Truncus Arteriosus

Truncus arteriosus was recognized in 1798,78 and the clinical and necropsy manifestations were described in 1864.10 Humphreys summarized the cases reported up to 1932,39 and Lev and Saphir critically reviewed published accounts during the following decade.46 The malformation accounts for approximately 1 to 2 of cases of congenital heart disease at necropsy and approximately 0.7 to 1.2 of all congenital cardiac malformations.13'54'69'79'87 In truncus arteriosus, a single great artery with a...

Uhls Anomaly

Parchment heart was so designated by Osler in 190592 and was redescribed by Segall in 1950.114 Two years later, X-ray from a symptomatic cyanotic female neonate with Ebstein's anomaly. The huge cardiac silhouette covers all but a fraction of oligemic lung fields. Cardiomegaly is due to a huge right atrium (RA) and the atrialized right ventricle (ARV). Diagnosis was confirmed at necropsy. X-ray from a symptomatic cyanotic female neonate with Ebstein's anomaly. The huge cardiac silhouette covers...

Situs Solitus Definition

In aortic regurgitation, 96, 97f in Taussig-Bing anomaly, 397, 397f in total anomalous pulmonary venous connection, 273, 274f-275f Salicylate(s), patent ductus arteriosus related to, 408 Scimitar sign, in total anomalous pulmonary venous connections, 274-275 Scimitar syndrome, in atrial septal defect characteristics of, 235-236, 238f-239f, 240 x-ray of, 253 Seckel syndrome, 289 Septal defect(s). See Atrial septal defect(s) Atrioventricular septal defect(s) Ventricular septal defect(s)....

Cor Triatriatum

Partition of the left atrium into two compartments was recognized by Andral in 1829.5 Four decades later, Church published the first detailed pathologic description of the malformation13 that Borst (1905) called cor triatriatum.9 The anomaly is rare, with a prevalence of about 0.1 of cases of congenital heart disease.60,85 Cor triatriatum is characterized by a membrane that partitions the left atrium into a proximal accessory chamber that receives the pulmonary veins and a distal true left...

Normal Continuous Murmurs The Venous Hum

The venous hum was described by Potain in 186755 and is the most common type of normal continuous murmur. It is universal in children and occurs in normal young adults26,33 even in the absence of thyrotoxicosis, anemia, or pregnancy.52 Maximal intensity is in the supraclavicular fossa just lateral to the sternocleidomastoid muscle. The hum may radiate widely and is often bilateral but is usually more prominent on the right (Fig. 2-11). A loud venous hum, especially in children, may radiate...

Congenital Coronary Arterial Fistula

Coronary arterial fistulas are the most frequent hemody-namically significant congenital malformations of the coronary circulation,6'68'69'75'99'132'141 comprising 14 of congenital coronary artery anomalies (see Chapter 32).141 In this anomaly' the right and left coronary arteries arise from their appropriate aortic sinuses' but a fistulous branch of one or more than one of the two coronary arteries drains into a cardiac chamber or into the pulmonary trunk' coronary sinus' vena cava' or a...

Precordial Movement And Palpation

In 1934' Roesler called attention to the conspicuous thrust of the right ventricle in atrial septal defect.357 The impulse is hyperdynamic but not sustained because the volume-overloaded right ventricle contracts vigorously and empties rapidly into a low resistance pulmonary vascular bed.136'317'335 The impulse is especially prominent at the left sternal border during held exhalation' and in the subxiphoid area during held inspiration.335 Anterior X-ray from a 32-year-old cyanotic woman with an...

Xax

In complete heart block, 49, 50f in pulmonary stenosis, 302, 303f, 304 cannon, in complete heart block, 49, 50f in congenitally corrected transposition of great arteries, 68 in mitral stenosis, 146, 147f in primary pulmonary hypertension, 220 in tricuspid atresia, 488 jugular venous. See Pulse, jugular venous, A waves in. Absence of pericardium, congenital. See Pericardium, congenital absence of. Accrochage, in complete heart block, 52 Air travel, pulmonary hypertension and, 218 Alagille...

The Echocardiogram

Transthoracic and transesophageal echocardiography with Doppler interrogation and color flow imaging exclude coexisting congenital or acquired heart disease that might cause pulmonary hypertension and establish the physiologic and morphologic consequences of elevated pressure in the lesser circulation.39,148,150Intravascular ultrasono-graphy has been introduced as a method of assessing the pulmonary circulation in pulmonary hypertension.146 Echocardiography defines the size of the right atrium...

What Is 75f Systolic Murmur

Gallavardin dissociation in aortic stenosis, 93, 94f aortic systolic murmur of, 11, 11f Gallop du bloc, in complete heart block, 52 Gastrointestinal bleeding, in aortic stenosis, 89 Goldenhar syndrome, Fallot's tetralogy associated with, 360 Gooseneck deformity, in atrioventricular septal defect, 279, 281f, 290 Graham Steell murmur. See Steell, Graham, murmur of. Great arteries. See also Aorta Pulmonary trunk Truncus arteriosus. definition of, 62 designations for, 18 in double outlet right...

P

In aortic arch interruption, 137, 137f-138f in aortic atresia with mitral valve hypoplasia, 575, 575f in aortic stenosis, 96, 97f in atrial septal defect, 249-250, 252f-253f in cardiac malposition, 27-28, 28f-29f dome and dart P wave, 28, 30f inverted, 28, 28f in coarctation of aorta, 128, 128f in complete heart block, 49f, 51f, 52 in cor triatrium, 152, 152f in coronary arterial fistula, 450, 450f in double outlet right ventricle, with subaortic ventricular septal defect with pulmonary...

Congenital Pulmonary Stenosis

Congenital obstruction to right ventricular outflow in hearts with two noninverted ventricles can originate in, below, or above the pulmonary valve. Stenosis originating in the pulmonary valve consists of three morphologic types12 (1) typical mobile dome-shaped, (2) dysplastic, and (3) bicuspid. Dome-shaped pulmonary valve stenosis was described in 1761 by John Baptist Morgagni77 and is characterized by a thin mobile valve mechanism with a narrow central opening at its apex (Fig. 11-1). Three...

Acquired Anomalies Of Coronary Arteries Secondary To Congenital Heart Disease

Coarctation of the aorta is associated with extramural and intramural abnormalities of the coronary arteries (see Chapter 8). Systemic hypertension causes intimal proliferation and medial thickening and is a risk factor for premature coronary atherosclerosis.131'154'155 Luminal size increases in proportion to medial thickness.88'155 Intramural coronary arteries and arterioles have thick walls, a rich adventitia, and dense collagen and elastic fibers.131 Supravalvular aortic stenosis (see...

Physical Appearance

Birth weights in infants with complete transposition are on average greater than normal, with a substantial proportion above 8 pounds,5,85 in contrast to newborns with other forms of congenital heart disease, who average less than normal birth weights for gestational age.58 The illusion of robust health is soon dispelled by the catabolic effects of congestive heart failure (Fig. 27-10).58,74 Increased anteroposterior chest dimensions are associated with hyperinflation of the lungs (see The...

Anomalies Of Aortic Origin Table 322

Congenital anomalies of the coronary arteries are included in comprehensive clinical and necropsy reviews.3'4'45'53'81'119 The widespread use of selective coronary angiography has helped establish the types and prevalence of many of these anomalies.25'43'45'53'60'72'84 Among 13'010 adults who underwent coronary angiography' 0.61 had congenital anomalies'43'146 and the incidence was 1.3 among 126'595 patients who underwent coronary arteriography at the Cleveland Clinic.159 Transesophageal...

Congenital Anomalies Involving The Coronary Sinus Table 325

The coronary sinus is the site of a variety of congenital abnormalities.8'91'137 Partial or complete absence results from absence of the roof' from absence of the entire coronary sinus' or from an atrial septal defect that occupies the site of the ostium of the coronary sinus (see Chapter 15). Hypoplasia of the coronary sinus is a response to diversion of blood from the sinus into dilated thebesian veins. Stenosis or atresia of the ostium is characterized by a coronary sinus that is hypoplastic...

The Arterial Pulse

Forceful carotid and suprasternal notch pulsations become increasingly apparent with age and exercise because of the disproportionate systolic hypertension associated with stiffness of the precoarctation aortic wall (see earlier).97,165,193 Abnormal differences in upper and lower extremity arterial pulses and blood pressure are hallmarks of coarctation of the aorta. If proper attention were paid to these physical signs, few or no diagnoses would be missed.169,233 Two methods have been advocated...

Atrial Septal Defect Simple and Complex

Chf Congestive Heart Failure Patient

Galen knew of the foramen ovale and its normal postnatal closure.123 Leonardo da Vinci wrote, I have found from left auricle to right auricle the perforating channel, and his subsequent account a true atrial septal defect is believed to be the first record of a congenital malformation of the human heart.145 Botallo described a persistent foramen ovale after birth but without understanding its function in the fetus.123 Gassendi composed an entire treatise based on observations of a patent...

Auscultation Sounds Congenital Single Ventricle

Nitrite Shunt

The physiology of Fallot's tetralogy is nicely reflected in the accompanying auscultatory signs. Ejection sounds originate in a dilated aorta (see Figs. 18-6A and 18-7A) and are therefore important auscultatory signs of severe pulmonary stenosis or pulmonary atresia (Fig. 18-19 (see Figs. 18-21 and 18-23 thru 18-25).133'136'211'212 The aortic ejection sound is maximum at the upper right sternal border but when loud' is heard along the left sternal border and toward the apex. The ejection sound...

References

In Nelson's Loose Leaf Medicine, vol 5. New York, Thomas Nelson and Sons, 1932. 2. Agustsson MH, Arcilla RA, Bicoff JP, et al Spontaneous functional closure of ventricular septal defects in 14 children demonstrated by serial cardiac catheterizations and angiocardiography. Pediatrics 31 958, 1963. 3. Albers HJ, Carroll SE, Coles JC Spontaneous closure of a membranous ventricular septal defect. Necropsy finding with clinical application. Br Med J 2 1162, 1962....

Congenital Pulmonary Arteriovenous Fistula

Chest Ray Fistula

In 1897, Churton described the necropsy findings of congenital pulmonary arteriovenous fistulae in a 12-year-old boy.31 Four decades later, the anomaly was recognized in a living subject.113 Pulmonary arteriovenous fistulae are the result of a developmental fault in the vascular complex that is responsible for pulmonary arteries and veins.6'84 The fis-tulae can be solitary or multiple, unilateral or bilateral, or minute and diffuse throughout both lungs.* Approximately 75 of congenital...

Coarctation of the Aorta

Elevation Effects Bicuspid Valve

In 1760, the Prussian anatomist Johann Friedreich Meckel characterized coarctation of the aorta as an extraordinary dilatation of the heart which came from the fact that the aortic conduit was too narrow.104Jarcho's informative historical papers underscored the clarity of early accounts of this congenital malformation.103-107 Coarctation of the aorta is typically located near the aortic attachment of the ligamentum arteriosum or patent ductus arteriosus (Figs. 8-1A and 8-2). An obtuse...

The Jugular Venous Pulse

The jugular venous pulse is elevated under two widely divergent physiologic circumstances. The first and most Physical appearance of a 15-month-old boy with complete transposition of the great arteries. Birth weight was 9 pounds 2 ounces. The child's head is large, although his body and arms have lost the robust appearance with which he was born. Physical appearance of a 15-month-old boy with complete transposition of the great arteries. Birth weight was 9 pounds 2 ounces. The child's head is...

The History

Congenital Heart Disease Cxr

Situs inversus with dextrocardia and a structurally normal heart is usually discovered by chance in a chest x-ray which is often considered normal because the film is inadvertently reversed when first read. There is a reported tendency for left handedness in complete situs inversus,22 but Matthew Baillie wrote The person seems to have used his right hand in preference to his left . . . which was readily discovered by the greater bulk and hardness of that hand as well as the greater fleshiness...

C

A, Illustrations of the various types of aortic valve stenosis. Illustrations on the left represent three types of congenitally abnormal aortic valves. The upper drawing shows a unicuspid acommissural valve. The middle drawing shows a unicuspid unicommissural valve with an eccentric orifice. The lower group of four drawings shows a functionally normal bicuspid aortic valve (upper center), a fibrocalcific stenotic bicuspid aortic valve (right), a bicuspid aortic valve that is inherently stenotic...

Coarctation Emotion

Coarctation of the aorta will not be overlooked if attention is paid to the upper and lower extremity arterial pulses and blood pressure during routine physical examination. The diagnosis is entertained in patients with systemic hypertension and reduced or absent femoral pulses. Disproportionate systolic hypertension heightens suspicion. Minor symptoms include headache, epistaxis, and leg fatigue. Major complications include congestive heart failure, especially in infants, rupture or dissection...

Auscultation

A soft first heart sound is a sign of the PR interval prolongation of first-degree heart block. Variation in intensity of the first heart sound is a sign of complete heart block (see Chapter 4). An ejection sound at the left base (see Fig. 6-13B) originates in the anterior aorta, especially when the aortic root is dilated (see Fig. 6-9). A short, soft basal midsystolic murmur originates in the anterior aorta but may also originate in the posterior pulmonary trunk across the deeply wedged...

Congenital Aortic Regurgitation

Aorta Fistula

The classic features of chronic severe aortic regurgitation have been familiar to clinicians since Corrigan's treatise in 1832 On Permanent Patency of the Mouth of the Aorta, or Inadequacy of the Aortic Valves.37 The remainder of this chapter deals with pure congenital aortic regurgitation when the regurgitant flow is through an incompetent aortic valve directly into the left ventricle (Tables 7-2 and 7-3). Regurgitant flow that reaches the left ventricle through channels other than the aortic...

Goose Neck Deformity

Goose Neck Deformity Angiogram

A large left-to-right shunt is established shortly after birth when total anomalous pulmonary venous connection is accompanied by a nonrestrictive atrial septal defect, low pulmonary vascular resistance, and no obstruction. Dyspnea, cardiac failure, and physical underdevelopment begin shortly thereafter, and the majority of infants do not reach their first birthday. The clinical picture subsequently resembles a nonrestrictive ostium secundum atrial septal defect with the notable exception of...

Summary

Ostium secundum atrial septal defects predominate in females and often come to light in asymptomatic children *See references 13, 39, 56, 58, 96, 121, 131, 216, 331, 353, 390. or young adults. Patients usually reach their fourth decade with little or no handicap. Preadolescents sometimes appear delicate and gracile. Dyspnea, fatigue, and recurrent lower respiratory infections are common. The left-to-right shunt in older adults is augmented by ischemic heart disease, systemic hypertension, and...

Percussion and Palpation

A right anterior chest bulge with asymmetry arouses suspicion of dextrocardia. Percussion and palpation are useful in the clinical recognition of cardiac malpositions because these physical signs are influenced by the malposition per se, and establish the right or left thoracic location of the heart and the abdominal location of hepatic dullness and gastric tympany. If the stomach is not sufficiently air-filled to generate a tympanitic percussion note, a carbonated beverage or deliberate...

Patent Ductus Arteriosus

Hypertrophic Osteoarthropathy

In 1593, Giambattista Carcano described the ductus arteriosus in his book on the great cardiac vessels of the fetus.39 However, Leo Bottali came to be associated with the arterial duct even though Bottali had misapplied the term ductus to the foramen ovale.39 It was not until Rokitansky's handbook of 1844 followed by his beautifully illustrated monograph of 1852 that patent ductus arteriosus was recognized as a specific congenital malformation.133 The incidence of isolated persistent patency of...