[Surgical treatment of pulmonary atresia with ventricular septal defect]. / Le traitement chirurgical des atresies pulmonaires a septum ouvert.

Pulmonary atresia with ventricular septal defect (PAVSD) is a complex cardiopathy represented by a complete obstruction between the right ventricle outflow and the pulmonary trunk associated with a ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA). The goal of the unifocalization in the PAVSD is to prepare the pulmonary tree for the complete repair by connecting the MAPCAs to the central pulmonary arteries that should be enlarged. After that we can made the VSD or other intracardiac repair. This is a retrospective study on 31 patients. We report our results discussing the PAVSD classification and the strategy of the complete repair in comparison with other reported results.

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects.

OBJECTIVES: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.

Changes in venous return parameters associated with univentricular Fontan circulations.

To clarify the physiology of venous return (Q(vr)) in Fontan circulations, venous return conductance (G(vr)) and mean circulatory filling pressure (P(mcf)) were determined in pentobarbital sodium-anesthetized pigs. Relationships between Q(vr) and right (biventricular, n = 8) or left (Fontan, n = 8) filling pressures are described by straight lines with significant correlation coefficients. Estimated P(mcf) values were correlated with observed P(mcf) values in either circulations (P

"Swiss cheese" septal defects: surgical closure using a single patch with intermediate fixings.

BACKGROUND: Residual ventricular septal defects and ventricular and septal dysfunctions are surgical drawbacks of "Swiss cheese" defects. We developed a technique that uses a single patch with intermediate fixings to cover the right side of the septum without producing a septal bulging, through a right atriotomy. METHODS: Since April 1993, 5 children with "Swiss cheese" defects have been operated on using this procedure (mean age, 17 +/- 12 months). Three patients had associated lesions including tetralogy of Fallot, Taussig Bing heart, and mitral stenosis. RESULTS: There have been no early or late deaths. The mean follow-up time is 29 +/- 18 months. All patients are asymptomatic. Echocardiography revealed either an intact septum (n = 4) or insignificant color jets at the apical portion of the septum (n = 1). The septal wall motion was preserved in 4 children and was hypokinetic in the fifth child. CONCLUSIONS: This technique can be an additional tool to provide a secure closure of "Swiss cheese" defects even in the presence of associated cardiac lesions. Long-term consequences of this procedure on septal wall motion remain to be determined.

[Value of the spiral angio-scanner with three-dimensional reconstruction in the surgical strategy of unifocalization. Apropos of a case]. / Apport de l'angioscanner hélicoïdal avec reconstruction tridimensionnelle dans la stratégie opératoire des unifocalisations. A propos d'une observation.

One of the difficulties of surgical treatment of pulmonary atresia with patent septum by unifocalisation resides in the accurate diagnosis of the different collateral vessels to the lung in order to optimise the surgical approach: anterior or posterolateral thoracotomy, and to determine the type of operation: one or two stages repair. Conventional angiography, even using different views, cannot always give an accurate representation of the anatomy of the different collateral vessels, especially their relationship to the bronchial structures. The authors report the contribution of spiral angioscanner with three dimensional reconstruction in the determination of the operative strategy of a case of pulmonary atresia with patent septum.

Surgical treatment of aortic root aneurysm related to Marfan syndrome in early childhood.

The prognosis of Marfan syndrome in both adult and pediatric patients is primarily related to the cardiovascular complications. In infantile Marfan syndrome, although involvement of the mitral valve is the most frequently encountered cardiovascular lesion, the aortic root can be more worrisome because of its excessive dilatation, leading to aortic insufficiency or dissection. If the role of elective surgery is relatively well defined for adult patients, it is still debated during childhood. We report two patients, aged 22 months and 5 years, each presenting an aortic root aneurysm related to Marfan syndrome, and each treated with the Bentall procedure without specific age-related mortality or morbidity. These two patients experienced normal growth and were free of any complication for a follow-up period of 8 and 2 years, respectively. More than an absolute value of the aortic root dimension, it is the conjunction of the rate of progression of the aortic root dilatation, the degree and the duration of the aortic valve regurgitation, and its resulting left ventricular dysfunction that must be taken into consideration in choosing the surgical option.

Bidirectional inferior vena cava-pulmonary artery shunt.

BACKGROUND: Bidirectional superior vena cava-pulmonary shunt is widely used as an interim palliation for patients with univentricular hearts. Bidirectional inferior vena cava-pulmonary artery shunt, as an alternative approach of partial Fontan circulation, may offer the advantage of performing the complete Fontan circulation more easily due to the already constructed inferior vena cava lateral tunnel. METHODS: We used bidirectional inferior vena cava-pulmonary artery shunt in 2 patients. Contraindications to a complete Fontan circulation were due to, respectively, a volume-overloaded systemic ventricle and an irregular pulmonary arterial tree. RESULTS: Postoperative courses were uneventful. There were no significant pleural effusions. Transcutaneous oxygen saturations were 77% and 78%. Pulmonary-to-systemic blood flow ratios were 0.57 and 0.63. A complete Fontan circulation was safely performed 8 and 12 months later, without any "Fontan-related" complications. CONCLUSIONS: Bidirectional inferior vena cava-pulmonary artery shunt can be useful in selected patients with univentricular hearts, although its place in the field of "partial Fontan operations" cannot be determined as yet.

[Late complications of percutaneous closure of atrial septal defects with the Sideris occluder]. / Complications tardives après fermeture percutanée des communications interauriculaires par prothèse boutonnée de Sideris.

Between June 1992 and January 1996, 27 patients aged 3.9 to 74 years with an ostium secundum (22 patients) or patent foramen ovale with right-to-left shunts (5 patients) underwent percutaneous closure of their atrial septal defects with the Sideris occluder. After a thromboembolic complication, transesophageal echocardiography was performed routinely after the procedure in 15 patients between 1 month and 2 years, and in 6 patients on the 15th day. Two patients died, on the 2nd day and 21st month, of non-related causes. After an average follow-up of 33 months, 59% of patients had complete occlusion of the atrial septal defects or only a minimal residual shunt. Displacement of the prosthesis was defects or only a minimal residual shunt. Displacement of the prosthesis was observed in 7 cases with no relationship to size: 4 parallel to the septum with reappearance or increase in shunt, 3 with tilting of the prosthesis. All of these patients had a large residual defect compared with 20% with a normally positioned prosthesis (p < 0.05). Tilting of the occluder was associated with left atrial thrombosis (present in 40% of these patients), complicated by systemic embolism in one case: there were no cases of left atrial thrombus in the 9 with complete occlusion and the 5 patients with an isolated residual defect (p < 0.05). Occlusion of atrial septal defect with the Sideris device is effective and a safe method in the majority of cases. However, a badly positioned prosthesis with a residual shunt should be extracted as seen as possible or within three weeks if displacement is observed at control echocardiography.

Total repair of congenital heart disease in the very young.

Total repair of congenital heart disease in the very young is nearly always feasible when two balanced ventricles are present. Refinements in antenatal and fetal cardiology, and progress in pediatric cardiac surgery have improved both the surgical results and the long-term outcome of these critically ill infants.

[Surgical treatment of atrial septal defects by right anterolateral thoracotomy]. / Cure chirurgicale des communications interauriculaires par thoracotomie antéro-latérale droite.

Motivated by esthetic considerations, the authors undertook surgical cure of atrial septal defects by a right antero-lateral thoracotomy in 80 patients with an average age of 24 +/- 13 years (range: 12-62 years) between 1984 and 1994. The pathologies operated were ostium secundum (62), superior sinus venosus defects (12), low atrial septal defects (2) and ostium primum lesions forming partial atrioventricular canals (4). Mortality rate was nil. The esthetic result was satisfactory overall. The authors suggest that a right antero-lateral thoracotomy provides an esthetic result whilst respecting the essential factor of maximal security.

Right anterolateral thoracotomy for repair of atrial septal defect.

BACKGROUND: To procure a cosmetic incision in female patients, we performed operation on atrial septal defects through a right anterolateral thoracotomy. METHODS: From 1984 to 1994, 80 female patients with a mean age of 24 +/- 13 years (ranging from 12 to 62 years) underwent right anterolateral thoracotomy for atrial septal defect repairs. Defects repaired included 62 ostium secundum, 12 sinus venosus, 2 low septal defect, and 4 ostium primum. The right iliac external artery was systematically used for arterial cannulation, through a cosmetic incision. Repairs were always performed under fibrillation, except in the 4 ostium primum defects, for which cardioplegia was used. RESULTS: There was no operative or late mortality, and no morbidity directly related to the thoracotomy approach. CONCLUSIONS: The right thoracotomy incision appears to be a safe and effective alternative to median sternotomy for repair of atrial septal defects.

"Classic" repair of congenitally corrected transposition and ventricular septal defect.

BACKGROUND: This study examined the results of "classic" repair of congenitally corrected transposition of the great arteries and ventricular septal defect. METHODS: From 1974 to 1994, 52 patients underwent a classic complete repair of lesions associated with congenitally corrected transposition. They were divided into two groups: ventricular septal defect plus left ventricular outflow tract obstruction (group I, 37 patients) and isolated ventricular septal defect (group II, 15 patients). Tricuspid plasty or replacement was performed primarily in 1 patient of group I (3%) and in 8 patients of group II (53%). RESULTS: The overall operative mortality was 15% (8/52 patients), and the incidence of postoperative atrioventricular block was 27% (14/52 patients). Eight patients died secondarily, 5 of heart failure. Survival rates were 83% +/- 6% at 1 year and 55% +/- 14% at 10 years for group I and 86% +/- 9% at 1 year and 71% +/- 12% at 10 years for group II (not significant). Redo tricuspid plasty or replacement was performed in 12 patients. CONCLUSIONS: Results of classic complete repair of lesions associated with congenitally corrected transposition are not satisfactory in our experience because (1) the operative mortality and the incidences of tricuspid valve replacement and atrioventricular block are high and (2) secondary heart failure is frequent. However, a retrospective review of morphologic findings shows that "anatomic" complete repairs would not have been feasible in 6 of our patients.

[Techniques of correction of partial right abnormal pulmonary venous return associated with atrial septal defect]. / Techniques de correction des retours veineux pulmonaires anormaux partiels droits associés à une communication interauriculaire.

Many techniques have been described for correcting partial right anomalous pulmonary venous drainage to avoid the possible complications of stenosis of the systemic or pulmonary venous return, residual shunt or arrhythmias. Between 1985 and 1994, 33 patients aged 1 to 69 years underwent repair of this malformation. The anomalous drainage was situated at the cavo-atrial junction or above in 25 cases and to the right atrium in 8 cases. Depending on the level of the drainage of the anomalous pulmonary veins, the size of the superior vena cava, the site of atrial septal defect and the age of the patient, 3 techniques were used: simple tunneling, tunneling with widening of the superior vena cava by a patch, tunneling with section of the superior vena cava and its transposition to the right atrium. There was no hospital mortality. Postoperative echocardiography showed a minimal residual shunt which regressed at the two months control examination. No cases of restriction of the systemic or pulmonary venous return were observed. Six patients developed arrhythmias during the hospital period. At the end of follow-up, all patients were asymptomatic without residual shunts or restriction of venous drainage. Persistent arrhythmias were observed in one case (3%). There were no differences in the results of the three techniques used. By using the most appropriate technique of repair for the anatomical form allows correction of this malformation with the minimal number of postoperative complications.

[Pulmonary arborization abnormalities in complex forms of pulmonary atresia with ventricular septal defect: unification, unifocalization and complete repair]. / Défauts d'arborisation pulmonaire des formes complexes d'atrésie pulmonaire à septum ouvert:unification, unifocalisation et réparation complète.

The presence of intrapulmonary arborization abnormalities in patients with pulmonary atresia and ventricular septal defect remains a therapeutic challenge. The aim of this study was to assess the value of procedures of pulmonary unifocalization, i.e. pulmonary unification, remodelling of the central pulmonary arteries and creation of an unifocal pulmonary blood supply, thereby resulting in complete repair. From october 1989 to october 1995, 27 unifocalization procedures were performed in 19 patients. The number of pulmonary segments dependant on non-communicating systemico-pulmonary collaterals was 14.7 +/- 5.4 per patient. The number of non-communicating systemico-pulmonary collaterals was 3.4 +/- 1.2 per patient. The Nakata index was 71 +/- 83 mm2/mm2. There were 3 deaths after an unifocalization procedure (mortality rate 15.8%). In 12 patients (63.2% of cases) a pulmonary arterial tree compatible with a complete repair was obtained. Eight complete repairs, with no mortality, following one or several pulmonary unifocalization procedures with a right to left ventricular systolic pressure ratio of 0.61 +/- 0.12 (range 0.4 to 0.75). Pulmonary unifocalization increases the recruitment of pulmonary segments and thereby the possibilities of complete correction in forms of pulmonary atresia with ventricular septal defect and arborization abnormalities of the pulmonary arterial tree.

Surgical treatment of diffuse supravalvular aortic stenosis.

Diffuse supravalvular aortic stenosis can be treated by a variety of surgical approaches. In this case of severe diffuse supravalvular aortic stenosis in a child, we used the combination of an apicoaortic conduit followed 6 years later by aortic valve replacement, replacement of the ascending aorta and aortic arch, and an ascending to thoracic descending aorta bypass graft.

Hemodynamics of different degrees of right heart bypass: experimental assessment.

BACKGROUND: Although their assessment could be of the utmost importance to determine the surgical treatment for patients with univentricular hearts, differences in ventricular performance between partial and complete right heart bypass remain to be defined. METHODS: Three different degrees of right heart bypass were investigated in 5 mongrel dogs: (1) superior vena cava to both pulmonary arteries shunt (SCP); (2) inferior vena cava to both pulmonary arteries shunt (ICP); and (3) both venae cavae to both pulmonary arteries shunt (BCP). Hemodynamic studies included evaluation of the cardiac index and left atrial pressure as a function of the degree of right heart bypass. RESULTS: By maintaining the mean left atrial pressure at 5 mm Hg, cardiac indexes were 1.98 +/- 0.25, 1.67 +/- 0.29, and 1.33 +/- 0.21 L.min-1.m-2 for SCP, ICP, and BCP shunts, respectively (p = 0.001). When keeping the cardiac index constant, mean left atrial pressures were 5.2 +/- 0.8, 5.5 +/- 0.9, and 7 +/- 0.7 mm Hg for SCP, ICP, and BCP shunts, respectively (p = 0.001). CONCLUSIONS: Increasing degrees of right heart bypass are associated with a significant decrease in ventricular performance in this experimental model.

Bypass graft for complex forms of isthmic aortic coarctation in adults.

BACKGROUND: Bypass grafting for complex forms of coarctation has been poorly documented as an alternative to decrease the high complication rate associated with anatomic repair. METHODS: Between mid-1980 and the end of 1994, 16 patients underwent bypass grafting for complex forms of isthmic aortic coarctation. Age ranged from 11 to 49 years (mean age, 28.4 +/- 13 years). Indications were atypical anatomic forms of coarctation (n = 12) and reoperation after multiple or complicated previous coarctation repair (n = 4). Lateroisthmic bypass grafts were performed in 14 patients and ascending aorta-descending aorta bypass grafts in 2. RESULTS: There was no hospital mortality. Morbidity consisted of postoperative paradoxical hypertension in 3 patients. There were no spinal cord complications. One death 10 years postoperatively was unrelated to the surgical technique. One patient successfully underwent ascending aorta-descending aorta bypass grafting for a false aneurysm 10 years after lateroisthmic grafting. All patients were asymptomatic and all grafts, patent after a mean follow-up of 5.7 +/- 4 years. CONCLUSIONS: On the basis of these results, bypass grafting appears to be a safe alternative in this select group of patients. The lateroisthmic bypass graft is the procedure of first choice, and the ascending aorta-descending aorta bypass graft should be reserved for failure of previous lateroisthmic bypass grafting.