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aneurysm Localized dilatation of either a blood vessel or a heart chamber. The most common cause of an arterial aneurysm is atherosclerosis. Anywhere in the aorta atrophy of the media (muscular layer) deep to an atherosclerotic plaque results in either a saccular or a fusiform (spindle-shaped) dilatation. The most common site is the abdomen distal to the renal arteries. Syphilis (lues) used to be the most common cause of thoracic aortic aneurysm, usually in the ascending aorta. When infection destroys a local area of any artery to cause a local dilatation, a mycotic aneurysm is said to have formed.

dissecting aneurysm In the correct pronunciation the "diss" rhymes with "kiss." Localized aortic dilatation that results from separation of the layers of the aortic wall by hemorrhage into the media secondary to degeneration of the media (cystic medial necrosis). In some patients it may begin with an intimal tear. It usually dissects distally, but when it dissects proximally, it may involve the aortic valve and produce aortic regurgitation, or it may dissect into the pericar-dial space, producing a fatal tamponade.

ventricular aneurysm A dilated segment of the left ventricle (LV). It is commonly caused by myocardial infarction. They vary in size from a few centimeters in diameter to a size one-half that of the LV. They rarely rupture but can cause heart failure. A thrombus often fills the aneurysm. It may bulge during systole (paradoxical motion or dyskinesis). A large area of damaged myocardium that fails to show any motion during systole is sometimes also called an aneurysm, although it is better simply to call this area akinetic or, if the movement is slight, hypokinetic.

anomalous pulmonary venous connection or drainage Drainage of one or more pulmonary veins, usually into the right atrium or superior or inferior vena cava. More rarely, a vein (or veins) from the left lung empties into the innominate vein, a left vertical vein, or the coronary sinus. The anomalous connection results in a left-to-right shunt with the same chambers overloaded as in atrial septal defect. In total anomalous pulmonary venous return, all of the pulmonary veins may enter any of the following: a left vertical vein, the innominate vein, the coronary sinus, the right atrium, the superior or inferior vena cava, or the portal vein. An atrial septal defect is essential for survival. The hemodynamics are similar to those of a large atrial septal defect.

aortic stenosis (AS) Obstruction to LV outflow. This may occur at valvular, supravalvular, or subvalvular levels. The subvalvular obstruction may occasionally be due to a congenital fibrous ring just below the aortic valve (discrete subvalvular AS), but usually it is due to a hypertrophied septum impinging on the anterior leaflet of the mitral valve during systole (hypertrophic subaortic stenosis, hypertrophic obstructive cardiomyopathy). (See figure, p. 203.) The supravalvular type is associated with a characteristic facies (see p. 21). Aortic valvular stenosis without any other valves involved is almost always congenital, and about half of these are due to calcification of a bicuspid aortic valve. The acquired ones are usually due to rheumatic valvulitis and are associated with some mitral valve disease. In subjects over age 70, degenerative calcification of the aortic valve may be the most common cause of aortic stenosis, especially in women.

arteriosclerosis 1. Atherosclerosis (the progressive laying down of lipid in the intima of an artery, starting with a fatty streak and ending with a plaque made up of lipids, fibrosis, and calcium). 2. Medial sclerosis (fibrosis of the media or muscular layers of arteries, which may end in "pipestem" arteries). Medial sclerosis affects only the larger peripheral arteries; i.e., the aorta and coronary arteries are subject to atherosclerosis but not to medial sclerosis. Arterioles are not usually affected either by medial sclerosis or atherosclerosis.

atrial myxoma A tumor made up of soft, loose, friable tissue that is usually on a pedicle (pendunculated) attached to the atrial septum in the region of the fossa ovalis. It is almost twice as common in the left atrium as in the right atrium. It can protrude through its respective atrioventricular (AV) valve in diastole to produce a partial obstruction that imitates mitral or tricuspid stenosis and occasionally causes syncope. It may merely prevent complete closure of the valve, resulting in various degrees of mitral or tricuspid regurgitation. If the tumor becomes calcified, it may act like a wrecking ball and completely destroy the AV valve, producing severe regurgitation. Emboli from the friable tumor are among the most common causes of clinical manifestations. For unknown reasons, the tumor acts as an inflammatory agent and commonly produces a high sedimentation rate and intermittent fevers, which, together with the occasional clubbing, mimics infective endocarditis. It may even produce reactions of an allergic type, resulting in puzzling skin and joint manifestations.

atrial septal defect (ASD) An opening between the atria, which may occur at three possible levels. The lower one is called a primum defect, the middle one is a secundum defect, and the upper one is called a sinus venosus defect. By far the most common is the secundum, or fossa ovalis defect.

The three levels of atrial septal defects are, in general, low, middle, and high. It may help your memory if you think of the lower two levels as the "first floor," or primum defect, and the "second floor," or secundum defect. The sinus venosus defect then remains and thus must be the "top floor," or high defect. The sinus venosus is the embryological site of the pacemaker of the heart, or the sinoatrial node. If you keep in mind that the sino-atrial node is at the junction of the superior vena cava and right atrium, it will be easy to remember that the sinus venosus defect is the high one. When the inferior wall of the fossa ovalis acts like a flap valve, it is called a patent foremen ovale. There will then be flow from the right to the left atrium only if the pressure rises abnormally high in the right atrium. The flap is normally closed by the higher pressure in the left atrium relative to that in the right atrium. Note that in the sinus venosus defect, two anomalous pulmonary veins are shown draining info the superior vena cava.

primum defect An atrial septal defect that is part of a possible spectrum of abnormalities caused by maldevelopment of the fetal heart that give rise to

1. The inferior part of the atrial septum above.

2. The upper part of the ventricular septum below.

3. The medial (anterior) leaflet of the mitral valve on the left.

4. The septal leaflet of the tricuspid valve on the right.

If the lower part of the atrial septum (AS) does not develop, an ostium primum ASD results. If the upper part of the ventricular septum (VS) is missing, a ventricular septal defect results. If the medial cushion parts of the mitral (MV) or tricuspid valves (TV) are missing, a cleft mitral or tricuspid leaflet results.

Any permutation or combination of endocardial cushion defects may occur. When defects in all four of the above structures are present, the condition is called a complete atrioventricular canal or complete endocardial cushion defect. A left axis deviation on ECG is so common with endocardial cushion defects and so rare with atrial septal defects at higher levels that for the clinical differentiation of the primum from the secundum and sinus venosus defects by ECG it is quite helpful.

sinus venosus defect A high atrial septal defect that is always associated with an anomalous drainage of one or two right pulmonary veins into the superior vena cava. (See anomalous pulmonary venous connection or drainage.)

The shunted blood in atrial septal defects travels from the left to the right atrium, from the right atrium to the right ventricle, from the right ventricle to the pulmonary artery, from the pulmonary artery to the pulmonary arterioles and veins, and from the pulmonary veins to the left atrium. Therefore, the right atrium, right ventricle, and pulmonary vessels all have a volume overload. The left atrium, however, serves only as a conduit and does not become enlarged except under exceptional circumstances. (See figure on p. 271.)

Enlarged RV

Note that with an atrial septal defect, the right atrium and ventricle receive blood from two sources. They are volume-overloaded at the expense of blood to the left ventricle. An increase in total blood volume is the compensatory mechanism by which the left ventricle receives a normal volume for average physiological needs. When an atrial septal defect or any other left-to-right shunt is closed surgically, the total blood volume of the body decreases by the exact amount of the shunt.

Enlarged RV

Note that with an atrial septal defect, the right atrium and ventricle receive blood from two sources. They are volume-overloaded at the expense of blood to the left ventricle. An increase in total blood volume is the compensatory mechanism by which the left ventricle receives a normal volume for average physiological needs. When an atrial septal defect or any other left-to-right shunt is closed surgically, the total blood volume of the body decreases by the exact amount of the shunt.

atrioventricular block (AV block) Conduction delay anywhere from the AV node to the ending of the bundle branches in the ventricle, i.e., the delay may occur in the AV node, the bundle of His, or the bundle branches. A first-degree AV block is recognized by seeing a long P-R interval. Second-degree AV block refers to intermittent complete AV block in which a dropped QRS occurs periodically. If the AV block is complete, the atria and ventricles are dissociated and an independent pacemaker for the ventricles occurs either at or below the bundle of His.

atrioventricular dissociation A condition in which the atria and ventricles have independent pacemakers. The lower pacemaker may be in the junctional area or deep in a ventricle. AV dissociation implies that atrial contraction has varying relationships to ventricular contraction, i.e., if the atria are in sinus rhythm, the P-R interval will be continually changing in a haphazard manner.

beriberi heart disease The effect on the heart of total body capillary dilatation caused by a deficiency of vitamin Bj (thiamine). In order to fill the enlarged vascular bed, a marked hypervolemic, hyperkinetic state is produced, with a high venous pressure, tachycardia, cardiomegaly, peripheral edema, and rapid circulation time. In the occident, it is seen almost entirely in alcoholic persons who resort to an enormous intake of beer. An acute, fulminant, nonedematous form characterized by cardiovascular collapse and death within hours or days has been called shoshin beriberi. (Sho is Japanese for "damage," and shin means "heart").

Bernoulli effect The drop in pressure on the surface of any structure caused by a flow over that structure. This tends to pull the structure toward the stream. An instrument utilizing the Bernoulli effect to measure flow is called a Venturi meter. The Bernoulli effect on the wings of an airplane raises it and keeps it airborne.

carcinoid heart disease Accumulation of grossly whitish yellow fibrous tissue on the inner surface of the right ventricle or atrium, as well as on the undersurface of the tricuspid and pulmonary valves (rarely, of the mitral valve). It can hold the tricuspid or pulmonary valve in the semiclosed position and so cause tricuspid or pulmonary stenosis and regurgitation. Carcinoid heart disease is usually associated with a carcinoid tumor of the bowel and with metastases to the liver. Bron-chospasm, diarrhea, and various types of flushing (general redness, bright red patches, or violaceous cyanosis) are all part of the carcinoid syndrome.

cardiomyopathy Myocardial damage from any cause. Therefore, it should have an adjective preceding it, e.g., idiopathic cardiomyopathy (often called primary myocardial disease or dilated cardiomyopathy), amyloid cardiomyopathy, or coronary or ischemic cardiomyopathy.

Cheyne-Stokes respiration The periodic breathing characterized by a gradually increasing depth of respiration, culminating in a period of apena that may last from a few seconds to as long as a minute. During the period of apnea, the oxygen saturation reaches its lowest level and the carbon dioxide tension its highest, causing the cycle to be repeated again. The most common associated condition for the cardiologist is severe low output due to heart failure. The neurologist more commonly sees it as a result of cerebral disease. Because it is exaggerated when dozing, and the hyperpneic phase can cause enough cerebral stimulation to prevent sleep, it is a possible cause of insomnia in a patient with heart failure.

Carcinoid deposits tend to form on the undersurface of the tricuspid valves and the upper surface of the pulmonary valves and so hold them in a rigid, semiclosed position. The compliance of the right atrium can be reduced by a lining of carcinoid material, thus raising its pressure conspicuously.

circulation time The time it takes for a marker material to travel from the site of injection to the site of appearance, usually after it passes through the lungs or lesser circulation.

clubbing A condition in which soft tissue of the terminal phalanges of the fingers or toes becomes hypertrophied and the nail finally curves excessively, giving a drumstick appearance. (See p. 17 for method of eliciting.) In cardiac patients, clubbing is usually associated with cyanosis, but if not, it should suggest the presence of acute infective endocarditis that has been present for a few weeks, suppurative lung lesions, anoxic cor pulmonale, or metastatic lung cancer. More rarely, it is caused by chronic diarrhea with ulcerative colitis or may even be familial. In cardiac patients, the most common causes of clubbing are tetralogy of Fallot and transposition of the great vessels. When clubbing and cyanosis are greater in the toes than in the hands, the condition is called differential cyanosis (see p. 237) and clubbing. (See illustrations at beginning of Chapter 2, Questions 3 and 4.)

coarctation Localized or diffuse narrowing of the aorta. The degree of constriction varies from slight to severe; rarely, it is complete. It is usually seen around the isthmus, which is the area just beyond both the left subclavian artery and the ductus arteriosus. It can occur proximal to the left subclavian artery and occasionally even in the abdominal aorta. Collateral vessels develop through the internal mammary and shoulder girdle arteries to the intercostal arteries and can become very large and even palpable. In all the vessels proximal to the coarctation, there is hypertension and increased pulse pressure as well as dilatation. There is a very low systolic pressure and pulse pressure beyond the coarctation.

The aortic valve is commonly bicuspid and regurgitant, but is occasionally stenotic.

Preductal coarctation is commonly associated with diffuse narrowing of the aortic arch, pulmonary hypertension, and an Eisenmenger syndrome, as well as with differential cyanosis (see p. 18). This is sometimes known as the infantile type, because patients rarely live beyond infancy.

compliance Elastic resistance or stiffness of a structure, e.g., the stiffer the left ventricle, the less the compliance. To physicists it is change of volume/change of pressure. Generally, a thick ventricle is a noncompliant or stiff ventricle.

constrictive pericarditis Thickening of the pericardium by dense, fibrous tissue that may calcify. It results primarily in restriction of expansion of the heart but also often causes a slight restriction of systole as well, especially if the duration of the constriction is long enough to allow much infiltration of the epicardium by the fibrous tissue. In past decades the most common cause was tuberculosis. The most common causes today are idiopathic, viral, bacterial, radiotherapy of the chest, and hemopericardium resulting from trauma.

Restrictive cardiomyopathy mimics constrictive pericarditis, but restrictive disease is more likely to have cardiomegaly (displaced apex beat), and its S3 is more likely loudest at the apex. In constriction the S3 may be loudest at the left lower sternal border.

cor pulmonale Right ventricular hypertrophy secondary to a lung abnormality, such as primary pulmonary hypertension. The term does not require that the patient be in right ventricular failure.

ductus arteriosus See persistent ductus arteriosus.

Ebstein's anomaly A downward-displaced, deformed tricuspid valve. One leaflet is displaced into the right ventricle (RV) so that some RV is in the right atrium. There is commonly tricuspid regurgitation, which can enlarge the outflow tract of the RV. The right atrium may be so large that it dominates the ECG (tall peaked P waves) and X-ray picture. A right-to-left shunt through an atrial septal defect or patent foremen ovale with resultant cyanosis is common. Atrial arrythmias or heart failure are the most common complications.

Eisenmenger syndrome or reaction Severe pulmonary hypertension due to high and fixed pulmonary arteriolar resistance caused by a large left-to-right shunt due to a ventricular septal defect (VSD), atrial septal defect (ASD), or persistent ductus arteriosus (PDA). The high right ventricular and right atrial pressures result in a right-to-left shunt through the ASD, VSD, or PDA. The right-to-left shunt may be dominant or it may be a balanced shunt, i.e., as much left-to-right as right-to-left. When a VSD is the cause, the term Eisenmenger complex is often used, because this is the original lesion described by Eisenmenger in 1897 [1]. The Eisenmenger syndrome usually begins in infancy when a PDA or VSD is responsible; it begins in the teens or later when the shunt is an ASD. The pulmonary hypertension of Eisenmenger syndrome with cyanosis is irreversible and prohibits surgical closure of the defect. A patient with irreversible pathological changes in the lung vessels (plexiform lesions) due to an Eisenmenger reaction is often said to have "pulmonary vascular disease."

ejection fraction Relationship between stroke volume (volume ejected) and end-diastolic volume (volume at the moment of greatest filling of the ventricle at the end of diastole). It is the volume at the end of diastole minus the volume at the end of systole divided by the volume at the end of diastole, times 100. (The normal range is 70 ± 10%.)

endocardial cushion defect See atrial septal defect.

endocarditis See infective endocarditis.

Fallot's tetralogy See tetralogy of Fallot.

filling pressure The pressure in the ventricle that distends it, especially toward the end of diastole, so that it is most related to the end-diastolic pressure in the ventricle. The change in volume that produces this diastolic pressure is called the preload. It is controlled on the right side by venous pressure and, in sinus rhythm, also by the power of the right atrial contraction. On the left side it is controlled by the left atrial pressure and, in sinus rhythm, also by the power of left atrial contraction or the "atrial kick."

gradient Difference in pressure along a conduit that results in flow from highest to lowest pressure. In cardiology, it usually refers to a difference in pressure across an obstruction, i.e., it is generally caused by a drop in pressure across an obstruction (usually a stenotic artery or valve), so that the pressure is higher proximal than distal to the obstruction.

hypertelorism Widely set eyes found in such syndromes as pulmonary stenosis with atrial septal defect and with supravalvular aortic stenosis.

hypertrophic obstructive cardiomyopathy Disproportionate hypertrophy of the septum that causes obstruction in midsystole as the anterior or septal mitral leaflet draws toward the septum in systole. (See figures on p. 203 for hemodynam-ics). The disproportionate septal hypertrophy has engendered the term asymmetric septal hypertrophy. It was called idiopathic hypertrophic subaortic stenosis in the first extensive report, but the term idiopathic seems an unnecessary appendage [2]. Asymmetric septal hypertrophy (commonly called ASH) does not necessarily imply obstruction to outflow, since ASH can occur without obstruction. Therefore, hypertrophic obstructive cardiomyopathy (HOCM) should be used when referring to a patient with obstruction due to asymmetric septal hypertrophy.

infective endocarditis An infection usually of regurgitant heart valves or of certain congenital defects that cause regurgitant or retrograde flows, such as ventricular septal defect or persistent ductus arteriosus. In former years, the infection was nearly always bacterial and thus the condition was called bacterial endocarditis. Because it could last for as long as 2 years before the diagnosis was made, it was known as subacute bacterial endocarditis. Today, fungi and Rickettsia are the causative organisms in a significant proportion of infections. Therefore, infective is a more embracing term.

The diagnosis used to be considered when there was fever and "changing murmurs." Because a regurgitant murmur is the only murmur likely to develop when a valve is destroyed, the term changing should be modified to mean a new or increasing regurgitant valvular murmur. Patients often have had recent dental work or other surgery and may present with severe night sweats, back pain, cerebral vascular accident, or other embolic phenomena.

inflow and outflow tract of the left ventricle The inflow tract of the left ventricle is the area just below the mitral valve. The outflow tract is made up of the septum anteromedially and the anterior or septal leaflet of the mitral valve, plus their chordae laterally. (See figure on p. 207.)

infundibulum Outflow tract of the right ventricle made up mostly of muscle called the crista supraventricularis. It is much like the spout of a teapot, the body of the right ventricle being the pot. (See figure on p. 207.)

intermittent claudication Pain in ischemic working muscle produced by certain metabolites; classically, pain in the legs due to inadequate arterial supply during walking. If the obstruction is high in the aortoiliac area, the pain may be in the hip or buttock. However, the pain may be felt in unusual sites, such as in the thighs or the arch of the foot. When the ischemia is due to thrombosis of the lower aorta (chronic aortoiliac occlusion), impotence and leg weakness may occur as well (Leriche's syndrome) [3]. If the celiac or mesenteric arteries are involved, the pain after meals is called "abdominal angina."

Note: Intermittent claudication is not related to the nocturnal leg or foot muscle cramps that occur in bed.

ischemia (pronounced is-ke-mi-ah) Inadequate blood supply to a part of the body.

isovolumic contraction The rise in LV pressure between closure of the mitral valve and opening of the aortic valve. This used to be called isometric contraction, but because the measurements of the ventricle change while the volume does not, the term isovolumic is more accurate.

isovolumic relaxation The fall in LV pressure between closure of the aortic valve and opening of the mitral valve.

Note: Isovolumic relaxation is an active process, i.e., the ventricle is capable of generating a negative pressure or suction" effect.

left lateral decubitus position Body position in which the subject is horizontal and lying on the left side.

malpositions of the heart Situs solitus (solitus = "usual") means a normal position of all chambers and vessels of the heart and viscera. In situs inversus or mirror-image dextrocardia all chambers, vessels, and viscera are inverted with the apex, etc. on the right. Dextrocardia or levocardia means that the heart and viscera are discordant and are almost always associated with other cardiac abnormalities. Dextroversion or levoversion means that the heart is in the same position as in dextrocardia or levocardia but the heart is rotated so that the apex is rotated to the left with dextroversion and to the right with levoversion.

Marfan syndrome See index for clinical descriptions in text.

medial sclerosis See arteriosclerosis.

Mueller maneuver An inspiratory effort against a closed mouth and nose or against a closed glottis that decreases intrathoracic pressure. It is the opposite of a Val-salva maneuver.

neurocirculatory asthenia A syndrome occurring in some patients with anxiety neurosis and consisting of palpitations and tachycardias, nondescript chest pains, shortness of breath, chronic fatigue, and other signs of sympathetic overactivity. It has been called DaCosta's syndrome (American Civil War), "soldier's heart" (World War II), "effort syndrome," "neurotic heart syndrome," "cardiac neurosis," and "vasoregulatory asthenia." If these patients are chronic hyperventilators, their breath-holding time will be less than 20 s.

outflow tract See inflow and outflow tract of the left ventricle.

patent ductus arteriosus See persistent ductus arteriosus.

pectus excavatum Posterior displacement of the lower sternum. It can be slight, or so severe that it not only displaces the heart to the left but also may interfere with cardiac function by raising the right ventricular diastolic pressure and may cause palpitations and dyspnea on strenuous exertions. It is commonly seen in the Marfan syndrome and in patients with the straight back syndrome (see p. 196). The three degrees of severity have been described as the "saucer," "cup," and "funnel."

persistent ductus arteriosus (PDA) Usually incorrectly called patent ductus arteriosus. The word ductus itself implies patency [4]. It refers to an opening between the aorta and pulmonary artery in which flow occurs between the higher-pressure aorta and the lower-pressure pulmonary artery. Thus some of the blood ejected by the LV into the aorta passes into the pulmonary artery, resulting in a left-to-right shunt. A volume overload occurs where the shunted blood circulates, i.e., in the pulmonary artery, pulmonary veins, left atrium, and LV. In uncomplicated PDA, the right-sided chambers should be normal.

A PDA represents persistence of the fetal ductus arteriosus that is designed to bypass the lungs in fetal life, i.e., the high pulmonary artery pressure in the fetus forces blood into the aorta through the ductus. In PDA severe pulmonary hypertension may develop if the fetal arterioles do not involute. When pulmonary hypertension develops, its onset usually occurs early in infancy or childhood. Then the right ventricle remains hypertrophied, resulting in an Eisenmenger syndrome or reaction with right-to-left shunting. If a patient with a large PDA reaches adulthood without developing pulmonary hypertension in infancy, heart failure with acute pulmonary edema may develop. Infective endocarditis can occur as one of the complications of even a small PDA. (See p. 237 under PDA for an explanation of differential cyanosis, which is one of the characteristics of the Eisenmenger reaction in persistent ductus arteriosus.)

postextrasystolic potentiation Increased contractility that occurs in the beat following a premature electrical depolarization of the heart. Although a pause after a premature beat can increase contractility by the long diastole, causing more fill-

The persistent ductus arteriosus is a slightly left-sided structure that usually connects the junction of the main and pulmonary artery to the aorta just distal to the origin of the left subclavian artery. Since normal aortic pressure (120/80 mmHg) is usually higher than pulmonary artery pressure (25/10 mmHg), the shunt flow is normally from aorta to pulmonary artery in both systole and diastole.

The persistent ductus arteriosus is a slightly left-sided structure that usually connects the junction of the main and pulmonary artery to the aorta just distal to the origin of the left subclavian artery. Since normal aortic pressure (120/80 mmHg) is usually higher than pulmonary artery pressure (25/10 mmHg), the shunt flow is normally from aorta to pulmonary artery in both systole and diastole.

ing and a Starling effect, an early depolarization itself produces increased contractility, i.e., contractility that is independent of the length of diastole following the early beat. The cause of this increased inotropism is thought to be a calcium flux phenomenon.

postmyocardial infarction syndrome (Dressler's syndrome [5]) A syndrome consisting of fever, pneumonitis, and painful pericarditis and pleuritis that may occur from about 2 to 11 weeks after myocardial infarction and is probably an autoimmune response to myocardial necrosis. It is only dangerous in the presence of anticoagulants, when it may produce a bloody effusion and tamponade. The syndrome closely resembles the postcardiotomy syndrome and may be recurrent for as long as 2 years.

primary pulmonary hypertension Irreversible pulmonary hypertension of unknown etiology, usually progressing to severe degrees, producing right ventricular hypertrophy and dilatation, as well as main pulmonary artery dilatation and atherosclerosis. It is most common in females under age 40. The small pulmonary arteries and arterioles show intimal fibrosis and proliferation as well as medial thickening.

pulse pressure or volume Amplitude of a pulse. In palpation, it refers to the distance your fingers are moved between the least and the greatest expansion of a vessel. On an arterial pulse tracing, it refers to the distance between the systolic and diastolic pressures.

Raynaud's phenomenon Intermittent constriction of small arteries and arterioles of the fingers, resulting in a change of color, usually produced by cooling but also by sympathetic stimulation of any kind. It begins with blanching, progresses to cyanosis, and often ends with a reactive redness (reactive hyperemia) that may be very painful. It is occasionally a precursor of a collagen or other connective-tissue disease. It is called Raynaud's disease when it is not secondary to trauma or to neurogenic lesions or other systemic disease.

Note: Acrocyanosis is a persistent blueness and coldness of the distal parts of the extremities, probably due to an abnormality of the small vessels.

semilunar valves The aortic or pulmonary valves. Their leaflets are half-moon-shaped (semilunar).

sinus arrhythmia Increase in heart rate with inspiration and decrease with expiration due to vagal inhibition during inspiration. (Remember "in" for "increase" on inspiration.)

sinuses of Valsalva The three bulges or sinuses at the root of the aorta, two of which give rise to the coronary arteries. They help to prevent the open aortic leaflets from occluding the orifice of the coronary arteries. Occasionally, they may be congenitally weak and rupture into adjacent chambers, or they may become aneurysmal, especially in the Marfan syndrome, producing any degree of aortic regurgitation. (See top figure on p. 280.)

Starling effect The effect of the Frank-Starling law of the heart, which states that if the heart muscle is stretched before it contracts, it will contract with more energy. This is equivalent to a bow-and-arrow effect, i.e., the tauter the bow, the farther the arrow will go.

sternal angle, or angle of Louis (pronounced Loo-ee) The first protuberance or hump in the sternum below the suprasternal notch. It is at the junction of the manubrium and body of the sternum. It marks the point where the second costal cartilage joins the sternum. Below this cartilage is the second intercostal space. (See bottom figure on p. 280.)

The right coronary sinus is anterior when viewed from above, but it is called the right coronary sinus, probably because it gives rise to the right coronary artery.

The sternal angle is one of the most important landmarks in cardiology, not only because it is the zero level for jugular pressures but also because it is the only accurate way to find the fourth right interspace in order to place the V1 electrode for an ECG.

Stokes-Adams attack or Adams-Stokes attack An episode of syncope secondary to complete AV block (see atrioventricular block). Any cerebral symptom secondary to complete AV block may probably also be considered a minor degree of Stokes-Adams attack because it may presage syncope and death. Syncope secondary to any other arrhythmia should probably be called cardiac syncope and not a Stokes-Adams attack.

subclavian steal Use of a vertebral artery as collateral circulation to feed a subclavian artery beyond an obstruction (usually on the left). Blood from a vertebral artery flows retrogradely into the distal subclavian, thus "stealing" blood from the brain.

If the left subclavian is blocked proximal to the entrance of the left vertebral artery, exercise of the ischemic left arm causes blood in the right vertebral artery to flow via intracerebral vessels into the left vertebral, which then flows retrogradely downward into the subclavian beyond the obstruction. The blood drawn from the cerebral circulation causes cerebral ischemia with resultant vertigo, ataxia, or dysarthria.

tamponade Restriction of cardiac diastole caused by fluid in the pericardium. Because the pressure in the pericardium is equal to venous pressure, the patient with tamponade must of necessity have a high venous pressure and usually also have peripheral edema when the tamponade becomes severe. Pericardial effusion without a high venous pressure is not tamponade. A combination of fluid and solid pericardial material causing constriction is called effusive-constric-tive pericarditis. A useful method of detecting pericardial fluid is to listen to the heart sounds and murmurs first lying supine, then while the patient is on his or her stomach and propped up on the elbows. If there is fluid gravitating anteriorly, the sounds become softer, instead of louder as in normal subjects.

If the left subclavian is blocked proximal to the entrance of the left vertebral artery, exercise of the ischemic left arm causes blood in the right vertebral artery to flow via intracerebral vessels into the left vertebral, which then flows retrogradely downward into the subclavian beyond the obstruction. The blood drawn from the cerebral circulation causes cerebral ischemia with resultant vertigo, ataxia, or dysarthria.

tetralogy of Fallot The most common congenital heart abnormality, it refers to a large ventricular septal defect, pulmonary stenosis, an overriding aorta, and right ventricular hypertrophy (RVH). Because pulmonary stenosis will always lead to RVH, the latter is a necessary result of the other congenital lesions and not really part of the basic abnormality.

The pulmonary stenosis may be either valvular, infundibular, or both, and is the cause of the murmur. The enlargement of the aorta by virtue of receiving blood from both ventricles may contribute to the overriding.

A right aortic arch is relatively frequent. Its pulsation should be sought just below the right sternoclavicular joint, which itself may be felt to pulsate.

thrill A vibratory sensation similar to what is felt when touching the head and neck of a purring cat. A long thrill is merely a palpable murmur and signifies that the murmur is at least grade 4/6 in loudness. A short thrill on the chest wall may be a juxtaposition of split heart sound vibrations. A short thrill felt on the carotid artery may be due to a mid-systolic dip or a minor degree of bisferiens pulse.

transposition of the great vessels It is often called "complete" transposition of the great vessels and means that the anteroposterior relationship of the aorta and pulmonary arteries is reversed, i.e., instead of the aortic root being posterior to the pulmonary artery, it is anterior (and often to the right). This results in the right ventricle giving rise to the aorta and the left ventricle giving rise to the pulmonary artery. One or more abnormal communications between the systemic and pulmonary circulation must exist for the patient to survive. Mixing may occur through either an atrial septal defect, a ventricular septal defect, a persistent ductus, or large bronchial arteries.

Cyanosis is usually present either from birth or within a few days of birth. The most common cause of cyanosis combined with shunt vascularity in the lungs on X-ray is transposition of the great vessels, especially if it is accompanied by congestive failure in infancy.

Turner's syndrome Female phenotype consisting of short stature, receding chin, webbed neck, low hairline over the back of the neck, broad shield chest resulting in widely separated nipples, exaggerated carrying angle, sparse axillary and pubic hair, lymphedema of the lower extremities (in infancy), and a short fourth metacarpal. It is sometimes simply described as short stature, neck webbing, and sexual infantilism in a female with a sex chromosome abnormality (absence of one of the two sex chromosomes). Coarctation of the aorta is the most common associated cardiovascular lesion. When patients with such an appearance have normal sex chromosomes and also have hypertelorism with a slight antimongoloid slant to the eyes as well as ptosis of the upper lids and exophthalmos, especially if the above physical characteristics are found in a male, they are likely to have pulmonary stenosis and are said to have Noonan's syndrome.

Valsalva maneuver (See also the section on blood pressure response on pp. 59-61.) A forced expiration against a closed glottis in order to raise intrathoracic pressure. There are several methods for helping patients perform this maneuver.

(1) Have patient blow into an anaeroid manometer until it registers 40 mmHg.

(2) Have patient push his or her abdomen against your hand. (3) Have patient seal his or her lips around his index finger and blow hard on the finger for about 10 s.

The rise in intrathoracic pressure (phase 1) decreases venous return to the heart, thus causing a gradual decrease in heart size, stroke volume, and pulse pressure. There is a tachycardia due to reflex sympathetic stimulation as the blood pressure falls (phase 2). On release of the strain there is a further sudden drop of blood pressure for a few beats because of the almost empty pulmonary venous reservoir (phase 3). The blood that had been dammed up in the venae cavae now pours into the lungs, LV, and aorta. The heart rate now slows and an excessive rise in blood pressure occurs for a few beats due to the reflex sympathetic stimulation caused by the strain that takes a few seconds to be "turned off," plus the effect of the sudden increase in volume distending the carotid baroreceptors.

ventricular aneurysm See aneurysm.

ventricular septal defect An opening or hole in the ventricular septum is the most common congenital lesion. It is most usually found in the translucent membranous portion of the ventricle, a few centimeters below the aortic valve. It may, however, be found in the muscular septum, in the supracristal area leading directly into the pulmonary artery (rare), or posterior and superior to the attachment of the tricuspid valve to the membranous septum and therefore may lead directly into the right atrium (Gerbode defect). Ventricular septal defect shunts occur mostly during systole from left to right, i.e., from the left to the right ventricle, and produce volume overloads in the right ventricle, pulmonary artery, pulmonary veins, left atrium, and left ventricle.

The defect varies in size from pinpoint to slightly more than a centimeter in diameter. When it involves the entire septum, a single ventricle is produced. When multiple muscular defects are present, it is known as a Swiss cheese defect. Unless the defect is very large, the hole may close in the first few years of life, often with a membranous septal pouch or aneurysm. (See figure on p. 284.)

A dreaded complication of large defects is severe pulmonary hypertension, which may become irreversible. When it becomes severe enough to reverse the shunt, this is known as an Eisenmenger complex (see Eisenmenger syndrome), and usually occurs within the first years of life.

Wolff-Parkinson-White (W-P-W) preexcitation This refers to an atrioventricular bypass pathway in which an abnormal conduction pathway between the atrium and ventricle bypasses the AV node, thus shortening the P-R interval and producing a wide QRS.

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Pulmonary veins

In most ventricular septal defects, only the right atrium is spared the volume overload caused by the left-to-right shunt. In the rare left ventricle-to-right atrium type of ventricular septal defect, however, all cardiac chambers are volume-overloaded. In this figure, the black portion of the arrow represents the shunt flow, and the dotted portion represents the normal flow that comes from the venae cavae.

Membranous VSD

Pulmonary veins

In most ventricular septal defects, only the right atrium is spared the volume overload caused by the left-to-right shunt. In the rare left ventricle-to-right atrium type of ventricular septal defect, however, all cardiac chambers are volume-overloaded. In this figure, the black portion of the arrow represents the shunt flow, and the dotted portion represents the normal flow that comes from the venae cavae.

xanthoma A cholesterol-filled nodule found either subcutaneously or over a tendon. Tuberous xanthomas are subcutaneous xanthomas on the extensor surfaces of the extremities. They are associated with an increase in coronary disease and high blood levels of both cholesterol and triglycerides. They are most commonly found in patients with type III (high-IDL) hyperlipoproteinemia but also with type II (high cholesterol) or type IV (high triglycerides). Since they are under the skin, their yellow pigment is visible. Tendon xanthomas are too deep to impart any change of color to the skin.

eruptive xanthomas Tiny yellowish nodules 1-2 cm in diameter on an erythema-tous base and found mostly on pressure areas. They are often transient and vary with the degree of hypertriglyceridemia, with which they are associated. The triglyceride level is usually at least 1000 mg per 100 mL, no matter what the cause, whether diabetes or pure type I hyperlipoproteinomia. There is some correlation with coronary disease.

palmer xanthomas Very small xanthomas found in the palmer crease and probably representing an early stage of the tuberous type.

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