Repair of truncus arteriosus and complete atrioventricular canal defect.
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The authors describe the rare case of an infant, 35 days old at operation, with truncus arteriosus in which the right pulmonary artery rose from the left posterolateral side of the truncus arteriosus above the truncal valve, and the left pulmonary artery from the inferior aspect of the proximal third of the aortic arch. At operation, the right ventricle-pulmonary artery continuity was reconstructed with an aortic homograft. The left pulmonary artery was anastomosed "end-to-side" to the left carotid artery of the aortic homograft, and the right pulmonary artery "end-to-side" to the joined openings of the left subclavian artery and descending thoracic aorta. It was difficult to understand if the first tract of the left pulmonary artery (2 mm in diameter) was a true pulmonary artery, a ductus arteriosus or an anomalous vessel. The infant survived the operation. Intensive postoperative treatment was necessary to counteract severe episodes of pulmonary hypertension. Enoximone 5 mcg/Kg/min and prostacyclin 5-10 mcg/Kg/min were effective in reducing pulmonary vascular reactivity. Four month follow-up shows optimal surgical correction and normal hemodynamic findings.
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A 32-year-old male with a diagnosis of truncus arteriosus (Van Praggh Type A3) underwent successfully a total correction by the modified Rastelli procedure. The bilateral pulmonary arteries (PA) were disconnected from the truncus, and the ventricular septal defect was rerouted with a Dacron patch from the left ventricle to the true aorta. A new outflow from the right ventricle to the bilateral PA was established by the use of a T tube graft which consisted of a Rygg's valved conduit and a Rygg's pericardium. The patient had recent cardiac catheterization with slight elevated pulmonary hypertension (Rt-PA 40/10 mmHg, Lt-PA 30/10 mmHg), but is doing well at 5 years postoperatively.
A surgical technique is described for correction of truncus arteriosus type II, in which the confluence of the pulmonary arteries is translocated anteriorly to the divided truncal root. In this technique, the truncal root is reconstructed, and the ventricular septal defect is closed through a right ventriculotomy. The outflow tract of the right ventricle is reconstructed by using a cryopreserved aortic homograft, with the pulmonary artery confluence lying anteriorly to the aorta. This positioning may facilitate future reoperation in patients with this anomaly by obviating dissection around the truncal artery.
This report described a case in which purulent mediastinitis involving a Hancock prosthesis developed after repair of truncus arteriosus in a 6-year-old boy. The graft and surrounding area became sterilized after having been irrigated with povidone-iodine and antibiotic solutions for 6 weeks. At his most recent visit, the child was completely active and asymptomatic.
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From September 1986 through December 1991, 63 patients with truncus arteriosus underwent surgical repair. The management approach evolved over the period of the study from elective primary repair at 3 months of age to elective primary repair in the early neonatal period. Thirty variables were examined as potential risk factors for the outcome events of death, reoperation, and presence of pulmonary vascular morbidity. According to both univariate and multivariate techniques, severe truncal valve regurgitation, interrupted aortic arch, coronary artery anomalies, and age at repair greater than 100 days were important risk factors for perioperative death. In the 33 patients without these risk factors, early survival was 100%. In the 30 patients with one or more of these risk factors, survival was 63%. Pulmonary hypertensive episodes were fewer, and duration of ventilator dependence and pulmonary artery pressure were significantly less in patients undergoing the operation before 30 days of age. Seven patients required reoperation for right ventricular outflow tract obstruction at a mean follow-up time of 23.4 months, with no deaths. In conclusion, interrupted aortic arch, severe truncal valve insufficiency, coronary anomalies, and repair later than 100 days of age were risk factors for death after repair of truncus arteriosus. In the absence of these associations, truncus arteriosus can be repaired with excellent surgical outcome in the neonatal and early infancy period. Repair in the early neonatal period reduces the prevalence of postoperative pulmonary vascular morbidity.
Although the early mortality for repair of truncus arteriosus has decreased in the modern era, routine correction in the neonate has not been widely adopted. To assess the results of our protocol of early repair, we reviewed 46 neonates and infants undergoing repair of truncus arteriosus at the University of Michigan Medical Center from January 1986 to January 1992. Their ages ranged from 1 day to 7 months (median 13 days) and weights from 1.8 kg to 5.4 kg (mean 3.1 kg). Repair was performed beyond the first month of life in only 8 patients, because of late referral in 7 and severe noncardiac problems in 1. Associated cardiac anomalies were frequently encountered, the most common being interrupted aortic arch (n = 5), nonconfluent pulmonary arteries (n = 4), hypoplastic pulmonary arteries (n = 4), and major coronary artery anomalies (n = 3). Truncal valve replacement was performed in 5 patients with severe regurgitation, 3 of whom also had truncal valve systolic pressure gradients of 30 mm Hg or more. The truncal valve was replaced with a mechanical prosthesis in 2 patients and with a cryopreserved homograft in 3 patients. Right ventricle-pulmonary artery continuity was established with a homograft in 41 patients (range 8 mm to 15 mm), a valved heterograft conduit in 4 (range 12 mm to 14 mm), and a nonvalved polytetrafluoroethylene tube in the remaining patient (8 mm). There were 5 hospital deaths (11%, 70% confidence limits 7% to 17%). Multivariate and univariate analyses failed to demonstrate a relationship between hospital mortality and age, weight, or associated cardiac anomalies. Only 1 death occurred among 9 patients with interrupted aortic arch or nonconfluent pulmonary arteries. Hospital survivors were followed-up from 3 months to 6.3 years (mean 3 +/- 0.4 years). Late noncardiac deaths occurred in 3 patients, all within 4 months after the operation. Actuarial survival was 81% +/- 6% at 90 days and beyond. Despite the prevalence of major associated conditions, early repair has resulted in excellent survival. We continue to recommend repair promptly after presentation, optimally within the first month of life.
Identification of the genetic basis for congenital heart defects has been a topic of inquiry for pediatric cardiologists for many years. Any genetic model proposed must account for three often puzzling features of congenital heart diseases, namely the high population incidence, the less than Mendelian recurrence risk, and low concordance rates of heart lesions within individual families. A multifactorial or polygenic inheritance model has been used to explain these observations. Although this model has been serviceable, it does not adequately explain the phenotypic variability within families. Further, the emphasis on teratogenic influences has not led to the elucidation of pathogenetic mechanisms. The multifactorial model should be modified to include the role of chance as originally proposed by Kurnit. Once the role of chance is acknowledged, the phenotypic variability of congenital heart diseases becomes less problematic, and the multifactorial model gives way to a model in which single genes are capable of producing congenital heart disease. Through recent work in animal models and humans, it is now clear that single congenital heart disease genes can be identified that produce phenotypic variability in families that is similar to that seen in the population as a whole. Examples demonstrating recent progress in the search for major congenital heart disease genes are discussed.
Physical examination, chest roentgenography, phonocardiography, and right heart catheterization were used to evaluate the function of composite aortic homografts inserted as right ventricular outflow tracts in 13 patients 1.8 to 7 years previously. All homografts in these patients who were followed for 2 or more years demonstrated calcification of the aortic wall, but valve leaflets were spared. Moderate pulmonary insufficiency was present in eight patients with single cusp partial homografts, but was well tolerated in all. Homograft valve insufficiency was mild in five patients with complete homografts. No patient had an outflow tract gradient greater than 32 mm Hg. The average gradient was 18.5 +/- 2.7 (SEM) mm Hg. Abnormality of pulmonary blood flow was found in three patients and could be related to previous Waterston or Potts anastomoses. No late morbidity due to homograft degeneration has occurred and the long-term function of these conduits appears to be good.
Eighty-five patients operated on before January 1976 have survived at least 30 days after insertion of a valved external conduit by the venous ventricle (usually the right) and the pulmonary arteries as a part of the repair of their congenital heart disease. Follow-up information is available on 83 patients, and mean follow-up time has been 27.7 months. Seven patients have required reoperation because of obstructive complications. Actuarial analysis indicates that the proportion of patients requiring reoperation is 4.0 +/- 2.8% at 2 years, 12.9 +/- 5.5% at 3 1/2 years, and 30.4 +/- 12.1% at 5 years. In five patients compression of the conduit between the sternum and heart was the primary cause of the obstruction. The proportion requiring reoperation for conduit compression is 2.0 +/- 2.0% at 2 years, 8.6 +/- 4.9% at 3 1/2 years, and 27 +/- 12.4% at 5 years. Age at operation is not related to the proportion of patients requiring reoperation, nor is the type of conduit. No patients have yet required reoperation for conduit compression whose operation has been done since we began systematically to position the conduit well away from the sternum. By actuarial analysis the proportion of patients surviving 5 years is 76.7%. Of those alive at follow-up examination, 78% were without limitation of activity.
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OBJECTIVE: The preventive efficacy of the periconceptional use of multivitamins is well established for neural tube defects, much less so for other birth defects. We conducted a population-based, case-control study to assess the effects of multivitamin use on the risk for conotruncal defects, a group of severe heart defects that includes transposition of the great arteries, tetralogy of Fallot, and truncus arteriosus. METHODS: From the population-based Atlanta Birth Defects Case-Control Study, we identified 158 case infants with conotruncal defects and 3026 unaffected, randomly chosen control infants, born from 1968 through 1980 to mothers residing in metropolitan Atlanta. Periconceptional multivitamin use was defined as reported regular use from 3 months before conception through the third month of pregnancy. We present the results of the crude analysis, because the multivariate model yielded essentially identical results. RESULTS: Mothers who reported periconceptional multivitamin use had a 43% lower risk of having infants with conotruncal defects (odds ratio [OR], 0.57; 95% confidence interval [CI], 0.33 to 1.00) than did mothers who reported no use. The estimated relative risk was lowest for isolated conotruncal defects (OR, 0.41; 95% CI, 0.20 to 0.84) compared with those associated with noncardiac defects (OR, 0.91; 95% CI, 0.33 to 2.52) or a recognized syndrome (OR, 1.82; 95% CI, 0.31 to 10.67). Among anatomic subgroups of defects, transposition of the great arteries showed the greatest reduction in risk (OR, 0.36; 95% CI, 0.15 to 0.89). CONCLUSIONS: Periconceptional multivitamin use is associated with a reduced risk for conotruncal defects. These findings could have major implications for the prevention of these birth defects.
The surgical treatment for truncal valve regurgitation is still controversial in patients with truncus arteriosus. A two-year-old girl with complaints of low weight gain and tachypnea was referred for treatment of truncal valve regurgitation. She had undergone an emergency pulmonary artery banding for severe congestive heart failure due to truncus arteriosus-type I at six months of age. This anomaly had been corrected by Barbero-Marcial method at seven months of age. But the truncal valve regurgitation started appearing at sixteen months of age with the progression of the stenosis of the pulmonary artery orifice and the right ventricular outflow tract regurgitation. Echo cardiography and cineangiography revealed the truncal valve to be bicuspid, and the regurgitation severe, especially through the prolapsed left sided cusp. The truncal valve was repaired by commissural suspension method, and the right ventricular outflow tract reconstructed with patch angioplasty of the pulmonary artery orifice and Carpentier-Edwards pericardial Bioprosthesis (19 mm). The post-operative course was uneventful. One year after, truncal valve regurgitation is small by color Doppler study. We conclude that valvoplasty is to be considered as the first choice of treatment for truncal valve regurgitation.
We report a case of myonephropathic metabolic syndrome (MNMS) and compartment syndrome caused by femoral arterial cannulation during long-term cardiopulmonary bypass. A boy who had been diagnosed with truncus arteriosus type 1 and who subsequently underwent a Rastelli operation at age 3 months had a second Rastelli operation at age 6 years due to graft stenosis (pressure gradient 82 mmHg between RV and PA). MNMS and compartment syndrome occurred postoperatively due to long-term femoral arterial cannulation during cardiopulmonary bypass. However, acute renal failure was successfully managed with CAPD. The patient recovered from acute renal failure 40 days postoperatively. CAPD is considered useful for postoperative acute renal failure in infants. We conclude that retrograde insertion of the cannula should be changed to anterograde insertion at an earlier time, or else the cannula should be inserted via the prosthetic graft.