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Biomedical subjects

C P Brizard

Publications and source records attributed to C P Brizard.

At least 19 recordsLinked to original sources

Damus-Kaye-Stansel connections in children with previously transected pulmonary arteries.

BACKGROUND: In patients with a univentricular arteriovenous connection, transection of the main pulmonary artery may be performed as part of a bidirectional cavopulmonary shunt or Fontan procedure. The proximal stump of the pulmonary artery may remain in the systemic circulation. In cases with a discordant ventriculoarterial connection, subsequent restriction of the bulboventricular foramen may lead to subaortic stenosis. The subaortic stenosis can be corrected in some patients by directing the systemic flow through a combined nonobstructed aortopulmonary outlet, as in the Damus-Kaye-Stansel connection. Previous closure of the pulmonary artery has been considered by some investigators to be a relative contraindication to the Damus-Kaye-Stansel procedure, unless an allograft root can be added to the circuit after excision of the closed pulmonary stump. METHODS: Three patients with previously transected pulmonary arteries underwent a modified Damus-Kaye-Stansel connection using the native pulmonary valve and the proximal pulmonary artery stump. RESULTS: The native pulmonary valves have functioned well despite thrombus formation in the proximal stump in 2 patients before Damus conversion. All 3 patients are alive and well after 108, 19, and 3 months, with competent nonobstructed ventriculoarterial connections. CONCLUSIONS: If transection and closure of the pulmonary artery as part of a previous palliation has spared the pulmonary valve, then the native pulmonary outlet might be used for a safe Damus-Kaye-Stansel connection.

Child↗

Does left ventricular outflow tract obstruction influence outcome of interrupted aortic arch repair?

BACKGROUND: In previous studies left ventricular outflow tract obstruction (LVOTO) has been recognized as an important factor affecting survival and reoperation probability in patients having repair of an interrupted aortic arch (IAA). METHODS: All 72 patients who underwent operation for IAA from January 1, 1985 to June 30, 1997 were reviewed. The presence or absence of LVOTO was noted and the immediate and long-term results were analyzed. RESULTS: Type A IAA was found in 23 patients and type B IAA in 49 patients. Anomalous right subclavian artery was noted in 15 patients, all of whom had type B IAA. LVOTO was identified in 36 patients before arch repair and was associated with the presence of type B IAA and anomalous right subclavian artery (p = 0.02 and 0.007, respectively). There were 2 hospital deaths (within 30 days) for a mortality of 2.8% (confidence limit [CL] = 0% to 6.6%). There were 7 late deaths over 3,737 patient-months of follow-up (9.7%, CL = 2.9% to 16.6%). Actuarial survival for the whole cohort was 84.8% (CL = 73.2% to 94.4%) at 12 years. There was 87% 10-year survival (CL = 60% to 93%) for patients with LVOTO at presentation compared with 83% (CL = 62% to 92%) for patients without LVOTO (p = 0.85, hazard function 0.87). Twenty-eight patients have required at least one reoperation. The use of conduits to restore right ventricular to pulmonary artery continuity predicted the need for reoperation (p = 0.0001). Patients with presence of a nonseptatable heart were also more likely to need reoperation (p = 0.027) when compared to the rest of the cohort. Freedom from reoperation was 47.3% (CL = 30% to 62%) at 12 years. In patients with LVOTO, freedom from reoperation (55% at 10 years, CL = 33% to 72%) was not significantly different from those patients without LVOTO at presentation (29% at 10 years, CL = 7% to 56%; p = 0.97, hazard function 0.7). Actuarial freedom from recurrent arch obstruction requiring reintervention was 82.7% (CL = 66.7% to 98.7%) at 12 years. CONCLUSIONS: Neonatal complete repair of patients with IAA is possible with low mortality. LVOTO was not a significant risk factor for hospital or late death in patients having complete repair of IAA during this period. The use of conduits for right ventricular to pulmonary artery continuity and the presence of a nonseptatable heart are important risk factors for further operation and will continue to provide added morbidity to these patients.

Aorta, Thoracic↗

IL-6 and IL-8 levels after cardiopulmonary bypass are not affected by surface coating.

UNLABELLED: BACKGROUND.:Contact of blood with the surfaces of the cardiopulmonary bypass (CPB) circuit has been implicated as a cause of the inflammatory response. We undertook a prospective randomized trial of 200 pediatric patients, all with a calculated total bypass flow of less than 2.3 L/min (< 0.96 L/m2/min). METHODS: Patients were randomly assigned to 1 of 4 CPB groups: (1) Nonheparin-bonded circuit with no albumin preprime; (2) Nonheparin-bonded circuit with albumin preprime; (3) Heparin-bonded circuit with no albumin preprime; (4) Heparin-bonded circuit with albumin preprime. Measurements of cytokines, (interleukin [IL]-6, IL-8) and blood cell counts were made prebypass and 6 and 24 hours after institution of cardiopulmonary bypass. RESULTS: Analysis of variance showed no significant difference in any of the clinical or biochemical characteristics of the 4 groups. The interaction between heparin-bonded oxygenators and albumin preprime was not significant. No important differences in IL-6 or IL-8 concentrations were noted after CPB using either heparin or nonheparin-bonded oxygenators with albumin or albumin free preprime using two-way analysis of variance. CONCLUSIONS: Albumin preprime and heparin-bonding do not attenuate the inflammatory response component attributable to the concentration of these markers.

Albumins↗

Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery.

OBJECTIVE: The aim of this study was to review the results of operations for anomalous left coronary artery from the pulmonary artery and the late outcome for exercise capacity, left ventricular function, and mitral regurgitation. METHODS: Twenty-one patients underwent operations over an 18-year period (median age, 9 months; range, 6 weeks-26 years) with a median follow-up of 6.5 years (range, 2 months-18 years). In addition to clinical and echocardiographic follow-up, patients at our institution were also investigated with radionuclide scans (n = 10) and treadmill exercise testing (n = 8). RESULTS: There were no operative or late deaths (0%; 95% confidence interval [CI], 0% and 16%). Five patients required support with a left ventricular assist device. Eighteen patients are currently in New York Heart Association class I, and 3 patients are mildly symptomatic. On nuclear gated scan at a mean of 6 years after the operation, the left ventricular ejection fraction was 64% (SD, 3%) at rest and increased to 74% (SD = 3%) on exercise (95% CI for the difference, 6%, 14%; P =.001). Treadmill endurance was normal for age (9.8-14.5 minutes) in those old enough to exercise. On echocardiography (n = 18), the current fractional shortening was 34% (SD, 4%) in the 15 patients with normal or only mildly abnormal ventricular septal motion. Three patients have undergone mitral valve operations. The left ventricular end-diastolic dimension fell from 48 mm (SD, 5.8 mm) before surgery to 35.1 mm (SD, 5.2 mm) at 1 year after the operation, and the fractional shortening increased over the first year from 19.6% (SD, 9.3%) to 32.8% (SD, 5.9%; both P <.001). CONCLUSIONS: Long-term clinical outcome and left ventricular function are good, despite severe left ventricular dysfunction at presentation.

Adolescent↗

Advances in pediatric cardiac surgery.

During the past year there have been many important scientific and clinical publications addressing important aspects of pediatric cardiac surgery. Herein we review some of the more significant contributions, with our own commentary added. Space limitations prevent a more comprehensive review.

Cardiac Surgical Procedures↗

Arterial switch in hearts with left ventricular outflow and pulmonary valve abnormalities.

BACKGROUND: Pulmonary valve and left ventricular outflow tract abnormalities (LVOT) may not be absolute contraindications to arterial switch operation (ASO). METHODS: In this study we analyze long-term outcome for 26 such transposition patients (6.3% of our ASO cohort). Median age and weight were 69 days (7 to 3,631 days) and 4.5 kg (2.6 to 34 kg). Pulmonary valve abnormalities included bicuspid valve (n = 4) and dysplastic valve (n = 5). The LVOT abnormalities (n = 17) included accessory atrioventricular valve/endocardial cushion tissue, fibromuscular ring, anomalous muscle bands, and septal malalignment. Patients with dynamic LVOT obstruction were excluded. The median preoperative left ventricular to pulmonary artery peak systolic pressure gradient was 30 mm (0 to 93 mm), or 50 mm (16 to 93 mm) if patients with isolated valve abnormalities are excluded. The ASO was performed according to our standard technique with or without LVOT resection or pulmonary valvotomy as required. RESULTS: There were two perioperative deaths (7.7%; 95% confidence interval, 0.9% to 25%), and no late deaths during 1,934 patient-months of follow-up time. Actuarial freedom from reoperation for neoaortic valve or LVOT problems is 87% (+/- 7) at 130 months, representing two reoperations. One was performed for neoaortic insufficiency plus LVOT obstruction, and the other for isolated LVOT obstruction. One patient currently has significant neoaortic insufficiency, and median gradient at last follow-up is 0 mm Hg (range, 0 to 35 mm Hg). CONCLUSIONS: The ASO can be performed in selected patients with transposition of the great arteries and with LVOT abnormalities with early and late survival and functional status similar to that of matched patients with normal pulmonary valves and LVOT (p > 0.05), but with a greater hazard for reoperation (p < 0.05). Selection for ASO should be based on anatomic criteria rather than left ventricular to pulmonary artery gradient alone, to avoid assigning these patients with transposition of the great arteries to treatment strategies less satisfactory than ASO.

Child↗

The surgical importance of coronary sinus orifice atresia.

Coronary sinus orifice atresia is rare. We describe two cases, one with an atrioventricular septal defect and another with supracardiac totally anomalous pulmonary venous drainage. The association with the latter has not been described previously. The importance of diagnosing the defect is emphasized and surgical treatment is discussed.

Coronary Vessel Anomalies↗

Transatrial-transpulmonary tetralogy of Fallot repair is effective in the presence of anomalous coronary arteries.

OBJECTIVES: The study's object was to analyze the outcomes of transatrial-transpulmonary repair in children with tetralogy of Fallot and anomalous coronary artery crossing the right ventricular outflow tract. METHODS: The transatrial-transpulmonary approach was used in 611 consecutive repairs, 36 (5.9%) of which were associated with a surgically relevant coronary artery anomaly. The median age and weight of the patients at repair were 23 months (2.8-170 months) and 9.9 kg (5.2-41 kg), respectively. Anomalies included left anterior descending coronary artery from right coronary artery or single right coronary artery (n = 22), right coronary artery from left coronary artery or left anterior descending coronary artery (n = 8), and large right coronary artery conal branch (n = 6). Diagnosis was established before the operation in 25 of 36 cases by angiography (n = 24) or echocardiography (n = 1). The approach was successful in 34 cases, in 25 of which placement of a limited transannular patch was necessary. Two patients had a right ventricle-pulmonary artery conduit as a result of proximity of the coronary branch to the pulmonary arterial anulus and inability to adequately relieve the right ventricular outflow tract obstruction. RESULTS: There have been no early or late deaths. Mean right ventricle-pulmonary artery gradient at last follow-up was 19 mm Hg (95% confidence interval 14.5-24 mm Hg), compared with 15 mm Hg (95% confidence interval 12.5-17.5 mm Hg) for patients with normal coronary arteries (P = .3). Actuarial freedom from reoperation at 120 months was 96.5% (95% confidence interval 79.8%-99.5%) and was also similar between patients with and without coronary artery abnormalities (P = .92). CONCLUSIONS: Surgically important coronary anomalies in tetralogy of Fallot can be dealt with through the transatrial-transpulmonary approach in most cases without major alterations in technique. Outcomes are similar to those of other patients with tetralogy of Fallot. The presence of anomalous coronary arteries does not impart incremental risk after this surgical strategy.

Adolescent↗

Centrifugal ventricular assist in children under 6 kg.

OBJECTIVE: The clinical application of centrifugal ventricular assist devices (VAD) has generally been limited to adults and large paediatric patients. In our experience neonates and small paediatric patients requiring ventricular support post-cardiopulmonary bypass are well supported by VAD. In this study we analyse our experience. METHODS: We have examined the records of our VAD patients who weighed less than 6 kg. Thirty-four patients, ranging in age from 2 to 258 days (median 60 days) and weight from 1.9 to 5.98 kg (median 3.7 kg), underwent 35 VAD procedures. One patient was supported on VAD twice. RESULTS: All patients had congenital heart lesions and were placed on VAD either because they could not be weaned from cardiopulmonary bypass after repair or palliation of the lesion (71.5%), or for support in the post-operative period due to refractory low cardiac output (28.5%). Twenty-two of the 35 VAD procedures (0.63, 95% CI: 0.45-0.78) resulted in successful weaning and decannulation, this was similar to the weaning probability for patients greater than 6 kg (P = 0.07). There were 10 late deaths in this group, with a 1-year KM survival of 0.31 (95% CI: 0.17-0.47). Most late deaths were related to irreversible cardiac disease processes as were the elective discontinuance of VAD outcomes. Neither weight, age, VAD duration, CPB duration, X clamp duration, univentricular anatomy or TGA anatomy predicted successful discharge from hospital (P > 0.05)--Weight P = 0.576; Age P = 0.532; VAD duration P = 0.181; CBP duration P = 0.549; X clamp duration P = 0.984; Univentricular anatomy P = 0.481; TGA anatomy P = 0.099. CONCLUSION: We believe centrifugal ventricular assist is a realistic option in very small patients who require post-cardiopulmonary bypass support. It is relatively easy to establish and manage, the results, although showing no factors predictive of successful discharge, are encouraging.

Cardiopulmonary Bypass↗

Senning plus arterial switch operation for discordant (congenitally corrected) transposition.

BACKGROUND: Congenitally corrected transposition of the great arteries is a complex cardiac lesion, usually associated with ventricular septal defect, left ventricular outflow tract obstruction, and tricuspid valve abnormalities. A subset of patients without left ventricular outflow tract obstruction have undergone Senning plus arterial switch operation in an attempt to place the left ventricle in the systemic circuit and the right ventricle in the pulmonary circuit. METHODS: Fourteen patients have had the operation performed since July 1989. Age and weight medians were 12 months (range, 0.5 to 120 months) and 8.2 kg (range, 3.2 to 34 kg). All but 1 patient had a left ventricular to right ventricular pressure ratio greater than 0.7, due to a large ventricular septal defect (with or without a previous pulmonary artery band), severe congestive heart failure caused by right ventricular dysfunction and tricuspid insufficiency, or a pulmonary artery band for left ventricular retraining. At least 10 patients had strong contraindications to "classic" repair, including right ventricular hypoplasia (n = 2), moderate to severe right ventricular dysfunction (n = 5), or moderate to severe tricuspid insufficiency (n = 9). RESULTS: There was one hospital death, occurring in a neonate (7%; 95% confidence interval = 0% to 34%). Actuarial survival beyond 10 months is 81% (95% confidence interval = 42% to 95%), currently with 389 patient-months of total follow-up time. The median grade of tricuspid insufficiency fell from 3/4 preoperatively to 1/4 postoperatively (p = 0.003). Right ventricular function is normal in 11/12 current survivors, all but 1 of whom are in New York Heart Association class I or II. CONCLUSIONS: Senning plus arterial switch operation is a good option for selected patients with congenitally corrected transposition of the great arteries with a similar or lower early risk (as compared with classic repairs). Some of the long-term problems associated with congenitally corrected transposition of the great arteries may be avoided with this strategy.

Cardiac Surgical Procedures↗

Management of the univentricular connection: are we improving?

OBJECTIVE: To assess the impact of the bidirectional cavopulmonary shunt, total cavopulmonary connection, and baffle fenestration on outcome of the Fontan operation in our unit. METHOD: We reviewed 123 bidirectional cavopulmonary shunts and 264 Fontan operations performed from 1980 to 1995. Analysis of pulmonary artery size (right and left main and lower lobe branches) before and after bidirectional cavopulmonary shunt was performed. Outcome of the Fontan operation was analysed in various time periods to assess the effect of prior bidirectional cavopulmonary shunt, use of the lateral tunnel, and fenestration. RESULTS: Operative risk for the bidirectional cavopulmonary shunt was 4% (CI = 2-10%) with a survival of 89% (CI = 83-95%) at 36 months. Probability of conversion to Fontan operation at 36 months was 49% (CI = 38-61%). Pulmonary artery size (Nakata and lower lobe indices) fell after bidirectional cavopulmonary shunt (P = 0.0006). Fontan risk dropped from 8.5% (1980-1987) to 1.8% (1988-1995) (P = 0.02), coinciding with the use of the bidirectional cavopulmonary shunt. There was no further risk reduction after introduction of the lateral tunnel and baffle fenestration, although these comparisons are limited by relatively small numbers. Duration of hospital stay related to pleural effusions was lowest for patients with a fenestrated lateral tunnel operation (P < 0.05). CONCLUSION: The bidirectional cavopulmonary shunt is a suboptimal stimulus for pulmonary artery enlargement, but may reduce the risk of Fontan operation in selected children. Fenestrated lateral tunnel operations have reduced the duration of postoperative pleural effusions.

Adolescent↗

Management strategy and long-term outcome for truncus arteriosus.

OBJECTIVE: Evaluation of a consistent policy of elective repair of truncus arteriosus at 2-3 months of age for the patients who are independent of hospital ward care, and long-term outcome. METHODS: Retrospective study of 82 patients with truncus arteriosus who underwent total repair at the Victorian Paediatric Cardiac Surgical Unit between 1979 and December 1995. The timing was based on a consistent policy of elective repair at 2-3 months of age for patients who were independent of hospital ward care. Earlier repair was performed when the patients were in uncontrolled congestive heart failure. RESULTS: Follow-up was complete for all patients with a mean of 76 months (1-183). There were 11 hospital deaths (13.4% CL 9-18.5), and five late deaths, actuarial survival at 80 months was 81% (CL 70-88%) with 39 patients uncensored at that point. For the purpose of this presentation, patients have been grouped according to their age at repair; 1, neonates n = 17 (hospital mortality = 5); 2, infants 1-6 months of age n = 48 (hospital mortality = 4); 3, patients beyond 6 months n = 17 (hospital mortality = 2). This series includes 10 patients with interrupted aortic arch with no mortality, and 10 patients with discontinuous pulmonary artery (hospital mortality = 2). Thirty-seven patients have had 54 conduits replaced to date. Using multiple regression, body weight < 3 kg was the only significant independent risk factor for hospital mortality. CONCLUSIONS: Our management policy tended to gather patients with risk factors described elsewhere into presentation group 1 and low risk truncus patients into presentation group 2. Deferral of surgery to 2-3 months of age is possible and lowers the surgical risk.

Actuarial Analysis↗

Arterial switch operation. Surgical solutions to complex problems.

The arterial switch operation is appropriate treatment for most forms of transposition of the great arteries. In this review we analyze indications, techniques, and outcome for various subsets of patients with transposition of the great arteries, including those with an intact septum beyond 21 days of age, intramural coronary arteries, aortic arch obstruction, the Taussig-Bing anomaly, discordant (corrected) transposition, transposition of the great arteries with left ventricular outflow tract obstruction, and univentricular hearts with transposition of the great arteries and subaortic stenosis.

Cardiac Surgical Procedures↗

[Follow-up of allogenic bone marrow graft with DNA polymorphisms. Use of two minisatellite MS31 and MS43 probes. Comparison with data of chromosomal polymorphisms].

OBJECTIVES: Allogenic bone marrow transplantation is widely used to treat many diseases of the haemopoietic system as well as metabolic disorders. Follow-up is essential to assess acceptance, rejection or post-graft relapse. This study was undertaken to evaluate the usefulness of the minisatellite probes MS31 and MS43 used as a routine follow-up test after bone marrow transplantation. METHODS: Twenty receivers of allogenic bone marrow transplants were followed-up. Two monoclonal minisatellite probes, MS31 and MS43, were used for comparison with the classical polymorphism methods. RESULTS: Fourteen cases of total chimeras, 3 cases of rejections and 3 cases of mixed chimeras were observed with the molecular probe techniques. In 19 of the 20 cases, this technique gave results compatible with classical polymorphism results. CONCLUSIONS: The minisatellite probes MS31 and MS43 were found to be sensitive, effective tests for bone marrow transplants which can be used in routine follow-up.

Adolescent↗

Trisomy 12 in B-cell chronic lymphocytic leukemia: interphase study by in situ hybridization in 75 patients.

Trisomy 12 is the most common cytogenetic abnormality in chronic lymphocytic leukaemia (CLL) and may be a prognostic indicator. In the present study, fluorescence in situ hybridization (FISH) is shown to be a method of choice for detection of trisomy 12 in interphase cells. Seventy-five cases of B-cell CLL were analysed with a chromosome 12 specific alpha satellite DNA probe and results compared with those from cytogenetic analysis. FISH showed the three hybridization spots characteristic of trisomy 12 in 32/75 patients (42.6%). Sixty-three patients were also studied by conventional cytogenetics: failure in 7 cases, normal karyotype in 28, trisomy 12 in 9 (14.3%) and in 19 cases abnormalities other than trisomy 12. In these same 63 patients, trisomy 12 was detected on 29 occasions by FISH (46%): in one case of failure by cytogenetic analysis, in 9 cases thought to have a normal karyotype, in 10 cases carrying abnormalities other than trisomy 12 and in all 9 cases showing trisomy 12 by conventional cytogenetic investigation. Correlation between trisomy 12 and the three stages of the Binet classification indicated an increasing proportion of trisomy 12 from stage A to stage C. It is concluded that fluorescence in situ hybridization is a powerful and sensitive technique for detection of trisomy 12 in CLL and although more cases will be required to confirm a correlation between the incidence of trisomy 12 and the stage of the disease, this link could be important from a prognostic point of view.

Adult↗

Cytogenetic experience in prenatal fra(X) detection on amniotic fluid cultures.

Since 1987, we have had experience with 13 prenatal diagnoses of 11 women at risk for the fragile X syndrome by cytogenetic studies on amniotic fluid cultures. The induction method included TC 199 medium and methotrexate. Results were obtained in all cases. Ten were males and three were prenatally diagnosed as being affected. Three were females and none of them was fra(X)-positive. Results were confirmed in 10/13 cases. In these cases, we had neither false-positive nor false-negative results.

Amniocentesis↗

t(2;18) and t(18;22) variant chromosomal translocations in B cell malignancies.

Variant translocations (2;18 and 18;22) are described in this review. The chromosomal and molecular findings of these translocation of BCL2 and their effect on possible BCL2 gene activation is discussed. Unanswered questions still remain and these include why this is so rare compared to the 25% incidence recorded for translocations in Burkitt's lymphoma. Further studies are obviously still needed in order to determine the true frequency of these findings and their distribution in the various B-cell disorders.

Animals↗

Chromosome studies in stimulated lymphocytes of 50 patients with B-cell chronic lymphocytic leukemia.

The chromosome constitutions of stimulated lymphocytes from 50 B-cell chronic lymphocytic leukemia patients were studied using different stimulation systems, i.e., TPA alone or associated with different cytokines. Adequate metaphases were obtained in 44 subjects (88%). Among 20 patients with abnormal karyotypes (45.5%), 7 had trisomy 12. The most frequent structural abnormality was a 14q+ resulting from translocations including one t(11;14) and two t(14;17), while deletions on the long arms of chromosomes 6 and 13 constituted a second common alteration. The most important finding in this series was the recurrence of a t(18;22) observed in two cases.

Adult↗