Fetal aortic valvuloplasty: investigating institutional bias in surgical decision-making.

OBJECTIVES: Fetal aortic valvuloplasty may prevent the progression of aortic stenosis to hypoplastic left heart syndrome and allow biventricular rather than univentricular postnatal treatment. This study aimed to investigate whether blinded simulation of a multidisciplinary team approach aids interpretation of multicenter data to uncover institutional bias in postnatal decision-making following fetal cardiac intervention for aortic stenosis. METHODS: The study included 109 cases of prenatally diagnosed aortic stenosis from 13 European countries, of which 32 had undergone fetal cardiac intervention. The multidisciplinary team, blinded to fetal cardiac intervention, institutional location and postnatal treatment, retrospectively assigned a surgical pathway (biventricular or univentricular) based on a review of recorded postnatal imaging and clinical characteristics. The team's decisions were the numerical consensus of silent voting, with case review when a decision was split. Funnel plots showing concordance between the multidisciplinary team and the local team's surgical choice (first pathway) and with outcome (final pathway) were created. RESULTS: In 105 cases the multidisciplinary team reached a consensus decision regarding the surgical pathway, with no decision in four cases because the available imaging records were inadequate. Blinded multidisciplinary team consensus for the first pathway matched the decision of the surgical center in 93/105 (89%) cases, with no difference in agreement between those that had undergone successful fetal cardiac intervention (n = 32) and no (n = 74) or unsuccessful (n = 3) valvuloplasty (no fetal cardiac intervention) (κ = 0.73 (95% CI, 0.38-1.00) vs 0.74 (95% CI, 0.51-0.96)). However, funnel plots comparing multidisciplinary team individual decisions with those of the local teams displayed more discordance (meaning biventricular-univentricular conversion) for the final surgical pathway following fetal cardiac intervention than they did for cases without such intervention (36/74 vs 34/130; P = 0.002), and identified one outlying center. CONCLUSIONS: The use of a blinded multidisciplinary team to simulate decision-making and presentation of data in funnel plots may assist in the interpretation of data submitted to multicenter studies and permit the identification of outliers for further investigation. In the case of aortic stenosis, a high level of agreement was observed between the multidisciplinary team and the surgical centers, but one outlying center was identified.

[Extracorporeal membranous oxygenation in severe infant pertussis: a case report]. / L'oxygénation extracorporelle artérioveineuse est-elle utile dans le traitement de la coqueluche maligne ?

Pertussis is a leading cause of death from community infections in infant. Life-threatening clinical presentations of pertussis can associate multiple organ system failure with respiratory distress. The question of the optimal management of these severe forms of pertussis, in order to reduce the high mortality rate, is raised by the clinicians caring for such patients. We report the case of a 1 month infant who was admitted to the pediatric intensive care unit (PICU) for a severe pertussis. He presented with an acute respiratory distress syndrome, a severe pulmonary hypertension was treated initially with mechanical ventilation and nitric oxide. At day 4 (D4), a cardiogenic shock occurred and, despite epinephrine and norepinephrine infusion, fluid expansion, the hemodynamic condition worsened with two episodes of cardiac arrest. The child was then successfully resuscitated, and, facing the extreme hemodynamic instability, extracorporeal membrane oxygenation (ECMO) was considered. ECMO allowed epinephrine and norepinephrine to be progressively discontinued, and protective mechanical ventilation. ECMO withdrawal was possible at D9, with milrinone as the sole inotropic agent. Weaning from mechanical ventilation was possible on D15 and the total length of stay in PICU was 20days. While the analysis of the literature, through limited experiences on the use of ECMO in children with severe pertussis does not allow concluding definitively on the utility of ECMO in this situation, the contribution of ECMO in the favourable outcome for our patient was considerable. This is an argument, to our opinion, for considering ECMO in the management of those very instable patients.

Vacuum-assisted closure in the treatment of poststernotomy mediastinitis in the paediatric patient.

INTRODUCTION: Delayed sternal closure after paediatric open heart procedure is often necessary. The risk of delayed sternal closure is infection: superficial wound or sternal and mediastinal infection. The incidence of sternal wound infection reported in the literature varies from 0.5 to 10%. The mortality for poststernotomy deep sternal infection continues to be high--from 14 to 47%. Established treatment includes surgical debridement, drainage and irrigation, antibiotics, frequent change of wound dressing and direct or secondary closure with omentum or pectoral muscle flap. PATIENTS AND METHODS: Between October 2003 and August 2005, three children, aged from 9 days to 2 years and who had developed severe mediastinitis after cardiac surgery were treated with the vacuum-assisted closure (VAC) system. RESULTS: The duration of VAC treatment ranged from 12 to 21 days. The response to VAC was rapid with local purulence and C-reactive protein (CRP) both decreasing within 72 h in all cases. After good granulation was obtained, two patients required a thin skin graft. DISCUSSION: All three children had peritoneal dialysis which did not permit omental use. The use of pectoralis major is a difficult technique in neonates and the haemodynamic conditions were poor in our cases. The VAC technique is a good indication in post-cardiotomy mediastinitis in children: it plays a role in the reduction of infection and provides good healing.

[A monocenter experience of ventricular septal defects treated by catherization]. / Expérience monocentrique de la fermeture par cathétérisme interventionnel des communications interventriculaires.

INTRODUCTION: Studies on ventricular septal defects closure by catheterization confirm its feasibility without reporting clearly the indications and difficulties encountered. PATIENTS AND RESULTS: From 2001 to end-2006, 22 patients benefited from 26 ventricular septal defects closure (15 muscular and 7 membranous) at a median age and weight of 2.1 years and 12.5 kg, respectively. A perventricular catheterization was performed in 2 cases. Eighteen patients (82%) benefited from 21 prostheses with success. The closure was associated to surgery in 9 cases (41%) whereas it substituted surgery in the other 13 cases (59%). The median duration of the procedure was significantly longer in case of muscular ventricular septal defects (215 min (175-510) vs. 170 min (120-225), p=0.04). Major complications are reported in 5 cases out of 26 catheterization (19%), including one death related to conduction block, occurring after the implantation of two prostheses in a patient with aortopulmonary transposition. All other associated cardiac diseases have been corrected. A prosthetic emboli occurred in one case, 1.5 months after implantation. It had been retrieved by catheterization. Two patients died afterwards from non-procedure-related causes. After a median follow-up of 1.1 years, the 17 other patients remained asymptomatic. One child with a perimembranous prosthesis presents a paroxystic atrio-ventricular block. CONCLUSION: Even though indispensable for the curative treatment of several congenital cardiac diseases including non-operable ventricular septal defects, this procedure is related to a substantial rate of mortality and morbidity. The risk of atrio-ventricular block must be adequately considered in case of membranous ventricular septal defects.

[Long-term results of complete atrio-ventricular canal correction]. / Résultats à long terme du traitement chirurgical du canal atrioventriculaire complet.

OBJECTIVES: To assess the prognosis of surgically corrected complete atrio-ventricular canal and determine risk factors for death and redo surgery. PATIENTS: and method: From January 1984 to March 2006, 126 patients were distributed in 3 groups according to their date of intervention: group I (1984-1991), group II (1992-1999) and group III (2000-2006). They were operated at mean age of 8.35 + 13.3 months. Down syndrome was present in 99 patients (79%). A partial or total closure of the left atrio-ventricular valvular cleft was performed in 47 patients (37%). RESULTS: The in-hospital mortality was at 14.28% (18 patients), including 23.7% in group I, 16.2% in group II and 4.4% in group III (p=0.06). The predictive factors of survival were the Down syndrome (p<0.05) and surgery of the left atrio-ventricular valvular cleft (p=0.05). An early reoperation was required in 6 cases, for a severe leak of this valve (n=4) or an important residual shunt (n=2). After a mean follow-up of 9.5+6.9 years, the survival rate was at 83.6% and 10 patients (9.9%) had a redo surgery for a leak of the left atrio-ventricular valve (n=6) or sub-aortic stenosis (n=4). The rate of no-redo surgery for valve insufficiency was at 94.2% at 5 years, 91.1% at 10 years and 87.6% at 15 years. CONCLUSION: The surgical treatment of complete atrio-ventricular canal became more and more efficient with poor rates of mortality and redo surgery during these last years. The closure of the left atrio-ventricular valve cleft, mostly partial, is frequently performed. Patients with the Down syndrome have a better vital prognosis and lower rates of long-term redo surgery.

[Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results]. / Réhabilitation des artères pulmonaires hypoplasiques dans l'atrésie pulmonaire avec communication interventriculaire. Résultats à moyen terme.

The treatment of pulmonary atresia with ventricular septal defect, hypoplasia of the native pulmonary arteries and major aorto-pulmonary collaterals is controversial with a wide range of options from symptomatic treatment to surgical correction, but usually unifocal surgery. However, the collateral vessels used in the reconstruction are often tortuous and stenosed, resulting in a problematic haemodynamic result. The authors report the results of medico-surgical rehabilitation of the native pulmonary arteries in three stages. Since 1990, patients with an average Nakata index of 27.5 +/- 16.7 mm2/m2 underwent elective surgical connection of the native pulmonary arteries to the right ventricle (1st stage) followed by their rehabilitation with catheter occlusion of the collaterals (2nd stage) with the aim of preparing surgical correction (3rd stage). The first surgical stage at an median age of 5 months (0.1 to 25.2) was complicated by one death. After an average of 2.1 +/- 1 catheterisations, 17 patients underwent surgical correction at an median age of 2.18 (0.6 to 10.3) years with an average Nakata index of 207 +/- 91 mm2/m2. All survived surgery with three late deaths in patients with poor haemodynamic results (right ventricular/left ventricular pressure ratio>80%). After an median follow-up of 9.2 (3.5 to 17) years, the 14 survivors are in NYHA Class I or II with a good haemodynamic result in 10 patients. The authors conclude that despite some late deaths and raised right ventricular and pulmonary artery pressures in a minority of patients, the rehabilitation of the pulmonary arteries is an effective management of this cardiopathy.

[Medium and long-term results of anatomical correction of transposition of the great arteries in Marseille]. / Résultats à moyen et long terme de la correction anatomique de la transposition des gros vaisseaux à Marseille.

The object of this study was to evaluate the results of anatomical correction of transposition of the great arteries (TGA) in an inter-regional centre. The characteristics of 220 patients operated between 1985 and 2002 were analysed. Prenatal diagnosis of TGA was made in 38 patients (17%) including 36 of the 109 patients operated since 1995 (33%) and leading to earlier atrial septostomy in this situation (p= 0.046). Since 1995, the global early mortality of 12.3% has been significantly reduced to 5.5% (N= 6) including 4.4% (3/68 cases) of simple TGA (p= 0.002). In multivariate analysis, the only independent risk factor for early mortality was the occurrence of a major per-operative complication (p< 0.0001). The average follow-up was 4.8 +/- 4.5 years with an 86% survival at 5, 10 and 15 years and 97% survival at 5 years for patients operated after 1995. There were 22 reoperations including 14 on the right ventricular outflow tract. The non-reoperation rate at 10 years was 80% for simple TGA versus 70% in complex TGA (p= 0.0001). Survivors are asymptomatic with a normal ECG and normal LV function on echocardiography. Five patients have significant pulmonary stenosis and 4 have mild aortic regurgitation. The exercise stress tests performed between the ages of 7 and 10 were all normal. The authors conclude that that modern and reproducible management of TGA is possible in an inter-regional centre with results comparable to those of surgical centres of reference.

[Value of the Norwood-Sano compared with the classical Norwood procedure in hypoplastic left heart syndrome]. / Intérêt de l'intervention de Norwood-Sano par rapport à l'intervention classique de Norwood dans l'hypoplasie du coeur gauche.

Palliative treatment of the hypoplastic left heart syndrome is rarely practiced in France because of the poor prognosis of the first surgical stage of the Norwood procedure. The recent modification proposed by Sano seems to give more promising results. The authors report their experience with the Norwood-Sano procedure compared with the classical Norwood procedure in 13 neonates with hypoplasia of the left heart. From 1999 to 2005, 8 patients underwent the classical Norwood procedure (1999-2001) and 5 had the Sano modification (2002-2005). During the preoperative period, there was 1 case of a very restrictive interatrial septum and 5 patients required mechanical ventilation. The patients were operated at an average age of 8.5 +/- 12 days. The results showed that survival after the first surgical stage was significantly better with the Sano modification (12.5% versus 80%; p= 0.03). All patients who survived the first stage went on to the second stage with a bi-directional right superior cavo-pulmonary derivation at an average of 6.1 +/- 2.4 months. Despite the infundibulotomy of the Sano modification, no arrhythmia or right ventricular dysfunction was observed after an average follow-up of 21.7 +/- 7.6 months. The authors conclude that the Sano modification improved survival of patients with hypoplasia of the left heart after the first palliative surgical procedure. The long-term follow-up of patients operated by this technique should particularly look out for arrhythmias and right ventricular dysfunction.

[Unusual outcome of mitral valve infectious endocarditis in a premature infant]. / Evolution inhabituelle d'une endocardite infectieuse mitrale chez un prématuré.

The authors report a case of Staphylococcus Aureus infectious endocarditis in a premature baby. Echocardiography on the 8th day of life showed a large vegetation on the anterior mitral leaflet without significant regurgitation. Intravenous antibiotics and platelet antiaggregant therapy were given. At three weeks of age the vegetation split into two, one part attached to the foramen ovale and the other to the anterior mitral leaflet. At one month, the development of severe mitral regurgitation led to surgical valvuloplasty in a 2 kg neonate, the vegetation on the foramen ovale having spontaneously fragmented. The ablation of the mitral vegetation associated with commissuroplasty reduced the mitral regurgitation. At 3 months after surgery, the child was asymptomatic with a minimal mitral regurgitation.

[Endomyocardial fibrosis: a rare case of restrictive cardiomyopathy in a Caucasian female]. / Fibrose endomyocardique: une cause rare de cardiomyopathie restrictive chez une patiente caucasienne.

The authors report the case of endomyocardial fibrosis diagnosed in a young Caucasian female presenting with progressive congestive cardiac failure. The diagnosis was suspected on the echocardiographic, magnetic resonance imaging and cardiac catheterisation findings in association with the clinical presentation. After a short course of symptomatic medical therapy, the patient underwent the only curative treatment of this pathology, surgical endocardectomy and combined valvular surgery. The confirmation of the diagnosis was obtained a posteriori by histopathological examination of the operative findings which showed appearances of endomyocardial fibrosis similar to those observed in tropical regions. The patient was discharged on the eighth postoperative day, much improved clinically, and follow-up at one year was very satisfactory.

[Results of surgery for coarctation of the aorta in children under one year of age]. / Résultats de la chirurgie de la coarctation aortique chez l'enfant de moins de 1 an.

The aim of this study was to determine the surgical results of repair of coarcta tion of the aorta in children under 1 year of age and to assess the risk of recoarctation. Between 1984 and 2004, 206 children under 1 were operated for coarctation of the aorta. Three groups of patients were identified: Group I (isolated coarctation, N=99), Group II (associated ventricular septal defect, N=63), and Group III (complex congenital heart disease, N=44). The transverse aorta was hypoplastic in 51% of cases. Uni- and multivariate analysis of the risk factors for mortality and recoarctation was performed. An extended resection anastomosis was performed in 206 patients. Reconstruction of the transverse aorta was performed in 32 cases. The hospital mortality was 3.9% (N=8). The presence of a complex intracardiac anomaly was a risk factor for mortality on multivariate analysis (p=0.023). In univariate analysis, a two-step management of patients in Group III was a significant risk factor for mortality (p=0.036). Thirty patients (14.6%) had recoarctation (gradient > 20 mmHg, follow-up 40 +/- 44 months). On multivariate analysis the severity of the immediate postoperative gradient was the only risk factor for recoarctation. The authors conclude that surgery for coarctation of the aorta is associated with excellent results in children under 1 year of age. The prognosis is related to the presence of associated severe cardiac malformations. Survival seems to be better if treatment is carried out in a single stage in this group. The risk of recoarctation is related to the immediate postoperative transisthmic gradient which justifies extensive repair of hypoplastic aortic arches.

[Extracorporeal assistance in paediatric intensive care]. / Assistance extracorporelle en réanimation pédiatrique.

OBJECTIVE: For 3 years we have used extracorporeal assistance in intensive care frequently. This study evaluates our results. METHOD: We studied the patient records for those treated between January 2002 and January 2005. The method used, indications and morbidity/mortality were analysed. RESULTS: We performed 24 circulatory assistance procedures in 20 patients (median age: 5 months), arterio-venous with oxygenation (n=18), veino-venous with oxygenation (n=3) or biventricular (n=3). The indications were post cardiotomy cardio-respiratory failure (Group I; n=20, 16 patients), pure respiratory failure (Group II: n=1), or pre-transplant/recovery (Group III: n=3). Five procedures (4 from group I and 1 from group III) required cardiac massage (no fatalities). The average duration of assistance was 7 +/- 6 days (2 to 20 days). Treatment was successfully discontinued in sixteen patients 80%), one of them thanks to heart transplant. Four (20%) died during assistance. The morbidity essentially consisted of further surgery for haemostasis, multiple transfusions, and infections. Three patients (15%) died later (1 at 17 months after discontinuation) from complications unrelated to the assistance. No neurological sequelae were noted in the survivors. CONCLUSION: These results confirm the usefulness of circulatory assistance when medical treatment has failed, particularly in the post-operative period of paediatric cardiac surgery or while awaiting transplantation.

[Results of the surgery of the truncus arteriosus: present implications]. / Résultats de la chirurgie du tronc artériel commun: implications actuelles.

We report our results of truncus arteriosus surgical reparation in a period of 10 years. From January 1993 to November 2003, 17 patients were operated with a median age and weight of 66 days and 3.5 kg respectively. According to the Van Praagh classification there were 13 cases of type A1, 2 cases of type A2 and 2 cases of A4. The connection between the right ventricle and the pulmonary artery was performed by a homograft (n = 12), a Contegra tube (n = 1) or a Barbero-Marcial intervention (n = 4). In one case, a plasty of the truncus was performed. Patients were classified in two groups: group A for those operated between 1993 and 1997 (n = 8) and group B for those operated after 1997 (n = 9). Five patients died (29%) in the post-operative period, 4 in the group A (50%) and one in group B (11%). The mean duration of intensive care stay was 12.2 (+ 7.4) days. Statistical analysis did not reveal any difference between both groups, especially concerning post-operative treatment or prognosis. In the multivariate analysis, an age below 30 days was a risk factor of post-operative death (OR = 16.5, 95% CI = 1.09 - 250; p = 0.043). After a mean follow-up of 3.9 (+ 3.5) years, 2 patients required a redo intervention for replacement of the pulmonary artery homograft. All 12 suvivors are asymptomatic without any pulmonary hypertension. In conclusion, the results of surgical reparation of the truncus arteriosus seem to improve with experience. According to recent progresses in surgery and intensive care, the intervention can be scheduled beyond the neonatal period without additive risk and with potentially less consecutive redo interventions.

[Medico-economic study of percutaneous and surgical closure of ostium secundum type interatrial communications]. / Etude médico-économique de la fermeture percutanée et chirurgicale des communications interauriculaires de type ostium secundum.

The aim of this study was the medico-economic evaluation of the closure of ostium secundum type interatrial communications in 32 patients, treated by cardiac catheterisation (group A: n = 17) or by surgery (group B: n = 15). The success rate was 100% with the 2 techniques. With national cost scale data we evaluated the overall cost of the 2 treatments using the information system medical program, starting from homogenous groups of patients: 150 catheterisation, 169 and 170 surgery. As the value for the homogenous group of 150 patients was much lower than the price of the material used for catheterisation, an adjusted group of 150 patients including the price of this material was created. An evaluation in terms of an artificial index of activity was also performed. The average duration of hospital stay for group A was reduced by 6.1 days compared to group B (p < 0.001). The overall cost was lower in group A than in group B (p < 0.001), using the real and "adjusted" homogenous group of 150 patients, with a respective reduction of 7,582 Euros and 3,731 Euros. Surgery and catheterisation scored 8,167 points (17,756 Euros) and 2,726 points (5,926 Euros) per patient respectively on the artificial activity index. In conclusion, catheterisation reduced the duration of hospital stay and brought an economic benefit for the Assurance Maladie compared to surgery. However, surgery is more profitable for the hospital than catheterisation because of the high cost of the prosthesis, which is not taken into account with a homogenous group of non-specific patients. This could hamper the development of this innovative technique in a hospital subjected to overall budgetary constraints.

[Case history of mitochondrial cytopathy with cardiac expression]. / A propos d'une cytopathie mitochondriale d'expression cardiaque.

Childhood dilated cardiomyopathies comprise a wide aetiological spectrum for which the prognosis and treatment sometimes vary considerably. We report the case of a patient affected by a rare form of mitochondrial cardiomyopathy in whom the diagnosis of acute myocarditis had initially been made. The progression was fatal even though the patient was awaiting a cardiac transplant. Beyond the difficulties of diagnosis and treatment of this pathology, this clinical case underlines the significance of an early aetiological diagnosis based on the results of an endo-myocardial biopsy. Cardiac transplantation should be envisaged for this type of patient based not only on the clinical status but equally by taking account of the prognosis of this disease for which deterioration can sometimes be very rapid.

[Dextrocardia with situ solitus, ventricular loop, double outlet hypoplastic right ventricle and L-malposition of the great arteries. Description and surgical treatment of a rare and complex cardiopathy]. / Dextrocardie avec situs solitus, coeur croisé, ventricule droit hypoplasique à double issue et L-malposition des gros vaisseaux. Description et traitement chirurgical d'une cardiopathie rare et complexe.

Despite the cardiac surgery improvements allowing the correction of the majority of congenital heart diseases with ventricle-great vessels discontinuity, some abnormalities increase the risk of bi-ventricular reparation. We herein report the case of a patient presenting a rare form of double outlet right ventricle with a ventricular loop, with moderate right ventricle hypoplasia. L-malposition of great vessels and pulmonary artery stenosis, and for whom we opted for a palliative surgical treatment including a systemic-pulmonary anastomosis followed by a upper right bi-directional cavo-pulmonary derivation. The last surgery was followed by recurrent right pleural effusions disappearing after the embolization of the systemic-pulmonary anastomosis by catheterism as it probably obstructed the draining of the cavo-pulmonary anastomosis. The relevance of this clinical case reported is, firstly the description of this ventricle loop resulted from a marked ventricular malposition which is a rare heart disease, and secondly the discussion about the surgical treatment, especially about the choice between palliative and curative surgery. Only comparative studies on long term morbidity and mortality between the bi-ventricular reparation and mono-ventricular palliation will allow the selection of the most appropriate surgical treatment.

Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals.

OBJECTIVE: Among 63 patients with pulmonary atresia and ventricular septal defect (VSD), 10 patients with extreme hypoplasia of the pulmonary arteries (PA) (mean Nakata index 20.6 mm(2)/m(2)), but with confluent arteries and a diminutive main PA, and major aorto-pulmonary collaterals (MAPCAS), have been submitted to a 'rehabilitation' of the PA with several stages: (i) connection between RV and PAs, (ii) interventional catheterizations, (iii) complete correction with or without unifocalisation. We report here the results of this approach. METHODS: The RV-PA connection was direct (nine cases) or with an homograft conduit (one case), done under normothermic cardiopulmonary by-pass in patients aged 4.9 months (range 0.1-18 months). Subsequently, six underwent interventional catheterizations (dilations and stents in the PA, MAPCAS occlusion by coils). Complete correction was done in seven patients (mean age 30 months, range 8-49). One patient is awaiting correction. RESULTS: One patient died after the first stage. All patients having had the third stage had a satisfactory development of the PA, had a complete closure of the VSD and a satisfactory reconstruction of the PA bifurcation. There was one death of severe pulmonary infection 6 months after repair. All other patients have been followed by catheterization and/or echocardiograms. With a follow-up of 83+/-65 months, all patients are improved, 50% have no cardiac medications, none has residual shunt, RV/LV pressure ratio is 0.6 (range 0.3-1). CONCLUSIONS: The strategy of 'rehabilitation' of PA allowing: (i) antegrade flow in the PA, (ii) interventional catheterizations, (iii) growth of the PA with possible angiogenesis, (iv) complete correction, is a logical approach to be undertaken in the young patient and is a valid alternative to strategies relying more on MAPCAS for pulmonary vascular supply. The therapeutic sequences depend upon the individual anatomy.

[Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication]. / Réhabilitation des artères pulmonaires hypoplasiques et correction anatomique des atrésies pulmonaires avec communication interventriculaire.

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.

Is lung cancer surgery justified in patients with direct mediastinal invasion?

OBJECTIVE: To assess the results of the surgical treatment of patients with stage IIIB non-small cell lung carcinoma (NSCLC) invading the mediastinum (T4). METHODS: Twenty-nine patients were operated on from 1986 to 1999. Histology was squamous cell carcinoma in 17 patients, adenocarcinoma in eight, large cell carcinoma in two and neuroendocrinal carcinoma in two. Three patients received a preoperative chemotherapy (n = 2) or radiochemotherapy (n = 1). The lung resection consisted of a pneumonectomy in 25 patients and a lobectomy in four. The procedure was extended to one of the following structures: superior vena cava (SVC) (n = 17), aorta (n = 1), left atrium (n = 5) and carina (n = 6). Seventeen patients had a postoperative regimen including radiochemotherapy (n = 12), radiotherapy (n = 4), or chemotherapy (n = 1). RESULTS: Complete R0 resection was achieved in 25 patients, whereas four patients had a microscopically (n = 1) or macroscopically (n = 3) residual disease. The operative mortality rate was 7% (n = 2). Non-fatal major complications occurred in eight patients (28%). Overall 5-year survival rate was 28% (median 11 months), including the operative mortality. The median survival of the 18 patients with an N0 or N1 disease was 16 months whereas the median survival of the 11 patients with an N2 disease was 9 months. At completion of the study, 22 patients have died, two postoperatively and 10 from pulmonary causes without evidence of cancer. CONCLUSIONS: Surgical management of T4 NSC lung cancer invading the mediastinum should be considered, in the absence of N2 disease, when a complete resection is achievable.

Surgical management of metachronous bronchial carcinoma.

OBJECTIVE: To assess the results of surgery for the treatment of metachronous bronchial carcinoma. METHODS: From 1985 to 1999, 38 patients were operated on for a metachronous lung carcinoma, accordingly to the criteria of Martini. All tumors were staged using the new International Classification System revised in 1997. RESULTS: Diagnosis of the second cancer was done at radiological follow-up in 30 asymptomatic patients. Seventeen metachronous locations were ipsilateral. Histology of the metachronous lesion was the same as that of the first tumour in 23 patients (60%). The first resection was a lobectomy (n=35), a pneumonectomy (n=2) and a carinal resection (n=1). The second one was a wedge resection (n=7), a segmentectomy (n=3), a lingulectomy (n=2), a lobectomy (n=9), a bilobectomy (n=1), and a pneumonectomy (n=16). There were five in-hospital deaths (13%). Completion pneumonectomy was performed in 15 patients, with one postoperative death (7%). The overall estimated 5 and 10-years actuarial survival rates from the treatment of the first cancer were 70 and 47% respectively. The 5-year survival rate after the treatment of the second cancer was 32% (median survival: 31 months), including the operative mortality. Survival was negatively affected by a resection interval of less than 2 years and the performance of atypical lung sparing pulmonary resection for the treatment of the second cancer. CONCLUSIONS: Good long-term results are achievable by the means of a second pulmonary resection in selected patients with metachronous lung cancer. Optimal cancer operations should be applied whenever functionally possible.