Origin of congenital coronary arterio-ventricular fistulae from anomalous epicardial and myocardial development.

Coronary Artery Fistulae (CAFs) are cardiac congenital anomalies consisting of an abnormal communication of a coronary artery with either a cardiac chamber or another cardiac vessel. In humans, these congenital anomalies can lead to complications such as myocardial hypertrophy, endocarditis, heart dilatation, and failure. Unfortunately, despite their clinical relevance, the aetiology of CAFs remains unknown. In this work, we have used two different species (mouse and avian embryos) to experimentally model CAFs morphogenesis. Both conditional Itga4 (alpha 4 integrin) epicardial deletion in mice and cryocauterisation of chick embryonic hearts disrupted epicardial development and ventricular wall growth, two essential events in coronary embryogenesis. Our results suggest that myocardial discontinuities in the embryonic ventricular wall promote the early contact of the endocardium with epicardial-derived coronary progenitors at the cardiac surface, leading to ventricular endocardial extrusion, precocious differentiation of coronary smooth muscle cells, and the formation of pouch-like aberrant coronary-like structures in direct connection with the ventricular lumen. The structure of these CAF-like anomalies was compared with histopathological data from a human CAF. Our results provide relevant information for the early diagnosis of these congenital anomalies and the molecular mechanisms that regulate their embryogenesis.

Accuracy and impact of prenatal diagnosis of common arterial trunk.

OBJECTIVES: Outcome of common arterial trunk (CAT) depends mainly on truncal valve function, presence of coronary artery abnormalities and presence of interrupted aortic arch. The main objective of this study was to evaluate the accuracy of prenatal diagnosis of CAT by analyzing prenatal vs postnatal assessment of: (1) anatomic subtypes and (2) truncal valve function. The secondary objective was to assess the potential impact of prenatal diagnosis of CAT on postnatal mortality and morbidity by comparing prenatally vs postnatally diagnosed patients. METHODS: This was a retrospective analysis of all CAT patients diagnosed either prenatally, with postnatal or fetopsy confirmation, or postnatally, from 2011 to 2019 in a single tertiary center. Cohen's kappa statistic was used to evaluate agreement between pre- and postnatal assessment of anatomic subtypes according to Van Praagh and of truncal valve function. Mortality and morbidity variables were compared between prenatally vs postnatally diagnosed CAT patients. RESULTS: A total of 84 patients (62 liveborn with prenatal diagnosis, 16 liveborn with postnatal diagnosis and six terminations of pregnancy with fetopsy) met the inclusion criteria. The accuracy of prenatal diagnosis of CAT anatomic subtype was 80.3%, and prenatal and postnatal concordance for subtype diagnosis was only moderate (κ = 0.43), with no patient with CAT Type A3 (0/4) and only half of patients with CAT Type A4 (8/17) being diagnosed prenatally. Fetal evaluation of truncal valve function underestimated the presence (no agreement; κ = 0.09) and severity (slight agreement; κ = 0.19) of insufficiency. However, four of five cases of postnatally confirmed significant truncal valve stenosis were diagnosed prenatally, with fair agreement for both presence and severity of stenosis (κ = 0.38 and 0.24, respectively). Mortality was comparable in patients with and those without prenatal diagnosis (log-rank P = 0.87). CAT patients with fetal diagnosis underwent earlier intervention (P < 0.001), had shorter intubation time (P = 0.047) and shorter global hospital stay (P = 0.01). CONCLUSIONS: The accuracy of prenatal diagnosis of CAT is insufficient to tailor neonatal management and to predict outcome. Fetal assessment of truncal valve dysfunction appears unreliable due to perinatal transition. Improvement is necessary in the fetal diagnosis of anatomic subtypes of CAT requiring postnatal prostaglandin infusion. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Evaluation of septal insertion of atrioventricular valves in fetuses by postmortem 4.7 Tesla cardiac MRI: A feasibility study.

PURPOSE: The purpose of this study was to compare non-invasive high-spatial-resolution postmortem cardiac magnetic resonance imaging (MRI) and autopsy findings for evaluating the septal insertion of atrioventricular valves in fetuses. MATERIALS AND METHODS: Five fetal heart specimens including two normal hearts, one heart with complete atrioventricular septal defect (AVSD) and two hearts with linear insertion of atrioventricular valves (LIAVV; gestational age 17 to 34 weeks) were studied with cardiac MRI using a 4.7 T MRI scanner without sample preparation. Three (3D) and two-dimensional (2D) turbo-RARE (rapid imaging with refocused echoes) sequences in four-chamber and left-ventricular long-axis planes were obtained with a minimal isotropic/in-plane resolution of 156µm. Nonparametric tests were performed to compare the distance between insertions of medial leaflets of the atrioventricular valves and the inlet/outlet distance ratio between MRI and autopsy findings in normal, complete AVSD and with linear insertion of atrioventricular valves (LIAVV) fetal hearts. RESULTS: Despite apparent differences between LIAVV/normal hearts, no significant differences were found between differential insertion of medial leaflets and inlet/outlet distance ratios with both techniques. Very good to excellent reliability between both techniques was found for differential insertion (ICC: 87.2%; 95% CI: -21.7%, 99.1%) (P=0.963) and inlet/outlet distance ratio (ICC 98.3%; 95%CI: 85.2%, 99.8%) (P=0.537) measurements. CONCLUSION: Postmortem cardiac MRI could replace autopsy for assessing normal or abnormal septal insertion of atrioventricular valves in fetuses without requiring specific preparation of the heart.

[Surgical repair of atrial septal defect in 1011 consecutive patients]. / Traitement chirurgical des communications interauriculaires chez 1011 patients consécutifs. Incidence des complications postopératoires précoces.

The immediate postoperative complications of 1011 consecutive patients undergoing surgical repair of atrial septal defects between 1980 and 1998 at Marie Lannelongue Hospital were analysedwith the ultimate objective of comparing their incidence with that of percutaneous closure. Five patients died (0.49%) of low cardiac output (N=3), pulmonary oedema (N=1) or pulmonary hypertension (N=1). A total of 356 patients (35.2%) had 448 postoperative complications. There were 77% minor and 23% major complications. The minor complications included arrhythmias and conduction defects (N=130), respiratory complications (N=90) and pericardial effusions (N=64). The main major complications were cardiac failure (N=27), cardiac tamponade (N=13), neurological complications (N=8) and reoperation (N=28). Of the survivors, 95.6% of patients were discharged from hospital with no residual problem and 41 (4.4%) had sequellae: arrhythmias (N=29, including one pacemaker implantation), neurological complications (N=4), acquired mitral regurgitation (N=2), phrenic nerve paralysis (N=1) and minimal residual shunt (N=10). Factors correlated with surgical morbidity were age, the severity of pulmonary hypertension, the type of atrial septal defect (less morbidity with ostium secundum defects), the presence of associated malformations, the surgical approach (less morbidity with the right postero-lateral thoracic approach). This study demonstrated the incompressible risk of open heart cardiac surgery. It will form a basis for a comparative study of the two modern methods of treating atrial septal defect: surgical and percutaneous closure.

[Placing an endoprosthesis to maintain arterial canal permeability in lamb neonates]. / Mise en place d'une endoprothèse pour maintenir le canal artériel perméable chez l'agneau nouveau-né.

Placing an endoprosthesis in the arterial canal in order to maintain permeability is a possible alternative to performing a modified Blalock-Taussig type surgical systemico-pulmonary anastomosis. This was studied in an animal model. Twelve newborn lambs, weighing from 1.8 to 3.5 kg, were catheterised in the neonatal period in order to place a stent. Three had a partial ligature of the pulmonary artery in utero. During the initial angiography, the canal was occluded in ten of them. Different types of coronary endoprosthesis were used: Multi link tetra TM and RX Herculink TM (Guidant Europe SA), Niroyal (Boston Scientific International), Bx Velocity (Cordis, Johnson and Johnson), Jostent (Jomed). The length of the endoprostheses varied from 12 to 18 mm and the diameter from 3.5 to 6 mm. Implantation was successful 10 out of 12 times: in one case, implantation was complicated by a fatal haemopericardium, and in another by pulmonary artery embolism. Nine animals out of 10 were followed up for 1 to 2 months. At autopsy verification, the canal was permeable in 7 cases with the development of a neointima and zones of moderate stenosis with intimal hyperplasia. In 2 animals the canal was occluded at the aortic level in a zone not covered by the endoprosthesis. Three animals died after implantation: the 2 implantation failures. A third animal with a well inserted prosthesis and a permeable canal died on day 1 from an unknown cause. Placing a stent in the arterial canal is a possible alternative to performing an aorto-pulmonary shunt. Coronary stents seem well adapted and this study does not allow any conclusions to be made at this time on the best type of stent. Further studies will be necessary in order to validate this concept before its use in congenital cardiopathies.

[Pediatric cardiac transplantation. Long-term results and problems]. / Transplantation cardiaque pédiatrique. Résultats à long terme et problèmes actuels.

The aim of this study was to establish an assessment for cardiac transplantation by retrospective analysis of data from two French centres. Screening for acute rejection was ensured by systematic myocardial biopsies for patients aged over 10 years, and by clinical non-invasive follow up, ECG and Doppler echo in the younger ones. Coronaropathy was screened for by coronarography or stress echography. Eighty three cardiac transplantations were performed in 82 patients aged 9.2 +/- 6 years. The follow up was 5.8 +/- 4.8 years. Survival was 86% at 1 month, 76% at 1 year, 73% at 5 years and 60% at 10 years and did not differ for age at transplantation (p = 0.88) or the initial pathology (p = 0.25). Twenty-nine patients died in the period between 3 days to 11 years, of which 13 were during the first month and 9 after one year (of which 4 were acute rejections and 2 coronaropathies). The incidence of acute rejection was 1.8 episodes per patient; 42% were late rejections, through non-compliance for half of them. Systematic biopsy was useful in the first 3 months post transplant. Eight patients (9.6%) presented with coronaropathy of the graft, progressive for 5 of them. Renal function was altered in 12% of cases, especially in the patients transplanted before the age of 2 years. The results of paediatric cardiac transplantation are satisfactory. Acute rejection and therapeutic compliance for adolescents, graft coronaropathy, and renal function of infants remain the principal elements for the long term prognosis.

[Neonatal cardiac transplantation. Long-term results]. / Transplantation cardiaque néonatale. Résultats à long terme.

The pioneering work of Bailey since 1985 has made cardiac transplantation an effective treatment of left heart hypoplasia in the neonatal period. At the Marie-Lannelongue surgical centre, 5 neonates with hypoplastic left hearts or an equivalent cardiac malformation, underwent cardiac transplantation between 1993 and 1998. There were no operative deaths. At the end of the follow-up, all children were treated with cyclosporine, without steroids. One child died of a secondary acute rejection resistant to treatment 7 years after the transplantation, 1 has a severe coronary problem 13 years after the transplantation, and three children (including the diseased child) have psychomotor development abnormalities. These results and those of the literature together with the psychological problems encountered by the children and their families, do not incite in the pursuit of the neonatal transplantation program until the introduction of new drugs for the treatment of chronic rejection which should prevent the programmed death of these children.

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch.

OBJECTIVES: Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta. This technique can be limited by the presence of tricuspid chordae and by the pulmonary artery to tricuspid valve distance. Furthermore, there is an important risk of late subaortic obstruction related to the long patch required that creates a potential akinetic septal area. Presented here is another technique; by VSD rerouting to the pulmonary infundibulum and arterial switch. METHODS: Ten patients, with DORVncVSD, underwent a VSD rerouting to the pulmonary infundibulum followed by arterial switch. Seven had a previous pulmonary artery banding and one a moderate infundibular stenosis. The median age at surgery was 16 months (range 3 weeks to 4.5 years). All patients had a bilateral infundibulum, with a large persistent subaortic conus, D malposition of the aorta, side-by-side vessels and double loop coronary patterns. The VSD was perimembranous with inlet or trabecular extension. Subaortic obstruction was constant. The VSD was severely distant from both the aortic and the pulmonary annulus. The operation was conducted through a combined approach. The VSD was constantly enlarged superiorly. The almost permanent subaortic obstruction was released. The VSD was always found quite close to the pulmonary infundibular ostium. The arterial switch technique was adapted to the complex coronary anatomy. RESULTS: There was one non-cardiac death. At a mean follow-up of 20 months, all nine survivors are in NYHA class I, in sinus rhythm, and have no subaortic gradient greater than 15 mm. CONCLUSION: This technique of VSD rerouting to the pulmonary artery and arterial switch limits greatly the size of the rerouting patch, respects the tricuspid chordae and is independent of the pulmonary artery-tricuspid valve distance. In this early series of biventricular repair of DORVncVSD, the VSDs were always found close to the pulmonary artery, allowing this new type of repair.

[Interarterial and intramural coronary trajectoris: anatomic aspects and surgical implications]. / Trajets coronaires interartériel et intramural: aspects anatomiques et implications chirurgicales.

Among the coronary trajectory anomalies found not only in congenital cardiopathies but also in otherwise normal hearts, the terms intramural and interarterial are still used in an ambiguous fashion too frequently. Study of the embryonic development of the coronaries allows a better understanding of the possibility of such a trajectory, (existence of the periarterial sheath). Certain anatomical factors favour the existence of an intramural coronary trajectory, the coronary always being tangential to the aorta in this case: paracommissural ostium, ostium situated too high, departure of the coronary at an acute angle, and ostium in the form of a slit. However, interarterial and intramural are not synonyms. In the case of a coronary trajectory tangential to the aorta, three anatomical and histological situations are possible: free trajectory, with the aorta and the coronary each having its own media and its own adventitia; adherent trajectory, with two separate media covered by a common adventitia: and true intramural trajectory, with fusion of the media without interposition of adventitia. These three situations have different surgical implications in the case of coronary reposition.

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.

Congenital mitral stenosis with or without associated defects: An evolving surgical strategy.

BACKGROUND: Congenital mitral stenosis (CMS) remains a surgical challenge, particularly when it is associated with other heart defects. As in other groups of heart defects, there is a trend toward early single-stage complete repair, but the optimal surgical approach remains unanswered. METHODS AND RESULTS: This study was designed to analyze the evolution of surgical strategies in patients with CMS and associated defects through single-stage and staged repair. Between 1980 and 1999, 72 children were operated on for congenital heart defects, including CMS. Preoperative transmitral gradient was 12.6+/-7 mm Hg. Preoperatively, all the patients were NYHA class III to IV. Thirteen patients had an isolated CMS; in 59, it was associated with other heart defects, mainly ventricular septal defect (n=28) or multilevel left ventricular obstruction (n=41). In this group of patients, 33 had a staged approach, and 26 had a single-stage approach. Early mortality was 12.5% (9 patients). There were no deaths in the isolated CMS and single-stage repair groups. Logistic regression revealed that early mortality was influenced by association with left ventricular outflow tract obstruction (P:<0.001) and by use of a staged approach (P:<0.01). There was no late mortality in isolated CMS; there were 2 late deaths in the group of single-stage repair and 6 late deaths in the staged approach group (P:<0.01). Reoperation was required in 24 patients, mainly for residual mitral valve dysfunction or residual left ventricular outflow tract obstruction. Including the reoperations, 10 patients received a prosthetic mitral valve. At 15 years after surgery, survival was 69.6+/-7.5%, freedom from reoperation was 70.8+/-6.3%, and freedom from mitral valve replacement was 69+/-6%. CONCLUSIONS: Surgery for isolated CMS gives excellent early and long-term results. In patients with associated heart defects, a single-stage operation seems superior to a staged approach. Mitral valve replacement in this category of patients should be reserved as a salvage procedure.

Transaortic closure of residual intramural ventricular septal defect.

BACKGROUND: Residual intramural ventricular septal defect is an unusual cause of left-to-right shunt after biventricular repair of conotruncal anomalies. It results from the insertion of the patch within the trabeculated right ventricular free wall related to the ventriculoinfundibular fold creating a communication through the intertrabeculated spaces to the right ventricular cavity. This complication often leads to unsuccessful reoperations unless the exact mechanism of the shunt has been identified. METHODS AND RESULTS: Five patients presented with residual intramural ventricular septal defects. Three had double outlet right ventricle, one pulmonary atresia with ventricular septal defect, and one tetralogy of Fallot. One patient was unsuccessfully reoperated on for closure of the residual ventricular septal defect through the right ventricular approach. The surgical treatment, which consisted of patch closure of the residual intramural ventricular septal defect through aortotomy, was successful in 3 patients. In the 2 remaining patients the hemodynamically insignificant residual intramural ventricular septal defect remained untouched. No mortality or morbidity occurred. CONCLUSIONS: Residual intramural ventricular septal defect should be suspected in presence of a residual ventricular septal defect after biventricular repair of conotruncal anomalies. It is not accessible through either atriotomy or right ventriculotomy. The transaortic approach allows an easy treatment of this rare complication.

Double-outlet right ventricle with non-committed ventricular septal defect.

OBJECTIVE: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. METHODS: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 21) and arterial switch operation with VSD to pulmonary artery baffle (n = 2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. RESULTS: There were two hospital deaths (9%, 70% CL: 3-19%). Eight patients (35%, 70% CL: 23-48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. CONCLUSIONS: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.

[Right postero-lateral thoracotomy for open heart surgery in infants and children. Indications and results]. / Thoracotomie postéro-latérale droite pour la chirurgie à coeur ouvert chez l'enfant. Indications et résultats.

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.

Biventricular repair of conotruncal anomalies associated with aortic arch obstruction: 103 patients.

BACKGROUND: Biventricular repair of conotruncal anomalies associated with aortic arch obstruction is a complex surgical procedure that combines a cardiac repair and a aortic arch reconstruction. METHODS AND RESULTS: From January 1984 to April 1996, such a repair was performed in 103 patients. The conotruncal anomalies included: 15 transpositions of the great arteries (TGAs) with intact ventricular septum, 44 TGAs with ventricular septal defect, 32 double outlet right ventricle with subpulmonary ventricular septal defect, 10 truncus arteriosus, one double outlet left ventricle, and one tetralogy of Fallot. The arch obstruction included 88 coarctation and 15 interrupted aortic arch. One-stage repair has been the favored technique since 1990 and was performed in 58 neonates, including 38 TGAs or double outlet right ventricle and ventricular septal defect, 10 TGAs with intact ventricular septum, and all of the 10 truncus arteriosus. The cardiac repair included 89 arterial switch operations, 2 Kawashima rerouting, 10 truncus arteriosus repairs, and one double-outlet left ventricle repair and one tetralogy of Fallot repair. The aortic arch was reconstructed by direct anastomosis in 85 patients, with a Gore-Tex conduit in three patients and more recently by an ascending aortic patch augmentation in 15 patients. The hospital mortality was 12% (7 of 58) for the one-stage repair and 20% (9 of 45) for the two-stage repair. There were six late deaths. Reoperations or angioplasties were mandatory for 12 right ventricle outflow tract obstructions after arterial switch, involving 10 patients with double outlet right ventricle (P=.02), 10 recurrent arch obstruction, and six miscellaneous lesions. CONCLUSIONS: One-stage biventricular repair of conotruncal anomalies associated with aortic arch obstruction can be achieved in selected patients with an 83% survival rate at 7 years.

Prevention of unbalanced lung perfusion and atrial arrhythmias after the lateral tunnel operation.

BACKGROUND: The lateral tunnel operation has become increasingly popular with pediatric cardiac surgeons, as it is technically reproducible, is relatively easy to perform, and can be used in a variety of patients with single-ventricle physiology. The main drawbacks of the original operation are uneven blood flow distribution to the lungs and increasing incidence of supraventricular arrhythmias over time. METHODS: In 1988, we modified this technique by avoiding narrowing of the tunnel at the superior vena cava-atrial junction, avoiding incorporation of the crista terminalis in the baffle suture line, and minimizing damage to the sinoatrial node. Between 1988 and 1995, 19 patients underwent this operation at Marie-Lannelongue Hospital in Paris. RESULTS: There was one early death and no late deaths. At a mean follow-up of 5.2 years, all survivors are in New York Heart Association class I. Early atrial flutter, related to atrial scarring secondary to multiple previous surgical procedures, developed in 1 patient, and late atrial flutter developed in 1 patient who had a previous Blalock-Hanlon atrial septectomy. All patients are currently in sinus rhythm. Atrial flutter did not occur in 17 patients who had had no previous atrial wall surgical procedure. CONCLUSIONS: We believe that the good long-term clinical results are directly attributable to our modifications, which ensure optimal hemodynamics and absence of rhythm disturbances. All patients who had not previously undergone operation on the atrial wall were free from supraventricular tachyarrhythmias at a mean follow-up of 5.2 years. This is a consequence of protecting the sinus node, crista terminalis, and Bachmann's bundle.

[Acquired pulmonary vein stenosis after surgery of complex partial anomalous pulmonary venous drainage in an adult. Diagnostic value of transesophageal echocardiography]. / Sténose acquise d'une veine pulmonaire après chirurgie d'un retour veineux pulmonaire anormal partiel complexe de l'adulte. Intérêt diagnostique de l'échocardiographie transoesophagienne.

Surgical correction of partial anomalous pulmonary venous drainage is difficult and may be complicated by acquired postoperative stenosis at the site of reimplantation of the pulmonary veins in the left atrium. Diagnosis should be made quickly because of the very poor prognosis due to acute pulmonary hypertension. The case described by the authors underlines the value of multiplane transesophageal echocardiography with two-dimensional and Doppler analysis for rapid and accurate diagnosis of this complication.

Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle.

Out of 180 patients who underwent biventricular repair of double-outlet right ventricle between 1980 and 1995, 9 (5%) required reoperation because of subaortic stenosis. Two other patients who initially underwent operation elsewhere underwent reoperation at our institution because of subaortic stenosis. The median age at biventricular repair was 4 months. Repair consisted of tunnel construction from the left ventricle to the aorta in nine patients; the remaining two patients received an arterial switch operation with ventricular septal defect closure. Subaortic stenosis developed with time: the mean postoperative left ventricle-to-aorta gradient after repair was 10 +/- 19 mm Hg (range, 0 to 50 mm Hg) and became 84 +/- 27 mm Hg (range, 40 to 124 mm Hg) in a mean delay of 45 +/- 66 months (range, 1 to 213 months). At reoperation, the obstruction was caused by the protrusion of the inferior rim of the ventricular septal defect into the left ventricular outflow tract associated with subaortic hypertrophied muscle and membrane. The 11 patients underwent 15 reoperations. Surgical technique consisted of an extended septoplasty in 6 reoperations. In this technique an incision was made in the septal patch and was extended into the muscle toward the apex until a large opening of the left ventricular outflow pathway was obtained. A new patch was then secured to streamline the left ventricular outflow tract. None of the patients who underwent extended septoplasty had to undergo reoperation. There were no early or late deaths. At 115 +/- 85 months after biventricular repair, all patients were in New York Heart Association functional class I or II and the mean postoperative left ventricle-to-aorta gradient was 20 +/- 24 mm Hg (range, 0 to 60 mm Hg). We conclude that after biventricular repair of double-outlet right ventricle, the subaortic region is at risk for the development of stenosis. Surgical treatment adapted to the anatomy of the obstruction can offer good early and midterm results. It seems that an aggressive approach by an extended septoplasty avoids multiple reoperations.