Truncal Valve Repair in Neonates Using Pericardial Leaflet Extension

Truncal valve insufficiency is a significant risk factor for post‐operative mortality following repair of truncus arteriosus. The surgical management of dysplastic and insufficient truncal valves remains an operative challenge. We report the cases of two infants with type 2 truncus arteriosus and severely dysplastic and insufficient quadricuspid truncal valves. At primary repair, their truncal valves were successfully repaired using pericardial leaflet extensions. This technique may be used in neonates with truncal valve insufficiency as part of the primary repair of truncus arteriosus.     

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.     

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-sided 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.     

Long-term results after repair of truncus arteriosus communis in neonates and infants

BACKGROUND: We reviewed our experience of truncus arteriosus communis (TAC) repair. METHODS: Between 05/90 and 10/01, 16 patients underwent complete repair of TAC (primary repair: group I, 12 patients, secondary repair: group II, 4 patients). Age was 2.4 months [5 days-8.8 months] (median [range]) in group I, and 8.3 [5.6-13.5] years in group II. Continuity from the right ventricle to the pulmonary artery was achieved using a valved conduit. All patients had regular follow-up examinations. RESULTS: There was one early death in each group (12.5%). Follow-up was 9 [1.2-12.7] years. Valved conduit failure occurred in 8 patients (67 %) in group I (group II, 1 patient, 33 %) requiring replacement at 2.5 [0.3-4.3] years (group II, 5.8 years). Severe neo-aortic valve regurgitation after truncal valve repair was observed in one patient, requiring valve replacement at 8.5 years in association with repeat homograft replacement (group I). Actual echocardiographic examination revealed normal ventricular function. Moderate conduit dysfunction was noted in 2 patients (group I). CONCLUSIONS: Complete repair of truncus arteriosus communis can be performed with excellent long-term results.     

Truncus arteriosus and previous pulmonary arterial banding: clinical and hemodynamic assessment.

Twenty-seven patients with truncus arteriosus and previous pulmonary arterial banding were evaluated 1 1/2 to 14 years (mean 7 years) after banding. Ages at the time of cardiac catheterization ranged from 3 to 18 years (mean 9 years). Current symptoms were severe in five patients and were related to truncal valve incompetence or decreased pulmonary blood flow (or both) rather than to age, duration of palliation or band location. Twenty-one of 22 patients with two pulmonary arteries were considered to be in a hemodynamically operable state at the time of study. The condition of three of five patients with a single pulmonary artery was subsequently found inoperable because of severe pulmonary vascular disease in the lung supplied by the single pulmonary artery. In patients with two pulmonary arteries, demonstration of low pressure in at least one normal-sized pulmonary artery established operability. Postoperative pressure measurements correlated well with preoperative prediction of operability, with 19 of 20 patients having a pulmonary arterial pressure less than 70 percent of systemic levels after repair. Bilateral pulmonary arterial binding may be more effective than central arterial banding (which frequently produces severe obstruction to the right pulmonary artery) in preventing pulmonary vascular obstructive disease in patients with truncus arteriosus who have two pulmonary arteries. Patients with truncus arteriosus and a single pulmonary artery with pulmonary arterial banding remain at high risk for the development of pulmonary vascular obstructive disease.     

Echocardiographic features of truncal abnormalities. Special emphasis to the evaluation of pulmonary arteries

9 patients with persistent truncus arteriosus, 11 patients with pseudotruncus and 2 patients with hemitruncus, ranging in age from 6 months to 29 years, have been studied by M-mode and two-dimensional echocardiography (2-D echo). In all patients the diagnosis was confirmed by cardiac catheterization and angiocardiography; in 10 of them surgical or autopsy documentation was available. Peripheral contrast echocardiography was performed in 13 patients. A variable degree of truncal or aortic override was observed in 7 of 9 patients with truncus arteriosus and in all patients with pseudotruncus by M-mode or two-dimensional echocardiography. By 2-D echo the truncal origin of the right and left pulmonary arteries was visualized in 7 of 9 of the patients with truncus arteriosus. Visualization of one aberrant pulmonary artery from the aortic wall was assessed retrospectively in 2 patients with hemitruncus. A pulmonary atretic valve region was recorded in 10 of 11 of the patients with pseudotruncus. Peripheral contrast echocardiography enabled to distinguish patients with complete obstruction to pulmonary flow from patients with unobstructed right ventricular outflow tract. By showing no direct connection between the pulmonary arteries and the ascending aorta or direct continuity between one or both pulmonary arteries and truncal or aortic root, differentiation of pseudotruncus arteriosus from truncus or hemitruncus may be achieved by 2-D echo.     

Severe pregnancy-induced deterioration of truncal valve regurgitation in an adolescent patient with repaired truncus arteriosus

Truncus arteriosus, a rare and complex congenital heart disease, is hallmarked by a single great vessel (truncus) that arises over a large ventricular septal defect and provides both the pulmonary and systemic circulation. Pregnancy reports after repair for truncus arteriosus are scarce. Therefore, the maternal and offspring outcomes are unknown. We report the outcome of a pregnancy in an 18-year-old woman with repaired truncus arteriosus. Despite severe and symptomatic deterioration of truncal valve regurgitation, she successfully delivered a healthy child, and the valve function recovered within 2 weeks postpartum.     

An Adult with Truncus Arteriosus and Unilateral Pulmonary Hypertension

We report a young man who has persistent truncus arteriosus (TA), severe truncal regurgitation and unilateral pulmonary hypertension. Our patient had palliative main pulmonary artery (PA) banding done during infancy that was not followed by definitive corrective surgery. Unilateral irreversible left sided pulmonary hypertension developed due to migration of the PA band to the right PA. The patient presented to us with infective endocarditis of the truncal valve. This had resolved with medical treatment. Discussion was made on general management of TA and specific difficult management issues of palliated TA in adult, as found in our patient.     

Fetal cardiovascular morphology of truncus arteriosus with or without truncal valve insufficiency in the rat

BACKGROUND. Recent advances in fetal echocardiography have necessitated further study on fetal in situ cardiovascular morphology of truncus arteriosus and the effects of truncal valve insufficiency. METHODS AND RESULTS. We studied 55 fetal rats with truncus arteriosus among 300 fetuses from 40 virgin females treated with 200 mg fertilysin on the 10th day of pregnancy. After rapid whole-body freezing on the 21st day, the fetuses were studied by means of serial cross-sectional photographs of the frozen thorax. Thirty-five fetuses with a normal heart treated with fertilysin served as controls. Truncus arteriosus was characterized by a large ventricular septal defect, a solitary artery (truncus arteriosus) overriding the ventricular septum, the right and left pulmonary arteries originating from the truncus arteriosus with or without a common trunk (main pulmonary artery), and absent ductus arteriosus. Fetuses with truncal valve insufficiency had thick truncal valves, a large truncus arteriosus, and large ventricles. The subgroup of 12 fetuses with a large truncus (truncal diameter greater than 160% of the ascending aorta diameter in the controls) showed significantly greater values for right ventricular volume (200% of control) and mass (120% of control), left ventricular volume (170% of control) and mass (110% of control), right (120% of control) and left (110% of control) atrial volume, and pericardial fluid (140% of control) than the controls. These changes were less prominent and ventricular volumes were not increased in the remaining subgroup with a truncal diameter of 160% or less of aorta diameter in the controls. CONCLUSIONS. In fetal truncus arteriosus, truncal valve insufficiency was associated with increased ventricular volume load and incomplete cardiac compensation in rats.     

The natural history of truncus arteriosus.

The cases of 23 patients whose condition was diagnosed as truncus arteriosus, type I or II, and who were seen at the Mayo Clinic during the decade preceding 1967, that is, before corrective operation became feasible, were reviewed. Ten were infants (through one year of age), and all ten have died. Eight ranged in age from more than one year through seven years of age, and all are living, except one, who diet 11 years after diagnosis. Five were older than seven years, and all had severe pulmonary vascular obstructive disease; three have died. Thus, 14 of the 23 have died, and all but one surviving patient have mild to moderate disability. The generally grave prognosis for patients with truncus arteriosus warrants continued use of corrective operation, but suggests that the greatest benefit can be realized by successful correction in the infant with congestive heart failure and in early childhood before the development of severe pulmonary vascular obstructive disease.     

Surgical experience with persistent truncus arteriosus in symptomatic infants under 1 year of age. Report of 13 consecutive cases.

Between January 1974 and November 1980, 13 symptomatic infants under 1 year of age with persistent truncus arteriosus type I or II underwent surgery. Pulmonary artery banding was performed in 10 cases, with five deaths. Among the survivors, one developed severe pulmonary vascular disease and only two underwent late intracardiac repair. Primary total correction was performed in three infants and all are well, though one required conduit replacement five and a half years after the initial procedure. Recently, antibiotic sterilised homograft conduits, rather than heterografts, have been preferred as extracardiac conduits in infancy. Early intracardiac repair followed, if necessary, by later conduit replacement appears to have significant advantages over "conventional" pulmonary artery banding and late total correction. Concern remains on the treatment of choice in infants under 3 months of age.     

Role of perioperative transesophageal echocardiography in the management of adolescent truncus arteriosus: Rare case report

Truncus arteriosus (TA) is a rare congenital heart disease defined as a single arterial vessel arising from the heart that gives origin to the systemic, pulmonary and coronary circulations. The truncal valve in majority of the cases is tricuspid though quadricuspid and bicuspid valves have been reported. Patients with TA typically have a large nonrestrictive sub truncal ventricular septal defect. Survival of these infants beyond 1-year is uncommon. Here, we report a unique case of 12-year-old female patient with persistent TA who underwent surgical repair by using transesophageal echocardiography as a monitoring device during the perioperative management.     

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 postoperative 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.     

Pathogenesis of persistent truncus arteriosus in light of observations made in a dog embryo with the anomaly

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.     

Persistent truncus arteriosus

Opinion statement Survival of patients with persistent truncus arteriosus, otherwise known as truncus arteriosus communis, has increased over the past several years with advances in congenital heart surgery and postoperative management. Despite the fact that ongoing debate regarding timing for surgical intervention, timing and indication for truncal valve replacement, and the ideal materials to use for the right ventricle to pulmonary artery conduit, perseverance on the part of cardiac surgeons, cardiologists, and the rest of the medical team caring for these patients has resulted in longer and improved quality of life. Early, accurate diagnosis, initial stabilization, and medical management in the perinatal period, and surgical intervention are most important in preventing or decreasing long-term morbidity and mortality.     

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, with 30 ° superior transducer angulation and parasternal long-axis views, the truncus arteriosus and the pattern of pulmonary arteries were demonstrated in each case.     

Stenotic semilunar valve in persistent truncus arteriosus.

The clinical, hemodynamic, and pathologic findings in two newborn infants with persistent truncus arteriosus and stenosis of the truncal valve are described. In one case the anatomic features of the basic condition were classic, with a dysplastic semilunar valve which was mainly stenotic and also incompetent, while in the other the truncus arteriosus arose exclusively from the right ventricle and was almost exclusively stenotic. A ventricular septal defect was the only outlet for the left ventricle. In this case, mitral stenosis was also present and associated with a left-to-right shunt at the atrial level.     

Bicuspidized pulmonary homograft for truncus arteriosus repair

Primary repair is preferable to palliation in infants with truncus arteriosus. At our institute, an appropriately small homograft valved conduit is not available for every patient; a bicuspidized pulmonary valve homograft is an alternative. Between December 1996 and August 2005, 24 patients aged 28 days to 21 months with truncus arteriosus underwent primary repair with a homograft valved conduit; bicuspidized homografts were used in 15 of them. In the 18 (75%) patients who survived to hospital discharge, 5-year survival was 94% (75% for tricuspid homografts and 100% for bicuspidized homografts, which was not significantly different). Freedom from reoperation or balloon angioplasty in all 18 survivors was 89% at 5 years. Freedom from reoperation in tricuspid and bicuspidized homograft groups at 5 years was 67% and 100%, respectively; the difference was not statistically significant. Bicuspidized homografts worked as well as tricuspid conduits in the intermediate term. The remodeled homografts showed excellent hemodynamic characteristics and appear to be a reasonable alternative when an appropriate size of valved homograft is unavailable.     

The pulmonary vascular bed in patients with complete transposition of the great arteries.

Histological material obtained at autopsy from 35 patients over three months of age with complete transposition of the great arteries (TGA) was examined. Two of six patients less than a year of age with an intact ventricular septum and closed ductus arteriosus were found to have pulmonary vascular changes of at least grade 3 severity; in addition, two of seven patients with a large ventricular septal defect in this age group showed changes of similar severity. The reported low incidence of marked pulmonary vascular changes in patients with only an interatrial communication dying during the first year of life would appear to be due in part to the high proportion of cases less than three months old in whom there was not sufficient time for such changes to develop. Although the incidence is not as high as that found beyond a year of age, it is sufficiently high to influence the management of infants beyond three months of age. Hemodynamic studies were performed following atrial baffle repair in 34 patients. All but one of the 16 patients operated upon prior to two years of age had a pulmonary arteriolar resistance (Rpa) less than 3 units M2 at the time of review, including three with a large communication at ventricular or great vessel level who underwent surgery before two months of age. Repair at an older age did not preclude a low Rpa on follow-up, but in 11 of the 18 patients who were two or more years of age at the time of repair it was greater than 3 units M2. None of these latter patients had a large ventricular septal defect or patent ductus arteriosus. Comparison of postoperative hemodynamic data with autopsy studies would suggest that advanced pulmonary vascular disease is associated with earlier death among patients with complete transposition of the great arteries.     

Surgical correction of truncus arteriosus in infancy

An 8 week old infant with severe heart failure from type 1 truncus arteriosus underwent successful corrective surgery employing the Rastelli procedure with use of deep hypothermia and total circulatory arrest. Postoperative hemodynamic studies showed complete closure of the septal defect, disappearance of truncal stenosis, but presence of mild porcine valve stenosis. This procedure is possible even in very small subjects and is preferable to palliative pulmonary arterial banding.