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PubMed2006

Truncus arteriosus with interrupted aortic arch: successful repair using modified cardiopulmonary bypass and surgical techniques.

A neonate with truncus arteriosus (type 1) and interrupted aortic arch (type A) associated with an excessively large gap between interrupted aortic segments underwent successful reconstruction of the aortic arch with a partial subclavian flap aortoplasty plus bovine jugular patch (Contegra 200, Medtronic, Inc, Minneapolis, MN) using continuous selective low-flow cardiopulmonary bypass without circulatory arrest. The ventricular septal defect was closed with a Dacron (IMPRA, Inc, Tempe, AZ) patch, and a Contegra prosthesis was used to re-establish right ventricle to pulmonary artery continuity. At an 18-month follow-up, the patient was free of symptoms with normal development. Echocardiography revealed a widely patent aortic arch and an unobstructed right ventricle to pulmonary artery connection.

Anastomosis, Surgical↗
PubMed2006

Truncus arteriosus communis: early and midterm results of early primary repair.

BACKGROUND: Repair of truncus arteriosus communis (TAC) in the neonatal and early infant period has become standard practice in many centers. We report our experience on early primary repair of TAC, with a focus on early and midterm results. METHODS: From July 1993 to December 2005, 29 patients with median age 28 days (range, 11 to 127), and median body weight 3.1 kg (range, 2.6 to 5.9 kg), underwent primary repair of TAC. The anatomical type of TAC was as follows: A1-2, 27; A3, 0; and A4, 2. Right ventricular outflow tract was reconstructed with an aortic (n = 7) or pulmonary homograft (n = 8), or a bovine (n = 11) or porcine valved xenograft (n = 3). Follow-up was complete for all patients. RESULTS: Hospital mortality was 3.4% (1 death due to respiratory infection). At a mean follow-up of 74 months (range, 2 to 149), 1 patient died suddenly 2 months after surgery (6-year actuarial survival 93%). Of the 27 midterm survivors, 14 (52%) underwent 30 interventional procedures including percutaneous balloon dilation with or without stenting for right ventricular outflow tract or branch pulmonary artery obstruction. Eight of them were reoperated on for right ventricle-to-pulmonary artery conduit replacement (n = 8, 23%), and aortic valve regurgitation (n = 1, 3.4%). The overall freedom from any reintervention at 6 years was 50%. Aortic valve regurgitation was trace in 15 patients, mild in 8, moderate in 4. All midterm survivors but 1 (26 of 27) had good ventricular function. CONCLUSIONS: Truncus arteriosus communis repair can be performed early with very low perioperative mortality and satisfactory midterm morbidity; the latter is mainly attributed to right ventricular outflow tract reconstruction. Interventional cardiac catheterization delays inevitable conduit replacement.

Cardiac Surgical Procedures↗
PubMed2006

Complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus communis.

Truncus arteriosus communis is a congenital heart malformation, which is usually repaired in the neonatal period or early infancy. Although results of repair are good, there is long-term morbidity caused by reoperations mainly owing to right ventricle to pulmonary artery conduit exchange or stenosis at the pulmonary artery bifurcation as recently reported for Contegra conduits. We present a new technique for complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus type I and II.

Anastomosis, Surgical↗
PubMed2005

Prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies.

OBJECTIVES: This study was designed to determine the prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies and to assess the clinician's ability to predict the presence or absence of 22q11.2 microdeletion on the basis of clinical features. BACKGROUND: It is known that 22q11.2 microdeletion is a chromosomal anomaly with cardiac and extracardiac manifestations. The prevalence and manifestations in adults have not been well characterized. METHODS: A total of 103 consecutive adults with either tetralogy of Fallot (TOF), pulmonary atresia/ventricular septal defect (PA/VSD), or truncus arteriosus (TA) were prospectively screened for 22q11.2 microdeletion using a fluorescence in situ hybridization (FISH) assay. Clinicians were asked to predict 22q11.2 microdeletion status on the basis of clinical features. A geneticist blinded to FISH assay results reviewed photographs of the patients for typical dysmorphic features of 22q11.2 microdeletion. RESULTS: Six patients (prevalence 5.8%, 95% confidence interval 1.3 to 10.3) had 22q11.2 microdeletion (3 with TOF, 2 with PA/VSD, 1 with TA). In two of these patients, the clinician incorrectly predicted absence of the deletion. In three, typical dysmorphic features of 22q11.2 microdeletion were absent. CONCLUSIONS: Our work showed that 22q11.2 microdeletion is under-recognized in adults with congenital heart disease. The absence of typical phenotypic features makes it difficult to correctly predict if the deletion is present. Screening for 22q11.2 microdeletion should be considered in adults with high-risk cardiac lesions, as it has important implications in reproductive counseling and surveillance for associated extracardiac manifestations.

Abnormalities, Multiple↗
PubMed2005

The effect of repair technique on postoperative right-sided obstruction in patients with truncus arteriosus.

OBJECTIVES: We reviewed our experience with repair of truncus arteriosus to assess the effect of type of right ventricular outflow tract reconstruction on perioperative morbidity, survival, and freedom from catheter-based interventions and reoperation. METHODS: Patients undergoing repair of truncus arteriosus from June 1990 through February 2004 were evaluated on the basis of operative procedure regarding preoperative and postoperative variables, the need for postoperative catheter-based intervention or reoperation, and survival on the basis of univariate, multivariable, and actuarial analyses. RESULTS: Of 54 study patients, 15 (28%) received a valved homograft, and 39 (72%) received a direct connection with a variety of hood materials. Five (9.1%) patients died. Valved homograft recipients were more likely to require reoperation than patients receiving direct connections (40% vs 15%, P = .046); however, valved homograft and direct connection recipients had a similar incidence of the combined end point of reoperation or catheter-based intervention (40.0% vs 37.5%, P = .865). Univariate and multivariable modeling demonstrated use of valved homografts or direct connections with an autologous pericardial hood to be predictive of the need for later catheter-based intervention or reoperation. Actuarial analysis demonstrated a trend toward improved outcomes in the direct connection group and within the direct connection cohort, a statistically significant difference on the basis of hood type. CONCLUSIONS: Although the direct connection technique might not prevent later catheter-based intervention, it does reduce the need for reoperation. Outcomes among direct connection recipients were associated with hood type: polytetrafluoroethylene hoods (W. L. Gore & Associates, Inc, Tempe, Ariz) had the lowest rate of reintervention, and untreated autologous pericardial hoods had the highest rate of reintervention. We report excellent outcomes with primary repair of truncus arteriosus. Where anatomically appropriate, we advocate the direct connection technique.

Cardiac Surgical Procedures↗
PubMed2006

Evaluation of downsized homograft conduits for right ventricle-to-pulmonary artery reconstruction.

OBJECTIVE: Although homograft conduits are frequently used to establish right ventricle-to-pulmonary artery continuity, the limited availability of small-size homografts is a significant constraint in pediatric cardiac surgery. We compared the performance of standard homograft conduits with that of surgically reduced bicuspid homograft conduits in patients undergoing repair of truncus arteriosus. METHODS: Forty infants undergoing complete repair of truncus arteriosus with either standard homografts (n = 26) or reduced-size bicuspid homografts (n = 14) were evaluated. RESULTS: The median downsized conduit diameter (13 mm) was similar to the standard homograft diameter (12 mm, P = .52). There were 6 early deaths and 5 late deaths, representing an overall 30-day mortality of 15% and a 5-year mortality of 25%. No deaths were directly related to homograft dysfunction. Four (29%) downsized conduits and 8 (31%) standard conduits required replacement at a median interval of 18.5 months and 42.4 months, respectively. Catheter-based interventions were required in 5 (36%) patients in the downsized group and in 3 (12%) patients in the standard group. There was no difference in freedom from surgical or catheter-based reintervention between the 2 groups (P = .42). Freedom from conduit failure (severe conduit stenosis, moderate or greater regurgitation) was 55.9% and 17.2% at 3 years in the downsized and standard groups, respectively. CONCLUSION: The surgically downsized homograft is an excellent option when an appropriate-sized homograft is not available and might prevent morbidity associated with the use of an oversized conduit.

Anastomosis, Surgical↗
PubMed2001

Echocardiographic features and outcome of truncus arteriosus diagnosed during fetal life.

There are few data on the outcome of truncus arteriosus when this diagnosis is made during fetal life. Such prognostic information is important to assist parental counseling during pregnancy. This study aimed to analyze, retrospectively, the echocardiographic features and outcome of fetuses with truncus arteriosus. A database of those presenting to a tertiary center for fetal cardiology between 1990 and 1999 was reviewed. Cases in which truncus arteriosus was identified as a firm or differential diagnosis were selected. Outcome data were derived from clinical records, and fetal echocardiograms were reviewed retrospectively. At presentation, truncus arteriosus was firmly diagnosed in 16 patients and was a differential diagnosis in 12. Fourteen of 16 (87%) of the firm diagnoses were correct. There were 17 confirmed cases of truncus arteriosus. Pregnancy was terminated in 4 patients (24%) and there were 13 live births. One child was not actively treated, 4 (31%) died preoperatively, and 8 (61%) underwent surgery. Thirty-day surgical mortality was 2 of 8 (25%). There was 1 late death after cardiac catheterization, and overall survival on an intention-to-treat basis was 5 of 12 (42%). Five of 6 patients with a prenatal truncal valve Doppler velocity above the normal aortic range were found to have postnatal truncal valve stenosis. Two fetuses with stenotic valves died preoperatively with sudden cardiovascular collapse. Counseling of parents for fetuses with truncus arteriosus should include the relatively high nonsurgical mortality as well as surgical results. Elevated prenatal truncal valve Doppler velocity predicts postnatal truncal valve stenosis. Fetuses with truncal valve stenosis may be at risk of early sudden death.

Female↗
PubMed1976

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.

Adolescent↗
PubMed1984

Pulmonary vascular disease in children with truncus arteriosus.

Pulmonary vascular structure was analyzed using quantitative morphometric techniques in lung biopsy (n = 17) and/or postmortem specimens (n = 18) from 23 patients aged 18 days to 13 years, with truncus arteriosus (TA) type I or II. All 14 patients younger than age 1 year, showed abnormal extension of muscle and an increase in pulmonary arterial medial thickness (p less than 0.01 in 18 of 24 biopsy and autopsy specimens), whereas 6 showed intimal proliferation. Structural abnormalities were usually potentially reversible, even in 5 infants with a pulmonary arteriolar resistance of greater than or equal to 8 units X m2. Abnormalities were more severe in 9 patients aged 3.5 to 13 years, 6 with medial atrophy and 4 with pulmonary arteriolar resistance of greater than 8 units X m2 that also showed intimal fibrosis, with or without plexiform lesions or hyalinized arteries. Findings indicate that biopsy specimens are helpful in assessing the severity of pulmonary vascular disease, and are representative of the entire pulmonary vascular bed (avoiding the lingula); however, interpretation can be difficult if a biopsy specimen is taken of only the peripheral intraacinar arteries, distal to the most damaged vessels. An intracardiac repair is recommended by 6 months, at which time severe pulmonary arterial medial hypertrophy and intimal proliferation can be expected to prejudice the repair.

Adolescent↗
PubMed1999

Truncus arteriosus with anomalous pulmonary venous connection.

The anatomic, diagnostic, and management findings of 6 patients with truncus arteriosus and anomalous pulmonary venous connections are described. Additional risk factors indicative of poor prognosis were found in 3 of 4 patients with truncus arteriosus and totally anomalous pulmonary venous connection and in 1 patient with partially anomalous pulmonary venous connection.

Cause of Death↗
PubMed1993

Clinical results of cryopreserved valved conduits in the pulmonary ventricle-to-pulmonary artery position.

Aortic valved homograft conduits (AVHC) have become valuable in the pulmonary ventricle (PV)-to-main pulmonary artery (MPA) reconstruction in congenital heart defects. Since 1985, 45 patients, ranging in age from 12 days to 32 years, underwent PV-to-MPA reconstruction utilizing cryopreserved AVHC. Operative deaths included seven patients (16%), six of whom died as a result of the complexity of their underlying heart defects. One late death (2%) occurred as a result of infective endocarditis 48 months after conduit placement. The 38 patients who survived the operation remained in the intensive care unit for a mean of 5.7 +/- 1.0 days (median: 4 days; range: 2 to 37 days). The mean hospital stay was 13.0 +/- 1.8 days (median: 9 days; range: 6 to 63 days). The mean follow-up was 40.0 +/- 3.6 months (median: 40 months; range: 10 months to 7.1 years). Only two patients (5%) required reoperation for conduit stenosis with systolic pressure gradients of 60 to 80 mm Hg at 10 and 14 months, respectively, after operation, and both reoperations were successful. During outpatient visits, 16 patients are totally asymptomatic, and 21 patients have minimal symptoms (New York Heart Association class II). Only 10 patients (26%) require digoxin, and 2 patients (5%) need diuretics as part of their medical regimen. Recent echocardiographic examinations show insignificant pressure gradients in all 37 currently surviving patients. Thus, barring operative mortality, which is almost always associated with the nature of the underlying heart defect, the use of cryopreserved AVHC is a safe and effective alternative for PV-to-MPA reconstruction.

Adolescent↗
PubMed2001

Repair of truncus arteriosus in early infancy with antibiotic sterilized aortic homografts.

BACKGROUND: To evaluate the fate of the truncal valve, the antibiotic sterilized aortic homografts, and the survival after repair of truncus arteriosus in the first 6 months of life. METHODS: Between 1974 and 1994, 23 infants (mean age 1.7 months, range 5 days to 6 months) underwent primary repair of truncus arteriosus by one surgeon (J.L.M). Sixteen were neonates (age range 5 to 30 days). Continuity between the right ventricle and the pulmonary artery was established with an aortic antibiotic sterilized homograft (mean diameter 14.9 mm, range 11 to 17 mm). Follow-up was 100% complete. RESULTS: Four neonates with severe truncal regurgitation died early (17.4%). Fourteen patients underwent reoperations. Five had a truncal valve replacement (mean time 7 years, range 6 months to 17 years). Ten-year freedom from truncal valve replacement was 78.2%. Eleven patients had homograft replacement (mean time 12.7 years, range 2 to 26.2 years). Ten-year freedom from homograft replacement for any cause was 77.1% (for homograft-related problems it was 86.7%). Seven patients retained the original homografts (mean time 14.3 years, range 6 to 18.7 years). There was one late death. Overall 10-year survival was 79% and for the hospital survivors it was 95%. All survivors are in New York Heart Association functional class I. CONCLUSIONS: Abnormal truncal valves pose serious early and late problems but the patients with normal truncal valves do well and seem unlikely to need replacement of these valves. The durability of the antibiotic sterilized aortic homograft even in sizes less than 14 mm is remarkably good. Late survival is excellent.

Adolescent↗
PubMed2001

Surgical management of severe truncal insufficiency: experience with truncal valve remodeling techniques.

BACKGROUND: Truncal valve insufficiency has been a significant short- and long-term risk factor for repair of truncus arteriosus. Recent reports have documented the virtues of truncal valve repair. The purpose of this report is to review our experience with truncal valve repair and illustrate our techniques. METHODS: Between 1995 and 2000, 8 patients had interventions for severe truncal valve insufficiency at primary repair (3 patients) or in conjunction with conduit replacement (5 patients). One neonate had truncal valve replacement at initial repair early in the experience. The other 7 patients had truncal valve repair, 3 by valvar suture techniques. The remaining 4 patients had leaflet excision and annular remodeling in 3 (coronary reimplantation was required in 2) and commissure resuspension in 1 patient. RESULTS: Trivial to mild truncal valve insufficiency is present in the patients who had leaflet excision and annular remodeling (n = 3) and commissure resuspension (n = 1). Of the 3 patients who had valvar suture truncal valve repair, there was one death and 2 patients required acute valve replacement. The 7 survivors are doing well 1 month to 6 years postoperatively. CONCLUSIONS: Truncal valve repair by valvar suture techniques has not been successful in our practice. Truncal valve remodeling by leaflet excision and reduction annuloplasty is an effective method for truncal valve repair. When leaflet excision of a coronary sinus of Valsalva is required, coronary artery translocation can be accomplished.

Adolescent↗