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Persistent truncus arteriosus (TA) is an uncommon congenital cardiovascular malformation, which comprises between 0.4% and 4% of all congenital heart defects. Occurrence of TA in siblings has been reported infrequently. Twins concordant for isolated TA appear to have been reported only once previously. In this paper, we describe dizygotic twin females who were concordant for isolated TA.
Four cases of persistent truncus arteriosus (PTA) type 1 are presented where echocardiography was helpful in making the diagnosis. The diagnosis was later confirmed by cardiac catheterisation and angiocardiography.
Persistent truncus arteriosus is a relatively rare cardiac anomaly which is associated with a single large artery arising from the ventricles. An interventricular septal defect is invariably present. The vessel gives origin to the pulmonary trunk, aorta and coronary arteries. A description of the angiocardiographic diagnosis of this condition is given as well as a general review of the relative developmental anatomy.
An infant with persistent truncus arteriosus associated with splenic agenesis and the asplenia syndrome is reported, including clinical, echocardiographic and autopsy findings. To the authors' knowledge this association has not been previously reported.
The Splotch mutant mouse shows defects in neural crest-derived cell populations. The septation of the truncus arteriosus and the development of the aortic arch-derived blood vessels was studied in homozygotes of the Splotch mutant allele Sp1H. It is shown that in homozygous mutant embryos, the septation of the truncus arteriosus does not proceed normally, resulting in persistent truncus arteriosus. The ostium of the persistent truncus arteriosus opens to the right ventricle. Frequently, variations of the aortic arch-derived blood vessels are observed. The development of the thymus, the parathyroid and the ultimobranchial bodies are also variably affected in mutants. These results provide indirect evidence, that cells contributing to the aortic arches and the septum of the truncus arteriosus in mice are derived from the neural crest. The Splotch mutant mouse is proposed to be an animal model for persistent truncus arteriosus. The implications of the vascular malformations for the midgestational death of this mutant are discussed.
Description of the history, the clinical findings, and the autoptic results in a 5-days-old newborn child with a truncus arteriosus (Type I) and an interrupted aortic arch (Type B). Perfusion of the distal aortic arch by a wide open Ductus arteriosus. Additional anomalies: Atrial septal defect with partial anomalous pulmonary venous drainage of the right lung into the right atrium. Persistant left superior vena cava which leads to the left atrium. Hypoplasia and dysplasia of the left kidney. A tear caused by friction in the left sensorium with intra-and subdural bleeding. Discussion of the clinical, electrocardiographic, radiologic, angiocardiographic and pathologic-anatomical peculiarities of the persistent Truncus arteriosus and of the interrupted aortic arch connected with both defects.
Specimens of heart from 30 subjects with persistent truncus arteriosus were studied for the nature and sites of coronary arterial origin. These factors were related to the sinuses of the truncus valve. Bicuspid truncal valve was observed in six cases (20%) and tricuspid in 21 cases (70%). Single coronary artery was observed in four cases (three with tricuspid and one with quadricuspid truncal valves). In three other cases the coronary arteries arose separately but near one another. There was a strong tendency for the left coronary artery to arise from a more posterior level than it does normally from the aorta. It was common for the posterior descending artery to arise from the left circumflex artery. This arrangement was noted in eight of 25 cases (32%) with single posterior descending coronary artery. In five other cases, two posterior descending arteries were present, one arising from the left circumflex and the other from the right coronary artery.
Nimustine hydrochloride (ACNU) is a nitrosourea derivative anticancer agent which has been shown to cause persistent truncus arteriosus in chick embryos. The objective of this study was to confirm the teratogenic effects of ACNU on the cardiovascular system of chick embryos and to determine whether ACNU induces persistent truncus arteriosus by interfering with neural crest cells. Various doses of ACNU ranging from 10 to 200 micrograms were injected under the chorioallantoic membrane of chick embryos on the third day of incubation. Saline solution was used as the control. After 10 to 11 days of incubation, 242 (46%) survivors of the 524 treated eggs were obtained. The survival rates of the embryos and the frequencies of cardiovascular anomalies were dose dependent. Of 146 embryos with cardiovascular anomalies, 104 (71%) had persistent truncus arteriosus. Ventricular septal defect and double-outlet right ventricle were seen in 37 (25%) and one (1%), respectively. Aortic arch anomalies were seen in 116 embryos (79%). Quail-chick chimeras (chick embryos with quail cardiac neural crest) were treated with 50 micrograms of ACNU and examined histologically 24 hours later. These chimeras showed dying neural crest cells in the pharyngeal arches. Dying cells were also noted in the neural tube, cranial ganglia, retina, and otocyst. These results suggest that persistent truncus arteriosus in chick embryos treated with ACNU is induced by neural crest cell death.
Among 36 embryos obtained from a strain of Keeshond dogs in which there is a large incidence of spontaneously occurring conotruncal anomalies, a specimen with persistent truncus arteriosus, type 1 was found. The embryo had a crown-rump length of 20 mm. The specimen was serially sectioned and a wax plate reconstruction was made of the heart and proximal great vessels at a magnification of X100. The truncal valve was quadricuspid and dysplastic; associated anomalies were a right subclavian artery arising anomalously from the descending aorta, a single coronary artery, an absent ductus arteriosus and a small persistent left cranial (superior) vena cava. The truncus cushions were hypoplastic, had failed to fuse and each had simply produced an arterial cusp. The observations made on this embryo support the view that in persistent truncus arteriosus there is failure of septation of the truncus arteriosus. No evidence was found in favor of the concept that persistent truncus arteriosus represents a form of tetralogy of Fallot with atresia of the subpulmonary infundibulum and partial or complete absence of the aorticopulmonary septum.
Sixteen specimens of heart with persistent truncus arteriosus were studied to evaluate the anatomic features. Using the Collet and Edwards classification, type I truncus arteriosus was the most frequent (62.5%). Using Van Praagh's classification type A1 was the most common (43.7%). There were two cases which could not be classified according to the Collet and Edwards classification. The truncal valve was tricuspid in 75% of the cases and bicuspid in the remaining 25%. In all 6 cases with interruption of the aorta, the truncal valve was committed to the right ventricle. The ventricular septal defect was subtruncal in all except 1 case. There was variation in the thickness of the posterior limb of the septal band and the ventriculo-infundibular fold. Absence of the ventriculo-infundibular fold in 3 cases led to truncal tricuspid continuity. Right-sided aortic arch and interruption of the aorta were frequently associated arch anomalies.
Calcium currents were examined in an experimental model in the embryonic chick heart with a congenital malformation known as persistent truncus arteriosus. This is a severe defect characterized by failure of conotruncal and aorticopulmonary septation of the embryonic heart tube. As a result, no separation into the aortic and pulmonary arteries occurs, and there is a common outflow tract. The hearts with persistent truncus arteriosus had a 26% greater ventricular to whole embryo weight, which indicated that the ventricles were enlarged. Both the low-threshold T-type (ICa.T) and the 1,4-dihydropyridine-sensitive L-type (ICa.L) Ca2+ currents were present in the ventricular myocytes from hearts at day 11 of incubation. However, day 11 hearts with persistent truncus arteriosus showed a twofold reduction in the peak magnitude of ICa.L at a test potential of + 10 mV without a concomitant reduction in the number of L channels detected by 1,4-dihydropyridine antagonist [(+)[3H]PN200-110] and agonist (Bay K 8644) receptor binding. The results indicated that an L channel regulatory mechanism other than protein synthesis was affected. These changes are consistent with responses to conditions of excessive hemodynamic burden that have been characterized in adult hearts.
Twenty-four patients with persistent truncus arteriosus who underwent total surgical correction at The Hospital for Sick Children in Toronto, Canada between October 1984 and December 1987 were investigated to determine whether the postoperative course is satisfactory even without performing replacement of the truncal valve. All patients but one were less than 6 months of age. There were 9 operative deaths with a mortality rate of 37%. The most significant incremental risk factor was age of less than 30 days at the time of surgery (p < 0.01). The operative mortality did not correlate with the degree of preoperative truncal valve regurgitation nor stenosis. Among 15 hospital survivors, 14 patients were followed by echocardiography within one week after surgery, 4 of whom were reexamined within 2 weeks. Four patients without truncal valve regurgitation and stenosis underwent successful surgery, however, mild regurgitation developed in one 2 weeks after surgery. Among 4 patients with solitary truncal valve regurgitation, 2 improved and the other 2 did not. Among 5 patients with truncal regurgitation and stenosis, 3 improved in both truncal valve regurgitation and stenosis, but improvement was observed in only stenosis in the other 2 patients. One patient with stenosis improved, but developed mild regurgitation 2 weeks later. After radical surgery, 8 of the 10 patients (80%) with regurgitation and/or stenosis showed improvement without performing replacement of the truncal valve.
Fifty-four specimens of heart with persistent truncus arteriosus (PTA) were reviewed anatomically. According to the Collett-Edwards classification there were 28 examples of type I and 26 type II. The sex distribution was equal. The number of the truncal cusps ranged from one to four (42% tricuspid, 30% bicuspid, 24% quadricuspid, and 4% unicommissural). A unicommissural truncal valve has not been previously reported. In 72% of cases, the truncal valve leaflets were thickened or dysplastic. Two valves were stenotic. The truncus arteriosus originated from both ventricles equally in 42% of the cases, predominantly from the right ventricle in 42%, and predominantly from the left ventricle in 16% of the cases. In unoperated cases of PTA originating predominantly from the right ventricle, it appeared to us that usual operative correction might result in left ventricular outflow obstruction. Variations in coronary arterial origins and patterns were present in nearly half of the cases. A single coronary artery was observed in ten cases (18.5%). Stenosis of the ostium of one coronary artery was seen in each of four cases (7%). High posterior origin of the left coronary artery was observed in ten cases (18.5%). Among the associated cardiovascular anomalies, the most common were right aortic arch (36%) and interruption of the aortic arch (11%). Three cases with the latter condition exhibited crossed pulmonary arteries. Isolated cases with tricuspid atresia, vascular sling (left pulmonary artery arising from right pulmonary artery), and persistent common atrioventricular canal were encountered.
Clinical, echo-Doppler, and pathologic data of a rare case of tricuspid atresia in association with truncus arteriosus are presented. There are only six patients (including the patient reported here) with this anomaly in whom detailed pathologic and/or clinical descriptions are available in the literature. An additional six cases were mentioned in the literature but without any details. A prevalence rate of 1.4% among tricuspid atresia cases is estimated. All infants presented with symptoms of cyanosis and/or congestive heart failure within a few days to 2 months after birth and died shortly thereafter. Cardiomegaly and increased pulmonary vascular markings on the chest x-ray film and left axis deviation with left ventricular hypertrophy on the ECG are usually present. Echocardiographic, catheterization, and angiocardiographic studies are helpful in documenting anatomic and physiologic features of this anomaly. Pathologically, the muscular type of tricuspid atresia, type I or II truncus arteriosus, a large subtruncal VSD, and a hypoplastic right ventricle are present. It is concluded that coexistence of tricuspid atresia and truncus arteriosus is rare; clinical, laboratory, and pathologic features are distinctive; the clinical presentation early in life with rapid demise is common; and prompt palliative surgery immediately after recognition of this anomaly should be undertaken.
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