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Persistent Truncus Arteriosus, Aortopulmonary Septal Defect, and Hemitruncus Arteriosus

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Persistent Truncus Arteriosus, Aortopulmonary Septal Defect, and Hemitruncus Arteriosus .ANOMALIES OF AORTOPULMONARY SEPTATION: PERSISTENT TRUNCUS ARTERIOSUS, AORTOPULMONARY SEPTAL DEFECT, AND HEMITRUNCUS ARTERIOSUS R.M. Freedom Persistent Truncus Arteriosus Incidence Persistent truncus arteriosus, also called com­ The Report of the New England Regional Infant mon aorticopulmonary trunk, is that anomaly Cardiac Program indicated that 33 of the 2251 where a single arterial trunk supported by the infants with heart disease or 1.4% had truncus ventricular mass gives origin to the coronary arteriosus [28]. The data also provided pre­ arteries, pulmonary trunk, and the brachio­ valence at livebirth of 0.030 per 1000 livebirths cephalic arteries [2, 9, 13, 14, 16, 17, 18, 52, 57, for this lesion. The prevalence at livebirth data 60, 69, 71, 72, 73, 74]. With very rare exception from the more current Baltimore-Washington a ventricular septal defect is present [14, 15]. Infant study was 0.056 per 1000 livebirths [25] Untreated, this condition carries a very high and the prevalence of this specific lesion is not mortality, with many babies dying in the neona­ stated in the survey conducted by the Alberta tal period or early infancy in congestive heart Heritage Pediatric Cardiology Program [30]. failure, often with an ischemic myocardium. Data from The Hospital for Sick Children in About 65% of patients treated medically fail to Toronto gave an incidence for truncus arterio­ survive beyond 6 months of life, and more than sus of 0.7% of congenital heart disease. 90% die before one year of age [14, 28, 34, 41, 47, 74]. Data from Butto et al. [13], Collett and Edwards [17] and Van Praagh and Van Praagh [74] summarized by Stanger [66] suggest that of 100 babies born with truncus and surgically Morphology and Morphogenesis untreated, 20 will die in the first week and at least 86 will have died by a year of age. A rare patient will survive with pulmonary vascular Three hypotheses have been used to explain the obstructive disease into the fourth decade of life development of this lesion: 1) This defect or beyond. represents partial or total absence of the aortico- R. M. Freedom et al., Neonatal Heart Disease © Springer-Verlag London Limited 1992 430 NEONATAL HEART DISEASE Fig. 26.1. Classification of truncus·arteriosus [17]. A single arterial trunk is supported by the ventricular mass, and this trunk gives origin to the coronary arteries, the pulmonary arteries, and the brachiocephalic arteries. With rare exception a ventricular septal defect is present. The bottom right is so-called truncus, Type 4, and there is discussion whether this is a true entity. TR = truncal root; AO = ascending aortic portion of truncus; PA = pulmonary artery; c = collaterols. Not shown is the form with coarctation or interruption. pulmonary septum, complete absence of fusion ate entirely above the right ventricle [3, 9, 14, of the truncal swellings and the conal ridges 16, 17, 18, 56, 60, 69, 71, 73, 74]. which normally join to form the arterial valves and the distal infundibulum. 2) Van Praagh and his colleagues consider truncus arteriosus to be Classification of truncus arteriosus closely related to tetralogy of Fallot and thus truncus arteriosus represents absence or severe The classification proposed by Collett and Ed­ attenuation of the distal pulmonary infundibu­ wards in 1949 [17] has been widely accepted, lum, with defects of the aortopulmonary sep­ although in the past 15 years some modifica­ tum and ventricular septum. Anderson and tions have been suggested. Calder and her Thiene [1] take issue with the view espoused colleagues have made a classification focusing by Van Praagh [73a] that tetralogy and com­ both on the presence of a ventricular septal· mon arterial trunk are morphologically closely defect (Type A) or its absence (Type B), as well related. 3) This defect represents the absence of as on the site of origin of the pulmonary arteries a common arterial vessel downstream from the [14] (Fig. 26.1). Type Al has a partially separate semilunar valve [14, 57, 72, 73, 74]. main pulmonary trunk because of the presence of an incompletely formed aortopulmonary sep­ tum (Fig. 26.2). In Type A2, the aortopulmonary Segmental Analysis septum is absent and the pulmonary arteries originate separately from the truncus. Type A3 Hearts exhibiting persistent truncus arteriosus is characterized by absence of either the right or are usually left-sided and the atrial situs is left pulmonary artery. The "absent" pulmonary sotitus. With rare exception, the hearts are artery will originate from either a ductus arter­ biventricular, the atrioventricular connections iosus or a major aortopulmonary collateral [9, are concordant, and there is fibrous continuity 17, 56, 60, 71, 74]. Those patients with truncus between the mitral valve and the truncal valve. arteriosus and either interruption of the aortic Occasionally, the single arterial root will origin- arch, atresia of the aortic arch, preductal coarc- ANOMALIES OF AORTOPULMONARY SEPTATION: PERSISTENT TRUNCUS ARTERIOSUS 431 A B C D Fig. 26.2. A Truncus (TRU) with its typical ventricular septal defect (white') viewed from the right ventricle. Note the short main pulmonary trunk bifurcating (arrows) into right and left pulmonary arteries. B Same heart seen from left ventricle. C Separate origins of pulmonary arteries (black'). D Truncus with interruption of aortic arch. The ascending aorta (asc ao) component of the truncus is relatively small. The ventricular septal defect is large (white') and there are separate origins of the pulmonary arteries (black'). The ductus arteriosus (') has constricted and is in continuity with the descending aorta. tation of the aortic arch, or severe diffuse pid, and hexacuspid valves are very uncom­ hypoplasia of the aortic arch are classified as mon. Amongst the 54 cases reported from the Type A4 (Fig. 26.1). United Hospital in Minneapolis, 42% had a tricuspid truncal valve, 30% a bicuspid valve, and 24% a quadricuspid valve [13]. Calder and The Truncal Valve her colleagues [14] from Boston Children's Hospital reported that 61% had a tricuspid The truncal valve can theoretically possess one truncal valve, 31 % a quadricuspid valve, and to six cusps, but the unicommissural, pentacus- 8% a bicuspid valve. 432 NEONATAL HEART DISEASE septal defect results from absence of the infun­ dibular septum and is found in the limbs of the trabecula septomarginalis (Fig. 26.4). Ventricu­ lar septal defects may be present remote from this area but are uncommon. The infundibular ventricular septal defect is usually large but may be potentially restrictive, especially in those instances where the truncus originates almost entirely above the right ventricle [55]. Pulmonary Stenosis Most patients with persistent truncus arteriosus have excessive pulmonary blood flow and, when untreated, die in congestive heart failure. In about 10% of patients with truncus arter­ A iosus, the pulmonary blood flow is restrictive. In most of these patients the mechanism res­ ponsible for limiting the pulmonary blood flow is ostial stenosis resulting from an obstructive truncal leaflet [9, 13, 14, 56, 73, 74] . The Coronary Arterial Circulation The coronary arteries exhibit a variable pattern of origin independent of the number of truncal valve leaflets [la, 21a, 61, 69, 74]. Abnormally high origin of a coronary artery, usually the right, has been observed in this disorder. Frank myocardial ischemia is usually the result of the disordered hemodynamics with a torrential pulmonary blood flow, a volume loaded left ventricle, and a low aortic diastolic or coronary artery driving pressure. Rarely, a coronary B ostium will be congenitally stenotic and this will Fig. 26.3. A and B Severely stenotic, quadricuspid truncal promote an ischemic myocardium. Uncommon­ valve in two different patients. Little commissural fusion is ly, the coronary ostium will be just superior to present. the truncal valve commissure, and a bulky fleshy truncal valve leaflet may obstruct the coronary ostium, resulting in coronary insuf­ The truncal valve may guard the ventricu­ ficiency. loarterial junction normally, or the valve may be stenotic or regurgitant, or both. Truncal valve leaflet thickening may be mild, moderate, or Laterality of the Aortic Arch severe (Fig. 26.3). In this last group the truncal valve may be nodular, polypoid and myxoma­ A right-sided aortic arch is present in 20%-30% tous [7, 12, 13, 14, 22, 29, 37, 38,48, 74]. Severe of patients with truncus arteriosus [13, 14, 69, truncal valve regurgitation has been reported as 74] . An occasional patient has a double aortic a very serious complication [21]. arch. The Ventricular Septal Defect Obstructive Anomalies of the Aortic Arch With very rare exception a ventricular septal Coarctation of the aorta, aortic arch atresia, and defect is present [9, 14, 15]. This ventricular interruption of the aortic arch, usually Type B, ANOMALIES OF AORTOPULMONARY SEPTATION: PERSISTENT TRUNCUS ARTERIOSUS 433 B A Fig. 26.4. Ventricular septal defect in truncus arteriosus (TRU). ALarge ventricular septal defect (white') cradled in limbs of trabecular septum in a baby with associated interruption of aortic arch. B Slightly smaller ventricular septal defect (white'). Note the thickened, fleshy, truncal valve (black'); pa = pulmonary artery. C Same specimen as in B viewed from the left ventricle; mv = mitral valve. C can complicate the basic morphologic expres­ 13, 14]. Crossed pulmonary arteries have been sion of truncus arteriosus. In this setting, the described in truncus arteriosus, usually but not ductus arteriosus is usually present [9, 13, 14, invariably with associated interruption of the 17, 18, 19, 46, 57, 68, 72, 74]. aortic arch [6, 36, 78]. Uncommon Lesions Clinical Features Persistent truncus arteriosus has been described in patients with associated complete form of atrioventricular septal defect, tricuspid atresia, Many patients with truncus arteriosus present single ventricle, hypoplastic left ventricle, and with features of congestive heart failure or mild total anomalous pulmonary venous return [9, cyanosis, or both, in the newborn period.
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