Monocusp homograft VSD patch in atrioventricular canal with tetralogy of Fallot

A monocusp aortic homograft was used to compensate for deficient right atrioventricular valve tissue during repair of complete atrioventricular canal defect with tetralogy of Fallot. The homograft was used to produce a comma-shaped ventricular septal defect patch together with the septal leaflet of the right atrioventricular valve, thus committing native leaflet tissue to left atrioventricular valve reconstruction. One year postoperatively the child is in New York Heart Association class I with no tricuspid regurgitation.     

Tooth enamel thickness in the mature dentition of domestic dogs and cats--preliminary study

Eleven patients underwent late repeated correction of tetralogy of Fallot in 1991-1993. The previous operation was repair of simple Fallot's tetralogy in seven cases, repair plus transannular patch in one case and repair of tetralogy and pulmonic atresia in three cases. The indications for reoperation were residual ventricular septal defect, right ventricular outflow tract (R.V.O.T.) obstraction, residual branch pulmonary artery stenosis, aneurysmal dilatation of R.V.O.T. Patch or combination of any of the above. At reoperation these defects were corrected. The post operative course was uneventful in eight patients. Two required mechanical ventilation for 2-3 days, and one underwent another operation for residual branch pulmonary artery stenosis. The functional and haemodynamic results were good in ten patients, and one had residual distal pulmonary artery stenosis. There were no death during 2 years of follow-up. Repeated correction of tetralogy of Fallot thus had low postoperative morbidity and good haemodynamic results. For the relatively few patients initially found to have tetralogy of Fallot and pulmonic atresia, the outcome may be less favorable.     

One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals

BACKGROUND: Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have traditionally required multiple unifocalization staging operations before undergoing complete repair. Recently, the feasibility of a single-stage unifocalization and repair was demonstrated by Hanley. In this report, we describe our experience with each approach. METHODS AND RESULTS: Since 1989, 11 of 12 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone complete surgical correction. The first seven patients were subjected to staged bilateral unifocalizations, with repair being achieved in six (group I). The last five patients have undergone a single-stage midline unifocalization and repair via a sternotomy (group II). Four of these were infants (2 weeks to 9 months) and one was 13 years old. All patients in group I had tetralogy of Fallot, whereas in group II three patients had tetralogy of Fallot, one patient had double-outlet right ventricle, and one patient had complete atrioventricular canal and transposition. In group I, the median age at the first operation was 43 weeks. Complete repair was performed at a median age of 3.5 years, with a mean number of 3.3 operations required. In group II, only one operation was required to achieve complete repair at a median age of 28 weeks. The postoperative right ventricular/left ventricular pressure ratio was 0.49 in group I and 0.45 in group II. One intraoperative death and one late death occurred in group I and no early or late deaths in group II. Currently, four patients in group I and all five patients in group II are alive and well. CONCLUSIONS: Early intervention with both surgical approaches can lead to complete biventricular repair in most patients. Because the single-stage midline unifocalization and repair can achieve a completely repaired heart in infancy with one operation, it is currently our approach of choice.     

Prevalence of the microdeletion 22q11 in newborn infants with congenital conotruncal cardiac anomalies

Conotruncal malformations account for about 50% of congenital heart defects diagnosed in newborns. We studied prospectively 104 patients admitted in our neonatal intensive care unit for conotruncal defects by fluorescence in situ hybridization to estimate the prevalence of the interstitial deletion in this category of congenital heart disease. Cardiac phenotypes were: truncus arteriosus (17), interrupted aortic arch (18), tetralogy of Fallot with or without pulmonary valve atresia (55), tetralogy of Fallot with absent pulmonary valves (5), ventricular septal defect with malalignment of the conal septum (9). We discovered a microdeletion 22q11 at loci D22S39 or D22S398 in 50 newborns (48%). The prevalence of this microdeletion in different groups of conotruncal defects was: truncus arteriosus 7/17, interrupted aortic arch 16/18, tetralogy of Fallot 19/55, absent pulmonary valves 2/5, and ventricular septal defect 6/9 respectively. Only two patients without any clinical or biological feature of the so called CATCH22 syndrome exhibited the deletion. Parental studies confirmed that the deletion occurred de novo in 47/50 cases (three parental microdeletions). On the other hand, recurrence of conotruncal heart defects in families of "undeleted probands" was higher than expected (13%). CONCLUSION: In 50/104 newborns with conotruncal defects, an interstitial deletion 22q11 was found. Fluorescence in Situ Hybridization should be performed in newborn infants with conotruncal defect and at least one additional manifestation of the CATCH22 phenotype.     

Assessment of the role and reliability of sonographic post-prandial flow response in grading Crohn''s disease activity

CONTEXT: The incidence of infective endocarditis after surgical repair of congenital heart defects is unknown. OBJECTIVE: To determine the long-term incidence of endocarditis after repair of any of 12 congenital heart defects in childhood. DESIGN: Population-based registry started in 1982. SETTING: State of Oregon. PARTICIPANTS: All Oregon residents who underwent surgical repair for 1 of 12 major congenital defects at the age of 18 years or younger from 1958 to the present. MAIN OUTCOME MEASURE: Diagnosis of infective endocarditis confirmed by hospital or autopsy records. RESULTS: Follow-up data were obtained from 88% of this cohort of 3860 individuals through 1993. At 25 years after surgery, the cumulative incidence of infective endocarditis was 1.3% for tetralogy of Fallot, 2.7% for isolated ventricular septal defect, 3.5% for coarctation of the aorta, 13.3% for valvular aortic stenosis, and 2.8% for primum atrial septal defect. In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. No children with secundum atrial septal defect, patent ductus arteriosus, or pulmonic stenosis have had infective endocarditis after surgery. CONCLUSION: The continuing incidence of endocarditis after surgery for congenital heart defect, particularly valvular aortic stenosis, merits education about endocarditis prophylaxis for children and adults with repaired congenital heart defects.     

Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot

OBJECTIVE:The objective was to explore the best management algorithm for atrioventricular septal defect in conjunction with tetralogy of Fallot. METHODS: We reviewed the cases of 38 children referred to our division (March 1981-August 1997) who had atrioventricular septal defect associated with tetralogy of Fallot; 32 (84%) had Down syndrome. Twenty-one received initial palliation with a systemic-to-pulmonary artery shunt; of these, 2 (9.5%) died before complete repair. Thirty-one children underwent complete repair; 14 of these (45%) had undergone initial palliation (mean age at shunt 20 +/- 24 months). Right ventricular outflow obstruction was relieved by a transannular patch in 22 (71%); 14 (64% of 22) had a monocuspid valve inserted. Four required an infundibular patch. RESULTS: Two children (6.4%) died early after repair; 1 had undergone previous palliation. Patients with palliation underwent repair at an older age (78 vs 36 months), required longer ventilatory support (8 vs 4 days) and inotropic support (8 vs 4 days), and had longer intensive care stays (11 vs 6 days) and hospital stays (24 vs 15 days). Eleven children (35%) underwent reoperation, 7 (58%) for right ventricular outflow reconstruction and pulmonary arterioplasty. Reoperation was more frequent in the palliation group than in the primary operation group (64% vs 12%). The single late death was related to a reoperation in the palliation group. CONCLUSIONS: Atrioventricular septal defect with tetralogy of Fallot can be repaired with a low mortality rate. Initial palliation with a shunt resulted in a more complex postoperative course and a higher reoperative rate. Primary repair is superior to initial palliation with later repair.     

Facile minimally invasive cardiac surgery via ministernotomy

BACKGROUND: The public's and surgeons' perception of minimally invasive operations are frequently at odds. Nevertheless, real or perceived benefits may result from limiting skin and skeletal trauma. METHODS: Beginning in January 1996, we began approaching most infant and pediatric open heart procedures through an upper sternal split incision using a 1- to 3-inch skin opening and then extended this technique using a 2.5- to 3.5-inch incision for adult aortic and mitral valve replacement. RESULTS: A total of 82 patients, 57 infants and children and 25 adults, have been operated on using this approach (age range, newborn to 81 years). Operations accomplished through ministernotomy have included aortic valvotomy, arterial switch, tetralogy of Fallot, atrial or ventricular septal defect closure, aortic valve replacement, mitral valve replacement and repair, redo aortic or mitral valve replacement, double valve replacement, aortic root replacement, and complex arch reconstruction. In adults, the sternum was divided and then a T incision was made at the second, third, or fourth intercostal space. The mitral valve was reached through the roof of the left atrium. In children, a lower sternal split was used for atrial septal defect repairs. All cannulas were introduced through the ministernotomy incision, eliminating femoral cannulation. No new instruments, retractors, or ports were used. Mediastinal drainage was accomplished through a Blake drain connected to Heimlich-valved grenade suction. All but 2 patients were extubated immediately. Hospital stay was from 1 to 20 days (median 2 days). Patient and family acceptance is very high. CONCLUSIONS: On the basis of this initial experience, we attempt all congenital cardiac and isolated adult valve operations through ministernotomy.     

Surgical treatment of ventricular septal defect and ruptured sinus of Valsalva associated with infective endocarditis

Two patients with ventricular septal defect of Kirklin type I and ruptured right coronary sinus of Valsalva associated with infective endocarditis were operated on. Both had bacillus vegetation clinging to the aortic and pulmonary valves and the right ventricular intimal wall around the septal defect. Aortic and pulmonary regurgitation were also found. The surgical approach included vertical incision of the right ventricular outflow tract and pulmonary trunk and transverse aortotomy. The right coronary sinus of Valsalva showed distinct aneurysmal change in one patient. The aortic valve and infected Valsalva sinus were excised in both cases, and the pulmonary valve and right ventricular wall where infection extended thoroughly débrided. The resulting defect, including the ventricular septal defect and excised right Valsalva sinus and aortic annulus, was closed with one patch, and the prosthetic valve inserted in the position of the original aortic valve using this patch as part of the annulus. Both patients had a good postoperative course and are doing well, although slight pulmonary regurgitation persists.     

Role of neutrophil elastase in stress-induced gastric mucosal injury in rats

To evaluate the growth of a pulmonary trunk reconstructed without an extracardiac conduit, the hemodynamics and diameter of a new pulmonary trunk were measured in 5 patients from the right ventriculogram and MRI at postoperative follow-up periods. There were tetralogy of Fallot with pulmonary atresia in two patients, tetralogy of Fallot with single coronary in one, truncus arteriosus type I in one and transposition of the great arteries with ventricular septal defect and pulmonary stenosis in one. The age at operation ranged from 26 days to 4.5 years. The posterior wall continuity of the right ventricle and pulmonary artery was established by the direct pulmonary-right ventricular anastomosis in three patients and by the interposition of the left atrial appendage in two. Postoperative follow-up periods ranged from 2 years and 6 months to 3 years and 10 months (median: 2 years and 11 months). In four of them, the postoperative right ventricular to aortic or left ventricular systolic pressure ratios were less than 0.4 without any significant systolic pressure gradients between pulmonary artery and right ventricle. In these four patients, the diameters of the reconstructed pulmonary trunks grew from 10-18 mm to 18-21 mm postoperatively. These diameters were more than 100% of normal values. In the remaining patient with tetralogy of Fallot and single coronary artery, the obstruction of the new pulmonary trunk by a bulged left atrial appendage, which was used as the posterior wall, was observed on the right ventricular outflow tract reconstruction without an extracardiac conduit has growth potential in the future.     

In-vitro isolation and antifungal susceptibility of amphotericin B-resistant mutants of Aspergillus fumigatus

BACKGROUND AND AIMS OF THE STUDY: Aortic valve disease in the pediatric population poses special problems to surgeons and cardiologists. The pulmonary autograft has proven to be a good alternative for aortic valve replacement and left ventricular outflow tract (LVOT) reconstruction in this special group. METHODS: Forty-one children (mean age 10.0 +/- 4.8 (SD) years; range: 35 days to 18.8 years) underwent aortic root replacement with a pulmonary autograft between February 1994 and April 1998. Twenty-one patients (51%) had previous cardiac surgery; seven (17%) had balloon valvulotomy. Aortic root replacement was combined with other techniques for various disorders, including tunnel LVOT obstruction, ventricular septal defect (VSD)-aortic insufficiency complex, neoaortic insufficiency following arterial switch procedure, and subvalvular stenosis following correction of type B interruption of the aortic arch (IAA) with VSD (IAA-B/VSD). RESULTS: The mean follow up was 1.7 +/- 1.0 years (range 44 days to 4.1 years). Total follow up time was 67.8 patient-years. Two patients, both after repair of interrupted aortic arch, died intraoperatively (4.9%). There was no late mortality. Two patients were reoperated on (5.1%), one for autograft insufficiency due to cuspal perforation and one for right ventricular outflow tract stenosis at the distal anastomosis. Thirty-eight patients (97%) are currently in NYHA class I; one child with a preoperatively poor left ventricular function did not improve and is in class II. At the latest echocardiographic follow up, neoaortic regurgitation was absent in 19% of patients, trivial in 69% and mild in 11%. Homograft insufficiency was absent in 64%, trivial in 31% and mild in 6%. All mean gradients for both autograft and homograft were < 15 mmHg. CONCLUSIONS: The Ross operation can be performed with good results in infants and children with different forms of LVOT obstruction and aortic insufficiency, though aortic stenosis following IAA-B/VSD repair poses a surgically difficult problem.     

Risk factors for higher cost in congenital heart operations

BACKGROUND: For many congenital heart defects, hospital mortality is no longer a sensitive parameter by which to measure outcome. Although hospital survival rates are now excellent for a wide variety of lesions, many patients require expensive and extensive hospital-based services during the perioperative period to enable their convalescence. These services can substantially increase the cost of care delivery. In today's managed care environment, it would be useful if risk factors for higher cost could be identified preoperatively so that appropriate resources could be made available for the care of these patients. The focus of this retrospective investigation is to determine if risk factors for high cost for repair of congenital heart defects can be identified. METHODS: We assessed financial risk by tracking actual hospital costs (not charges) for 144 patients undergoing repair of atrial septal defect (58 patients), ventricular septal defect (48 patients), atrioventricular canals (14 patients), or tetralogy of Fallot (24 patients) at Duke University Medical Center between July 1, 1992, and September 15, 1995. Furthermore, we were able to identify where the costs occurred within the hospital. Financial risk was defined as a large (> 60% of mean costs) standard deviation, which indicated unpredictability and variability in the treatment for a group of patients. RESULTS: Cost for atrial septal defect repair was predictably consistent (low standard deviation) and was related to hospital length of stay. There were factors, however, for ventricular septal defect, atrioventricular canal, and tetralogy of Fallot repair that are identifiable preoperatively that predict low- and high-risk groups using cost as an outcome parameter. Patients undergoing ventricular septal defect repair who were younger than 6 months of age at the time of repair, who required preoperative hospital stays of longer than 7 days before surgical repair, or who had Down's syndrome had a less predictable cost picture than patients undergoing ventricular septal defect repair who were older than 2 years, who had short (< 4 days) preoperative hospitalization, or who did not have Down's syndrome ($48,252 +/- $42,539 versus $15,819 +/- $7,219; p = 0.008). Patients with atrioventricular canals who had long preoperative hospitalization (> 7 days), usually due to pneumonia (respiratory syncytial virus) with preoperative mechanical ventilation had significantly higher cost than patients with atrioventricular canals who underwent elective repair with short preoperative hospitalization ($83,324 +/- $60,138 versus $26,904 +/- $5,384; p = 0.05). Patients with tetralogy of Fallot had higher costs if they had multiple congenital anomalies, previous palliation (combining costs of both surgical procedures and hospital stays), or severe "tet" spells at the time of presentation for operation compared with patients without these risk factors ($114,202 +/- $88,524 versus $22,241 +/- $7,071; p = 0.0005). One patient (with tetralogy of Fallot) with multiple congenital anomalies died 42 days after tetralogy of Fallot repair of sepsis after a gastrointestinal operation. Otherwise, hospital mortality was 0% for all groups. CONCLUSIONS: Low mortality and good long-term outcome for surgical correction of congenital heart defects is now commonplace, but can be expensive as some patients with complex problems receive the care necessary to survive. This study demonstrates that it is possible to identify factors preoperatively that predict financial risk. This knowledge may facilitate implementation of risk adjustments for managed care contracting and for strategic resource allocation.     

Focus on bovine products

Echocardiographic (ECHO) findings in 211 consecutive examinations performed at Eldoret Medical Services from August 1992 to May 1994 were analysed. Most patients were referred by physicians from Eldoret and from the surrounding hospitals for assessment of heart failure or a heart murmur. Thirty three echocardiograms were judged as being normal and 177 abnormal. Of the abnormal ECHOs, congenital heart disease accounted for 38.4% while rheumatic heart disease accounted for 40.7%: Of the 68 patients with congenital defects, 21 (30.1%) had ventricular septal defects; 19 (27.9%) persistent ductus arteriosus; five (7.5%) mitral valve; four (5.9%) tetralogy of Fallot; and three (4.4%) atrial septal defects. In addition, there were two (3.0%) patients each with complete atrio-ventricular septal defects, tricuspid atresia, complete transposition of the great arteries, truncus arteriosus, single ventricle and bicuspid aortic valve. Of the 67 patients with uncomplicated rheumatic heart disease, mitral valve involvement alone was seen in 39 (58.2%), mitral and aortic in 26 (38.8%) and aortic valve alone in two (3.0%). Rheumatic heart disease was complicated by bacterial endocarditis in five patients. Nine patients had pericardial effusion. The possible role of colour-flow ECHO as a feasible, non-invasive and, in the long term, cost-effective means of allowing for early detection and timely institution of secondary prophylaxis while rheumatic heart disease is still asymptomatic is highlighted.     

The clamshell approach for the surgical treatment of complex cardiopulmonary pathology in infants and children

BACKGROUND: The surgical approach to children with complex cardiovascular and pulmonary anomalies is still controversial. Staged operations through multiple incisions are often performed in this setting. OBJECTIVE: The different applications and clinical advantages of a bilateral thoracosternotomy approach to complex cardiothoracic disease requiring surgical repair were reviewed retrospectively. METHODS: Between January 1993 and June 1995, 33 patients, aged between 2 months and 17 years (mean 7.8 +/- 5.3) underwent surgical treatment of complex cardiovascular or pulmonary disease using a clamshell approach. Twenty-one patients (64%) had undergone 1-5 previous surgical procedures (mean 2.5 +/- 1.0/patient). The technique involved supine position placement, submammary incision, access to the pleural space bilaterally through the fourth intercostal space and transverse division of the sternal body. RESULTS: Four groups of patients were operated on via this approach: (1) patients undergoing lobar, lung or heart-lung transplantation (40%); (2) patients undergoing repair of tetralogy of Fallot/pulmonary atresia (36%); (3) patients with previously corrected miscellaneous procedures (12%), including completion of Fontan, one-stage repair of left main bronchial stenosis and atrial septal defect, one-stage repair of partial anomalous pulmonary venous connection and aortic coarctation, and repair of congenital pulmonary venous stenosis. There were two early (< 30 days) deaths, giving a perioperative mortality of 6% for the entire series. Complications included postoperative hemorrhage in 4 patients (12%), prolonged ventilation time due to mechanical failure in 4 (12%). There were no wound infections. Analysis of complications by group showed the lung transplant group to be more affected (18% of patients experienced complications). Except for 2 infants undergoing complete unifocalization and presently awaiting completion of repair of tetralogy of Fallot/pulmonary atresia, in the remaining 31 (94%) a definitive surgical treatment could be performed in one-stage. CONCLUSIONS: The bilateral thoracosternotomy allows optimal exposure of all intrathoracic anatomic structures making one-stage surgical repair possible in a variety of complex cardiovascular and pulmonary anomalies. Early mortality and technique-related morbidity do not differ from those reported with the conventional approaches to the different disease conditions. A wider application of the clamshell approach for the management of complex intrathoracic pathology in infants and children is advocated.     

Homografts in congenital heart disease: current applications and future directions

The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.     

Conal anatomy in aortic atresia, ventricular septal defect, and normally developed left ventricle

Although aortic valve atresia is usually associated with severe underdevelopment of the mitral apparatus and left ventricle in rare cases of aortic atresia, the left ventricle may be of normal size, or even enlarged. This occurrence seems related to the presence of a significant ventricular septal defect. We have presented the morphologic findings in seven patients with aortic atresia and normally developed left ventricle, (six necropsied patients, and one studied angiocardiographically). Four autopsied patients had conal type ventricular septal defects, characterized in three by conoventricular malalignment. Subaortic atresia in these patients resulted from leftward deviation of the conal septum. One patient with aortic atresia and well-developed left ventricle had a membranous defect, and one patient had a complete A-V canal. The ventricular septal defect in the patients with conoventricular malalignment are very similar to the conal VSD observed in patients with aortic arch interruptions. Although ultimate survival with these uncommon groupings of anomalies necessitates patency of the ductus arteriosus, clinical recognition rests on (1) awareness of its existence, (2) ultrasonography, and (3) selective biventricular and aortic angiography. It is possible that some of these patients might be candidates for ventriculo-aortic reconstitiution.     

Provoked enuresis-like episodes in healthy children 7 to 12 years old

A 15-year-old boy with tetralogy of Fallot and pulmonary atresia without central pulmonary arteries who was successfully treated using a staged approach is presented. The first stage consisted of the creation of central pulmonary arteries. In the second stage, the continuity between the right ventricle and the pulmonary arteries was established with closure of the ventricular septal defect. Some patients previously considered to be unsuitable candidates for correction may be successfully repaired using this approach.     

Influence of pulmonic position on durability of bioprosthetic heart valves

BACKGROUND: The insertion of bioprosthetic valves into the pulmonic position is not performed commonly because of uncertainty concerning the necessity and durability of such valves. METHODS: We reviewed the long-term outcome of 10 patients who underwent pulmonary valve replacement with bioprostheses between March 1985 and March 1997. A Carpentier-Edwards supraannular bioprosthesis was used in 7 patients, a Hancock II bioprosthesis was used in 2 patients, and a Carpentier-Edwards pericardial bioprosthesis was used in 1 patient. The mean patient age at the time of pulmonary valve replacement was 38.9 +/- 16.3 years (range, 15 to 63 years). The diagnoses were pulmonary valvular regurgitation after corrective surgery for tetralogy of Fallot in 7 patients, right ventricular outflow tract stenosis and absent right pulmonary artery combined with a double-outlet right ventricle in 1 patient, pulmonary valvular regurgitation with pulmonary artery dilatation in 1 patient, and aortic valve stenosis treated with our modification of the Ross procedure using a pulmonary bioprosthesis in 1 patient. Survivors were followed up for a mean of 5 years and 5 months. RESULTS: One patient underwent reoperation because of infective endocarditis of the bioprosthesis. No bioprosthetic valve dysfunction has been observed on Doppler echocardiography during a maximum follow-up period of 12.2 years, except in the patient who underwent replacement at 15 years of age. CONCLUSIONS: Bioprostheses in the pulmonic position are durable in adult patients because they face a minimal hemodynamic load, but they may undergo early leaflet degeneration in younger patients.     

Congenital malformations of the mitral valve in children. Pathology and surgical treatment

A systematic study of congenital mitral valve malformations was undertaken on a surgical basis in an attempt to develop techniques of valvular reconstruction adapted to the various lesions. Forty-seven children between the ages of 4 months and 12 years (average 6 years, 4 months) have been operated upon between January, 1970, and March, 1976. Valvular lesions were classified into four group: Group I, mitral insufficiency owing to valvular lesions: Group II mitral insufficiency with subvalvular lesions; Group III, mitral insufficiency and stenosis; Group IV, stenosis. Associated lesions (ventricular or atrial septal defects, coarctation, or aortic valve stenosis) were present in 31 patients (65 per cent) and were corrected during the same operation. Valve reconsruction was possible in 38 patients whereas valve replacement was necessary in 9 patients. In the valve repair group there were three operative deaths (8 per cent), no late deaths, one reoperation for residual ventricular septal defect, and one myocardial infarction. In the valve replacement group of 9 patients, there were three operative deaths, three late deaths, and one case of repeated embolization. Thirty-one of 38 patients in the valve repair group were classified into Functional Class I after the operation (86 per cent), 2 were in Class II, and one in Class III. Minimal or moderate regurgitation and cardiomegaly persisted in the majority of the patients. Pulmonary artery pressure significantly decreased, however, as demonstrated by postoperative catheterization in 17 patients.     

A modified repair technique for tricuspid incompetence in Ebstein's anomaly

OBJECTIVE: A modified technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. Midterm results of this therapeutic approach for patients with Ebstein's anomaly and tricuspid valve incompetence are reported. METHODS: Between October 1988 and April 1997, the incompetent tricuspid valve was repaired with our technique in 19 patients (12 female, 7 male; 2 to 54 years, mean 21 years). The indication for operation was congestive heart failure of various degrees in all patients. Tricuspid incompetence was grade II in two patients, grade III in 14, and grade IV in three. Associated congenital malformations were simultaneously repaired (interatrial communication in 18, ventricular septal defect in two, pulmonary stenosis in two, mitral valve prolapse in one). Follow-up ranged between 10 and 103 months (median 28 months) and was complete for all patients. RESULTS: There were no operative deaths. One patient with active endocarditis and pulmonary abscess died 2 months after the operation of recurrent sepsis; there were no late deaths. During follow-up, New York Heart Association functional class improved from 2.8 before the operation to 1.9 without recurrent cyanosis, and tricuspid incompetence decreased from a mean grade of 3.1 to one of 0.9, without any echocardiographic deterioration of the tricuspid valve function or right ventricular dilation. CONCLUSIONS: Our technique allows tricuspid valve repair in patients with Ebstein's anomaly, even in cases usually reserved for primary valve replacement, without late functional deterioration.     

Sub-trochanteric fractures. A comparative study between gamma nail and angular osteosynthesis with lateral cortical support]

METHODS: Thirty-three children and young adults with congenital aortic valve disease underwent pulmonary autograft replacement of the aortic valve between October 1993 and March 1997. There wer six females and 27 males; at operation, median age was 16 years (range: 3 to 41 years) and median body weight 60 kg (range: 14 to 121 kg). Fifteen patients (46%) had undergone one or more previous cardiac surgical procedures. A bicuspid aortic valve was present in 31 patients (94%); moderate to severe aortic stenosis and regurgitation was present in 10 (30%) and 26 (79%), respectively. RESULTS: All patients underwent the Ross procedure while in NYHA class I (64%) or class II (36%). A preoperative shortening fraction of 41 +/- 1.4% suggested well-preserved systolic function, but the mean left ventricular end-diastolic pressure of 16.6 +/- 1.3 mmHg was consistent with preoperative left ventricular pressure and volume overload. The aortic root was replaced using an interrupted suture technique in two patients and with three separate running sutures in 31. The right ventricular outflow tract was reconstructed in all classes with a cryopreserved pulmonary homograft valved conduit (median diameter 23 mm; range 19 to 30 mm). Intraoperative complications included transient atrioventricular dissociation (one), permanent atrioventricular dissociation (one), and left coronary artery distortion relieved by shortening the distal ascending aorta (one). Postoperatively, postpericardiotomy syndrome developed in six patients (18%), supraventricular tachycardia in three (9%), and ventricular tachycardia in one (3%). At three days after surgery, one patient developed ischemic left ventricular dysfunction requiring repositioning of the distorted left coronary artery higher on the neo-aortic root. Hospital survival rate was 100%. During a median follow-up of 17 months (range: 1 to 41 months) one patient suffered a non-cardiac death due to blunt trauma. there has been a significant postoperative improvement in NYHA class among surviving patients (class I, 94%; class II, 6%; p = 0.004 versus preoperative). Postoperative aortic regurgitation was absent or trivial in 17 (60%) and mild in the remaining 11 (40%) patients for whom follow-up echocardiographic data are available. One patient required reoperation 16.5 months after the Ross procedure to replace a rapidly degenerating pulmonary homograft, and one with moderately severe homograft stenosis and five with mild homograft stenosis are being monitored. Postoperatively, a gradual early expansion in the diameter of the neo-aortic root and reduction in echocardiographic indices of left ventricular hypertrophy and dilatation occurred. CONCLUSIONS: Pulmonary autograft replacement of the aortic valve in young patients with congenital aortic valve disease has produced excellent short-term anatomic/physiologic results and symptomatic relief with no mortality. Indices of left ventricular dilatation and hypertrophy regress after repair when the Ross operation precedes important deterioration in preoperative ventricular function. Important technical considerations include: (i) the native distal ascending aorta should be sufficiently shortened before performing the distal aortic anastomosis; and (ii) the left coronary anastomosis should be positioned relatively high on the neo-aortic root with a slight amount of tension. Both of these maneuvers reduce the likelihood of coronary artery distortion. Rapid degeneration of the pulmonary homograft and the propensity towards progressive dilatation of the neo-aorta are important postoperative considerations. Until more is known about the etiology and natural history of these two potential complications, postoperative anti-inflammatory and/or immunosuppressive therapy and strict control of hypertension should be strongly considered.