Square,

London WC1H 9JR www.bmjbooks.com

British Library Cataloguing in Publication Data

A catalog record for this book is available from the British Library

ISBN 0 7279 1690 4

Typeset by SIVA Math Setters, Chennai, India

Printed and bound in Spain by Graphycems, Navarra

Tayo Addo, John D Rutherford

Victor A Ferrari, Luis Araujo, Martin G St John Sutton

Rao H Naseem, Michael L Main, John D Rutherford

David J Kessler, Richard L Page

13 Pericardial disease, disease of the aorta, and heart tumors 404

John A Osborne, Leonard S Lilly

14 Pulmonary embolism and pulmonary hypertension 445

Carlos M Sotolongo, James D Marsh

Khether E Raby

Charles Landau

Assistant Professor of Clinical Medicine, Columbia University, College

of Physicians and Surgeons, Cardiologist, Cardiac Associates of SouthernConnecticut, Bridgeport, Connecticut, USA

Thomas H Lee

Chief Medical Officer, Partners Community Healthcare, Inc.,Associate Professor of Medicine, Harvard Medical School, Boston,Massachusetts, USA

Leonard S Lilly

Cardiologist, Chief, Brigham and Women’s/Faulkner Cardiology,Associate Professor of Medicine, Harvard Medical School, Boston,Massachusetts, USA

Cardiologist, Head, Division of Cardiology, Robert A Bruce Professor

of Medicine, University of Washington School of Medicine, Seattle,Washington, USA

John D Rutherford

Cardiologist, Vice President of Clinical Operations, Professor ofInternal Medicine, Gail Griffiths Hill Chair in Cardiology, UTSouthwestern Medical Center, Dallas, Texas, USA

Carlos M Sotolongo

Cardiologist, Diagnostic Cardiology, PA Jacksonville, Florida, USA

Martin G St John Sutton

Cardiologist, Director, Cardiovascular Imaging Program, Hospital

of the University of Pennsylvania, John Bryfogle Professor ofCardiovascular Diseases, University of Pennsylvania Health System,Philadelphia, Pennsylvania, USA

Clyde W Yancy Jr

Cardiologist, Medical Director of Heart Failure and Cardiac TransplantProgram, Associate Professor of Medicine, Carl H WestcottDistinguished Chair in Medical Research and Dallas Heart Ball Chair inCardiac Research, UT Southwestern Medical Center, Dallas, Texas, USA

Christina Karaviotis, Lotika Singha and Andy Baker of BMJ Books fortheir advice, support, and the rapid response to trans-Atlantic emailsand attachments For administrative support I remain indebted toShirley Crook and Doris Matthews.

John D Rutherford

UT Southwestern Medical Center

Dallas, Texas

history should uncover risk factors that predispose to coronary arterydisease In general, angina pectoris will tend to occur in males over

40 years of age and females over 50 years of age; a history of cigarettesmoking is common, and the presence of diabetes mellitus,hypertension, hypercholestrolemia, or peripheral vascular disease

is highly relevant Similarly, a prior history of myocardial infarction,

or a family history of myocardial infarction or sudden death in a maleparent or sibling before age 55 years, or a female parent or siblingbefore age 65 years is important

Palpitations may be reported by patients as sensations of flipping,stopping, pounding, or fluttering of their heart Abrupt onset andoffset of rapid, sustained palpitations suggests supraventricular orventricular tachycardia If the sensation associated with thepalpitations is accompanied by dizziness, presyncope, or syncope,then ventricular tachycardia needs to be excluded If rapid irregularrhythms are reported then atrial fibrillation or flutter or tachycardiawith varying block are suggested Palpitations arising during exercise

or in situations of catecholamine excess may be due to ventriculartachycardia arising from the right ventricular outflow tract, and thosethat arise immediately after exercise may be due to atrial fibrillationand a relative increase in vagal tone A family history of prematuredeath or arrhythmias is highly relevant When acquiring the history

it can be helpful if the patient can tap out, with their fingers, theirperception of normal rhythm and the rhythm associated with theirsymptoms (A 12-lead electrocardiogram taken during the abnormalrhythm can be diagnostic An electrocardiogram taken in normalsinus rhythm can give clues as to the possible primary diagnosis For

Table 1.1 Severity of angina (Canadian Cardiovascular Society)

activity; it occurs only with strenuous or prolonged exer tion

with rapid walking on level ground or up inclines

or steps

angina occurs when walking

without angina, which also may occur briefly

at rest

example, a short PR interval and δ waves suggest pre-excitation andsupraventricular tachycardia; left ventricular hypertrophy, atrialpremature beats, or P-wave abnormalities of mitral valve disease mayrepresent substrates for atrial fibrillation; Q waves of prior myocardialinfarction may be the substrate for ventricular arrhythmias; and along QT interval is possibly associated with polymorphic ventriculartachycardia.2)

Shortness of breath, or dyspnea, is a normal accompaniment ofphysical exertion and is accentuated by obesity, pregnancy, lack ofphysical fitness, and advanced age Normally, shortness of breathlimits exercise performance, or physical activity, and individuals have

a certain expectation of what is normal for them The shortness ofbreath caused by heart disease is similar to that caused by normalexertion, but it is provoked by lower levels of physical activity thanhas been experienced previously by the individual patient Newsymptoms of nocturnal cough or episodic difficulty with breathingwhen lying supine may also be associated with cardiac dysfunction.Anxious patients may at times experience the sensation that a deeperbreath than normal is required to feel comfortable but have normalbreathing with physical activity The clinician must listen carefully tothe description provided by the patient and decide whether thesymptoms fall into a normal or abnormal category

Appearance of the patient

The patient may appear to be breathless at rest, which suggests thepresence of heart failure or severe pulmonary disease The appearance

of a patient may suggest specific systemic or developmental disorders.For example, in Marfan’s syndrome there is a defect in the region ofchromosome 15 that encodes the connective tissue protein fibrillin-1;this defect is associated with a well recognized phenotype, including tallstature, joint hypermobility, pectus excavatum, and arachnodactyly.Rarely, arterial pulsations may be immediately obvious after looking

at a patient For example, prominent arterial pulsations might be seen

in the neck or the patient’s head may be bobbing with each heartbeat, possibly indicating the presence of wide pulse pressures as areseen in conditions such as severe aortic regurgitation

Examination of the skin and mucous membranes is important(Table 1.2) The presence of pale, cool extremities might suggestcirculatory insufficiency; the presence of warm extremities withperspiration might suggest increased sympathetic activity (for example,associated with hyperthyroidism) and dry mucous membranes mightsuggest volume depletion The patient may exhibit obvious edema,

might suggest cyanosis, which is usually due to an incr

variety of diseases or may be a congenital abnor

dorsum of the distal phalanx The shape of the finger is alter

shunting of the blood associated with congenital hear

and on palms and soles They fade and become painless over several days

especially of the lower extremities, due to hepatic, renal, or cardiac causes,including congestive heart failure and constrictive pericardial disease.Examination of the optic fundi may provide direct information on

a variety of conditions that affect the cardiovascular system Inpatients with hypertension the retinal changes are classifiedaccording to the Keith–Wagner–Barker method These changes reflectboth atherosclerotic and hypertensive retinopathy Initially, arterialnarrowing is seen (grade 1), and subsequently the arteriolar diametersincrease in relation to the venous diameters, manifested asarteriovenous nicking (grade 2 changes) As uncontrolledhypertension progresses, small vessels rupture, and exudates andhemorrhages are seen (grade 3 changes) Eventually, sustained,accelerated, or extreme hypertension is associated with raisedintracranial pressure seen as papilledema, usually associated withretinal exudates and hemorrhages (grade 4 changes) Grade 2 changescorrelate with other evidence of clinical cardiovascular disease or end-organ damage (left ventricular hypertrophy, renal disease, arterialdisease) Overall risk in patients with hypertension correlates withpresence, or absence, of conventional cardiovascular risk factors andevidence of uncontrolled hypertension or end-organ damage Inpatients with diabetes mellitus (an important cardiovascular riskfactor) retinopathy may develop Increased capillary permeabilityleads to capillary closure and dilatation, microaneurysms, and dilatedveins Cotton wool spots (microinfarcts), hemorrhages, and hardexudates are seen New vessel formation and scarring are seen withproliferative retinopathy Finally, in infective endocarditis evidence offocal retinal hemorrhage (i.e Roth spots – retinal hemorrhage with aclear center) or embolic phenomena (retinal arterial occlusion) may

be found

Arterial blood pressure and pulses

Blood pressure should initially be measured in both arms Theblood pressure is obtained by sphygmomanometry, and the bladderassociated with the cuff should encircle and cover approximately 50%

of the length of the upper arm (If the cuff bladder is too small theninaccurate, abnormally high blood pressure readings may beobtained.) The cuff is inflated rapidly while palpating the pulse untilabout 20–30 mmHg above the point at which the palpated radialpulse disappears The cuff is then deflated slowly as the brachial artery

is auscultated The sounds heard are described in five phases(Korotkoff sounds) The initial phase (phase 1) begins with theappearance of clear, tapping sounds and represents systolic blood

pressure Diastolic blood pressure is represented by the disappearance

of sounds (phase 5) Just before the sounds disappear they becomemuffled (phase 4) A discrepancy between blood pressures measured

in both arms (of more than 5–10 mmHg) may indicate involvement

of the great arteries leaving the heart, in a disease process (arterialocclusive disease, aortic dissection or coarctation)

The major arterial pulses (carotid, brachial, radial, femoral,popliteal, posterior tibial, and dorsalis pedis) should be examinedbilaterally Diminished or absent arterial pulses suggest occlusivedisease The radial and femoral arteries should be palpatedsimultaneously during the cardiovascular examination A significantradial–femoral delay (the appearance of the pulses in the lowerextremities is delayed as compared with that in the upper extremities)suggests coarctation of the aorta, which needs exclusion In thisinstance, an initial step is to measure blood pressures in the lowerextremities and compare them with pressures in the upperextremities Again, if there is a major discrepancy (i.e the bloodpressures measured in the lower extremities are lower) thencoarctation of the aorta must be excluded

When the arterial pulse is palpated (typically in the radial, carotid,and brachial locations initially), rate, rhythm, and quality of the pulseare noted The rhythm may be regular or irregular Sinus arrhythmia,

a normal finding, may be noted when there is a slight acceleration ofthe pulse during inspiration and slowing during expiration Thisdetermination can usually be made only when the pulse is relativelyslow An irregularly irregular pulse suggests atrial fibrillation, andoccasional dropped or skipped beats may suggest atrial or ventricularpremature beats A rhythm strip, or a 12-lead electrocardiogram, is theonly sure way to distinguish these irregularities The shape of thepulse wave (quality) and the volume of the pulse wave (the amplitude

of the pulse is determined by the difference between the systolic anddiastolic blood pressures = pulse pressure) each provide importantinformation Large volume pulses suggest a high cardiac stroke outputand may generally be associated with the dilatation of the peripheralblood vessels that is seen normally with exercise, fever, pregnancy,aortic regurgitation, and hyperthyroidism A small volume pulsemight suggest a low cardiac stroke output or obstruction to cardiacoutflow, for example significant aortic valve stenosis Duringpalpation of the arterial pulse or blood pressure measurement anexaggerated decrease in systolic blood pressure with inspiration(pulsus paradoxicus) may suggest cardiac tamponade or may beassociated with asthma or, rarely, morbid obesity

Clinical experience will alert the clinician to any deviation from theshape of the normal arterial pulse wave, which has a smooth, quick

rise; a momentary peak; and a smooth, quick fall In aortic stenosisthe upstroke of the pulse may be delayed and associated with a smallvolume pulse if significant outflow obstruction exists In patients withsignificant aortic regurgitation a rapidly rising and falling pulse with

a wide pulse pressure may be observed

Finally, palpation of the pulse (especially the radial and femoralpulses) may provide some indication of “hardening of the arteries”,which occurs with advanced atherosclerosis Certainly in olderpatients, with loss of elasticity of the arterial walls, the sensation offirmness or hardening of the arteries may be appreciated

Jugular venous pulse (Figure 1.1)

The level of the jugular venous pulse (JVP) allows estimation of thefilling pressure of the right heart (i.e the central venous fillingpressure).4 It is elevated with heart failure, hypervolemia, conditionsthat reduce compliance (or increase “stiffness”) of the right ventricle,constrictive pericarditis, and obstruction of the superior vena cava Toassess the JVP the right internal jugular vein is usually examined withthe patient in a supine position, with back, head, and neck inclined

at a 30–45° angle In the absence of obesity the pulsations of thejugular veins are transmitted to the skin In contrast to the carotidpulse, which is a fast, localized, single, outward deflection, the JVP isdiffuse, usually has two waves, and is a slow rostral deflection Twophases are evident in the pulsation An “a” wave occurs just before thefirst heart sound (S1) and a “v” wave occurs simultaneously with orjust after the second heart sound (S2; Table 1.3 and Figure 1.1) The “a”wave reflects right atrial contraction and the “v” wave reflects passiveatrial filling after systole

The maximal height of the JVP above the heart, with the patientlying at 45°, closely reflects mean right atrial pressure Aperpendicular plane is extended from the highest point at which thejugular venous pulsation is seen to the sternal angle The height fromthe sternal angle is measured Because the right atrium isapproximately 5 cm below the sternal angle, 5 cm is added to theheight of the JVP seen above the sternal angle This approximates JVP

in centimeters of water (cmH2O) The upper limit of normal for JVP is

4 cm above the sternal angle or a central venous pressure of 9 cm.With a patient sitting in a 90° upright position, any visible venouswaveform is abnormally elevated Some patients, with severeelevation of right heart filling pressures, will need to sit upright sothat the pulses can be visualized and the right atrial pressureestimated

contraction; it occurs late in ventricular diastole just befor

venous wave The “a” wave follows the P wave of the electr

passive filling of the emptied right atrium in late diastole Nor

passive filling of the right atrium continues and blood backs up in the jugular veins while the tricuspid valve is closed during ventricular systole

ge “a” waves occur when diastolic filling of the right ventricle is r

atrium contracts and the tricuspid valve is closed; for example, thir

fibrillation and in this situation the JVP consists of a “v” wave and Y descent

T Wave

which begins as the tricuspid valve opens and the blood empties into the right ventricle; it is a measur

often has a rapid and deep Y descent followed by a sudden, rapid r

be palpated in patients with major obesity or pulmonary conditionssuch as emphysema, which distend the lungs more than usual andthus increase the gap between the heart and the chest wall.

Although the value of the position of the apex beat as a physicalsign of heart enlargement is limited, some useful information can be

Ventricular diastole

Figure 1.1 The jugular venous pulsation Two phases are evident in the pulsation.

An “a” wave occurs just before the first hear t sound (S1) and a “v wave” occurs simultaneously with, or just after, the second hear t sound (S2) The “a” wave reflects right atrial contraction and precedes ventricular systole and the the “v” wave reflects passive atrial filling after ventricular systole

obtained by determining its exact position In normal persons theapex beat pulsation is produced by a small area of left ventricle, most

of which lies behind and to the left of the right ventricle With leftventricular pressure overload the apex beat is forceful and sustained.With left ventricular dilatation and volume overload the apex beat isprolonged, sustained and, when the heart is enlarged, it is displacedleftward and downward A parasternal lift felt by palpation over thethird, fourth, and fifth left intercostal spaces close to the sternumusually indicates right ventricular pressure or volume overload ormitral regurgitation (due to the forceful backflow of blood into theleft atrium during left ventricular systole) In patients with abnormalhearts, especially those with valvular heart disease, murmurs may beloud or turbulent enough to be felt as vibrations known as “thrills”(for example, in pulmonic stenosis)

Cardiac auscultation

Successful auscultation of the heart involves recognition of normalheart sounds and detection of abnormal heart sounds associated withcardiovascular disease In order to concentrate on the appropriatephase of the cardiac cycle, the heart sounds can be timed by assessingtheir relationship to simultaneous palpation of the carotid pulse insystole Two heart sounds are heard in all normal people (Figure 1.2).The first heart sound (S1) is associated with closure of theatrioventricular valves (mitral and tricuspid) and occurs at the start ofventricular systole It is usually best heard at the apex of the heart,using the “bell of the stethoscope” S1 is normally heard as a singlesound but actually has two successive components associated withclosure of the mitral valve and later closure of the tricuspid valve Inpatients with complete right bundle branch block, the delay of thetricuspid component may result in a widely split S1

The second heart sound (S2) is produced by closure of the aortic (A2)and pulmonary (P2) valves S2for clinical purposes marks the end ofventricular systole Normally, the first component (i.e A2) and thesecond component (i.e P2) of S2separate or “split” during inspirationbecause P2 is delayed as a result of the inspiratory increase incapacitance of the pulmonary vascular bed This splitting is usuallybest heard in the second left intercostal space (pulmonary area),whereas the louder A2can be heard at the base, left sternal edge, andcardiac apex (see Figure 1.2) P2 is almost never heard at the apex.Fixed splitting of S2 is heard in most patients with an atrial septaldefect, and wide splitting is associated with right bundle branch blockand pulmonary valve stenosis When the patient stands, fixedsplitting persists and wide splitting will decrease

The “loudness” of the heart sounds can vary in health and disease.The sound associated with S1 is probably mainly due to mitral valveclosure, and its intensity depends on how far into the left ventricularcavity the mitral leaflets are at the onset of the ventricular systole.Therefore, S1 may be loudest in the presence of a rapid heart rate, ashort PR interval, at times in mitral valve prolapse when the mitralvalve leaflets are at their maximal point of excursion into the leftventricular cavity at the onset of ventricular systole, and in mitralstenosis with a pliable valve The intensity of S1decreases in situations

in which this mobility of the mitral valve leaflets into the ventricularcavity is lost (for example, longstanding mitral valve stenosis with anon-pliable, calcified mitral valve, mitral regurgitation, and a long PRinterval) The intensity of S2increases with both systemic hypertension(increase in intensity of A2) and pulmonary hypertension (increase inintensity of P2)

Added diastolic sounds

Third heart sounds (S3) are mid-diastolic sounds that are best heardwith the stethoscope bell applied lightly and with the patient in theleft lateral decubitus position S3occurs during the rapid filling phase

of the ventricle, occurring after closure of the aortic and pulmonaryvalves, and coinciding with the wide ascent of the atrial pressurepulse In children, young adults, and women during pregnancy, an S3may be a normal finding An abnormal S3 may be generated if theventricle is dysfunctional (heart failure) or if there is an increase inflow during the rapid filling phase of the ventricles (mitral andtricuspid regurgitation)

Figure 1.2 Normal hear t sounds S1, first hear t sound; A2, aor tic component of the second hear t sound; P2, pulmonar y component of the second hear t sound

The fourth heart sound (S4) is probably never a normal finding An

S4is a late diastolic (or presystolic) sound and is generated within theventricle during the atrial filling phase Active atrial systole is required

to generate an S4 and the sound is not heard in patients with atrialfibrillation when coordinated atrial contraction is absent An S4 istypically generated in situations in which the ventricle is “stiff” or

“non-compliant”, and augmented atrial contraction is needed to fillthe ventricle appropriately (for example, left ventricular hypertrophyassociated with hypertension or aortic valve stenosis; right ventricularhypertrophy associated with pulmonary hypertension or pulmonarystenosis; and acute myocardial ischemia with a stiff, non-compliantleft ventricle) When these sounds originate from the left ventriclethey are best heard with the patient in the left lateral decubitusposition, applying the bell of the stethoscope lightly to the cardiacapex When the sounds originate from the right ventricle they may bebest heard at the left lower sternal edge with the patient supine andmay possibly be accentuated by inspiration

Cardiac murmurs

In general cardiac murmurs are sounds that result from turbulentblood flow of blood in the heart or great vessels, producing vibrations.When blood vessels or valves are narrowed by disease or when valvesleak because of disease the rate of blood flow may result in turbulenceand vibrations, and therefore murmurs are heard It is important tonote that turbulence may also occur when blood viscosity is low orwhen the rate of blood flow through a normal heart or blood vessel isaccelerated, as in anemia, fever, or pregnancy

A grading system for intensity or loudness was proposed by Samuel

A Levine on a scale of 1 to 6 A grade 1 murmur is so faint that it isonly just heard, a grade 3 murmur is easily heard but not loud, and agrade 5 murmur is very loud Murmurs may also be describedaccording to their time of onset and termination (for example, mid-systolic, holosystolic [beginning with S1 and ending with S2], earlysystolic, or late systolic) and according to whether they are crescendo,decrescendo, crescendo/decrescendo, or heard as a “plateau”

Murmurs associated with the aortic and pulmonary valves areusually best heard at the base of the heart, those associated with themitral valve are best heard at the apex, and those associated with thetricuspid valve at the lower left sternal edge In general, right sidedheart murmurs are accentuated by inspiration, aortic diastolicmurmurs are best heard with the patient leaning forward in theexpiratory phase of respiration, and diastolic mitral murmurs are best

heard with the patient lying in the left lateral decubitus position withthe bell of the stethoscope lightly applied at the cardiac apex.

Examination of the chest includes the following:

• inspection (shape of the chest – kyphosis, scoliosis; respiratoryrate; amplitude and symmetry of chest movement; use of accessorymuscles of neck and chest wall with labored respiration)

• listening for noisy breathing (stridor with laryngeal or trachealobstruction, wheezing with partial bronchial obstruction)

• palpation (position of trachea and upper mediastinum – displaced

by masses, collapse or fibrosis of the lung drawing the trachea tothe affected side, or tension pneumothorax or a large pleuraleffusion displacing the trachea to the opposite side)

• measurement of chest expansion (normally 5–10 cm)

• assessment of tactile vocal fremitus

Vocal tactile vibrations are absent or diminished over a pleuraleffusion, but are transmitted with greater ease through consolidatedlung (for example, in pneumonia) than normally aerated lung.Percussion of the lung yields a resonant note over normally aeratedlung, a dull note over fluid, and a tympanitic note or hyperresonantnote over collections of gas Auscultation of normal breath sounds(which are produced in the larynx or throat and conducted down thetrachea and bronchial system to the alveoli) reveals bronchial breathing

if one listens over the trachea (harsh, low pitched inspiration andlonger, high pitched expiration) and normal vesicular breathing overthe lungs (lower pitched, less harsh inspiration and faint, shortexpiration) as normal lung modifies the laryngeal sounds Normalbreath sounds are diminished or absent distal to an occluded bronchus

or if fluid or air is between the lung and the chest wall (effusion orpneumothorax) Normal breath sounds change to bronchial sounds if asegment of lung consolidates and allows laryngeal breath sounds topass with less modification to the chest wall A variety of abnormalsounds can accompany breath sounds and are known as adventitioussounds There are two main types of adventitious sounds: crepitations

or rales, which are crackling or rustling sounds heard mainly ininspiration when there is fluid in bronchioles and alveoli; and rhonchi,which are louder, coarser sounds associated especially withinflammation or partial obstruction of bronchi (asthma, bronchitis).The abdomen is examined for evidence of edema, ascites, liverenlargement (heart failure), pulsatile liver (tricuspid regurgitation),enlargement of the abdominal aorta (pulsatile abdominal aorticaneurysm), and bruits (associated with renal artery stenosis orabdominal aortic aneurysm)

Specific cardiac conditions

Certain features of the history, clinical examination,

electrocardiogram, and chest x ray film will almost always lead to the

correct cardiac diagnosis if carefully and correctly assessed by theclinician Although many the following conditions5 are discussed indetail in the relevant chapters of this book, a few are discussed herewith particular emphasis on auscultatory findings It is our opinionthat the skilled clinician is very familiar with these findings, activelyconfirms or excludes their presence during the cardiac examination,and is able to arrive at an informed conclusion regarding the presenceand severity of specific cardiac diseases Furthermore, the physicianwho has detailed knowledge of the history and clinical examination,

electrocardiogram, and chest x ray findings for a patient will be able

to interpret accurately the meaning of the non-invasive and invasivecardiac studies and place their findings in perspective Armed with all

of this information, the physician is then in a position to discuss thecardiac diagnosis and options for therapy with the patient Reliance

on the results of non-invasive or invasive testing alone withoutsynthesis of all available clinical information may lead to poor clinicaldecisions and suboptimal patient care

Mitral stenosis

In many parts of the world (India, Central America, South Pacificcountries) mitral stenosis, caused by rheumatic pancarditis, presentspredominantly in young women of child-bearing age, often inassociation with pregnancy Elsewhere, the disease presents one ortwo decades later The predominant symptom is breathlessness,caused by pulmonary congestion or edema, at times unmasked by theonset of atrial fibrillation

The clinical examination features of mitral stenosis depend on thefollowing:

• whether the mitral valve apparatus remains “pliable” or mobile,and is fused predominantly along the valve edges, or is “non-pliable” and immobile (a fibrosed, thickened, distorted valve withassociated fusion and shortening of the chordae tendinae)

• the presence or absence of pulmonary hypertension

• the presence or absence of atrial fibrillation

“Pliable” or mobile mitral valve apparatus in sinus rhythm

On physical examination the patient appears normal or isbreathless Pulse is normal or low volume Blood pressure is normal

JVP is normal, or there may be an increased “a” wave if pulmonaryhypertension is present Cardiac impulse is normal or a leftparasternal lift is present in pulmonary hypertension.

On auscultation (Figure 1.3), S1 is loud S2 splits normally oninspiration P2 is accentuated with pulmonary hypertension Anopening snap (OS) of the mitral valve immediately follows S2 (heard

at the apex with the diaphragm), followed by a mid-diastolic, lowpitched rumble (with or without presystolic accentuation) that is bestheard at the apex with the bell lightly applied and the patient in theleft lateral recumbent position The OS occurs when the openingmovement of the mitral apparatus or “doming” into the left ventricleabruptly stops The loud S1is the reciprocal sound of the OS and is due

to abrupt completion of atrioventricular valve closure Both soundsarise when the mitral valve apparatus is mobile and pliable, and arelost when it becomes relatively immobile

On chest examination, crepitations or rales are present withpulmonary congestion Abdominal examination reveals a normalliver span or pulsatile liver with pulmonary hypertension andsecondary tricuspid regurgitation Peripheral edema may be noted ifthe patient is pregnant or in the presence of tricuspid regurgitation.The electrocardiogram may indicate left atrial enlargement, whichmay be manifested as a negative terminal force in the P wave in V1, or

as a notched or wide P wave in II The chest x ray film may show left

atrial enlargement with elevation of the left main stem bronchus.Evidence of pulmonary congestion may be seen with prominentupper lobe vessels, and later there may be evidence of interstitial

Figure 1.3 Mitral stenosis with pliable valve S1, first hear t sound; A2, aor tic

the OS MDM, mid-diastolic murmur; OS, opening snap; P2, pulmonar y component

of the second hear t sound

edema and fluid within the interlobular septa (Kerley B lines, whichare short, dense, horizontal lines often seen near the lung periphery

in the costophrenic angles)

Immobile mitral valve apparatus

The clinical examination features associated with an immobile mitralvalve apparatus when the patient has atrial fibrillation, pulmonaryhypertension, and secondary tricuspid regurgitation are as follows

In addition to symptoms of breathlessness and palpitations, thepatient may complain of reduced energy and leg swelling Onphysical examination the patient may appear normal or be breathless.Pulse is normal or low volume and irregularly irregular (atrialfibrillation) Blood pressure is normal JVP is elevated to the angle ofthe jaw with “v” waves Cardiac impulse is a left parasternal lift due

to right ventricular pressure/volume overload

On auscultation (Figure 1.4), S1is soft (and variable) At the base aloud P2 is heard; S2 may become single with severe pulmonaryhypertension (reduced compliance of the pulmonary vascular bedassociated with earlier closure of P2) A mid-diastolic, low pitchedapical rumble is heard at the apex with the patient in the left lateralrecumbent position At the lower left sternal edge a grade3–4 holosystolic murmur, which increases with inspiration, is heard(tricuspid regurgitation) With right ventricular hypertrophy anddilatation, the murmur of tricuspid regurgitation may be heard at theapex as the enlarging ventricle becomes displaced leftward Incontrast to the patient with the pliable mitral stenotic valve, once themitral valve becomes fibrosed, distorted, and relatively immobile, S1becomes soft and the OS is lost With the onset of atrial fibrillationthe loudness of S1becomes variable

Chest examination reveals crepitations or rales with pulmonarycongestion Abdominal examination indicates increased liver spanand a pulsatile liver Peripheral edema is present Electrocardiogramfindings are atrial fibrillation, evidence of right ventricular hyper-trophy with a rightward shift of the mean frontal plane axis greaterthan 80°, and an R : S ratio greater than 1·0 in V1 The chest x ray film

may show left atrial enlargement, evidence of pulmonary congestion,Kerley B lines, and enlargement of the pulmonary arteries and rightventricle

Aortic stenosis

Aortic valve stenosis seen in adults is usually secondary to acongenital bicuspid valve or age-related degenerative calcification and

results in left ventricular pressure overload and concentrichypertrophy The condition usually presents in patients older than

50 years, with a triad of possible symptoms: angina, increasedbreathlessness, and exertional syncope (or dizziness)

On physical examination the patient appears normal Pulse is slowrising, low volume, and sustained Blood pressure is normal, as is JVP.Cardiac impulse is prominent but usually not displaced (when heartfailure ensues late in the natural history, the cardiac impulse becomesdisplaced inferiorly and laterally)

On auscultation (Figure 1.5), S1is normal S2sound is single and/orsoft An ejection systolic murmur is heard at the base of the heart(upper right sternal edge) and is often transmitted to the carotids andcardiac apex Chest and abdominal examinations are normal.Peripheral arterial pulses are all normal volume and equal Carotidpulses are slow rising and sustained A transmitted ejection systolicmurmur, from the aortic valve, is often heard over the right carotid

On electrocardiography, left ventricular hypertrophy is found in the

majority of patients with severe aortic stenosis Chest x ray film

appearances are often normal

S1 variable

MDM Apex

P2 loud Base

LSE

Holosystolic murmur

increases with inspiration

Figure 1.4 Mitral stenosis, pulmonar y hyper tension, and tricuspid regurgitation.

S2, second hear t sound; P2, pulmonar y component of the second hear t sound

If the pulse is slow rising, low volume, and sustained, S2 is single,and the typical ejection systolic murmur is long and late peaking,then it is likely that severe, aortic valve stenosis is present The mostuseful clinical findings for diagnosing aortic stenosis, in order ofimportance, are a slow rate of rise of the carotid pulse, a mid to latepeaking systolic murmur, and a decreased intensity or absent S2.

Hypertrophic cardiomyopathy

In this condition, also known as idiopathic hypertrophic subaorticstenosis, there is cardiac hypertrophy, usually involving theinterventricular septum, which in a minority of patients can lead to adynamic left ventricular outflow obstruction In the patients withobstruction any situation that results in a reduction in left ventricularvolume (hypovolemia, Valsalva maneuver) increases the obstruction

of the thickened, non-compliant, “restrictive” ventricle The diseasehas a genetic basis, at times affecting families, and involves mutations

in the coding of β cardiac myosin heavy chains The majority ofpatients are asymptomatic but the condition can present, usually inyoung adults, with syncope, sudden death, or breathlessness.Abnormal cardiac rhythms are poorly tolerated and patients maypresent with atrial tachyarrhythmias, leading to hypotension, as aresult of the loss of the atrial contribution to filling the “restrictive”ventricle

On physical examination, the pulse is bisferiens or “jerky”, with abrisk arterial upstroke Blood pressure is normal and JVP is usuallynormal There is a prominent, forceful, left ventricular impulse

Figure 1.5 Aor tic valve stenosis S1, first hear t sound; S2, second hear t sound;

S4, four th hear t sound Single S2 (because A2 is delayed and merges with P2); ejection systolic murmur; S4 often present A2, aor tic component of the second hear t sound; P2, pulmonar y component of the second hear t sound