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PubMedDate not supplied

Persistent truncus arteriosus: twenty years experience in a tertiary care center in Taiwan.

BACKGROUND: Persistent truncus arteriosus (PTA) is a rare congenital heart disease. The disease spectrum and outcome in the Oriental are still unclear. METHODS AND RESULTS: A total of 35 patients with PTA were identified from the Pediatric Cardiology Database of this institution, giving an incidence of 0.47%. According to the Van Praagh classification, we found type A1 in 16, A2 in 10, A3 in 4 and A4 in 5 patients. The most common truncal valves were still tricuspid (57.1%) and quadricuspid (28.5%), with the latter being associated with moderate to severe truncal regurgutation. Eleven patients had not received surgery and all died. We classified the era of operation as early (between 1980 and 1995) or late (1996-2001), and further classified the type of PTA as simple (type A1 or A2) or complex (interrupted aortic arch-A4, absent orifice of one PA from truncal root-A3 or moderate truncal valve insufficiency). The overall surgical mortality was 67%. Statistical analysis revealed that age at operation (older than 6 months), early operation era and complex PTA were risk factors for survival. CONCLUSIONS: PTA is a rare form of congenital heart disease in Taiwan and probably also in the Oriental. The surgical reparation seems to improve with experience. Early operation may prevent pulmonary vasculopathy. However, the results in complex PTA remain poor.

Age Factors↗
PubMed1990

Reduced L-type calcium current in the embryonic chick heart with persistent truncus arteriosus.

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.

3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethy↗
PubMed1990

Repair of a cerebral artery aneurysm in a patient with persistent truncus arteriosus.

We describe the anesthetic management of a 19-year-old woman with persistent truncus arteriosus who presented for cerebral artery aneurysm clipping. The anatomy and pathophysiology of persistent truncus arteriosus are reviewed to provide a basis for the choice of anesthetic agents and monitoring devices in this patient. The hazards of induced hypotension in the patient with truncus arteriosus are also discussed.

Journal Article↗
PubMed1991

[Truncal valve regurgitation and stenosis in persistent truncus arteriosus: echocardiographic evaluation of pre- and postsurgical states].

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.

Age Factors↗
PubMed1995

Polysplenia syndrome with common atrioventricular canal and persistent truncus arteriosus.

A case of an infant with a rare combination of polysplenia syndrome with common atrioventricular canal and persistent truncus arteriosus is presented. In our present case, severe common atrioventricular valve regurgitation was identified, as in previous cases. To our knowledge, echocardiographic and autopsy findings of this association has not been previously reported. The persistent truncus arteriosus is extremely rare in the setting of the polysplenia syndrome, but the present case report demonstrates that these anomalies may, at times, occur.

Abnormalities, Multiple↗
PubMed1995

Persistent truncus arteriosus in a diprosopic newborn calf.

A newborn diprosopic female calf had a partially duplicated head with two faces each exhibiting a mouth, a snout, an anomalous incomplete mandible, two eyes and a lateral ear. A single ear with two small auditory canals was present on the midline between the two medial eyes. A type 1 persistent truncus arteriosus and hypoplasia of the thoracic portion of thymus were the most outstanding extracranial defects. In the heart, a persistent foramen secundum and a large patent foramen ovale allowed communication between the right and left atria. In the right ventricle, the small conus arteriosus was separated in part from the inflow tract by an anomalous 'septomarginal muscular septum'. An interventricular septal defect was also present. A large undivided truncus arteriosus, exhibiting a tricuspid truncal valve at its origin, arose for the most part from the conus arteriosus of the right ventricle. The truncus gave rise to the brachiocephalic trunk, the aortic arch, a small pulmonary trunk, from which the left and right pulmonary arteries emerged, and two coronary arteries. The etiology and pathogenetic mechanisms implicated in the appearance of persistent truncus arteriosus are reviewed. It is suggested that a deficit or insufficiency in the cranial neural crest may play a role in the pathogenetic mechanisms leading to the production of persistent truncus arteriosus and related defects in cephalic duplications.

Animals↗
PubMed1999

Factors associated with outcomes of persistent truncus arteriosus.

OBJECTIVES: The purpose of this study was to identify trends and factors associated with outcomes of persistent truncus arteriosus (PTA). BACKGROUND: Although there have been significant improvements, PTA continues to be associated with significant morbidity and mortality. METHODS: We undertook a review of all consecutive cases of PTA (n = 205) presenting at our institution from 1953 to 1997. Data were collected regarding demographics, anatomy, management (surgical palliation and repair) and outcomes (mortality and reoperation). RESULTS: Significant trends (p < or = 0.001) related to groups defined by year of birth were as follows: number of cases (1953-1967, n = 13; 1968-1977, n = 42; 1978-1987, n = 69; 1988-1997, n = 81), median age at first assessment (8 months, 42 days, 7 days and 2 days, respectively), proportion who did not have any surgery (58%, 27%, 22% and 11%), proportion who had an initial palliative procedure (25%, 37%, 6% and 2%), proportion who underwent PTA repair (31%, 59%, 72% and 88%), median age at PTA repair (11.2 years, 1.1 years, 1.6 months and 12 days) and proportion dying before hospital discharge after repair (50%, 63%, 56% and 41%). Since 1995, mortality before hospital discharge after repair has further decreased to 2/11 (18%). Increasing time to initial conduit replacement in hospital survivors was significantly related to larger sized conduit at repair (p = 0.02) and use of pulmonary homografts (vs. aortic homografts or xenografts; p = 0.002). Interventional catheterization to address conduit obstructions significantly increased conduit longevity. CONCLUSIONS: Significant improvements in PTA outcomes are evident with trends toward earlier age at assessment and complete repair.

Child, Preschool↗
PubMed1999

Persistent truncus arteriosus in monozygotic twins: case report and literature review.

We report on a pair of monozygotic twins with persistent truncus arteriosus. They had no evident clinical signs of DiGeorge syndrome. Pathologic examination of the placenta and DNA analysis in chromosomes 7, 8, and 15 was consistent with monozygosity. Fluorescence in situ hybridization test was negative for chromosome 22q11 microdeletion. Family history revealed a female cousin with tetralogy of Fallot. The isolated presence of this conotruncal abnormality in monozygotic twins is extremely rare. The genetic considerations are discussed.

Diseases in Twins↗
PubMed1986

Persistent truncus arteriosus: pathologic anatomy in 54 cases.

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.

Adolescent↗
PubMed1982

Two-dimensional echocardiographic evaluation of persistent truncus arteriosus.

Using a phased-array, wide angle sector-scanner, 2-dimensional echocardiograms were obtained in 3 patients with persistent truncus arteriosus. Type I was found in 2 patients and type II in 1 patient. In the patient with type II truncal valve stenosis was also present. Using a combination of short-axis views at the cardiac base, within 30 degrees superior transducer angulation and parasternal long-axis views, the truncus arteriosus and the pattern of pulmonary arteries were demonstrated in each case.

Angiocardiography↗
PubMedDate not supplied

Persistent truncus arteriosus operated during infancy: long-term follow-up.

Between July 1974 and October 1988 19 consecutive infants (mean age 2.7 months, range 5 days to 11.7 months) underwent surgical correction for persistent truncus arteriosus by one surgeon (J.L.M.). Continuity between the right ventricle and pulmonary arteries was achieved with an antibiotic-sterilized aortic homograft (diameter 13-18 mm) together with patch closure of the ventricular septal defect. There were 3 early postoperative deaths (16%): 1 patient had severe aortic regurgitation, the other 2 had preoperative cardiac arrests. Of the latter, 1 had suffered severe cerebral damage, and the other developed recurrent pulmonary hypertensive crises and low cardiac output. The 16 survivors have been followed for 3.1-17.3 years (mean 7.8 years). Four patients required subsequent replacement of the homograft for stenosis, aortic valve replacement for regurgitation, or both (3.0, 4.0, 8.5, and 12.0 years postoperatively). Of the 16 survivors, 15 are in NYHA class I. Of the 13 patients who have not had aortic valve surgery, 9 have no evidence of stenosis or regurgitation. In the 14 children with the original homograft the median of the residual peak gradient across the right ventricular outflow tract is 15 mmHg (range 10-40 mmHg), and no patient has severe homograft regurgitation at follow-up. Repair of persistent truncus arteriosus has been achieved with low early mortality and no late mortality, which reflects excellent long-term function of the homograft. Furthermore, if truncal valve function is good at presentation, patients are unlikely to require aortic valve surgery.

Aortic Valve↗
PubMed1997

Excitation-contraction coupling in the day 15 embryonic chick heart with persistent truncus arteriosus.

Ca2+ transients were examined in embryonic chick hearts with an experimentally induced cardiac neural crest-related outflow tract defect known as persistent truncus arteriosus (PTA). In all of the animal models of neural crest-related heart defects, prenatal mortality is too high to be attributed to structural defects of the heart alone, suggesting that there is altered development of the myocardium. Earlier reports indicating reduced L-type Ca2+ current in hearts with PTA suggest that poor viability may be related to impairment of cardiac excitation-contraction coupling. To test this hypothesis, direct measurements of the systolic Ca2+ transient in fura-2-loaded myocytes from normal hearts and hearts with PTA were carried out. We found that Ca2+ transients were severely depressed in hearts with PTA and difficult to measure above background noise unless signal averaged or treated with isoproterenol (ISO). We confirmed that the reduced Ca2+ transients were due, at least partly, to a reduction in L-type Ca2+ current. In addition we found that although ISO could raise the L-type current in hearts with PTA to the level found in normal hearts in the absence of ISO, it could not fully restore the Ca2+ transient. Furthermore, caffeine-stimulated Ca2+ transients were diminished in size and the time-to-peak and the decaying phase were significantly slowed. Interestingly, these observations were not accompanied by a reduction in the number of Ca2+ release channels. These results indicated an impairment of SR function in addition to the reduction in L-type Ca2+ current. These results strongly support our hypothesis that the poor viability of embryos with PTA is due to impaired cardiac excitation-contraction coupling.

Animals↗
PubMed1991

Tricuspid atresia: association with persistent truncus arteriosus.

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.

Echocardiography↗