[Apoptotic deregulation of endothelial cells and intimal proliferation in pulmonary hypertension of congenital heart disease]. / Dysrégulation apoptotique des cellules endothéliales et prolifération intimale dans l'hypertension artérielle pulmonaire des cardiopathies congénitales.

OBJECTIVES: To assess the cellular and histological basis of irreversible pulmonary hypertension (PHT) in the clinical setting of congenital heart disease (CHD). BACKGROUND: Although many children with CHD develop pulmonary vascular disease, it is unclear why this complication is reversible after complete repair in some cases but irreversible in others. As failure of endothelial cell apoptosis might lead to intimal proliferation and lack of reversibility of PHT, we investigated this and other key markers of vasoactivity and angiogenesis, in subjects with PHT and CHD. METHODS: We assessed anti- and pro-apoptotic markers in vascular and perivascular cells in lung biopsies from 18 patients with CHD; 7 with reversible and 11 with irreversible PHT, and from 6 controls. Immunostaining for eNOS, VEGF and CD34 (markers of vasoactivity and neoangiogenesis) was also performed. RESULTS: The anti-apoptotic protein Bcl-2 was highly expressed by pulmonary endothelial cells in all cases of irreversible PHT but in no cases of reversible PHT, nor in controls (p<0.001). Intimal proliferation was present in 10/11 irreversible PHT cases but never observed in reversible PHT (p<0.001). Similarly, perivascular inflammatory T-cells expressed more anti-apoptotic proteins in irreversible PHT (p<0.01). Irreversible PHT cases were also more likely to show compensatory up-regulation of VEGF and new small vessel formation at the sites of native vessel stenosis or occlusion (p<0.001). CONCLUSION: Irreversible PHT is strongly associated with impaired endothelial cell apoptosis and anti-apoptotic signalling from perivascular inflammatory cells. These changes are associated with intimal proliferation and vessel narrowing and thereby may contribute to clinical outcomes associated with pulmonary hypertension. Markers of apoptosis and angiogenesis were assessed in lung biopsies of subjects with pulmonary hypertension (PHT) due to congenital heart disease (CHD). The anti-apoptotic protein Bcl-2 was strongly expressed by pulmonary endothelial cells in irreversible PHT (n=11) but never in reversible PHT (n=7) (p<0.01). Irreversible PHT was also associated with up-regulation of VEGF and new vessel formation around occluded native vessels (p<0.01).

Late coronary complications after arterial switch operation for transposition of great arteries. Clinical and therapeutic implications.

The arterial switch operation is the procedure of choice for correction of transposition of the great arteries. Although offering excellent long term results, this procedure is burdened by the risk of acute and subacute coronary occlusion. No guide-lines exist for the management of acute or chronic ischemia in this setting. We briefly review the literature and present the results of our institution.

Delayed surgery in pericardial teratoma with neonatal hydrops.

Timing of neonatal surgery in cases of pericardial teratoma with hydrops is not standardised. We report two cases of hydropic premature newborns with pericardial teratoma in which surgery was delayed until respiratory and haemodynamic stabilisation. Mature teratoma was removed on day 3. The newborns were weaned from the ventilator on postoperative day 5 and 10, respectively. Both infants were doing well at 18 months, suggesting delayed surgery may be feasible and effective.

[Endothelial vasoactive factors: predictive markers of the results of the Fontan intervention?]. / Facteurs vasoactifs endothéliaux: marqueurs prédictifs du résultat de l'intervention de Fontan?

UNLABELLED: We retrospectively analyzed the pulmonary biopsies of a Fontan intervention in order to identify predictive markers of its result. The pulmonary biopsies of 17 patients who benefited from the Fontan intervention and 6 controls were studied. The histomorphometric measurements and endothelial expression of vasoactive factors (NO synthase, endothelin-1) and angiogenic factors (VEGF) were analyzed with immunohistochemistry at the level of intra-acini pulmonary arterioles. Eight patients presented a good surgical result (group 1). Distal arterioles presented a thin and regular wall: the NO synthase, endothelin and VEGF expression was low. Nine patients presented a failure of the Fontan operation (group 2). The distal arterioles were slightly musculated (p<0.01 vs. group 1 and group control). The endothelin-1 expression was comparable to that observed in group 1. Conversely, the NO synthase and VEGF expressions were significantly increased in that group (<0.001 vs. group 1 and group control). CONCLUSION: In case of Fontan operation failure, the histological modifications are subtle with a muscular extension of the distal pulmonary arterioles wall. Conversely, a marked increase of NO synthase and VEGF expression is observed which can witness of an endothelial dysfunction. This study could be able to define predictive factors of the evolution and refine the selection of patients candidate for a Fontan operation.

Surgical reconstruction of occluded pulmonary arteries in patients with congenital heart disease: effects on pulmonary artery growth.

BACKGROUND: This study was undertaken to determine outcomes and best strategies for treatment of occluded pulmonary arteries in patients with congenital heart disease. METHODS AND RESULTS: Between 1998 and 2002, occlusion of a previously patent pulmonary artery was established in 23 patients. Data were obtained retrospectively. Diagnoses were pulmonary atresia and ventricular septal defect in 11, tetralogy of Fallot in 6, and other forms of pulmonary stenosis or atresia in 6. Median age and weight at diagnosis were 9 years (range, 6 days to 43 years) and 24 kg (range, 2.6 to 60 kg). Fourteen patients had had a previous surgery. The occluded pulmonary artery was visualized at angiography by wedge injection or injection into the collateral circulation. The left pulmonary artery was occluded in 20 patients and the right pulmonary artery in 3. Criteria for reconstruction were estimated duration of occlusion <6 months and ratio of occluded to contralateral artery >0.2. Twelve patients fulfilled these criteria and underwent pulmonary artery reconstruction at a mean interval of 2 months (range, 6 days to 6 months) from evidence of occlusion. Six patients had pericardial patch reconstruction, 3 terminoterminal anastomosis, 2 thrombectomy, and 1 a Blalock-Taussig shunt. There was 1 late death. At a median follow-up of 4 years (2 months to 5 years), all patients underwent cardiac catheterization: in 8 patients the reconstructed artery was patent, in 3 reoccluded. Hypoplasia of the occluded artery was reversed in 6 patients. CONCLUSIONS: Our data show that in selected patients, reconstruction of an occluded pulmonary artery can restore pulmonary vascularization and reverse hypoplasia. Strict surveillance is mandatory to prevent pulmonary artery loss.

Pericardial teratoma complicated by hydrops: successful fetal therapy by thoracoamniotic shunting.

Pericardial teratoma is a potentially curable lesion that may become life threatening when it induces mediastinal compression and fetal hydrops. So far, cases with fetal hydrops have been managed by elective delivery or pericardial needle decompression. We report a case in which pericardial teratoma resulted in fetal hydrops. Following transpleural needling of the fetal pericardium at 29 weeks and 6 days, pericardial effusion decreased but hydrops persisted, while major unilateral pleural effusion appeared. A thoracoamniotic shunt was placed at 30 weeks and 5 days. Hydrops resolved, although incompletely. The baby was delivered at 32 weeks and was operated upon on day 3. This observation suggests that fetal hydrops associated with pericardial teratoma may improve following thoracoamniotic shunting. Fetal therapy may limit the risks of respiratory distress arising from the combined effect of airways compression and lung immaturity.

[Percutaneous pulmonary valve replacement: towards a modification of the prosthesis]. / Remplacement valvulaire pulmonaire par voie percutanée: vers une modification de la prothèse.

Percutaneous pulmonary valve replacement has recently been introduced as an alternative to surgery, pushing back the frontiers of interventional catheterisation. The authors report their experience in this field and focus on the complications of the method. From September 2000 to November 2002, 14 patients with a failing artificial pulmonary outflow tract were included in this protocol of clinical research. The authors attempted pulmonary implantation of a bovine jugular vein valve fitted in a vascular stent under general anaesthesia. The implantation was possible in all patients. Three had dynamic obstruction due to the implanted valve. This was due to infiltration of blood between the valve and the stent. The problem was resolved by: implanting a second valve in one patient: redialating the valve stent in two other patients. However, the last patient had to be reoperated 7 weeks after the implantation because of recurrence of the obstruction. One valve had to be explanted one year after implantation because of severe infectious endocarditis after unprotected dental treatment. Two patients experienced fracture of the stent but without valve dysfunction. Despite these complications, the clinical state of all patients improved during follow-up. The authors conclude that non-surgical pulmonary valve replacement is feasible, thereby increasing the therapeutic arsenal of interventional catheterisation. Simple modifications should result in fewer problems of dynamic obstruction and stent fracture, and lead to proposing this technique for native pulmonary outflow tracts.

Intrapericardial teratoma in newborn babies.

We report two cases of intra pericardial tumor with pericardial effusion, diagnosed in utero by echocardiography at 21 and 28 weeks of gestation. Both fetuses underwent an intra uterine pericardiocentesis to treat a hydrops fetalis. Surgical resection of the tumor was undertaken immediately after birth and histological description reported cystic teratoma. Both babies had a favorable post operative course.

[Surgery for congenital heart disease in the adult: characteristics]. / La chirurgie des cardiopathies congénitales de l'adulte: particularités.

Surgery for congenital cardiopathy in adulthood presents at least four distinctive characteristics: rarity (less than 1% of all cardiac surgical procedures), extreme diversity of anatomo-clinical situations, the customary multivisceral disorder (pulmonary in particular) and certain specific technical differences (concerning the conduction of extracorporeal circulation and the surgical procedure itself). Because of these features, the operative indications are difficult to define in this population. They must be decided case by case, arriving at a well-tuned equilibrium between exaggerated intervention and excessive expectancy. Such an objective has the most chance of being realised in a specialised medico-surgical team.

[Followup of adults with a Fontan-type cavopulmonary derivation]. / Surveillance d'un adulte ayant eu une dérivation de type Fontan ou cavopulmonaire.

The Fontan procedure is a technique used in single ventricle syndromes and initiates a circulation which bypasses the right ventricle. It was described by Fontan in 1971. Many variations of this operation have been described and classified under the term of total cavopulmonary derivation. These operations result on long-term functional benefit. However, the 15 year survival rate is only 55 to 80%. This procedure (often carried out in childhood) is considered to be palliative rather than curative. The mortality is due to long-term complications, the frequency of which increases with the length of follow-up of the patient. The causes are: the Fontan circulation itself (arrhythmias, thrombosis, inferior caval stasis (hepatic, exsudative enteropathy), recurrence of cyanosis): the underlying cardiac disease (ventricular failure, atrioventricular valvular regurgitation); other complications (endocarditis and extracardiac, in particular orthopedic problems). Any of these complications may be an indication for cardiac transplantation, a high risk procedure because of previous multiple surgical interventions.

[Marfan syndrome in the newborn and infants less than 4 months: a series of 9 patients]. / Syndrome de Marfan chez le nouveau-né et le nourrisson de moins de 4 mois: une série de 9 patients.

We report a series of 9 children with neonatal Marfan syndrome. The diagnosis was made on a striking facial dysmorphia associated with arachnodactyly type skeletal anomalies. They all had cardiovascular anomalies: aortic dilatation 9 times, and mitral and/or tricuspid valve prolapse 8 times. Three children underwent ascending aorta replacement with the Yacoub technique. There was one operative death in a young infant and the two survivors required further surgery for gross mitral insufficiency and an aneurysm of the horizontal aorta. Three children died before the age of 1 year from cardiac insufficiency linked to massive leaks of all the valves. The three youngest children in the series did not undergo any procedure and were followed up for a slowly growing dilatation of the initial aorta. The prognosis of neonatal Marfan syndrome is weighted with a heavy mortality. The timing of surgical intervention in case of excessive dilatation of the aortic root is difficult to determine. It depends not only on the aortic diameter but also on the aortic valve function and the existence of other mitral or tricuspid lesions. Surgical treatment for lesions of the initial aorta and the atrioventricular valves improves the short-term prognosis but the risk of re-intervention is higher.

A new percutaneously adjustable, thoracoscopically implantable, pulmonary artery banding: an experimental study.

BACKGROUND: In patients who undergo left ventricular retraining, multiple reoperations are often necessary to adjust the pulmonary artery banding. The availability of a percutaneously adjustable band would be very useful. METHODS: Ten lambs (10 to 25 kg) underwent pulmonary artery banding using a new device, 7 by thoracotomy and 3 by thoracoscopy. The possibility of percutaneously adjusting the band was evaluated immediately after operation in 10 animals and at 3 months in 8 animals. RESULTS: One death occurred on the day of the procedure from displacement of the device and another death was from infection. Immediate hemodynamic studies proved the feasibility of increasing right ventricular afterload in a precise and reversible way. After 3 months the band could still be precisely loosened or tightened in all but 1 animal. Autopsy revealed that all the devices were in the correct position and no fibrosis or adhesions were present around the devices, and there was no residual stenosis noted on the pulmonary artery. CONCLUSIONS: This new device may be a valuable alternative to the repeated pulmonary artery banding needed for ventricular preparation.

Results of the arterial switch operation in neonates with transposed great arteries.

BACKGROUND: The arterial switch operation is judged the best palliative operation for neonates with transposed great arteries. We aimed to assess the value of analysing a large series of unselected cases by this technically demanding operation and formulate a realistic prognosis. METHODS: We reviewed all 432 neonates (mean age at operation 7 days, mean weight 3.25 kg) who underwent an arterial switch operation between 1987 and 1999. Follow-up (mean time: 4.9 years) was complete in 412 patients. FINDINGS: Survival probability and freedom from reoperation was 94% and 78% at 10 years, respectively. 26 patients died, 16 because of myocardial ischaemia. Risk factors for death included early experience, low weight, associated cardiovascular malformations (especially hypoplasia of the right ventricle or aortic arch), and difficult patterns of coronary arteries. The risk of the coronary artery pattern was greatly reduced in those who had recent operations. At last follow-up, 90% of patients had normal life without treatment, and 94% a normal heart function on echocardiography. INTERPRETATION: The arterial switch operation in neonates achieves excellent results mid-term. Obstruction of the translocated coronary arteries is responsible for most deaths and a substantial number of reoperations. Although confirmation is needed, these results allow anticipation of a favourable long-term prognosis.

Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction.

OBJECTIVE: Assessment of the Lecompte procedure, our repair method of choice in malposition of the great arteries with pulmonary stenosis. METHODS: A retrospective analysis of 42 patients (median age at operation, 1.4 years) operated on between 1986 and 1999 for various forms of great artery malposition, ventricular septal defect, and pulmonary stenosis. Relevant associated findings included the insertion of a tricuspid papillary muscle on the conal septum (nine patients), absence of conal septum (six patients), hypoplasia of a side pulmonary artery (four patients), and hypoplasia of the right ventricle (one patient). A preliminary systemico-pulmonary shunt was created in 28 patients and a cavo-pulmonary anastomosis in one patient. At operation, the conal septum (whenever present) was resected (36 patients), the pulmonary bifurcation was usually translocated over the ascending aorta (37 patients), and the main pulmonary artery was enlarged with a patch of pericardium. A monocusp valve was fashioned within the patch in 40 patients. The follow-up information was complete in 32 patients and ranged from 0.4 to 14 years (mean, 5.4+/-3.2 years). RESULTS: The survival rate at 5 years was 92+/-5%. Three patients died post-operatively (mortality, 7%) and none during follow-up. The freedom from reoperation was 86+/-8 and 51+/-22% at 5 and 10 years, respectively. Six patients were reoperated, all for a pulmonary stenosis. Calcification of the monocusp patch was present in all. Pulmonary stenosis developed in three further patients: one underwent percutaneous dilatation and two are awaiting surgery. No procedural factors thought to have a potential correlation with the development of right ventricular outflow tract stenosis could be individualized on univariate analysis. CONCLUSIONS: The Lecompte procedure, which allows early repair of these defects, provides overall good results. The need to reoperate on the right or left ventricular outflow tract seems reduced in comparison with the Rastelli operation, the other alternative.

Absorbable pulmonary artery banding in tricuspid atresia.

A child with tricuspid atresia, concordant ventriculoarterial connections, large ventricular septal defect, and elevated pulmonary artery pressure underwent pulmonary artery banding with a polydioxanone ribbon. This procedure was successful in this patient as the ventricular septal defect became restrictive while the banding was fully absorbed after 5 months. This technique could be included in the panel of surgical strategies for patients with single ventricle physiology and potential but insufficient subpulmonary stenosis in early infancy.

Tricuspid insufficiency after blunt chest trauma in a nine-year-old child.

The case of a traumatic tricuspid insufficiency in a child, due to an anterior and septal leaflet rupture at the annulus level is reported for the first time. The early diagnosis 2 months after the trauma enabled a rapid and simple tricuspid valvuloplasty by laeflet reinsertion on the annulus associated with annuloplasty with a good result 6 months after the repair.

A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band.

OBJECTIVES: We propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations. BACKGROUND: The alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery. METHODS: Eleven infants (mean weight 2,560 +/- 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 +/- 0.7 mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure. RESULTS: There were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients. CONCLUSIONS: Provided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.