Search

Truncus arteriosus (TA) is a major congenital cardiac malformation that requires surgical repair in the first few weeks of life. Interrupted aortic arch (IAA) is an associated malformation that significantly impacts the complexity of the TA operation. The aim of this study was to (1) define the comorbid conditions associated with TA and (2) determine the hospital survival and morbidity of patients with TA with and without an IAA. Data was collected from the Vizient Clinical Database/Resource Manager, formerly University HealthSystem Consortium, which encompasses more than 160 academic medical centers in the United States. The database was queried for patients admitted from 2002 to 2016 who were ≤ 4 months of age at initial admission, diagnosed with TA, and underwent complete surgical repair during that hospitalization. Of the 645 patients with TA who underwent surgery, 98 (15%) had TA with an interrupted aortic arch (TA-IAA). Both TA and TA-IAA were associated with a high prevalence of comorbidities, including DiGeorge syndrome, prematurity, and other congenital malformations. There was no difference in mortality between TA and TA-IAA (13.7–18.4%, p value = 0.227). No comorbid conditions were associated with an increased mortality in either group. However, patients with TA-IAA had a longer post-operative length of stay (LOS) compared to those without IAA (30 versus 40.3 days, p value = 0.001) and this effect was additive with each additional comorbid condition. In conclusion, the addition of IAA to TA is associated with an increased post-operative LOS, but does not increase in-hospital mortality.

Although the clinical outcomes of truncus arteriosus (TA) repair have been improving, few data are available on long-term outcomes after truncus arteriosus repair in the current era. This study evaluated long-term outcome after repair of TA. Fifty-one patients underwent total correction from April 1982 to June 2018. Since 2003, perioperative strategy has changed to minimal priming volume, modified ultrafiltration, and early total repair (n = 26). Mortality and reoperation rates were analyzed before and after 2003. There were 8 hospital deaths after initial operation, all before 1997. During the mean follow-up of 9.8 years, there were 2 deaths. The Kaplan-Meier estimate of survival among all hospital survivors was 94.7% at 5 years and 88.0% at 20 years. A significant independent risk factor for early mortality was operation before 2003 (Hazard ratio (HR) 9.710, p = 0.041) and REV operation (HR 8.000, p = 0.028). Freedom from reoperation for conduit change and TV repair were 88.3% and 41% at 1 and 5 years, and 96.2% and 85.4% at 1 and 5 years, respectively. After 2003, younger age and conduit choice were risk factors for conduit-related reoperation. Initial preoperative TV regurgitation was independent risk factor for sequential TV repair. Patients with TA can undergo total repair of TA with excellent results, especially in current era. Most of the patients require conduit-related reoperations. Younger age and the methods of RVOT reconstruction were risk factors for conduit-related reoperations. TV repair is necessary in limited patients, and initial regurgitation was a risk factor.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016. Multivariable competing risk analysis was performed to determine risk factors for later truncal valve intervention. We reviewed 252 patients. Forty-two patients (17%) underwent truncal valve intervention during their initial surgery. Postoperative extracorporeal support, CPR, and operative mortality for patients who underwent truncal valve interventions were statistically similar to the rest of the cohort. Truncal valve interventions were performed in 5 of 64 patients with mild insufficiency; 5 of 16 patients with mild-to-moderate insufficiency; 17 of 35 patients with moderate insufficiency; 5 of 9 patients with moderate-to-severe insufficiency; and all 10 patients with severe insufficiency. Twenty patients (8%) underwent later truncal valve intervention, five of whom had no truncal valve intervention during initial surgical repair. Multivariable analysis revealed truncal valve intervention during initial repair (HR 11.5; 95% CI 2.5, 53.2) and moderate or greater truncal insufficiency prior to initial repair (HR 4.0; 95% CI 1.1, 14.5) to be independently associated with later truncal valve intervention. In conclusion, in a multicenter cohort of children with truncus arteriosus, 17% had truncal valve intervention during initial surgical repair. For patients in whom variable truncal valve insufficiency is present and primary intervention was not performed, late interventions were uncommon. Conservative surgical approach to truncal valve management may be justifiable.

Children seem to be less affected by SARS-CoV-2 infection. High risk categories should include patients with Congenital Heart Disease (CHD), both children and adults. We describe the case of a newborn with a postnatal diagnosis of Truncus Arteriosus (TA) type A1 without 22.q.11 deletion syndrome. Soon after birth, SARS-CoV-2 infection was transmitted by the father. Due to the onset of heart failure symptoms, diuretic therapy has been set up. For worsening of clinical conditions, inotropic support with milrinone was added. A progressive reduction of N-terminal-pro hormone BNP over the days has been observed. Fourteen days after the negativization of the nasopharyngeal swab, the patient underwent surgical repair with Cardiopulmonary Bypass (CPB). Postoperative course was not complicated and the patient was discharged in good clinical conditions. There is very little evidence suggesting the optimal timing for surgery in SARS-CoV-2 positive patients. With a lack of specific guidelines, current strategy suggests a symptom-based or a polymerase chain reaction (PCR) test-based approach. In our case it was challenging to determine COVID-19 impact on heart failure symptoms. Our case is the first describing the surgical correction of CHD in a 40 days year old patient, performed in CPB after 14 days from SARS-CoV-2 infection negativization.

The purpose of this report is to assess the mid- and long-term outcomes of right ventricular outflow tract (RVOT) reconstruction for children with persistent truncus arteriosus. Between September 2006 and 2016, 105 patients with persistent truncus arteriosus (PTA) received surgical treatment at Shanghai Children's Medical Center. Direct right ventricle-pulmonary artery anastomosis (pulmonary artery pull-down) was performed in 51 patients; a left auricle or pericardial conduit was inserted between the RVOT and pulmonary artery as a connection in 17 patients; heterograft (bovine jugular vein or Gore-tex) conduits and homograft conduits were used in 30 and 7 cases, respectively, to connect the distal pulmonary and right ventricle outflow tract; and pulmonary valve reconstruction was performed in 38 patients using a Gore-tex monocusp. There were six in-hospital deaths and one delayed death 5 months after operation. After a mean follow-up of 55.8 ± 16.5 months (6-113 months), 19 patients underwent reoperation (3 with pulmonary patch enlargement, 14 with conduit replacement and 2 with aortic valve replacement) 10-89 months after the first operation, with 1 hospital death. The actuarial survival rates were 94.2, 93.3 and 93.3% at 1, 5 and 10 years, respectively. Freedom from reoperation was 98.0, 87.8 and 82.7% at 1, 5 and 10 years, respectively. The follow-up variables included echocardiography, chest radiography, cardiac CT and cardiac function. At the last examination, most of the patients exhibited an improvement of New York Heart Association functional class from III or IV preoperatively to I or II at follow-up. Surgical treatment for PTA has an acceptable survival rate and satisfactory outcomes. Most patients exhibited an improvement in cardiac function during follow-up. Aortic arch deformity, truncal valvular regurgitation and long cardiopulmonary bypass time were regarded as risk factors for hospital mortality. Autologous tissue has a lower reoperation rate and better growth potential than extracardiac conduits. A monocusp valve effectively reduces pulmonary regurgitation in the early postoperative stage.

We performed this study to determine the frequency of 22q11 deletions and associated phenotypic features and abnormalities in conotruncal heart defects. Sixty-one patients with conotruncal heart defects, including tetralogy of Fallot (TOF; n = 32), pulmonary atresia/ventricular septal defect (PAVSD; n = 12), double-outlet right ventricle (DORV; n = 5), transposition of the great arteries (TGA; n = 4 ), truncus arteriosus (TA; n = 4), subpulmonary ventricular septal defect (SPVSD; n = 3), and interrupted aortic arch (IAA; n = 1), were enrolled in this study and screened for 22q11 deletions by the fluorescence in situ hybridization technique. Phenotypic features and associated abnormalities, including submucosal cleft palate, abnormal facies, square nose, nasal voice, abnormal ears, long and slender fingers, delayed development, mental retardation, delayed growth, short stature, and hypocalcemia, were examined in these patients. Nine of 61 patients (14.8%) had 22q11 deletions, including 100% of IAA, 50% of TA, 33.3% of SPVSD, 33.3% of PAVSD, and 3.1% of TOF. Deletions were not detected in DORV and TGA. In all patients with 22q11 deletions, > or =1 phenotypic features or associated abnormalities were observed. A subgroup of patients with IAA, TA, SPVSD, and PAVSD associated with phenotypic features or abnormalities warrants evaluation for the presence of 22q11 deletions.

We present a case of congenital pulmonary systemic collateral vein associated with truncus arteriosus. Pulmonary systemic collateral vein with nonobstructed left atrial egress is different from those with obstructed left atrial egress in that it is functionally redundant. Including this case, 8 patients among 33 reported cases with pulmonary systemic collateral veins have had nonobstructed left atrial egress. Association with conotruncal anomalies in 4 of these 8 patients, as well as the reported finding that neural crest cells are distributed not only in the conotruncus but also in the cardinal vein, indicates that neural crest cells play roles in remodeling of the systemic venous system.

The association between truncus arteriosus and chromosome 22q11 deletion is well recognized, but the frequency of a chromosome 22q11 deletion has not been characterized in a large series of patients with truncus arteriosus, and little is known about cardiovascular morphologic features associated with a chromosome 22q11 deletion in this group of patients. We prospectively enrolled 50 consecutive patients with truncus arteriosus who were admitted to The Children's Hospital of Philadelphia between November 1991 and December 2001. Patients were studied for chromosome 22q11 deletion using fluorescence in situ hybridization. Correlations between anatomic features and chromosome 22q11 deletion were assessed. A chromosome 22q11 deletion was detected in 20 of the 50 patients (40%). Anatomic features that were significantly associated with a chromosome 22q11 deletion included a right-sided aortic arch, an abnormal aortic arch branching pattern, both abnormal sidedness and branching of the aortic arch, and the combined category of either abnormal sidedness or branching of the aortic arch. There was a trend toward the association of discontinuous pulmonary arteries with a chromosome 22q11 deletion. Interruption of the aortic arch and truncal valve morphology and function did not correlate significantly with the presence of a chromosome 22q11 deletion. In conclusion, a chromosome 22q11 deletion is common in patients with truncus arteriosus, and those with abnormal sidedness and/or branching of the aortic arch are significantly more likely to have a deletion. Clinically important anatomic variables, such as abnormalities of the truncal valve and interrupted aortic arch, were not associated with a chromosome 22q11 deletion in this series.

This report describes the extremely rare case of a 42-day-old boy with anomalous origin of a single coronary artery from the right branchiocephalic trunk associated with hypoplastic left ventricle, mitral atresia, truncus arteriosus, total anomalous pulmonary venous drainage, and patent foramen ovale.

We report a rare association of persistent truncus arteriosus with double aortic arch in a 34-day old neonate.

Extant epidemiologic results for pediatric congenital heart disease (CHD) are dated. Given the degree of variability in previous prevalence estimates and the rapid changes in pediatric cardiology diagnostic and treatment procedures, a reexamination of these rates represents a potentially important update in this area of inquiry. This report characterizes the prevalence rates of children with CHD in one state’s treated pediatric population by type of lesion and in comparison with published rates from previous studies. Two 15-year data sets (1996–2010) are analyzed. The inclusion criteria for the study required the participants to be 17 years or younger, enrollees in the South Carolina State Medicaid or State Health Plan, and recipients of a CHD diagnosis on one or more service visits to a pediatrician or pediatric cardiologist. A 15-year accrued prevalence rate for pediatric CHD of 16.7 per 1,000 was found among 1,145,364 unduplicated children served. The annual incidence rates varied from 6.3 to 8.6 per 1,000, with an initial downward trend from 1996 to 2002 followed by an upward trend from 2003 to 2008. A higher prevalence of atrioventricular canal/endocardial cushion defects, common/single ventricle, double-outlet right ventricle, tetralogy of Fallot, and truncus arteriosus and a lower prevalence of dextro-transposition of the great arteries were diagnosed in South Carolina than in other states and countries according to published results. The study results underscore the need for periodic updating of prevalence data for pediatric CHD, both in total and for specific lesions, to anticipate and provide more specialized care to young patients with CHD, especially in the more rare and complex cases.

A 31-year-old primigravida was referred to our unit due to suspicion of a cardiac anomaly on obstetric screening ultrasound at 28 weeks of gestation. Although fetal echocardiography in the four-chamber view showed atrioventricular concordance and normal cardiac size, an outflow tract abnormality was noted. A single large vessel arising from the left and right ventricles and a ventriculoseptal defect (VSD) were identified (Fig. 1a; Movie 1). The main pulmonary trunk was not detected, and bilateral pulmonary arteries arose from opposite sides of the single large vessel. This unique outflow tract was therefore identified as a truncus arteriosus (TA). Color Doppler sonography showed mild truncal valve regurgitation and no truncal valve stenosis (Movie 2). In the sagittal view, the ascending aorta appeared hypoplastic, and there was interruption between the left subclavian artery and the descending aorta arising from the ductus arteriosus (Fig. 1b, c; Movie 3). Taken together, these findings led a diagnosis of TA associated with interrupted aortic arch (IAA), which corresponded to Van Praagh classification type 4A. No associated anomalies were identified, and fetal cardiac function showed no remarkable changes. At 34 weeks gestation, a female neonate weighing 1,968 g was delivered vaginally after rupture of membranes, with Apgar scores of 6 at 1 min and 9 at 5 min. Neonatal echocardiography and computed tomography confirmed the prenatal diagnosis of TA-IAA (Fig. 2). TA is an uncommon cardiac anomaly accounting for 0.7 % of all congenital heart disease, and concomitant IAA is found in approximately 15 % of children with TA [2]. Thus, the combination of TA-IAA is an extremely rare clinical entity, and a case report of prenatally diagnosed TA-IAA is limited. Marasini et al. [4] described a case of TA-IAA with severe truncal valve regurgitation. However, the case was electively terminated by severe hydrops fetalis. Van Praagh and Van Praagh proposed a classification system for TA according to two commonly associated abnormalities of the great arteries. Type 1 is characterized a short pulmonary trunk originating from the TA. Type 2 is defined by right and left pulmonary arteries originating separately from the TA. Type 3 includes cases with no truncal origin of one pulmonary artery, with the blood supply to that lung from the ductus arteriosus or from a collateral artery. Finally, type 4 is associated with underdevelopment of the aortic arch, including tubular hypoplasia, discrete coarctation, or complete interruption. The Van Praagh classification also specifies the presence of a VSD (type A) or the absence of a VSD (type B) [5]. Our case, the common form of TA with IAA and an associated VSD, is thus classified type A4. Although surgical TA repair during the neonatal and early infant period has become standard practice, the management of neonates with TA-IAA remains challenging [2]. The largest study reported a mortality rate of 68 % during the neonatal and infant period for a cohort of 50 TAIAA patients [3]. Several studies have identified factors Electronic supplementary material The online version of this article (doi:10.1007/s00246-012-0469-3) contains supplementary material, which is available to authorized users.

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.

Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at ≤60 days of life from 1992 to 2007. Forty-three (32%) of 136 TA patients had prenatal diagnosis. Five patients with TA were prenatally misdiagnosed, and 5 with other congenital heart diseases were misdiagnosed with TA prenatally. Of 28 fetuses diagnosed at

A neonate presented to the C.S. Mott Children’s Hospital at the University of Michigan with truncus arteriosus and interrupted left aortic arch, with associated postductal origin of the left carotid, left subclavian, and aberrant retroesophageal right innominate arteries. In addition, the patient was diagnosed with DiGeorge syndrome. This unique anomaly has not been previously reported. The anatomy, pathophysiology, embryology, and successful surgical management of this anomaly are reviewed in this report.

Truncus arteriosus is a relatively uncommon congenital malformation. It accounts for approximately 1% of congenital heart diseases. The defect occurs sporadically but 22q11 deletion is frequently noted in such patients with conotruncal defects. We studied six cases of TA in four closely related families. Analysis of karyotypes in these cases was normal. Family 1 has one affected male infant who was born in 1998. Family 2 has two affected children (one male and one female) who were born in 1989 and 1995, respectively. They have four other normal children. Family 3 has two affected children (one male and one female) who were born in 1981 and 1984, respectively. They have three other normal children. Family 4 has one affected male born in 1998 and another healthy child. All parents of all affected children are double cousins. The data in this study are compatible with an autosomal-recessive inheritance, but multifactorial inheritance may also play a role.

The lower weight limit for infants undergoing intraoperative transesophageal echocardiography (TEE) with current commercially available probes has not been determined. A review of the literature reveals that infants as small as 1.6 kg have been studied successfully. This report describes the first intraoperative TEE reported in a 1.4-kg infant during truncus arteriosus/interrupted aortic arch repair. Successful pre- and postoperative images of the cardiac abnormality were obtained. Probe insertion was performed in this small patient after predilating the esophagus with a 14-F suction catheter.

The association of truncus arteriosus with congenital left heart abnormalities is rare. We describe a case of truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle. In 11 other patients diagnosed with truncus arteriosus, none had left heart hypoplasia. Despite the truncal root being primarily or entirely committed to the right ventricle, more right than left heart abnormalities have been found. This is in contrast to double-outlet right ventricle in which left heart abnormalities are more often associated.

The objective of this study was to ascertain the prevalence and survival rate of children born with a heart defect. A total of 816, 569 children live-born between 1980 and 1990 in Bohemia (52,478 km(2), population 6.314 million, western Czech Republic) were followed up and those with suspected heart disease referred to a center. Echocardiography was done in all of them. All dead children were autopsied. Congenital heart disease was found in 5030 of 816, 569 children (6.16 per 1000 live births). The most frequent conditions were ventricular septal defect (41.59%), atrial septal defect (8.67%), aortic (7.77%) and pulmonary (5.81%) stenoses, transposition of the great arteries (5.39%), coarctation of the aorta (5.29%) and persistent ductus arteriosus (5.07%). The first week was survived by 92.46%, the first month by 89.14%, 6 months by 82.42%, and the first year of life by 80.02%, and 77.11% (95% CI 75. 91-78.31%) survived to age 15 years. The best prognosis was found in pulmonary stenosis (15-year survival 95.55%), atrial septal defect (92.04%), persistent ductus arteriosus (90.59%), ventricular septal defect (89.37%) and aortic stenosis (88.39%). The worst results were attained in hypoplastic left heart, truncus arteriosus and pulmonary atresia with intact ventricular septum. In conclusion, the prevalence of congenital heart disease was 6.16 per 1000 live births; 77.11% of patients survived to age 15 years.

Chromosome 22q11 deletion or CATCH 22 is associated with DiGeorge syndrome, conotruncal anomaly face syndrome, and velocardiofacial syndrome. Associated congenital heart diseases include tetralogy of Fallot, truncus arteriosus, and ventricular septal defect. Associated anomalies of the aortic arch, aortic branches, ductus arteriosus, and pulmonary arteries are more frequent in patients with the deletion than in those without the deletion. Associated anomalies include right aortic arch, cervical aorta, aberrant origin or isolation of the subclavian artery, the absence of the ductus arteriosus, major aortopulmonary collateral arteries, isolation of the left pulmonary artery, and vascular ring formed by the right aortic arch, retroesophageal aortic arch, and left descending aorta.

The spectrum of cardiac anomalies in trisomy 18 typically includes septal defects and polyvalvular disease and only rarely complex malformations. We report the first case of trisomy 18 with truncus arteriosus type II.

To the Editor, We read with interest the case report by Liu et al. regarding an extremely rare occurrence in the medical literature, the anomalous origin of a coronary artery from the right brachiocephalic trunk [1]. Two previous cases were cited in their report. During our research on truncus arteriosus with a single ventricle, we encountered an additional case to those already reported by Liu et al. In 1956 Cree reported a single coronary artery that arose from the innominate artery and divided into a right coronary artery, running down the atrioventricular groove to supply branches to the right atrium and anterior surface of the ventricle, and a left marginal artery, running down the left margin of the ventricle to the apex and supplying branches to the posterior surface of the ventricle, in a patient with single ventricle and truncus arteriosus [2]. With this case, there are now four case reports of this type of coronary artery origin abnormality in the medical literature.

The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life--mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septal defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life--mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70-80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%. The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life.

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 1965 reclassification of truncus arteriosus by Van Praagh and Van Praagh greatly enhanced our understanding of this interesting anomaly. This brief review article attempts to illustrate the various types of truncus arteriosus identified in this classification by demonstrating their angiographic features. Reemphasis of the usefulness of this classification should help students of congenital heart disease recognize the advantages of a uniform diagnostic approach to this entity.

A study of 26 patients with truncus arteriosus showed a high prevalence of facial dysmorphism, aortic arch abnormalities, extracardiac malformations, and significant prenatal risk factors. There was little evidence of parathyroid or thymic abnormalities. However, there was laboratory evidence of immune deficiency, especially T-helper lymphocytes, and clinical evidence of predilection to infection. These findings suggest that patients with truncus arteriosus belong to the spectrum of the Di George syndrome.

This report describes the echocardiographic findings of truncus arteriosus, right aortic arch, and persistent left superior vena cava draining into the coronary sinus in a 32-week-old fetus, in whom the diagnosis was confirmed by autopsy.

The chances of survival for patients with interruption of the aortic arch depend mainly on early recognition and medical treatment before symptoms of cardiogenic shock appear, and specifically on early operative repair after the exact diagnosis has been established. Accurate angiography with visualization of the interrupted aortic arch and delineation of associated cardiac malformations is of prime importance for the surgical management and prognosis. Among our 36 patients with interrupted aortic arch, 27 were operated on. The operative mortality in 22 patients with associated ventricular septal defect (VSD) and persistent ductus arteriosus (PDA) was reduced from 43% (in seven patients seen up until 1979) to 7% (in 15 patients seen since 1980). One patient with interrupted aortic arch type C and another infant with associated truncus arteriosus were successfully corrected on day 9 and day 17 of life, respectively. In the patients with associated VSD and PDA as well as in the one patient with associated truncus arteriosus, the primary correction--direct anastomosis of the interrupted segments without prosthesis and VSD closure and in the case with truncus, the additional positioning of a valve-bearing allograft conduit from the right ventricle to the pulmonary artery--has been more successful than a two-step approach with initial pulmonary artery banding.

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.