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Papers by Jaroslav Stark

Research paper thumbnail of A case for anatomic correction in atrioventricular discordance? Effects of surgery on tricuspid valve function

The Journal of Thoracic and Cardiovascular Surgery, Jun 1, 2001

To assess tricuspid valve function in atrioventricular discordance after palliative procedures (p... more To assess tricuspid valve function in atrioventricular discordance after palliative procedures (pulmonary artery banding and Blalock-Taussig shunt) and corrective procedures (anatomic and physiologic repair). Methods: Tricuspid valve dysfunction was assessed by transthoracic echocardiography and graded as no regurgitation (0), mild (1), moderate (2), and severe (3) before and after palliative and corrective procedures performed in 97 patients with atrioventricular discordance between 1988 and 1999. Thirty-two percent had an isolated ventricular septal defect, 43% had a ventricular septal defect and pulmonary stenosis, and 16% had pulmonary stenosis. Twenty-six patients underwent pulmonary artery banding and 28 had a Blalock-Taussig shunt. Seventy patients underwent physiologic and 19 underwent anatomic repair. Six patients underwent one-ventricle repair. Results: After pulmonary artery banding, the tricuspid regurgitation score decreased from 1.7 ± 0.8 to 0.9 ± 0.6 (P < .001). In patients who underwent a Blalock-Taussig shunt, the tricuspid regurgitation score increased from 0.7 ± 0.5 preoperatively to 1.4 ± 0.6 postoperatively (P < .001). After physiologic repair, there was no significant change in the tricuspid regurgitation score; however, 7 patients required additional repair or replacement. The regurgitation score was significantly reduced from 1.5 ± 0.8 to 0.4 ± 0.5 (P < .001) after anatomic repair. The operative mortality in patients who underwent physiologic repair was 7% as compared with 0% in the anatomic repair group (P = .59). The median follow-up was 3.2 years. Conclusions: Right ventricular volume loading (shunt) worsens tricuspid regurgitation, whereas volume reduction (banding) or left-to-right septal shift (anatomic repair) has beneficial effects. We have not observed a significant change in the tricuspid regurgitation score after physiologic repair. Anatomic repair can be performed in selected patients with atrioventricular discordance and provides superior functional results.

Research paper thumbnail of Fibrin Seal (Tisseel/Tissucol) in Paediatric Cardiothoracic Surgery. The Experience of the Hospital for Sick Children, Great Ormond Street, London

Fibrin Seal (Tisseel/Tissucol) in Paediatric Cardiothoracic Surgery. The Experience of the Hospital for Sick Children, Great Ormond Street, London

Over the last 5 years Tisseel has become an established part of our armamentarium. We have previo... more Over the last 5 years Tisseel has become an established part of our armamentarium. We have previously reported our preliminary experience and now update those data. Tisseel has been used increasingly for the prophylaxis of haemorrhage control rather than for treatment of established haemorrhage. Since 1980, Tisseel has been utilised in 85 patients with a variety of paedatric cardiac surgical problems, and in 72 of the 85 (85%) excellent haemostasis was achieved. In ten patients (11%) a good result was achieved, and in only three patients (4%) was an unsatisfactory result achieved. One of these was early in our experience, and the remaining two were caused by the application of Tisseel to relatively severe established haemorrhage. Tisseel has also been used to seal serous leak from a Gore-tex graft. In our experience, Tisseel has represented a major advance in the safety of paediatric cardiothoracic surgery and may now be considered an essential part of certain procedures, particularly, for example, the arterial switch and repair of truncus arteriosus.

Research paper thumbnail of Mixed total pulmonary venous drainage: Still a surgical challenge

The Journal of Thoracic and Cardiovascular Surgery, Dec 1, 1996

The aim of this report is to review the surgical experience of a single institution with a relati... more The aim of this report is to review the surgical experience of a single institution with a relatively large series of patients with mixed total pulmonary venous drainage. Patient population: Between January 1, 1971, and December 31, 1994, 232 patients with total pulmonary venous drainage underwent surgical correction. Twenty of these patients (8.6%) had mixed type total pulmonary venous drainage. Ages at operation ranged from 1 day to 46 months, with a median of 2.3 months. Results: Both cardiac catheterization and echocardiography were performed before operation in 12 patients. Four patients underwent only cardiac catheterization, and another four patients underwent only echocardiography. The sensitivity and specificity for catheterization were 94% and 99%, respectively; they were 31% and 100%, respectively, for echocardiography. Severe pulmonary venous obstruction was present in three patients, all of whom underwent emergency operation. Three patients (15%), all of whom had preoperative pulmonary venous obstruction, died after operation. There were two late deaths, one of pulmonary vein stenosis and the other of probable pulmonary hypertension. The actuarial survival at 10 years was 73% for all patients; patients who survived the initial operation had a 10-year survival of 87%. Conclusion: The diagnosis of mixed total pulmonary venous drainage can be difficult to establish by echocardiography or at the time of operation. For patients in stable condition, cardiac catheterization may be considered if fewer than three pulmonary veins are identified by echocardiography. Pulmonary venous obstruction is relatively infrequent in this group of patients but when present impacts patient survival significantly. The long-term results with this lesion are excellent.

Research paper thumbnail of Atrioventricular septal defect and tetralogy of Fallot: a 15-year experience

European Journal of Cardio-Thoracic Surgery, Aug 1, 1997

Aim: Atrioventricular septal defect and tetralogy of Fallot is a relatively uncommon lesion in wh... more Aim: Atrioventricular septal defect and tetralogy of Fallot is a relatively uncommon lesion in which there is a risk of right ventricular dysfunction related to inlet and outlet valve problems. For this reason, conservative management involving an initial palliative procedure is often chosen. The aim of this report is to retrospectively review our experience with this lesion. Patient population: 35 patients with atrioventricular septal defect and tetralogy of Fallot have been surgically managed at this institution between January 1980 and June 1995. Twenty-one (60%) of these patients underwent 28 initial palliative shunt procedures. Fourteen (40%) patients underwent primary definitive repair. The criteria for choosing one management strategy over another was based on a number of factors, including age at presentation, anatomy of the lesion, and severity of symptoms. Of the 21 patients who underwent an initial shunt procedure, 15 have undergone definitive operation. Of the 6 patients who did not undergo definitive operation, three died (two directly related to complications of the shunt procedure), two are awaiting operation, and one was lost to follow-up. Results: The primary indication for operation in all patients was cyanosis. Freedom from reoperation at 5 years after definitive operation was 65.1% for all patients; most reoperations were related to left atrioventricular valve regurgitation or residual leaks across the ventricular septal defect patch. The operative mortality at definitive operation was 10.3% (70% CL 4.5-20%) for all patients. The actuarial estimate of survival 7 years following definitive repair was 77.3% (70% CL 68.7-85.9%) for all patients. The actuarial estimate of survival at 7 years was 84.4% (70% CL 73.8-95%) in the patients undergoing primary repair and 65% (70% CL 52.4-77.6%) in patients initially palliated if the mortality of the palliative shunt procedure is included (P=0.35). Conclusion: Patients with atrioventricular septal defect and tetralogy of Fallot can be successfully managed with a variety of surgical strategies. Primary repair may be a reasonable option in carefully selected patients, as this eliminates the morbidity and mortality of an initial shunt procedure and the subsequent interval between initial palliation and definitive repair.

Research paper thumbnail of Outcomes After Delayed Sternal Closure in Pediatric Heart Operations: A 10-Year Experience

The Annals of Thoracic Surgery, Feb 1, 1997

Background. Open heart operations in young children may lead to myocardial swelling and increased... more Background. Open heart operations in young children may lead to myocardial swelling and increased lung water. Decreased intrathoracic space may then make sternal closure difficult. Delayed sternal closure may be beneficial in this setting. Potential risks of delayed sternal closure are sepsis and sternal instability. Methods. To assess these risks, we reviewed retrospectively 150 consecutive children who underwent delayed sternal closure after repair of complex congenital cardiac defects. Results. Diagnoses included transposition of the great arteries (66), total anomalous pulmonary venous drainage (11), and complete atrioventricular septal defects (10). Age at operation was 229 ؎ 51 days (mean ؎ standard error of mean). Sixteen patients required extracorporeal membrane oxygenation. Survival was 88% (133 patients). The sternum was left open for 3.86 ؎ 0.29 days. Fifteen patients had minor wound infections requiring antibiotics. No patient required reexploration for mediastinitis and no patient had an unstable sternum. Conclusions. Delayed sternal closure with sternal stenting and silicone membrane skin closure is a safe procedure in infants and children with compromised cardiac output after repair of congenital cardiac defects.

Research paper thumbnail of Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome

Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome

The Annals of Thoracic Surgery, Jun 1, 1991

ABSTRACT

Research paper thumbnail of Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts

Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts

PubMed, 1981

Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried ... more Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried out at The Hospital for Sick Children, Great Ormond Street. The operation consists of interposing between the subclavian artery and the pulmonary artery a prosthesis of greater diameter than that of the subclavian artery. The first 13 operations were performed with a prosthesis of woven Dacron. Conduits of expanded polytetrafluorethylene (PTFE) were used for 86 MBTSs, and these form the basis of this report. Forty-four (51.1%) shunts were performed in infancy, 25 in patients under the age of 1 month (29.0%). Prostheses of 4 mm were used in 34 cases, 5 mm in 14, and 6 mm in 38. There were five shunt failures (5.8%) documented at postmortem or angiocardiographic investigation. Including two late deaths for which postmortem examinations were not obtained, the overall failure rate was 8.1% (seven patients). All these patients were operated upon in infancy, four of them in the neonatal period. Although a longer follow-up is necessary to assess the validity of these shunts, the early results are encouraging. We believe we can now recommend MBTS as an alternative when the classical Blalock-Taussig shunt is considered unsuitable.

Research paper thumbnail of Allografts in the treatment of absent pulmonary valve syndrome and complex tetralogy of Fallot

Allografts in the treatment of absent pulmonary valve syndrome and complex tetralogy of Fallot

The absent pulmonary valve syndrome comprises agenesis of the pulmonary valve, annular stenosis, ... more The absent pulmonary valve syndrome comprises agenesis of the pulmonary valve, annular stenosis, pulmonary insufficiency, ventricular septal defect (VSD) and, usually, right ventricular (RV) hypertrophy. The pulmonary arteries (PA) are aneurysmal and usually compress the tracheobronchial tree. Several surgical techniques have been described, and many have been used in our unit (1). Our current policy is described in this short review.

Research paper thumbnail of Congenital left ventricular inflow obstruction: is the outcome related to the site of the obstruction?

Congenital left ventricular inflow obstruction: is the outcome related to the site of the obstruction?

European Journal of Cardio-Thoracic Surgery, 1988

Between 1978 and 1987, 39 patients aged 1 day to 15 years underwent surgery for symptomatic left ... more Between 1978 and 1987, 39 patients aged 1 day to 15 years underwent surgery for symptomatic left ventricular inflow obstruction. Four diagnostic groups were identified: cor triatriatum (6 patients), supravalvar mitral membrane (SVMM) with a normal mitral valve (7 patients), SVMM with an abnormal mitral valve (9 patients) and mitral stenosis (17 patients). Associated cardiac anomalies occurred in 26 patients (67%). There were 8 deaths (21%), 3 in patients with SVMM and an abnormal mitral valve and 5 in patients with mitral stenosis. Survival for patients with normal mitral valves was significantly better than that for patients with abnormal mitral valves (13/13 vs 18/26, P less than 0.05). There was also high morbidity in patients requiring prosthetic mitral valve replacement. These data suggest that the outcome of surgical treatment for left ventricular inflow obstruction may be predicted according to the site of the obstruction. This is best determined preoperatively by cross-sectional echocardiography which allows optimal planning of surgical strategy.

Research paper thumbnail of Pulmonary artery sling

Pulmonary artery sling

The Annals of Thoracic Surgery, Nov 1, 1992

ABSTRACT

Research paper thumbnail of Tricuspid valve abnormalities in patients with atrioventricular discordance: Surgical implications

The Annals of Thoracic Surgery, Apr 1, 1994

From 1975 to 1990,90 patients (age, 6 months to 30 years; mean, 9.1 years) underwent operation fo... more From 1975 to 1990,90 patients (age, 6 months to 30 years; mean, 9.1 years) underwent operation for defects associated with atrioventricular discordance. Twenty-one had an anatomically abnormal tricuspid (systemic) atrioventricular valve (SAVV) of the following types: Ebstein, 11; straddling, 6; and dysplastic, 4. Sixteen valves were regurgitant: regurgitation was trivial to mild in nine and moderate to severe in seven. Two patients underwent a successful Fontan-type operation. None of the 5 patients with a competent SAVV underwent valve repair or replacement; 1 of these patients died. A ventricular septal defect was closed in 14 and an extracardiac valved conduit was placed in 7. Sixteen had a regurgitant valve: it was replaced in 10 and repaired in 2 (early mortality, he results from the surgical treatment of lesions asso-T ciated with atrioventricular (AV) discordance have improved in recent years [14]. Techniques for preventing injury to the conduction tissue during ventricular septal defect (VSD) closure [5-71 and for conduit placement and replacement [8, 91 have all been well described. The tricuspid valve functions as a systemic AV valve (SAVV) in these patients. It is of interest that the results of operation remain much less satisfactory in patients with either anatomically or functionally abnormal SAVVs [ 1, 41. We have reviewed our experience in 90 patients who underwent intracardiac repairs of anomalies associated with AV discordance [lo]. In that study, 20 variables were analyzed statistically, and the most significant risk factor for early death was an anatomically abnormal SAVV (p < 0.0001) (Table 1). Less than optimal results in patients with an abnormal SAVV contrasted strongly with the excellent results achieved in patients with a normal SAVV (Table 2). This report analyzes in greater detail a subgroup of 21 patients with AV discordance associated with a morphologically abnormal SAW. Material and Methods Definitions A discordant AV connection is a cardiac anomaly in which the right atrium connects to a morphologically left ventri

Research paper thumbnail of Intracardiac repair of lesions associated with atrioventricular discordance

European Journal of Cardio-Thoracic Surgery, 1996

Objective. Discordant atrioventricular (AV) connection is a rare congenital cardiac anomaly. Asso... more Objective. Discordant atrioventricular (AV) connection is a rare congenital cardiac anomaly. Associated cardiac defects modify the physiology, clinical presentation, and surgical management of these patients. We have reviewed our overall experience with 90 patients operated for lesions associated with AV discordance between 1975-1990. Methods_ 90 patients, consecutively

Research paper thumbnail of Expanded PTFE Membrane to Prevent Cardiac Injury During Resternotomy for Congenital Heart Disease

The Annals of Thoracic Surgery, Dec 1, 1996

Background. Resternotomy for repair of congenital cardiac defects can result in cardiacinjurj, Cl... more Background. Resternotomy for repair of congenital cardiac defects can result in cardiacinjurj, Closureof the pericardiumduring the initial operationmay prevent thia,and severalpericardialsubstituteshavebeentried, with variableresults,in patientsin whomprimarypericardial closure is not possible.We conducteda multicenter obsewationalstudy of the use of the expanded polytetrafluoroethylene membrane(PrecludePericardial Membrane, formerlycalledthe Gore-TexSurgicalMembrane; W. L. Gore & Associates, Flagstatl, AZ) in patients likely to undergo reoperation for treatment of congenital heart disease. Methods. Data were collectedretrospectivelyon all patientsin whomthe expandedpolytetrafluoroethylene s ince its popularization by Julianand associates[1]in 1957, the median sternotomy has become the most commonly used incision in cardiac surgery. Moreover, because the number of patients with congenital heart defects requiring reoperation is growing the need for a second and even a third or fourth stemotomy is also increasing. The risks involved in reoperative stemotomy are welj known [2-5]. Pericardial and mediastinal adhesions can predispose the heart to iatrogenic injury during reopening of the sternum by making it difficult for the surgeon to identify anatomic features during dissection. The sequelae of such an injury include catastrophic bleedin~decreased right ventricular function, and death. Because primary closure of the pericardium is not always possible, various materials have been suggested for use as a pericardial substitute to decrease adhesion formation and the potential for cardiac injury at reoperation. These include silicone, bovine pericardium, and expanded polytetrafluoroethylene (ePTFE) [6-10]. The O.1-mm-thick low-porosity ePTFE membrane (Preclude Pericardial Membrane, formerly called the Gore-Tex Surgical Membrane; W. L. Gore & Associates, Flagstaff, AZ) was initially found to reduce pericardial adhesion formation in animal models [11]. Subsequent reports described favorable results with the device in Acceptedfor publication June2S,19%.

Research paper thumbnail of Surgical treatment of aortic coarctation in infants younger than three months: 1985 to 1990. Success of extended end-to-end arch aortoplasty

PubMed, 1994

There remains controversy regarding the appropriate surgical treatment of coarctation of the aort... more There remains controversy regarding the appropriate surgical treatment of coarctation of the aorta in infants. In 1985 we introduced the extended end-to-end repair into our practice and now wish to present a review of our recent experience. One hundred fifty-one infants younger than 3 months of age underwent repair of coarctation between 1985 and 1990. In 25% and 33% of the patients, there was hypoplasia of the isthmus and of the transverse arch, respectively. Surgical procedures were as follows: subclavian flap angioplasty in 15 patients, resection with a traditional end-to-end anastomosis in 43, and resection with an extended end-to-end anastomosis into the arch in 77. In 30 patients, the extension was proximal to the origin of the left carotid artery (radically extended end-to-end anastomosis). Other procedures were used in 16 patients. Mortality (13 early and 12 late deaths) was related on multivariate analysis to the presence of an associated major heart defect, preoperative resuscitation, and direct postoperative gradient over the arch. This immediate postoperative gradient was significantly lower after both extended and radically extended end-to-end anastomosis if there was a hypoplastic isthmus, and after radically extended end-to-end anastomosis if the transverse arch was hypoplastic. Actuarial freedom from recoarctation at 4 years was 57% (confidence limits 28% to 78%) after subclavian flap angioplasty, 77% (confidence limits 60% to 87%) after end-to-end anastomosis, 83% (confidence limits 66% to 92%) after extended end-to-end anastomosis and 96% (confidence limits 77% to 100%) after radically extended end-to-end anastomosis. We conclude that the extended end-to-end anastomosis and radical end-to-end anastomosis appear to offer the best prognosis for all infants with coarctation. The technique can be applied successfully to almost all types of arch anomalies.

Research paper thumbnail of Surgical treatment of aortic coarctation in infants younger than three months: 1985 to 1990

The Journal of Thoracic and Cardiovascular Surgery, 1994

There remains controversy regarding the appropriate surgical treatment of coarctation of the aort... more There remains controversy regarding the appropriate surgical treatment of coarctation of the aorta in infants. In 1985 we introduced the extended end-to-end repair into our practice and now wish to present a review of our recent experience. One hundred fifty-one infants younger than 3 months of age underwent repair of coarctation between 1985 and 1990. In 25 % and 33% of the patients, there was hypoplasia of the isthmus and of the transverse arch, respectively. Surgical procedures were as follows: subclavian flap angioplasty in 15 patients, resection with a traditional end-to-end anastomosis in 43, and resection with an extended end-to-end anastomosis into the arch in 77. In 30 patients, the extension was proximal to the origin of the left carotid artery (radicaUy extended end-to-end anastomosis). Other procedures were used in 16 patients. Mortality (13 early and 12 late deaths) was related on multivariate analysis to the presence of an associated major heart defect, preoperative resuscitation, and direct postoperative gradient over the arch. This immediate postoperative gradient was significantly lower after both extended and radically extended end-to-end anastomosis if there was a hypoplastic isthmus, and after radically extended end-to-end anastomosis if the transverse arch was hypoplastic. Actuarial freedom from recoarctation at 4 years was 57 % (confidence limits 28 % to 78 %) after subclavian flap angioplasty, 77% (confidence limits 60% to 87%) after end-to-end anastomosis, 83% (confidence limits 66 % to 92 %) after extended end-to-end anastomosis and 96 % (confidence limits 77% to 100%) after radically extended end-to-end anastomosis. We conclude that the extended end-to-end anastomosis and radical end-to-end anastomosis appear to offer the best prognosis for aU infants with coarctation. The technique can be applied successfuUy to almost aU types of arch anomalies.

Research paper thumbnail of Allografts in the Rastelli procedure: Techniques

Allografts in the Rastelli procedure: Techniques

Steinkopff eBooks, 1988

Children with transposition of the great arteries (TGA) (VA discordance), ventricular septal defe... more Children with transposition of the great arteries (TGA) (VA discordance), ventricular septal defect (VSD) and anatomical left ventricular outflow tract obstruction (LVOTO) used to be treated by the Mustard operation, closure of the VSD and surgical relief of the obstruction. This carried out a mortality in excess of 50% (1, 2), largely due to the difficulty in relieving the LVOTO. The prognosis of children with this complex of disorders was improved by the application of the Rastelli operation (3). This operation consists in enlargement of the VSD, diversion of the LV outflow to the aorta by an intraventricular tunnel/patch and connection of right ventricle (RV) to pulmonary artery (PA) by an external valved conduit (Figs. 1–3). Details of the operative technique we use have been published elsewhere (4, 5).

Research paper thumbnail of Unusual vascular ring in infant with pulmonary atresia and ventricular septal defect

Heart, Aug 1, 1982

A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syn... more A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.

Research paper thumbnail of Modified Blalock-Taussig shunt

Modified Blalock-Taussig shunt

The Journal of Thoracic and Cardiovascular Surgery, 1981

Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried ... more Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried out at The Hospital for Sick Children, Great Ormond Street. The operation consists of interposing between the subclavian artery and the pulmonary artery a prosthesis of greater diameter than that of the subclavian artery. The first 13 operations were performed with a prosthesis of woven Dacron. Conduits of expanded polytetrafluorethylene (PTFE) were used for 86 MBTSs, and these form the basis of this report. Forty-four (51.1%) shunts were performed in infancy, 25 in patients under the age of 1 month (29.0%). Prostheses of 4 mm were used in 34 cases, 5 mm in 14, and 6 mm in 38. There were five shunt failures (5.8%) documented at postmortem or angiocardiographic investigation. Including two late deaths for which postmortem examinations were not obtained, the overall failure rate was 8.1% (seven patients). All these patients were operated upon in infancy, four of them in the neonatal period. Although a longer follow-up is necessary to assess the validity of these shunts, the early results are encouraging. We believe we can now recommend MBTS as an alternative when the classical Blalock-Taussig shunt is considered unsuitable.

Research paper thumbnail of Utilização de valvas homólogas e heterólogas em condutos extracardíacos The use of homograph and heterograph valves in extracardiac conduits

DOAJ (DOAJ: Directory of Open Access Journals), Aug 1, 1988

RESUMO: O conceito do uso de um conduto extracardíaco para estabelecer uma via de saída, conectan... more RESUMO: O conceito do uso de um conduto extracardíaco para estabelecer uma via de saída, conectando o ventrículo direito com o tronco pulmonar, ou seus ramos, foi desenvolvido na década de 60. Entre 1971 e 1986,335 pacientes receberam , no The Hospital for Sick Children, de Londres, condutos extracardíacos para o lado direito do coração; 176 destes foram homoenxertos aórticos, preservados em solução antibióticonutriente; 140 heteroenxertos (Hancock, Ross, Carpentier-Edwards, lonescu-Shiley e 19 tubos não valvulados. Estes condutos foram usados na correção de defeitos cardíacos complexos. A idade média foi de 6,34 anos e o peso médio, de 17,8 kg. O diãmetro interno dos condutos variou de 8 a 30 mm. A mortalidade hospitalar foi de 29 ,2% e o seguimento dos sobrevivente teve uma duração máxima de 14,3 anos, sendo que apenas 40% delas foram relacionadas ao conduto extracardíaco. A curva atuarial, livre de obstrução, dos condutos extracardíacos foi significativa, quando se analisaram os homoenxertos, face a cada grupo de heteroenxertos (p < 0,005). Os fatores de risco mais importantes foram : o número de complicações pós-operatórias, para óbitos tardios ; a severidade das lesões associadas, para as reoperações ;. a data da cirurgia, para a troca do conduto extracardíaco, e a severidade das lesões associadas, para a obstrução. Assim, conclui-se que, a longo prazo, o uso de condutos valvulados extracardíacos tem bons resultados , especialmente no que se refere aos homoenxertos. Pelos dados apresentados, os homoenxertos aórticos, preservados em solução antibiótico-nutriente, continuam a ser nossa primeira escolha na reconstrução da via de saída ventrículo-pulmonar. DESCRITORES : condutos extracardíacos, cirurgia; valvas cardíacas, cirurgia; próteses valvulares cardíacas, cirurgia. INTRODUÇÃO O conceito do uso de condutos extracardíacos (CE) na correção de defeitos cardíacos congêntos. caracterizados pela descontinuidade entre o ventrículo direito. ou átrio direito. e o tronco pulmonar. ou os seus ramos, foi estabelecido com o trabalho experimental de ROB-BARD & WAGNER 20, em 1949. Porém, somente na década de 60 iniciaram-se as primeiras cirurgias. quando RASTELLI et a/Ii 19 confeccionaram um conduto não valvulado de pericárdio. para estabelecer a continuidade entre o ventrículo direito e o tronco pulmonar. numa forma complexa de tétrade de Fallot; ROSS & SOMERVILLE 2 1. 2 anos após. descreveram o primeiro uso de homoenxertos aórticos na correção total da atresia pulmonar com' defeito do septo ventricular. Trabalho realizado no Hospital for Sick Children. Londres , Inglaterra. Desde então. o uso de CE tem-se popularizado e várias são as instituições 4. 7. 8. 10. 16 que apresentam

Research paper thumbnail of Myocardial adenine nucleotide metabolism in pediatric patients during hypothermic cardioplegic arrest and normothermic ischemia

The Annals of Thoracic Surgery, May 1, 1989

Quantitative assessment of high-energy phosphate levels, including degradation or utilization dur... more Quantitative assessment of high-energy phosphate levels, including degradation or utilization during ischemia, has not previously been performed in infants and children. Animal experiments suggest that high-energy phosphate metabolism varies with maturation. To help answer these questions, 24 patients aged 2 months to 8 years underwent myocardial biopsy immediately after the institution of cardiopulmonary bypass (16 to 20°C). Additional samples were obtained at 16 and 45 minutes after aortic cross-clamping and administration of cardioplegia (St. Thomas's solution) (in vivo ischemia). Seven patients also underwent major myocardial resection. Resected specimens were placed in a 37°C bath and divided into equal-sized samples that were removed at ten-minute intervals (in vitro ischemia). All samples were immersed in liquid nitrogen and analyzed for adenine nucleotide pool metabolites using highperformance liquid chromatography. Levels of adenosine triphosphate were high before cross-clamping but uring the past decade, major advances have been D made that extend fundamental observations in myocardial metabolism in such a way as to bring new knowledge into direct clinical application [ 11. Controlled circumstances in a bloodless field with unimpaired visualization directly affect the technical performance of cardiac surgical procedures, which must be reflected in results. This is especially true in congenital cardiac procedures, where the size of the field is small, technical demands considerable, and time limited. The price one pays for a bloodless, immobile operative field is myocardial ischemia. Despite extraordinary advances in cardiac surgery for infants and children with congenital heart defects, operative morbidity and mortality remain high for repair of many defects. Diminished ventricular function is a principal determinant of outcome in these patients [2, 31. An important correlate of myocardial function after ischemia is the level of adenosine triphosphate (ATP). The

Research paper thumbnail of A case for anatomic correction in atrioventricular discordance? Effects of surgery on tricuspid valve function

The Journal of Thoracic and Cardiovascular Surgery, Jun 1, 2001

To assess tricuspid valve function in atrioventricular discordance after palliative procedures (p... more To assess tricuspid valve function in atrioventricular discordance after palliative procedures (pulmonary artery banding and Blalock-Taussig shunt) and corrective procedures (anatomic and physiologic repair). Methods: Tricuspid valve dysfunction was assessed by transthoracic echocardiography and graded as no regurgitation (0), mild (1), moderate (2), and severe (3) before and after palliative and corrective procedures performed in 97 patients with atrioventricular discordance between 1988 and 1999. Thirty-two percent had an isolated ventricular septal defect, 43% had a ventricular septal defect and pulmonary stenosis, and 16% had pulmonary stenosis. Twenty-six patients underwent pulmonary artery banding and 28 had a Blalock-Taussig shunt. Seventy patients underwent physiologic and 19 underwent anatomic repair. Six patients underwent one-ventricle repair. Results: After pulmonary artery banding, the tricuspid regurgitation score decreased from 1.7 ± 0.8 to 0.9 ± 0.6 (P < .001). In patients who underwent a Blalock-Taussig shunt, the tricuspid regurgitation score increased from 0.7 ± 0.5 preoperatively to 1.4 ± 0.6 postoperatively (P < .001). After physiologic repair, there was no significant change in the tricuspid regurgitation score; however, 7 patients required additional repair or replacement. The regurgitation score was significantly reduced from 1.5 ± 0.8 to 0.4 ± 0.5 (P < .001) after anatomic repair. The operative mortality in patients who underwent physiologic repair was 7% as compared with 0% in the anatomic repair group (P = .59). The median follow-up was 3.2 years. Conclusions: Right ventricular volume loading (shunt) worsens tricuspid regurgitation, whereas volume reduction (banding) or left-to-right septal shift (anatomic repair) has beneficial effects. We have not observed a significant change in the tricuspid regurgitation score after physiologic repair. Anatomic repair can be performed in selected patients with atrioventricular discordance and provides superior functional results.

Research paper thumbnail of Fibrin Seal (Tisseel/Tissucol) in Paediatric Cardiothoracic Surgery. The Experience of the Hospital for Sick Children, Great Ormond Street, London

Fibrin Seal (Tisseel/Tissucol) in Paediatric Cardiothoracic Surgery. The Experience of the Hospital for Sick Children, Great Ormond Street, London

Over the last 5 years Tisseel has become an established part of our armamentarium. We have previo... more Over the last 5 years Tisseel has become an established part of our armamentarium. We have previously reported our preliminary experience and now update those data. Tisseel has been used increasingly for the prophylaxis of haemorrhage control rather than for treatment of established haemorrhage. Since 1980, Tisseel has been utilised in 85 patients with a variety of paedatric cardiac surgical problems, and in 72 of the 85 (85%) excellent haemostasis was achieved. In ten patients (11%) a good result was achieved, and in only three patients (4%) was an unsatisfactory result achieved. One of these was early in our experience, and the remaining two were caused by the application of Tisseel to relatively severe established haemorrhage. Tisseel has also been used to seal serous leak from a Gore-tex graft. In our experience, Tisseel has represented a major advance in the safety of paediatric cardiothoracic surgery and may now be considered an essential part of certain procedures, particularly, for example, the arterial switch and repair of truncus arteriosus.

Research paper thumbnail of Mixed total pulmonary venous drainage: Still a surgical challenge

The Journal of Thoracic and Cardiovascular Surgery, Dec 1, 1996

The aim of this report is to review the surgical experience of a single institution with a relati... more The aim of this report is to review the surgical experience of a single institution with a relatively large series of patients with mixed total pulmonary venous drainage. Patient population: Between January 1, 1971, and December 31, 1994, 232 patients with total pulmonary venous drainage underwent surgical correction. Twenty of these patients (8.6%) had mixed type total pulmonary venous drainage. Ages at operation ranged from 1 day to 46 months, with a median of 2.3 months. Results: Both cardiac catheterization and echocardiography were performed before operation in 12 patients. Four patients underwent only cardiac catheterization, and another four patients underwent only echocardiography. The sensitivity and specificity for catheterization were 94% and 99%, respectively; they were 31% and 100%, respectively, for echocardiography. Severe pulmonary venous obstruction was present in three patients, all of whom underwent emergency operation. Three patients (15%), all of whom had preoperative pulmonary venous obstruction, died after operation. There were two late deaths, one of pulmonary vein stenosis and the other of probable pulmonary hypertension. The actuarial survival at 10 years was 73% for all patients; patients who survived the initial operation had a 10-year survival of 87%. Conclusion: The diagnosis of mixed total pulmonary venous drainage can be difficult to establish by echocardiography or at the time of operation. For patients in stable condition, cardiac catheterization may be considered if fewer than three pulmonary veins are identified by echocardiography. Pulmonary venous obstruction is relatively infrequent in this group of patients but when present impacts patient survival significantly. The long-term results with this lesion are excellent.

Research paper thumbnail of Atrioventricular septal defect and tetralogy of Fallot: a 15-year experience

European Journal of Cardio-Thoracic Surgery, Aug 1, 1997

Aim: Atrioventricular septal defect and tetralogy of Fallot is a relatively uncommon lesion in wh... more Aim: Atrioventricular septal defect and tetralogy of Fallot is a relatively uncommon lesion in which there is a risk of right ventricular dysfunction related to inlet and outlet valve problems. For this reason, conservative management involving an initial palliative procedure is often chosen. The aim of this report is to retrospectively review our experience with this lesion. Patient population: 35 patients with atrioventricular septal defect and tetralogy of Fallot have been surgically managed at this institution between January 1980 and June 1995. Twenty-one (60%) of these patients underwent 28 initial palliative shunt procedures. Fourteen (40%) patients underwent primary definitive repair. The criteria for choosing one management strategy over another was based on a number of factors, including age at presentation, anatomy of the lesion, and severity of symptoms. Of the 21 patients who underwent an initial shunt procedure, 15 have undergone definitive operation. Of the 6 patients who did not undergo definitive operation, three died (two directly related to complications of the shunt procedure), two are awaiting operation, and one was lost to follow-up. Results: The primary indication for operation in all patients was cyanosis. Freedom from reoperation at 5 years after definitive operation was 65.1% for all patients; most reoperations were related to left atrioventricular valve regurgitation or residual leaks across the ventricular septal defect patch. The operative mortality at definitive operation was 10.3% (70% CL 4.5-20%) for all patients. The actuarial estimate of survival 7 years following definitive repair was 77.3% (70% CL 68.7-85.9%) for all patients. The actuarial estimate of survival at 7 years was 84.4% (70% CL 73.8-95%) in the patients undergoing primary repair and 65% (70% CL 52.4-77.6%) in patients initially palliated if the mortality of the palliative shunt procedure is included (P=0.35). Conclusion: Patients with atrioventricular septal defect and tetralogy of Fallot can be successfully managed with a variety of surgical strategies. Primary repair may be a reasonable option in carefully selected patients, as this eliminates the morbidity and mortality of an initial shunt procedure and the subsequent interval between initial palliation and definitive repair.

Research paper thumbnail of Outcomes After Delayed Sternal Closure in Pediatric Heart Operations: A 10-Year Experience

The Annals of Thoracic Surgery, Feb 1, 1997

Background. Open heart operations in young children may lead to myocardial swelling and increased... more Background. Open heart operations in young children may lead to myocardial swelling and increased lung water. Decreased intrathoracic space may then make sternal closure difficult. Delayed sternal closure may be beneficial in this setting. Potential risks of delayed sternal closure are sepsis and sternal instability. Methods. To assess these risks, we reviewed retrospectively 150 consecutive children who underwent delayed sternal closure after repair of complex congenital cardiac defects. Results. Diagnoses included transposition of the great arteries (66), total anomalous pulmonary venous drainage (11), and complete atrioventricular septal defects (10). Age at operation was 229 ؎ 51 days (mean ؎ standard error of mean). Sixteen patients required extracorporeal membrane oxygenation. Survival was 88% (133 patients). The sternum was left open for 3.86 ؎ 0.29 days. Fifteen patients had minor wound infections requiring antibiotics. No patient required reexploration for mediastinitis and no patient had an unstable sternum. Conclusions. Delayed sternal closure with sternal stenting and silicone membrane skin closure is a safe procedure in infants and children with compromised cardiac output after repair of congenital cardiac defects.

Research paper thumbnail of Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome

Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome

The Annals of Thoracic Surgery, Jun 1, 1991

ABSTRACT

Research paper thumbnail of Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts

Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts

PubMed, 1981

Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried ... more Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried out at The Hospital for Sick Children, Great Ormond Street. The operation consists of interposing between the subclavian artery and the pulmonary artery a prosthesis of greater diameter than that of the subclavian artery. The first 13 operations were performed with a prosthesis of woven Dacron. Conduits of expanded polytetrafluorethylene (PTFE) were used for 86 MBTSs, and these form the basis of this report. Forty-four (51.1%) shunts were performed in infancy, 25 in patients under the age of 1 month (29.0%). Prostheses of 4 mm were used in 34 cases, 5 mm in 14, and 6 mm in 38. There were five shunt failures (5.8%) documented at postmortem or angiocardiographic investigation. Including two late deaths for which postmortem examinations were not obtained, the overall failure rate was 8.1% (seven patients). All these patients were operated upon in infancy, four of them in the neonatal period. Although a longer follow-up is necessary to assess the validity of these shunts, the early results are encouraging. We believe we can now recommend MBTS as an alternative when the classical Blalock-Taussig shunt is considered unsuitable.

Research paper thumbnail of Allografts in the treatment of absent pulmonary valve syndrome and complex tetralogy of Fallot

Allografts in the treatment of absent pulmonary valve syndrome and complex tetralogy of Fallot

The absent pulmonary valve syndrome comprises agenesis of the pulmonary valve, annular stenosis, ... more The absent pulmonary valve syndrome comprises agenesis of the pulmonary valve, annular stenosis, pulmonary insufficiency, ventricular septal defect (VSD) and, usually, right ventricular (RV) hypertrophy. The pulmonary arteries (PA) are aneurysmal and usually compress the tracheobronchial tree. Several surgical techniques have been described, and many have been used in our unit (1). Our current policy is described in this short review.

Research paper thumbnail of Congenital left ventricular inflow obstruction: is the outcome related to the site of the obstruction?

Congenital left ventricular inflow obstruction: is the outcome related to the site of the obstruction?

European Journal of Cardio-Thoracic Surgery, 1988

Between 1978 and 1987, 39 patients aged 1 day to 15 years underwent surgery for symptomatic left ... more Between 1978 and 1987, 39 patients aged 1 day to 15 years underwent surgery for symptomatic left ventricular inflow obstruction. Four diagnostic groups were identified: cor triatriatum (6 patients), supravalvar mitral membrane (SVMM) with a normal mitral valve (7 patients), SVMM with an abnormal mitral valve (9 patients) and mitral stenosis (17 patients). Associated cardiac anomalies occurred in 26 patients (67%). There were 8 deaths (21%), 3 in patients with SVMM and an abnormal mitral valve and 5 in patients with mitral stenosis. Survival for patients with normal mitral valves was significantly better than that for patients with abnormal mitral valves (13/13 vs 18/26, P less than 0.05). There was also high morbidity in patients requiring prosthetic mitral valve replacement. These data suggest that the outcome of surgical treatment for left ventricular inflow obstruction may be predicted according to the site of the obstruction. This is best determined preoperatively by cross-sectional echocardiography which allows optimal planning of surgical strategy.

Research paper thumbnail of Pulmonary artery sling

Pulmonary artery sling

The Annals of Thoracic Surgery, Nov 1, 1992

ABSTRACT

Research paper thumbnail of Tricuspid valve abnormalities in patients with atrioventricular discordance: Surgical implications

The Annals of Thoracic Surgery, Apr 1, 1994

From 1975 to 1990,90 patients (age, 6 months to 30 years; mean, 9.1 years) underwent operation fo... more From 1975 to 1990,90 patients (age, 6 months to 30 years; mean, 9.1 years) underwent operation for defects associated with atrioventricular discordance. Twenty-one had an anatomically abnormal tricuspid (systemic) atrioventricular valve (SAVV) of the following types: Ebstein, 11; straddling, 6; and dysplastic, 4. Sixteen valves were regurgitant: regurgitation was trivial to mild in nine and moderate to severe in seven. Two patients underwent a successful Fontan-type operation. None of the 5 patients with a competent SAVV underwent valve repair or replacement; 1 of these patients died. A ventricular septal defect was closed in 14 and an extracardiac valved conduit was placed in 7. Sixteen had a regurgitant valve: it was replaced in 10 and repaired in 2 (early mortality, he results from the surgical treatment of lesions asso-T ciated with atrioventricular (AV) discordance have improved in recent years [14]. Techniques for preventing injury to the conduction tissue during ventricular septal defect (VSD) closure [5-71 and for conduit placement and replacement [8, 91 have all been well described. The tricuspid valve functions as a systemic AV valve (SAVV) in these patients. It is of interest that the results of operation remain much less satisfactory in patients with either anatomically or functionally abnormal SAVVs [ 1, 41. We have reviewed our experience in 90 patients who underwent intracardiac repairs of anomalies associated with AV discordance [lo]. In that study, 20 variables were analyzed statistically, and the most significant risk factor for early death was an anatomically abnormal SAVV (p < 0.0001) (Table 1). Less than optimal results in patients with an abnormal SAVV contrasted strongly with the excellent results achieved in patients with a normal SAVV (Table 2). This report analyzes in greater detail a subgroup of 21 patients with AV discordance associated with a morphologically abnormal SAW. Material and Methods Definitions A discordant AV connection is a cardiac anomaly in which the right atrium connects to a morphologically left ventri

Research paper thumbnail of Intracardiac repair of lesions associated with atrioventricular discordance

European Journal of Cardio-Thoracic Surgery, 1996

Objective. Discordant atrioventricular (AV) connection is a rare congenital cardiac anomaly. Asso... more Objective. Discordant atrioventricular (AV) connection is a rare congenital cardiac anomaly. Associated cardiac defects modify the physiology, clinical presentation, and surgical management of these patients. We have reviewed our overall experience with 90 patients operated for lesions associated with AV discordance between 1975-1990. Methods_ 90 patients, consecutively

Research paper thumbnail of Expanded PTFE Membrane to Prevent Cardiac Injury During Resternotomy for Congenital Heart Disease

The Annals of Thoracic Surgery, Dec 1, 1996

Background. Resternotomy for repair of congenital cardiac defects can result in cardiacinjurj, Cl... more Background. Resternotomy for repair of congenital cardiac defects can result in cardiacinjurj, Closureof the pericardiumduring the initial operationmay prevent thia,and severalpericardialsubstituteshavebeentried, with variableresults,in patientsin whomprimarypericardial closure is not possible.We conducteda multicenter obsewationalstudy of the use of the expanded polytetrafluoroethylene membrane(PrecludePericardial Membrane, formerlycalledthe Gore-TexSurgicalMembrane; W. L. Gore & Associates, Flagstatl, AZ) in patients likely to undergo reoperation for treatment of congenital heart disease. Methods. Data were collectedretrospectivelyon all patientsin whomthe expandedpolytetrafluoroethylene s ince its popularization by Julianand associates[1]in 1957, the median sternotomy has become the most commonly used incision in cardiac surgery. Moreover, because the number of patients with congenital heart defects requiring reoperation is growing the need for a second and even a third or fourth stemotomy is also increasing. The risks involved in reoperative stemotomy are welj known [2-5]. Pericardial and mediastinal adhesions can predispose the heart to iatrogenic injury during reopening of the sternum by making it difficult for the surgeon to identify anatomic features during dissection. The sequelae of such an injury include catastrophic bleedin~decreased right ventricular function, and death. Because primary closure of the pericardium is not always possible, various materials have been suggested for use as a pericardial substitute to decrease adhesion formation and the potential for cardiac injury at reoperation. These include silicone, bovine pericardium, and expanded polytetrafluoroethylene (ePTFE) [6-10]. The O.1-mm-thick low-porosity ePTFE membrane (Preclude Pericardial Membrane, formerly called the Gore-Tex Surgical Membrane; W. L. Gore & Associates, Flagstaff, AZ) was initially found to reduce pericardial adhesion formation in animal models [11]. Subsequent reports described favorable results with the device in Acceptedfor publication June2S,19%.

Research paper thumbnail of Surgical treatment of aortic coarctation in infants younger than three months: 1985 to 1990. Success of extended end-to-end arch aortoplasty

PubMed, 1994

There remains controversy regarding the appropriate surgical treatment of coarctation of the aort... more There remains controversy regarding the appropriate surgical treatment of coarctation of the aorta in infants. In 1985 we introduced the extended end-to-end repair into our practice and now wish to present a review of our recent experience. One hundred fifty-one infants younger than 3 months of age underwent repair of coarctation between 1985 and 1990. In 25% and 33% of the patients, there was hypoplasia of the isthmus and of the transverse arch, respectively. Surgical procedures were as follows: subclavian flap angioplasty in 15 patients, resection with a traditional end-to-end anastomosis in 43, and resection with an extended end-to-end anastomosis into the arch in 77. In 30 patients, the extension was proximal to the origin of the left carotid artery (radically extended end-to-end anastomosis). Other procedures were used in 16 patients. Mortality (13 early and 12 late deaths) was related on multivariate analysis to the presence of an associated major heart defect, preoperative resuscitation, and direct postoperative gradient over the arch. This immediate postoperative gradient was significantly lower after both extended and radically extended end-to-end anastomosis if there was a hypoplastic isthmus, and after radically extended end-to-end anastomosis if the transverse arch was hypoplastic. Actuarial freedom from recoarctation at 4 years was 57% (confidence limits 28% to 78%) after subclavian flap angioplasty, 77% (confidence limits 60% to 87%) after end-to-end anastomosis, 83% (confidence limits 66% to 92%) after extended end-to-end anastomosis and 96% (confidence limits 77% to 100%) after radically extended end-to-end anastomosis. We conclude that the extended end-to-end anastomosis and radical end-to-end anastomosis appear to offer the best prognosis for all infants with coarctation. The technique can be applied successfully to almost all types of arch anomalies.

Research paper thumbnail of Surgical treatment of aortic coarctation in infants younger than three months: 1985 to 1990

The Journal of Thoracic and Cardiovascular Surgery, 1994

There remains controversy regarding the appropriate surgical treatment of coarctation of the aort... more There remains controversy regarding the appropriate surgical treatment of coarctation of the aorta in infants. In 1985 we introduced the extended end-to-end repair into our practice and now wish to present a review of our recent experience. One hundred fifty-one infants younger than 3 months of age underwent repair of coarctation between 1985 and 1990. In 25 % and 33% of the patients, there was hypoplasia of the isthmus and of the transverse arch, respectively. Surgical procedures were as follows: subclavian flap angioplasty in 15 patients, resection with a traditional end-to-end anastomosis in 43, and resection with an extended end-to-end anastomosis into the arch in 77. In 30 patients, the extension was proximal to the origin of the left carotid artery (radicaUy extended end-to-end anastomosis). Other procedures were used in 16 patients. Mortality (13 early and 12 late deaths) was related on multivariate analysis to the presence of an associated major heart defect, preoperative resuscitation, and direct postoperative gradient over the arch. This immediate postoperative gradient was significantly lower after both extended and radically extended end-to-end anastomosis if there was a hypoplastic isthmus, and after radically extended end-to-end anastomosis if the transverse arch was hypoplastic. Actuarial freedom from recoarctation at 4 years was 57 % (confidence limits 28 % to 78 %) after subclavian flap angioplasty, 77% (confidence limits 60% to 87%) after end-to-end anastomosis, 83% (confidence limits 66 % to 92 %) after extended end-to-end anastomosis and 96 % (confidence limits 77% to 100%) after radically extended end-to-end anastomosis. We conclude that the extended end-to-end anastomosis and radical end-to-end anastomosis appear to offer the best prognosis for aU infants with coarctation. The technique can be applied successfuUy to almost aU types of arch anomalies.

Research paper thumbnail of Allografts in the Rastelli procedure: Techniques

Allografts in the Rastelli procedure: Techniques

Steinkopff eBooks, 1988

Children with transposition of the great arteries (TGA) (VA discordance), ventricular septal defe... more Children with transposition of the great arteries (TGA) (VA discordance), ventricular septal defect (VSD) and anatomical left ventricular outflow tract obstruction (LVOTO) used to be treated by the Mustard operation, closure of the VSD and surgical relief of the obstruction. This carried out a mortality in excess of 50% (1, 2), largely due to the difficulty in relieving the LVOTO. The prognosis of children with this complex of disorders was improved by the application of the Rastelli operation (3). This operation consists in enlargement of the VSD, diversion of the LV outflow to the aorta by an intraventricular tunnel/patch and connection of right ventricle (RV) to pulmonary artery (PA) by an external valved conduit (Figs. 1–3). Details of the operative technique we use have been published elsewhere (4, 5).

Research paper thumbnail of Unusual vascular ring in infant with pulmonary atresia and ventricular septal defect

Heart, Aug 1, 1982

A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syn... more A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.

Research paper thumbnail of Modified Blalock-Taussig shunt

Modified Blalock-Taussig shunt

The Journal of Thoracic and Cardiovascular Surgery, 1981

Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried ... more Between April, 1975, and December, 1979, 99 modified Blalock-Taussig shunts (MBTSs) were carried out at The Hospital for Sick Children, Great Ormond Street. The operation consists of interposing between the subclavian artery and the pulmonary artery a prosthesis of greater diameter than that of the subclavian artery. The first 13 operations were performed with a prosthesis of woven Dacron. Conduits of expanded polytetrafluorethylene (PTFE) were used for 86 MBTSs, and these form the basis of this report. Forty-four (51.1%) shunts were performed in infancy, 25 in patients under the age of 1 month (29.0%). Prostheses of 4 mm were used in 34 cases, 5 mm in 14, and 6 mm in 38. There were five shunt failures (5.8%) documented at postmortem or angiocardiographic investigation. Including two late deaths for which postmortem examinations were not obtained, the overall failure rate was 8.1% (seven patients). All these patients were operated upon in infancy, four of them in the neonatal period. Although a longer follow-up is necessary to assess the validity of these shunts, the early results are encouraging. We believe we can now recommend MBTS as an alternative when the classical Blalock-Taussig shunt is considered unsuitable.

Research paper thumbnail of Utilização de valvas homólogas e heterólogas em condutos extracardíacos The use of homograph and heterograph valves in extracardiac conduits

DOAJ (DOAJ: Directory of Open Access Journals), Aug 1, 1988

RESUMO: O conceito do uso de um conduto extracardíaco para estabelecer uma via de saída, conectan... more RESUMO: O conceito do uso de um conduto extracardíaco para estabelecer uma via de saída, conectando o ventrículo direito com o tronco pulmonar, ou seus ramos, foi desenvolvido na década de 60. Entre 1971 e 1986,335 pacientes receberam , no The Hospital for Sick Children, de Londres, condutos extracardíacos para o lado direito do coração; 176 destes foram homoenxertos aórticos, preservados em solução antibióticonutriente; 140 heteroenxertos (Hancock, Ross, Carpentier-Edwards, lonescu-Shiley e 19 tubos não valvulados. Estes condutos foram usados na correção de defeitos cardíacos complexos. A idade média foi de 6,34 anos e o peso médio, de 17,8 kg. O diãmetro interno dos condutos variou de 8 a 30 mm. A mortalidade hospitalar foi de 29 ,2% e o seguimento dos sobrevivente teve uma duração máxima de 14,3 anos, sendo que apenas 40% delas foram relacionadas ao conduto extracardíaco. A curva atuarial, livre de obstrução, dos condutos extracardíacos foi significativa, quando se analisaram os homoenxertos, face a cada grupo de heteroenxertos (p < 0,005). Os fatores de risco mais importantes foram : o número de complicações pós-operatórias, para óbitos tardios ; a severidade das lesões associadas, para as reoperações ;. a data da cirurgia, para a troca do conduto extracardíaco, e a severidade das lesões associadas, para a obstrução. Assim, conclui-se que, a longo prazo, o uso de condutos valvulados extracardíacos tem bons resultados , especialmente no que se refere aos homoenxertos. Pelos dados apresentados, os homoenxertos aórticos, preservados em solução antibiótico-nutriente, continuam a ser nossa primeira escolha na reconstrução da via de saída ventrículo-pulmonar. DESCRITORES : condutos extracardíacos, cirurgia; valvas cardíacas, cirurgia; próteses valvulares cardíacas, cirurgia. INTRODUÇÃO O conceito do uso de condutos extracardíacos (CE) na correção de defeitos cardíacos congêntos. caracterizados pela descontinuidade entre o ventrículo direito. ou átrio direito. e o tronco pulmonar. ou os seus ramos, foi estabelecido com o trabalho experimental de ROB-BARD & WAGNER 20, em 1949. Porém, somente na década de 60 iniciaram-se as primeiras cirurgias. quando RASTELLI et a/Ii 19 confeccionaram um conduto não valvulado de pericárdio. para estabelecer a continuidade entre o ventrículo direito e o tronco pulmonar. numa forma complexa de tétrade de Fallot; ROSS & SOMERVILLE 2 1. 2 anos após. descreveram o primeiro uso de homoenxertos aórticos na correção total da atresia pulmonar com' defeito do septo ventricular. Trabalho realizado no Hospital for Sick Children. Londres , Inglaterra. Desde então. o uso de CE tem-se popularizado e várias são as instituições 4. 7. 8. 10. 16 que apresentam

Research paper thumbnail of Myocardial adenine nucleotide metabolism in pediatric patients during hypothermic cardioplegic arrest and normothermic ischemia

The Annals of Thoracic Surgery, May 1, 1989

Quantitative assessment of high-energy phosphate levels, including degradation or utilization dur... more Quantitative assessment of high-energy phosphate levels, including degradation or utilization during ischemia, has not previously been performed in infants and children. Animal experiments suggest that high-energy phosphate metabolism varies with maturation. To help answer these questions, 24 patients aged 2 months to 8 years underwent myocardial biopsy immediately after the institution of cardiopulmonary bypass (16 to 20°C). Additional samples were obtained at 16 and 45 minutes after aortic cross-clamping and administration of cardioplegia (St. Thomas's solution) (in vivo ischemia). Seven patients also underwent major myocardial resection. Resected specimens were placed in a 37°C bath and divided into equal-sized samples that were removed at ten-minute intervals (in vitro ischemia). All samples were immersed in liquid nitrogen and analyzed for adenine nucleotide pool metabolites using highperformance liquid chromatography. Levels of adenosine triphosphate were high before cross-clamping but uring the past decade, major advances have been D made that extend fundamental observations in myocardial metabolism in such a way as to bring new knowledge into direct clinical application [ 11. Controlled circumstances in a bloodless field with unimpaired visualization directly affect the technical performance of cardiac surgical procedures, which must be reflected in results. This is especially true in congenital cardiac procedures, where the size of the field is small, technical demands considerable, and time limited. The price one pays for a bloodless, immobile operative field is myocardial ischemia. Despite extraordinary advances in cardiac surgery for infants and children with congenital heart defects, operative morbidity and mortality remain high for repair of many defects. Diminished ventricular function is a principal determinant of outcome in these patients [2, 31. An important correlate of myocardial function after ischemia is the level of adenosine triphosphate (ATP). The