Echocardiographic identification of thymic tissue in neonates with congenital heart disease.
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Aorta, Thoracic↗
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Results for “Truncus Arteriosus, Persistent”
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Truncal or aortic valve stenosis in functionally single arterial trunk. A clinical, hemodynamic and pathologic study of six cases.
Stenosis of the semilunar valve in the presence of a functionally single arterial trunk is uncommon. Three patients with truncus arteriosus, two with tetralogy of Fallot and pulmonary atresia and one with pulmonary atresia and intact septum were diagnosed as having stenosis of the truncal or aortic valve on the basis of clinical, echocardiographic, hemodynamic and angiocardiographic findings. Echocardiograms consistently showed multiple diastolic closure lines and abnormal semilunar valves in addition to the aortic override in five patients and hypoplastic right ventricle in the patient with pulmonary atresia and intact septum. Peak systolic gradients between the left ventricle and truncus (or aorta) at cardiac catheterization ranged from 20 to 47 mm Hg. Retrograde aortography confirmed a domed and stenotic semilunar valve. Cyanosis was progressive in the three patients with pulmonary atresia. Two patients had arterial anastomosis, and one had a right ventricle-pulmonary arterial graft in addition to aortic valvotomy. One of the three patients with truncus arteriosus underwent complete repair in addition to truncal valvotomy but he died in the post-operative period. The other two patients with truncus arteriosus died of intractable congestive cardiac failure before surgical intervention. It is suggested that the presence of semilunar valve stenosis in these patients adversely affects the prognosis. The myocardium is already jeopardized as a result of hypoxia in pulmonary atresia and left ventricular diastolic overload in patients with truncus arteriosus. The added burden of semilunar valve stenosis may further compromise the functional status of the myocardium.
Angiocardiography↗
Surgical significance of the coronary arterial anatomy in truncus arteriosus communis.
In truncus arteriosus communis, as in other anomalies of conotruncal development, the coronary arterial anatomy not only differs from that found in the normal heart but also is subject to unpredictable variations. A consistently distinctive pattern was found in roughly two thirds of 31 cases, a frequency approximating that with which a distinctive coronary arterial pattern occurs in complete transposition of the great vessels. Surgical injury to a major coronary artery was identified in 2 of the 30 hearts from patients with truncus arteriosus communis who died in the early postoperative period. Of particular significance are large branches of the right coronary artery crossing the upper anterior surface of the right ventricle to supply the anterobasal surface of both ventricles and the upper part of the interventricular septum. These arteries are at special risk in surgical procedures utilizing a conduit anastomosed to a right ventriculotomy.
Adolescent↗
Two dimensional echocardiography in congenital heart disease.
Two dimensional echocardiography has enhanced the ability of the clinician to define accurately intracardiac anatomy and great vessel relations. By visualizing relations of the great arteries and determining ventricular situs in the parasternal short axis planes and by assessing the relation of the great arteries to ventricles atrioventricular (A-V) valves and the interventricular septum in the parasternal and apical long axis view, complex congenital heart disorders can readily be differentiated. The subcostal approach allows accurate identification and localization of interatrial and interventricular septal defects. Utilizing the apical parasternal four chambered or subcostal four chambered view, complex malformations of the A-V valves such as complete endocardial cushion defect and Ebstein's anomaly can readily be appreciated. The suprasternal approach has allowed direct visualization of a coarcted aortic segment. The differentiation of left and right ventricular outflow obstruction is also possible. In some patients, it is possible to achieve an accurate diagnosis with two dimensional echocardiography alone. In others, two dimensional echocardiography provides accurate and detailed information with regard to spatial anatomy and valve morphology so that invasive studies can be performed more expeditiously, with less contrast agent and hence with greater safety. With regard to future developments, better resolution capability with newer instrumentation and combined two dimensional echocardiography with Doppler blood flowmeter studies may provide even greater diagnostic accuracy in the evaluation of patients with congenital heart disorders.
Aorta↗
Late results after extracardiac conduit repair for congenital cardiac defects.
Analysis of the late results in 352 patients surviving insertion of an extracardiac conduit before mid 1977 has provided a mean follow-up interval of 65 months. Three fourths of the patients remain in improved condition after operation. Serial measurements of transconduit gradient are available in 90. The median change was +7 mm Hg and the mean +21 mm Hg. Reoperation was required in 16 percent of patients (mortality rate 9 percent), most commonly (77 percent) because of progressive conduit stenosis, more commonly for transposition of the great arteries than for other types of anomalies, and more commonly after use of a homograft aortic conduit than a Hancock conduit. The side of the aorta on which the conduit was placed exerted no significant influence. The postrepair transconduit gradient did not affect the need for reoperation. Late survival was 95 percent at 1 year, 85 percent at 5 years, and 73 percent at 10 years and was significantly better (probability [p] less than 0.006) for patients with pulmonary atresia than for the others. The hospital mortality rate was highest, and the late mortality rate lowest, for children less than 5 years of age; the overall survival rate in this age group was lower. The postrepair right ventricular to left ventricular pressure ratio, together with age, was a principal prognostic indicator of late survival, being less good when more than 0.73. THe most frequent causes of late death were progressive congestive heart failure and sudden death.
Adolescent↗
Conotruncal malformations: diagnosis in infancy using subxiphoid 2-dimensional echocardiography.
Subxiphoid 2-dimensional echocardiography was performed and interpreted before the first cardiac catheterization of 109 of 113 infants less than 1 year of age with conotruncal malformations, situs solitus of the atria, and d-ventricular loop. The 4 remaining infants could not be examined using this technique for technical reasons. In 104 of 109 patients successfully examined a correct diagnosis was obtained with the use of subxiphoid 2-dimensional echocardiography. All patients with d-transposition of the great arteries had a correct diagnosis, and in 10 of 11 patients various types of double-outlet right ventricle were recognized by direct visualization of ventriculoarterial connection. Tetralogy of Fallot was correctly diagnosed in 34 of 36 patients and tetralogy of Fallot and pulmonary atresia were correctly diagnosed in 8 of 9 patients, on the basis of the position of the infundibular septum and presence or absence of continuity between the main pulmonary artery and the right ventricular. Five of 6 patients with truncus arteriosus communis were correctly diagnosed by demonstrating a connection between the pulmonary arteries and the ascending portion of the single large semilunar root. Subxiphoid 2-dimensional echocardiography proved to be a sensitive and specific technique for diagnosing conotruncal malformations in infancy.
Cardiac Catheterization↗
Two-dimensional echocardiographic prospective diagnosis of common truncus arteriosus in infants.
Two-dimensional echocardiographic prospective diagnosis of truncus arteriosus was made in 7 infants. Two infants had truncus arteriosus type I, 3 patients had truncus arteriosus type II, 1 infant had truncal valve stenosis with an interrupted aortic arch, and 1 had type IV truncus arteriosus with pulmonary hypertension. Multiple imaging views were utilized to confirm the diagnosis. The parasternal long-axis view demonstrated the great vessel-ventricular septal override and the origin of the pulmonary arteries from the posterior aspect of the ascending truncus. The suprasternal notch image facilitated identification of the left- or right-side aortic arch and the origin of the pulmonary arteries from the truncus. Transverse imaging sections at the base of the heart facilitated identification of the pulmonary artery origin of truncus arteriosus type I. Subcostal coronal and sagittal views imaged the common truncus and the ventricular septal defect. These echocardiographic images were contrasted with and discriminated from those of an infant with aorticopulmonary window with intact ventricular septum. Although cardiac catheterization and angiography may be required to assess pulmonary arterial pressure, pulmonary vascular resistance, and the distal pulmonary arterial anatomy in truncus arteriosus, 2-dimensional echocardiography can be used to correctly establish the morphologic diagnosis of truncus arteriosus in infants.
Angiocardiography↗
Cardiac malformations in relatives of children with truncus arteriosus or interruption of the aortic arch.
Familial recurrence risks for congenital cardiac malformations have been estimated at 1 to 4%, but little is known of recurrence risks for uncommon congenital cardiac malformations. This study determined the incidence of congenital cardiac malformations in relatives of patients with either truncus arteriosus (TA) or interruption of the aortic arch (IAA) seen between 1955 and 1985. Prior to this study, only 2 cases of IAA in siblings had been reported and the recurrence risk of TA had been estimated at 1.2%. In the families of 36 index cases of IAA, there were 98 siblings, 2 with congenital cardiac malformations (2.1%). All recurrences were in IAA type B families. Single cases of congenital cardiac malformations were found in second- and third-degree relatives and 2 cases in more distant relatives. In the families of 49 index cases of TA, there were 106 siblings, 7 with congenital cardiac malformations (6.6%). Two had TA, 3 had other conotruncal anomalies and 2 had other congenital cardiac malformations. Two parents had congenital cardiac malformations, 1 with TA and 1 with conotruncal malformation. Among second- and third-degree relatives, 7 had congenital cardiac malformations (5 were conotruncal, including 1 half sibling). Among greater than third-degree relatives, 3 had congenital cardiac malformations. Thus, in IAA, the recurrence rate of congenital cardiac malformations (2.1%) was higher than expected and could be related to the etiology of IAA type B as a conotruncal malformation. In TA, the recurrence rate of congenital cardiac malformations was 6.6%, higher than previously reported. When TA was complex the recurrence risk was higher (13.6%). These findings must be taken into consideration for satisfactory genetic counseling.
Adult↗
Coronary arteries in truncus arteriosus.
The origin and distribution of the coronary arteries was described in 39 autopsy specimens of truncus arteriosus (TA). The specimens were classified according to the number and the patterns of the truncal cusps. The position of the truncal cusps was defined in relation to intracardiac structures, namely, the atrioventricular orifices. Bicuspid truncal valves were observed in 8 cases (21%), tricuspid in 22 cases (56%) and quadricuspid in 9 cases (23%). All tricuspid valves had 2 anterior and 1 posterior cusp. Great variability in the origin of the coronary arteries was observed, with a tendency for the right coronary artery to arise from the anterior right quadrant and for the left coronary artery to arise from the anterior and left quadrant. Such a tendency was observed in 50% of the bicuspid, in 59% of the tricuspid and in 66% of the quadricuspid valves. The anatomic right ventricle was always observed to be vascularized by a right coronary artery, and the anatomic left ventricle by a left coronary artery, even in cases in which there was a single coronary trunk. The anterior surface of the right ventricle was crossed by a right coronary artery in 5 cases. A single coronary artery was observed in 7 cases (18%). Embryologic considerations are offered, especially regarding the relation between the observed variability in coronary artery patterns in TA and the absence of the truncal septation.
Coronary Vessel Anomalies↗
An unusual variant of common arterial trunk.
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Abnormalities, Multiple↗
Congenital heart disease in a male adult.
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Adult↗
Successful diagnosis and surgical treatment of single ventricle, truncus arteriosus.
The association of single ventricle and truncus arteriosus is rare. We present the successful surgical palliation of a patient who echocardiographically and angiographically has a truncus arteriosus type I and single left ventricle. At operation, the main pulmonary artery was dissected off the truncus arteriosus, and a 4-mm polytetrafluoroethylene graft was placed from the ascending aorta to the left pulmonary artery. The patient is currently 13 months old and growing well.
Echocardiography↗
Truncus arteriosus.
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Abnormalities, Multiple↗
Repair of truncus arteriosus in the neonate and young infant.
Infants with truncus arteriosus present a difficult management issue. Because of the high operative mortality, repair is often delayed beyond the first 3 to 6 months of age. We reviewed our experience with 11 neonates and young infants with truncus arteriosus undergoing repair (median age, 21 days). Five patients also had major truncal valve insufficiency, and 2 required valve replacement. Right ventricle-pulmonary artery continuity was established with a porcine valved conduit in 3 patients and an aortic or pulmonary homograft in 8. There was 1 operative death (9%; 70% confidence limits, 3%-22%) and 1 late death over a mean follow-up of 21 months (range, 4 to 32 months). Eight of the 9 late survivors are growing normally. Echocardiographic examination revealed normal ventricular function in all patients (mean shortening fraction, 39%). Doppler assessment demonstrated trivial prosthetic or homograft valve regurgitation in 7 patients and mild to moderate obstruction in 5 patients. This recent experience with repair of truncus arteriosus indicates that the operative risk is low even in the neonate. Repair in the first month of life should be recommended before the development of critical congestive heart failure or irreversible pulmonary vascular disease.
Abnormalities, Multiple↗
Internal banding for palliation of truncus arteriosus in the neonate.
A method for surgically limiting pulmonary blood flow in the critically ill neonate with truncus arteriosus is described. Two recent cases utilizing this technique are presented. Comparisons are made between this and other palliative surgical procedures used in truncus arteriosus.
Constriction↗
Coronary arterial and sinusal anatomy in hearts with a common arterial trunk.
Eighty-four specimens of common arterial trunk were studied with special reference to the arrangement of the leaflets in relation to the atrioventricular valves, the origin of the coronary arteries in relation to the arterial sinuses, and the epicardial course of the coronary arteries. Fourteen normal hearts were used for comparison. In the hearts with common arterial trunk, the location and level of the coronary artery orifices (as well as the relationship of the truncal root to the area of fibrous continuity with the mitral valve) are different from those in normal hearts. In none of the hearts with common arterial trunk (particularly the 53 hearts with three leaflets in the truncal valve) did the appearance of the truncal valve approximate that of a normal aortic valve. Among the 22 hearts with four leaflets, there was a high incidence of coronary artery orifices in opposite sinuses (17/22 or 77.3%) and a low incidence of coronary artery orifices in adjacent sinuses (2/22 or 9.1%). These results suggest that the formation of the truncal valve is independent of the formation of the coronary orifices. Its leaflets are not predestined to become part of either the aortic valve or the pulmonary valve.
Coronary Vessel Anomalies↗
Construction of an aortic homograft conduit for right ventricle to pulmonary artery continuity.
Over the past decade the aortic homograft has become the extracardiac conduit of choice for repair of many congenital cardiac defects. Anastomosis of the homograft annulus to the right ventricle may distort the valve and render it incompetent. Prosthetic tube grafts have been used to bridge the right ventricle and distal homograft. We propose and have use successfully a method for establishing right ventricle to pulmonary artery continuity that is free of prosthetic materials, is technically simple, and places the homograft valve in the hilum, away from the distorting right ventricle free wall.
Aorta↗
Surgical repair of type II truncus arteriosus without a conduit.
A technique for reconstructing the right ventricular outflow tract in an infant with type II truncus arteriosus without using a prosthetic conduit is described. The truncal root is transected, and the confluence of branch pulmonary arteries is brought anterior to the aorta by using Lecompte's maneuver. The aorta is then reconstructed directly with an end-to-end anastomosis. The right ventricular outflow tract is reconstructed by anastomosing the posterior wall of the confluence directly to the apex of the vertical right ventriculotomy. A glutaraldehyde-preserved pericardial patch is then placed anteriorly to complete reconstruction of the right ventricular outflow tract.
Anastomosis, Surgical↗