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In order to avoid the aesthetic prejudice of median sternotomy in young children undergoing open heart surgery for isolated congenital heart disease, a right posterolateral thoracotomy was performed in 146 children aged 5 months to 14 years. The large majority (140/146, 96%) were atrial septal defects: 130 ostium secundum, 5 sinus venosus, 1 low septal defect and 4 ostium primum (partial endocardial cushion defect). Six children had isolated perimembranous ventricular septal defects. One patient died of a probable lesional pulmonary oedema. Minor complications were observed in 15 cases and one had to be reoperated for a residual shunt. With an average follow-up of 2.6 years, all survivors are asymptomatic. The scar is normal, without cheloid or thoracic deformation, invisible to the patient when he looks in a mirror. The use of this approach requires a very accurate anatomical diagnosis, especially with regards to systemic and pulmonary drainage. The presence of a left superior vena cava draining into the coronary sinus is a contra-indication. The right posterolateral thoracotomy is now the approach of choice in its standard indication, the repair of ostium secundum atrial septal defects with large shunts, in young girls under 10 years of age.

The aim of this study was to assess the results of mitral valvuloplasty for chronic asymptomatic or paucisymptomatic mitral regurgitation. Of 584 patients operated for chronic mitral regurgitation between January 1989 and December 1994, 175 were in NYHA Classes I and II and made up the study population. All had chronic grade 3 or 4/4 mitral regurgitation suitable for mitral valvuloplasty. The average follow-up was 34.3 months. Mitral valvuloplasty was performed in 174 patients, the other patient requiring mitral valve replacement. Three patients died (1.7%) and the actuarial 5 year survival was 98.2 +/- 1.0%. The probability of absence of reoperation and absence of thrombo-embolic complications at 5 years were 97 +/- 0.8% and 96.3 +/- 1.7% respectively. The residual regurgitation at Doppler echocardiography was minimal or absent in 94% of patients at the last follow-up control. The mean end-systolic and end-diastolic left ventricular dimensions decreased from 40.0 +/- 6.8 mm and 64.8 +/- 7.0 mm before surgery to 34.6 +/- 6.7 mm (p < 0.001) and 52.7 +/- 7.4 mm (p < 0.001) at the last control. The authors conclude that conservative mitral valve surgery for NYHA Classes I and II patients with chronic mitral regurgitation is feasible with a low risk and is associated with a significant reduction in ventricular volumes and stability of valvular continence at medium-term. When performed by teams trained in techniques of mitral valvuloplasty, these results suggest that surgery should be performed early.

The Physio-Carpentier-Edwards ring is a new prosthetic ring developed to allow mitral annuloplasty associating remodelling and flexibility of the native mitral annulus. The object of this study was to assess the feasibility and reliability of mitral valvuloplasty with the Physio ring. Between December 1992 and October 1995, 100 patients with an average age of 56.8 years suffering from mitral insufficiency underwent mitral valvuloplasty with a Physio ring. The mitral insufficiency was degenerative in 94% of cases. The degree of regurgitation was scored 3+ or 4/+4/+ in 94 patients. Mitral valve prolapse was observed in 83 patients. Mitral reconstruction was undertaken using Carpentier's techniques. One patient died in the immediate postoperative period. Two patients were reoperated for valve replacement because of systolic anterior motion (SAM). One patient had SAM which regressed with medical treatment. There were no deaths after the hospital period. There were no late reoperations of thrombo-embolic complications. The average follow-up period was 19 +/- 8 months; 77 patients were followed up for over 1 year and all underwent control Doppler echocardiography. Sixty-one patients had no residual mitral insufficiency: 15 patients had grade 1/4 regurgitation and one patient had grade 2/4 regurgitation. The average mitral valve surface area was 2.8 +/- 0.3 cm2. The average left ventricular end diastolic volume decreased from 186 +/- 59 cm3 before surgery to 129 +/- 37 cm3 at the last control (p < 0.001). The authors conclude that the Physio ring enables reliable and effective mitral valvuloplasty with excellent short term results. The benefits of the flexibility of the Physio ring remain to be evaluated by a randomised trial.

The authors report echocardiographic appearances of subaortic obstruction secondary to correction of double outlet right ventricle in 8 children reoperated between January 1994 and June 1996. The initial repair of the double outlet right ventricule was performed before 3 months of age in 6 cases. Forms with subaortic ventricular septal defects (4 cases) and those with ventricular septal defects not connected to the great arteries (2 cases) were treated by tunneling between the left ventricule and the aorta, and forms with subpulmonary ventricular septal defects (2 cases) by tunneling between the left ventricle and pulmonary artery and detransposition. Reoperation for subaortic obstruction was performed after 18 to 33 months. The instantaneous maximal gradient measured by Doppler echocardiography was high, ranging from 60 to 145 mmHg. The causes of the secondary subaortic obstruction were stenosis of the tunneling patch (n = 2), subaortic fibrous ring (n = 3), muscular septal hypertrophy (n = 1), antero-lateral muscular hypertrophy (n = 1), relics of tricuspid tissue inserted on the infundibular septum (n = 1). Subaortic obstruction was diagnosed in the echocardiographic subcostal views in all cases; the nature of the obstruction was determined in 6 of the 8 cases. The mechanism of obstruction by stenosis of the tunneling patch was only detected in 1 of the 2 cases. This study show, 2D echocardiography to be the investigation of choice for follow-up of operated double outlet right ventricle for the diagnosis of secondary subaortic obstruction.

Between January 1980 and September 1995, 175 children were operated for closure of isolated multiple ventricular septal defects (VSD) (mean age 20 +/- 8 months, mean weight 8.1 +/- 4.4 kg). Eighty-nine patients had pulmonary protection: pulmonary artery banding (n = 76) and pulmonary valvular stenosis (n = 13). The remainder had severe pulmonary hypertension (mean pulmonary systolic pressures: 75.7 +/- 20.5 mmHg). The surgical strategy was based on the site of the VSD and the ventricular dominance determined preoperatively or at surgery. The VSD were perimembranous in 141 cases, muscular in 175 cases, of the inlet septum in 19 cases and infundibular in 13 cases. A surgical approach through a right atriotomy was adequate for complete repair in 122 patients; a second approach was necessary by right (n = 35) or left ventriculotomy (n = 4) or both (n = 2) or via the pulmonary artery in 2 cases. The operative mortality was 7.4% (13 patients). The causes of death were: residual (VSD (n = 6), pulmonary hypertension (n = 2), ventricular hypoplasia (n = 2), myocardial infarction (n = 3). Of the 162 survivors, 33 had residual VSDs, 12 were reoperated once (n = 11) or twice (n = 1). The mortality was 54.5%. The patients were classified in two groups: Group I (n = 130), those operated before 1990, and Group II (n = 45), the ones operated afterwards. The low muscular VSDs and left ventriculotomy were risk factors for mortality in univariate analysis for Group I and the "Swiss Cheese" type for Group II.

Eighty-six children under one year of age with univentricular hearts associated with increased pulmonary flow underwent pulmonary artery banding as the first stage of palliative therapy. Fifteen patients also had treatment of coarctation of the aorta at the same time and an atrial septal defect was treated in 13 patients. Twenty-seven patients underwent a Fontan procedure secondarily. The mean follow-up was 42.6 +/- 45.5 months; 8 patients were lost to follow-up. Twenty-three patients died during the whole of the study period. The global actuarial survival rate was 69.6 +/- 5% at 5 years. The 3 year survival rate was 56 +/- 12% in patients with anatomical right ventricles compared with 74.4 +/- 5.7% and 69.5 +/- 7.3% at 3 and 5 years respectively in those with anatomical left ventricles (p < 0.01). The presence of coarctation of the aorta reduced the 5 year actuarial survival rate to 25.4 +/- 12.8% (p < 0.01). Subaortic stenosis either at the time of initial presentation or occurring during pulmonary banding was associated with a 5 year survival of 58.3 +/- 13.7% (p < 0.01). Uni- and multivariate analysis demonstrated poor prognostic risk factors. On univariate analysis, they were the residual mean pulmonary pressures after banding, coarctation of the aorta, subaortic stenosis and a restrictive atrial septal defect. Independent risk factors on multivariate analysis were the residual pulmonary pressures after banding, coarctation of the aorta, the necessity of operation in the neonatal period and the need for reoperation for reason other than for a cavopulmonary connection. The feasibility of a Fontan procedure was reduced when subaortic stenosis was observed at any time. Only 17.5% of patients were free of reoperation during this study for whom banding remained the only palliative procedure. In conclusion, a programme of cavopulmonary connection should be envisaged from the initial presentation of these patients. The different stages of treatment with this objective in mind should aim to preserve myocardial function and keep pulmonary resistances low. Therefore, pulmonary artery banding should be rejected in cases with coarctation of the aorta and/or subaortic stenosis. Similarly, early conversion with a Glenn type bidirectional anastomosis allows adaptation of myocardial function for a secondary total cavopulmonary connection.

The authors report the echocardiographic appearances of 27 cases of transposition of the great arteries (TGA) with ventricular septal defect (VSD) (including the Taussig-Bing malformation) complicated by displacement of one or both atrioventricular valves and/or chordae tendinae diagnosed between September 1985 and September 1994. An anatomical repair was performed in 21 cases and allowed accurate correlation of the echocardiographic and peroperative findings; a cavo-bipulmonary conduit or a palliative procedure was performed in 6 cases. The cases were classified in 3 groups: group I: 16 children with isolated tricuspid valve abnormalities; group II: 7 cases of mitral valve straddling; group III: 4 cases of displacement of both atrioventricular valves and/or their chordae. The cases of group I were divided into 3 subgroups: subgroup Ia with insertion of the tricuspid chordae on the infundibular septum (7 cases); subgroup Ib with isolated overriding of the tricuspid valve (2 cases); subgroup Ic straddling with or without overriding of the tricuspid valve (7 cases). In all three groups, there were 10 cases of straddling of the tricuspid and 11 cases of straddling of the mitral valve: 9 type A, 3 type B and 9 type C of Tabry's classification. Echocardiography misdiagnosed one case of type A straddling of the tricuspid valve for a type A overriding and straddling of the mitral valve. The ventricular septal defect was situated in the admission septum in the 10 cases of straddling of the tricuspid; in the 11 cases of straddling of the mitral valve the chordae passed through the superior anterior part of the interventricular septum.(ABSTRACT TRUNCATED AT 250 WORDS)

Fibromuscular dysplasia is a non-inflammatory disease unrelated to atherosclerosis of the small and medium sized arterial walls, which often affects the renal and carotid arteries and occurs mainly in women. The authors report a case of atypical coarctation of the thoracic aorta due to fibromuscular dysplasia confirmed histologically in a 27 year old man. Arteriography was performed because of hypertension and asymmetry of blood pressure measurements and showed irregular stenosis of the aortic isthmus and of the origin of the left subclavian artery. Surgery comprised resection of the pathological segment of the aorta which was replaced by a prosthetic tube with reimplantation of the left subclavian artery. Histological examination showed fibromuscular dysplasia in perimedial areas and diffusely throughout the media. To the author's knowledge this is the first case to be reported in the medical literature.

One hundred and five survivors after the 30th day of complete cure of transposition of the great arteries with intact ventricular septum between 1980 and 1985 were followed up. Fifty-four had an arterial switch (AS) in a single stage at an average age of 10 +/- 9 days and 51 had Senning's procedure (S) at an average age of 4 +/- 2.5 months. The average follow-up (97% of patients) was 5.8 +/- 1.1 years for the AS group and 9.3 +/- 2.3 years for the S group. The actuarial survival at 5 years was 100% in the AS group and 85.8% in the S group (p < 0.01) (8 late deaths). In the AS group, 3 patients were reoperated for stenosis of the pulmonary artery and, in the S group, 4 patients underwent 6 reoperations. All but 3 patients in the S group and all but 1 patient in the AS group are in functional Class I of the NYHA classification. Doppler echocardiographic studies have shown mild to severe dysfunction of the systemic ventricle in 2% of the AS group and 26% of the S group (p < 0.001). Holter monitoring, performed in 70% of patients in the S group showed sinus node dysfunction in 60% and sinus rhythm in 40% of cases. In conclusion, good functional results were observed at over 5 years in both groups. However, the absence of late mortality and the minimal incidence of systemic ventricular dysfunction in the AS group confirm the authors' choice of indication of arterial switch for the treatment of transposition of the great arteries with intact ventricular septum.

Five patients, 3 months to 13 year old with atresia (4) or stenosis (1) of the left coronary artery underwent myocardial revascularisation at Marie Lannelongue Hospital. The preoperative symptoms were dominated in one case by cardiac failure due to myocardial infarction and in the other cases by effort angina with syncope. The operative technique of revascularisation in one case was angioplasty with enlargement of the left main coronary artery with a patch and, in the others, bypass graft of the left coronary artery with the left internal mammary artery. All 5 patients survived surgery and are asymptomatic with a follow-up of 6 months to 7 years. Control coronary angiography confirmed the patency of the different procedures of myocardial revascularisation. In 3 children, exercise electrocardiography was normal. Myocardial revascularisation of a child with a congenital stenotic abnormality of the left coronary network is possible, the technique of which depends on the diameter of the left main stenosis when the vessel is absent or atresic, left internal mammary artery bypass graft is the only solution. When the left main coronary is stenosed but patent, direct reconstructive surgery is an interesting alternative.

Immediate and long-term results of the Fontan procedure depend on the value of the pulmonary vascular resistance. This parameter cannot always be quantified accurately during preoperative evaluation. Cavo-pulmonary bypass using an extra corporeal circulation allowed simulation a Fontan procedure in 5 patients, and prediction of the pressure changes in the pulmonary artery and the systemic atrium according to the pulmonary to systemic resistances ratio. This technique could be valuable in border line cases for deciding on the optimal therapeutic solution.

The authors report their experience of cardiac surgery in 9 carriers of the human immunodeficiency virus (HIV). Eight HIV seropositive patients underwent surgery under cardiopulmonary bypass for valve repair or replacement. Eight patients were asymptomatic with respect to their viral infection: one patient had generalised lymphadenopathy. The hospital mortality was 1/9 (11.1%). There was no infectious morbidity. Five patients are alive with an average follow-up of 29.6 months and in NYHA Stages I or II. One patient deteriorated and presented symptoms of an AIDS-related complex. These results suggest that if the operative indications are justified, cardiac surgery under cardiopulmonary bypass may be performed in asymptomatic HIV seropositive patients.