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A rare case of truncus arteriosus associated with mitral valve atresia, hypoplastic left ventricle, and intact ventricular septum is reported. Successful medical management and surgical palliation of this defect is described. The possible embryology, pertinent hemodynamics, and clinical concerns with this unusual case are discussed.
BACKGROUND: The identification of moderate to severe preoperative truncal valve regurgitation has been synonymous with significant postoperative mortality after neonatal repair of truncus arteriosus. Spurned by the deficiencies of current truncal valve substitutes surgeons are once again reexamining the option of truncal valve reparative techniques. METHODS: From May 1996 until June 1997, 8 children underwent correction of truncus arteriosus. A retrospective analysis was conducted. RESULTS: There was one in-hospital death secondary to a postoperative massive coronary air embolism. Moderate-to-severe truncal valve regurgitation was identified clinically and confirmed with cardiac ultrasound in 3 neonates with a mean age of 7 days (range, 4 to 12 days) all with quadracusp truncal valves. Successful truncal valve repair was accomplished in 2 infants, with a third neonate requiring homograft replacement with coronary reimplantation for failure in achieving valvular competence after attempted valvuloplasty. Postoperative echocardiograms in those neonates who underwent truncal valve repair confirmed a functional "tricuspid" valve with only mild to mild-plus regurgitation. CONCLUSIONS: We give further credence to the hypothesis that primary neonatal truncal valve repair is feasible and may be successful in the avoidance and delay of serial truncal valve replacements using either mechanical or allograft substitutes.
We describe successful management of pulmonary hypertension with a reversible aorto-pulmonary (central) shunt and inhaled nitric oxide gas after truncus arteriosus repair. A temporary central shunt may provide a lifeline in those cases refractory to pharmacologic pulmonary vasodilation as long as marginal systemic oxygenation can be maintained.
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BACKGROUND: Early complete repair of truncus arteriosus with homograft reconstruction of the right ventricular outflow tract was performed and long-term results were evaluated. METHODS: Review of 46 consecutive patients with truncus arteriosus who underwent primary correction between June 1987 and May 1997 was performed. Ages ranged from 21 days to 7.2 years (median, 62 days) and weights from 1.8 to 21.5 kg (median, 3.4 kg). Ten patients were operated on before 1 month of age, 20 between 1 and 3 months, 13 between 3 months and 1 year, and 3 at an older age. Associated cardiac conditions were encountered frequently, the most common being coronary artery anomalies (n = 16), truncal valve incompetence (n = 12), and interrupted aortic arch (n = 5). The right ventricular outflow tract was reconstructed with an aortic (n = 28) or a pulmonary homograft (n = 18). RESULTS: There were two hospital deaths (4.3%). Both patients had severe truncal valve regurgitation and interrupted aortic arch together with other unfavorable conditions. Survival in uncomplicated truncus arteriosus was 100%. Follow-up was from 3 months to 10 years (mean, 36 months). There was one late death 4 months after the initial repair, presumably because of cardiac reason. Actuarial survival was 93% at 4 months and beyond. Actuarial freedom of reoperation in the 27 hospital survivors with aortic homografts was 43% at 75 months; it was 73% at 62 months in the 17 patients surviving with pulmonary homografts. CONCLUSIONS: Neonatal or early infancy complete repair is the treatment with the best potential for survival. The homograft remains our conduit of choice to establish continuity between the right ventricle and the pulmonary artery. Management of severe truncal valve incompetence remains a surgical challenge.
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A 98-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type B) using a one-stage anterior approach. The interrupted aortic arch was reconstructed by direct anastomosis between the ascending and descending aorta. The ventricular septal defect was closed, and a 12-mm porcine valved conduit was placed on the right side of the ascending aorta to establish continuity between the right ventricle and pulmonary artery. Surgical procedures for the repair of this malformation are the focus of discussion.
BACKGROUND: Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. METHODS: Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. RESULTS: There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. CONCLUSIONS: Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.
BACKGROUND: Valved homograft conduit repair in neonates and young infants creates a physiologically normal biventricular circulation, and unlike shunts, avoids surgery on the branch pulmonary. METHODS: Retrospective chart review was used for 84 patients operated on between 1990 and 1995 (mean age 26+/-28 days, mean weight 3.3+/-0.8 kg) undergoing homograft conduit repair in the first 3 months of life. Cases were divided into simple and complex, eg, absent pulmonary valve syndrome or associated interrupted arch. Mean homograft size was 9.0+/-2 mm. RESULTS: Early mortality was 4.7% (simple) and 30% (complex). Mean hospital stay was 18 days. Mean follow-up was 34 months. Thirty-seven (47%) patients underwent conduit replacement. Median time to reoperation was 3.1 years. Mean size of replacement homograft was 17+/-2 mm. There were no deaths at reoperation. Mean hospital stay at conduit change was 6.3 days. Probability of survival at 5 years is 75%. CONCLUSIONS: Biventricular repair employing a conduit can be performed safely in noncomplex anomalies in the first 3 months of life. Time interval until repeat surgery is relatively short but equal or greater than that with most palliative procedures.
The coexistence of tricuspid atresia and common arterial trunk is extremely rare. We present a successful three-stage surgical treatment of this defect. The first stage included disconnection of the pulmonary arteries from the common trunk, atrial septectomy and systemic-to-pulmonary shunt; the second stage, a hemi-Fontan procedure; and the third stage, a fenestrated Fontan completion. The child is now 8 years old and is developing well.
The extant nomenclature for truncus arteriosus (TA) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A modified Van Praagh (VP) classification is proposed involving three main categories of TA: TA with confluent or near confluent pulmonary arteries (large aorta type, VP A1, A2), TA with absence of one pulmonary artery (VP A3), and TA with interrupted aortic arch or coarctation (large pulmonary artery type, VP A4). A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
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A family is presented in which both siblings and their father had evidence of third-fourth pharyngeal pouch syndrome (DiGeorge syndrome). All three individuals had hypocalcemia and unusual facies. Both infants had truncus arteriosus. One infant had evidence of impaired cell-mediated immunity; the father had a relatively decreased number of T-lymphocytes. The syndrome is uncommon, most cases being isolated, and familial presentations are even rarer. Two recent reports described several affected individuals who also had partial deletions of chromosome 22. Chromosome banding studies in our family were normal. Thus our family demonstrates an autosomal dominant pattern of inheritance, although it cannot be proved that this is a single gene defect. We propose that inasmuch as the presentation of the syndrome is quite varied, thorough family investigation including high-resolution cytogenetic analysis is necessary. Familial cases may be more common and require genetic counseling.
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OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.
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