Invited letter concerning: total cavopulmonary connection.
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Anastomosis, Surgical↗
Biomedical subjects
A R Castaneda
Publications and source records attributed to A R Castaneda.
The results of a surgical program for interrupted aortic arch.
Seventy-one patients with interrupted arch entered the Boston Children's Hospital between Jan. 1, 1974, and Jan. 1, 1987, of whom 63 underwent an operation. Type B was the most prevalent form of interrupted arch, and ventricular septal defect alone was the commonest coexisting cardiac anomaly. Among the 63, the 30-day and the 1-, 5-, and 10-year survival rates were 61%, 52%, 48%, and 47%, respectively. The mortality rate declined strikingly during the experience, and by multivariate analysis in patients with coexisting ventricular septal defect operated on in 1986, the probability of death within 2 weeks of repair was only 7%. Also, preoperative therapy became progressively more intense and more prolonged. The complication of left ventricular outflow tract obstruction developed in eight of the 33 patients undergoing repair of interrupted arch and of isolated ventricular septal defect. The time-related freedom from this complication was 97%, 78%, and 58% at 1 month, 1 year, and 3 years, respectively. Seven of the eight patients underwent a surgical procedure directed against the left ventricular outflow tract obstruction, and all have survived. Recurrent or persistent aortic arch obstruction became evident after repair in 15 patients and appeared more frequently and earlier after direct anastomosis than after tube graft repair. All patients had either reoperation or balloon dilation, but all were alive at follow-up. Most surviving patients are active and without symptoms. Inferences: An aggressive surgical program can result in survival and a good clinical state for at least 10 years after birth of over 40% of patients born with interrupted arch. Multiple anatomic bases account for the development of left ventricular outflow tract obstruction in about 50% of the patients undergoing repair of interrupted arch with coexisting ventricular septal defect. Repair by direct anastomosis combined with repair of the coexisting defect whenever possible is optimal therapy.
Aorta, Thoracic↗
The early results of treatment of simple transposition in the current era.
A total of 187 neonates with simple transposition of the great arteries (TGA) have been entered into a twenty institution cooperative study between Jan. 1, 1985, and June 1, 1986. Eighty-two percent entered within the first 2 days of life. Seventy-six of the 187 patients were initially entered into a treatment protocol leading to an arterial switch repair, 45 into one leading to an atrial switch (Mustard) repair, and 49 into one leading to a Senning repair. Five (7%) of the patients entered initially into an arterial switch protocol later were crossed over to an atrial switch protocol, and 7% of those entered into an atrial switch (Mustard) protocol were crossed over to an arterial switch protocol. Formal follow-up information is available on all patients. Only 5% of patients entered into an arterial switch protocol were without repair 2 weeks after entry, whereas 96% and 100%, respectively, of those entered into a Mustard or Senning atrial switch protocol were without repair at that time. Ninety-seven percent of the first group had no preliminary procedures other than balloon atrial septostomy, which was true of only 76% and 90%, respectively, of the last two groups. No deaths before repair occurred in the first group, whereas five occurred in the latter two groups (P = 0.06). Overall survival rate among the 187 patients was 81% at 1 year. The only risk factors for death were birth weight, date of entry into the study, and an arterial switch protocol in the group of institutions at high risk for arterial switch repair; neither an arterial nor an atrial switch protocol was a risk factor per se. According to the multivariate equation, the 12-month predicted survival rate of a patient with a birth weight of 3.4 kg, entered currently into atrial or arterial switch protocols (except in the high risk for arterial switch group of institutions) is 92%.
Humans↗
Infundibular septal resection: surgical anatomy of the superior approach.
A technique for extensive resection of the infundibular septum through the superior transarterial approach is proposed. Infundibular septal resection facilitates intraventricular rerouting for anatomic correction of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle. This technique also may be used to enlarge progressively obstructive but physiologically advantageous ventricular septal defects, for example, with tricuspid atresia. Through the superior transaortic or transpulmonary approach, the landmarks of the infundibular septum are the intercoronary commissure of the aortic valve and the septal commissure of the pulmonary valve, both of which are directly above the middle of the infundibular septum. With this exposure, the infundibular septum may then be resected easily and relatively completely and a large opening created immediately beneath the semilunar valves. The feasibility of infundibular septal resection by the superior approach was assessed post mortem in 25 cases of transposition of the great arteries with ventricular septal defect and in 20 autopsied cases of double-outlet right ventricle. This technique was subsequently applied successfully to enlarge the obstructive ventricular septal defect of a 2-year, 11-month-old girl with D-transposition of the great arteries. The advantages of the proposed technique include technical ease, relative completeness of infundibular septal resection, and the facilitation of intraventricular repair of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle.
Adolescent↗
Anterior chest wall deformities and congenital heart disease.
Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.
Adolescent↗
Obstructed pulmonary venous drainage with total anomalous pulmonary venous connection to the coronary sinus.
Obstructed pulmonary venous drainage is generally considered extremely rare with total anomalous pulmonary venous connection (TAPVC) to the coronary sinus (CS). A retrospective review of 27 operated patients with TAPVC to CS revealed 6 cases of obstruction (22%). Two of 6 patients who died early had evidence of obstruction at the pulmonary vein confluence at autopsy. Among the 21 hospital survivors, obstruction proximal to the point of CS unroofing developed in 4 (19%), necessitating reoperation and resulting in death in 3. One other patient died late. Mean follow-up of the 17 long-term survivors, who are generally doing well, is 85 months (range 2 to 212). There have been no cases of late obstruction at the site of unroofing among 10 patients who underwent the fenestration procedure suggested by Van Praagh. The incidence of early bradyarrhythmias (60%) does not appear to be decreased by this procedure. Review of 13 autopsy specimens suggests that if the right and left pulmonary veins did not drain directly to the CS but converged to form a short common vertical vein (4 cases), obstruction was likely. When pulmonary artery pressure approaches systemic levels preoperatively, careful echocardiographic and intraoperative assessment of the junction of the pulmonary vein confluence with the CS should be made. If there is evidence of obstruction, consideration should be given to anastomosing the horizontal right and left pulmonary veins directly to the left atrium rather than performing a simple unroofing procedure.
Coronary Vessel Anomalies↗
Accuracy of subcostal two-dimensional echocardiography in prospective diagnosis of total anomalous pulmonary venous connection.
Since reparative surgery without cardiac catheterization has been advocated for certain lesions such as total anomalous pulmonary venous connection (TAPVC), it is important to assess the accuracy of two-dimensional (2-D) echocardiography in the prospective diagnosis of this lesion. Abandoning traditional cardiac catheterization and angiography would be justified only if all surgically relevant anatomic features could be consistently displayed noninvasively. Consequently, we reviewed our experience with TAPVC to determine the suitability of subcostal 2-D echocardiography as the sole prospective diagnostic tool. Over a 5-year period, of 2444 infants under 2 years of age who underwent subcostal 2-D echocardiography, 38 were diagnosed as having TAPVC as the only major cardiac defect. There were no false-negative or false-positive cases during the time of the study. Drainage sites were diagnosed correctly in 36 (95%). Of the five cases of mixed-type TAPVC, the second drainage site was missed by 2-D echocardiography in two cases.
Echocardiography↗
Nonoperative removal of entrapped thoracic intracardiac monitoring catheters: a new bedside technique.
Flexible guidewires introduced into transthoracic intracardiac monitoring catheters can release entrapped areas of acute angulation caused by fascial closure sutures. This technique has been successfully applied to adult and pediatric patients with various sized guidewires and catheters, thus obviating the need for surgical removal of the entrapped monitoring catheters. Because entrapment with subsequent fracture of these monitoring catheters represents the most common complication of their use, lessening of this risk supports their continued widespread implementation.
Catheters, Indwelling↗
Biological sealants and knitted Dacron conduits: comparison of collagen and fibrin glue pretreatments in circulatory models.
To assess the healing characteristics of knitted Dacron conduits sealed with fibrin glue, collagen cross-linked with glutaraldehyde, and collagen cross-linked with formaldehyde, composite conduits, each constructed from two of the three test materials, were implanted in the pulmonary and systemic circulations of sheep and dogs for 3 and 6 months. Relative rates and morphologies of sealant resorption and other histological findings were predicted by a previous study involving subcutaneous implantation of the same graft materials in the rat. In particular, both studies revealed that delayed resorption of collagen cross-linked with glutaraldehyde and collagen cross-linked with formaldehyde resulted in lack of adhesion between inner capsule and conduit, thereby causing focal hemorrhagic dissection in the circulatory implants. There were no important qualitative or quantitative differences in healing between sheep and dogs or in pulmonary versus systemic location of the conduit. Rendering an animal cyanotic by placing a pulmonary arterial-left atrial shunt or by increasing or decreasing the intraconduit systolic pressure did not obviously affect healing. The results emphasize that the biological consequences of sealed grafts are strongly dependent on the sealant material. Porosity control of knitted Dacron with biological sealants that undergo minimal or delayed resorption may lead to poor tissue adhesion and resultant complications.
Animals↗
Atrioventricular valve repair or replacement in atriopulmonary anastomosis: surgical considerations.
Three patients who underwent a modified Fontan-Kreutzer (F-K) operation required additional procedures to correct left atrioventricular valve regurgitation. Valve replacement was performed in two additional procedures, and initial annuloplasty with a Carpentier ring was carried out in the third. All 3 survived the combined procedures and are doing well after a follow-up period ranging from five months to three years. More frequent presentation of these types of patients can be expected with broadening indications for F-K procedure to more complex lesions.
Adolescent↗
Surgical management of subaortic obstruction in single left ventricle and tricuspid atresia.
Subaortic obstruction caused by either a restrictive bulboventricular foramen in single left ventricle with an outflow chamber or by a restrictive ventricular septal defect in tricuspid atresia with transposition of the great arteries can lead to a hypertrophied, noncompliant ventricle and excessive pulmonary blood flow. This combination is disadvantageous to potential Fontan procedure candidates because they are dependent on good ventricular function and low pulmonary vascular resistance for survival. The results of surgical procedures to directly or indirectly relieve significant subaortic obstruction (gradient greater than 30 mm Hg) in 24 patients, 16 with single left ventricle and 8 with tricuspid atresia, were reviewed. Four patients had a left ventricular apex to descending aorta valved conduit; none survived. Seven patients had resection of subaortic tissue; four survived and four developed heart block at surgery. Adequate gradient relief was evident in only one of the four survivors. Thirteen patients had a main pulmonary artery to ascending aorta anastomosis or conduit; six survived. All survivors had adequate gradient relief. The overall survival was 42% (10 of 24). None of seven patients with a subaortic gradient greater than 75 mm Hg survived. These data show that: Surgical relief of established subaortic obstruction in patients with single left ventricle and tricuspid atresia carries a high mortality rate, especially if the subaortic gradient is greater than 75 mm Hg. The best procedure appears to be the pulmonary artery to ascending aorta anastomosis. A clearer understanding of the factors leading to the development of significant subaortic obstruction is necessary to prevent it or to devise improved therapeutic strategies.
Adolescent↗
Current results of management in transposition of the great arteries, with special emphasis on patients with associated ventricular septal defect.
Two hundred forty-five patients less than 15 days of age with transposition of the great arteries with or without a ventricular septal defect or pulmonary stenosis were entered into an ongoing 20 institution treatment study between January 1, 1985 and June 1, 1986. Complete follow-up is available on all patients. The ventricular septal defect narrowed in only 1 of 36 patients with combined transposition of the great arteries and ventricular septal defect; pulmonary stenosis developed or worsened in 3 of these 36 patients and in 3 of the 187 patients with simple transposition. Twelve month overall survival among the 245 patients was 80%. No morphologic feature of transposition was a risk factor for death but major associated cardiac and noncardiac anomalies (more common in patients with combined transposition and ventricular septal defect) and low birth weight were risk factors. Neither arterial switch repair (n = 86), atrial switch (Mustard) repair (n = 21) nor atrial switch (Senning) repair (n = 39) was a risk factor for death, but results in all surgical groups were better in the last part of the experience. Death before repair was less frequent late in the study. Possibly, in low birth weight infants, survival was better with the arterial than with the atrial switch repair. These data suggest that survival at 1 year is similar with either the arterial or the atrial switch repair. The early results of repair of combined transposition of the great arteries and ventricular septal defect are as good as those of simple transposition. Special institutional efforts are required to attain good results with the arterial switch repair and to prevent death before repair.
Clinical Trials as Topic↗
The influence of a right-to-left cardiac shunt on lidocaine pharmacokinetics.
The pharmacokinetics of lidocaine were studied in 1-2-month-old lambs with surgically created, intracardiac right-to-left shunts (RLS) and in age-matched control lambs. Shunts were prepared by anastomosing the pulmonary artery to the left atrial appendage to achieve arterial oxygen saturation of 65-75%. Catheters were implanted both in the right atrium for drug infusion and in the ascending aorta for blood sampling. Lidocaine, 1 mg/kg, injected as a rapid bolus, or 12 mg/kg, injected as a continuous infusion over 15 min, was delivered into the right atrium. Serial samples of arterial blood were obtained every 2.5 s for 1 min following the bolus injection and up to 4 h following the continuous infusion. Samples were analyzed for lidocaine by gas chromatography. Peak arterial whole blood concentration of lidocaine in the shunted animals was 37.0 +/- 2.1 micrograms/ml compared to 21.1 +/- 0.1 microgram/ml in the control animals; P less than .01. The peak arterial concentrations during the lidocaine infusion were 12.6 +/- 3.5 micrograms/ml in the RLS and 5.8 +/- 1.5 micrograms/ml in the controls. Total body clearance of lidocaine was decreased in the shunted animals to 30.7 +/- 13.2 ml.kg-1.min-1 from 68.1 +/- 12.1 ml.kg-1.min-1 in the control animals; P less than .001. The steady-state volume of distribution was also decreased in the shunted animals, 1.0 +/- 0.2 l/kg versus 2.0 +/- 0.7 l/kg in the controls; P less than .02. To induce convulsions 4.75 +/- 0.46 mg/kg of lidocaine was required in the shunted animals and 7.37 +/- 0.44 mg/kg in the control animals (P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Unsatisfactory clinical experience with a collagen-sealed knitted Dacron extracardiac conduit.
A clinical trial of a collagen-sealed knitted Dacron conduit (Tascon Medical Technologies) in 86 patients has revealed a high incidence of early reoperation for conduit stenosis. At 3 years, the actuarial incidence of freedom from conduit replacement was 67 +/- 14% for valved conduits, and 66 +/- 20% for nonvalved conduits. Seven of eight conduits that were replaced had a thick, weakly adherent pseudointima. Comparison of the current series with a previous series of patients receiving tightly woven low-porosity Dacron conduits is complicated by the young age, small size, and greater complexity of the current group. Nevertheless, the findings are consistent with the results of two laboratory studies performed at this hospital which suggest that the collagen used in the Tascon conduit undergoes particularly slow resorption, resulting in weak adhesion between the pseudointima and conduit. This allows hemorrhagic dissection to occur deep to the pseudointima. These data suggest that alternative methods of sealing knitted Dacron conduits should be used.
Adolescent↗
Childhood systemic-pulmonary shunts: subsequent suitability for Fontan operation.
We investigated whether or not a systemic arteriopulmonary shunt (SAPS) after 1 year of age affects a child's chances for a successful Fontan procedure. From 1967 to 1986, 50 patients more than 1 year old with tricuspid atresia or other complex lesions amenable to a Fontan operation received a SAPS. Follow-up was incomplete in six patients. Two patients died within 1 week of surgery, and two died within 9 months for a total mortality of 8%. Each of the 40 remaining patients was catheterized 6 months to 10.8 years (mean 4.9 years) after placement of a SAPS. By the criteria of Choussat and Fontan, there were one or more adverse SAPS-induced changes in nine patients (23%). Three developed pulmonary hypertension, one with associated elevation of pulmonary arteriolar resistance and pulmonary artery distortion, and one in conjunction with severe congestive heart failure. Six additional patients developed isolated distortion of the ipsilateral pulmonary artery. Of these nine patients, three had successful Fontan operations (33%), two died after the Fontan operation, and four are no longer candidates. In contrast, of 31 patients without documented SAPS-induced complications, 27 have undergone Fontan operations, 23 successfully (74%). Palliation with a SAPS after 1 year of age can place children at increased risk for a subsequent Fontan operation.
Age Factors↗
Anomalous systemic and pulmonary venous connections in conjunction with atriopulmonary anastomosis (Fontan-Kreutzer). Technical considerations.
Anomalous systemic or pulmonary venous connections can coexist with certain forms of complex cyanotic heart diseases that are reparable only by atriopulmonary anastomotic procedures, thus complicating the intraatrial separation of systemic and pulmonary venous pathways. Anomalous systemic or pulmonary venous connections were encountered isolated or in combination in 17 patients (10%) among a series of 170 modified Fontan-Kreutzer procedures. Fourteen of these 17 patients (82.3%) survived their operations, which utilized different techniques to deal with the various forms of anomalous systemic and pulmonary venous connections. There was one late death (5.8%). Extracardiac exclusion of a left superior vena cava with an end-to-side left cavopulmonary shunt proved to be a more successful alternative than the use of complicated intratrial baffles. Because of the complexity of the anatomic variables, repair of anomalous systemic or pulmonary venous connections in conjunction with a modified Fontan-Kreutzer procedure requires a detailed preoperative anatomical and physiologic diagnosis, and an individualized plan for each patient must be formulated to provide unobstructed venous pathways.
Adolescent↗
Anomalous origin of left coronary artery from pulmonary artery. Ligation versus establishment of a two coronary artery system.
Between 1959 and 1985, 24 patients (mean age 38 months, range 15 days to 13 years) with anomalous origin of the left coronary artery from the pulmonary artery as an isolated lesion were treated surgically at Children's Hospital, Boston. In 11 cases a left coronary-to-aortic tunnel was created with a pulmonary artery baffle (Takeuchi) with no deaths either early or late over a mean follow-up period of 18 1/2 months. Late complications of this procedure include moderate aortic regurgitation (one), supravalvular pulmonary stenosis (two-one required a second operation), obstructed baffle (one-asymptomatic). In 11 cases of coronary ligation or ostial closure there was a 27% early mortality and a 25% late mortality over a mean follow-up period of 10 1/2 years. Late complications include residual shunt (three-two required a second operation), severe mitral regurgitation (one), and recurrence of angina (one). Two patients had other procedures. Both early and late deaths occurred in the group who had congestive heart failure and who had simple ligation. Five infants who had profoundly depressed ventricular function and moderate to severe mitral regurgitation, together with widespread Q waves on electrocardiogram, showed a dramatic improvement in ventricular function after the Takeuchi procedure. The Takeuchi procedure is a simple and effective means of establishing a two coronary artery system in the child with anomalous origin of the left coronary artery from the pulmonary artery. This procedure is particularly recommended over coronary ligation in patients in congestive heart failure.
Adolescent↗
Long-term follow-up of valvotomy before 1968 for congenital aortic stenosis.
The clinical course of 59 patients who underwent valvotomy for aortic stenosis before 1968 was reviewed. All were older than 1 year at the time of operation. Mean follow-up period was 17.7 years. Forty-six patients are alive; 26 (57%) are 30 to 40 years and 6 (13%) are older. Actuarial analysis indicated that the probability of survival was 94% at 5 years and 77% at 22 years. Thirteen patients died, 7 suddenly. Among the latter, significant obstruction or regurgitation was present in the 4 who underwent catheterization 0.9 to 7.2 years before death, 2 of whom were symptomatic and 2 with progression of a strain pattern on electrocardiogram. Surgery was recommended but declined by the latter 2 patients. Reoperation was carried out in 21 patients (36%), 3 (12%) of whom died. Actuarial analysis revealed the probability of reoperation to increase from 2% at 5 years to 44% at 22 years. Bacterial endocarditis occurred on 4 occasions in 3 patients, 1 of whom died suddenly during treatment. The incidence of endocarditis was 3.8 episodes/1,000 patient-years. Actuarial analysis of serious events, defined as death, reoperation and endocarditis, with the most serious of these and each patient being represented only once, indicated the probability of being free of such an episode to be 92% at 5 years, decreasing to 39% at 22 years. These data emphasize the palliative nature of valvotomy and the meticulous follow-up so necessary in these patients.
Actuarial Analysis↗