Quality of Life of Children with Congenital Heart Diseases: A Multicenter Controlled Cross-Sectional Study.

To assess the health-related quality of life (QoL) in children with congenital heart diseases (CHD) with a validated questionnaire in comparison with control children. We prospectively recruited 282 children with CHD aged from 8 to 18 years in two tertiary care centers (France and Belgium) and 180 same-age controls in randomly selected French schools. Children's QoL was self-reported with the KIDSCREEN-52 questionnaire and reported by parents with the KIDSCREEN-27. QoL scores of each dimension were compared between CHD and controls and between the classes of disease severity. Both centers were comparable for most demographic and clinical data. Age- and gender-adjusted self-reported QoL scores were lower in CHD children than in controls for physical well-being (mean ± SEM 45.97 ± 0.57 vs 50.16 ± 0.71, p < 0.0001), financial resources (45.72 ± 0.70 vs 48.85 ± 0.87, p = 0.01), peers/social support (48.01 ± 0.72 vs 51.02 ± 0.88, p = 0.01), and autonomy in the multivariate analysis (47.63 ± 0.69 vs 49.28 ± 0.85, p = 0.04). Parents-reported scores were lower in CHD children for physical (p < 0.0001), psychological well-being (p = 0.04), peers/social support (p < 0.0001), and school environment (p < 0.0001) dimensions. Similarly, the disease severity had an impact on physical well-being (p < 0.001), financial resources (p = 0.05), and peers/social support (p = 0.01) for self-reported dimensions, and on physical well-being (p < 0.001), psychological well-being (p < 0.01), peers/social support (p < 0.001), and school environment (p < 0.001) for parents-reported dimensions. However, in multivariate analysis on self-reported QoL, disease severity was significantly associated with the self-perception dimension only. Self-reported QoL of CHD children was similar to that of same-age healthy children in seven of 10 dimensions, but parents-reported QoL was impaired in four of five dimensions.

Impact of COVID-19 disease on clinical research in pediatric and congenital cardiology.

BACKGROUND: COVID-19 triggered an unprecedented crisis affecting society at every level. Research in pediatric and congenital cardiology is currently in full development and may have been disrupted. The aim of the study was to determine the impact of COVID-19 on pediatric and congenital cardiology clinical research and to analyze decision-making and adaptation processes, from a panel of ongoing academic and industry-sponsored research at the time of the pandemic. METHODS: This observational study was carried out in April 2020, from a CHD clinical research network involving five tertiary care pediatric and congenital cardiology centers. Investigators and clinical research assistants from each participating research center completed an online survey questionnaire, and each principal investigator underwent a 1-h web-based videoconference interview. RESULTS: A total of 34 study questionnaires were collected, reporting that 18 studies were totally suspended. Upon the investigator's decision, after discussion on ethical issues and with facilitating support from health authorities, 16 studies were resumed. The rate of study suspension in interventional research (53%) was similar to that in non-interventional research (56%). Logistical problems were predominantly reported in both continued and suspended trials. Research protocols were adapted, largely thanks to telemedicine, which in some cases even improved the course of the study. CONCLUSION: The impact of the COVID-19 pandemic on clinical research in pediatric and congenital cardiology has been limited by a rapid adaptation of all research structures and an extensive use of telemedicine at all stages of the studies.

Patients requiring pediatric palliative care for advanced heart disease in France: A descriptive study.

INTRODUCTION: Pediatric palliative care (PPC) teams address unmet needs and improve the quality of life of patients with life-limiting conditions across pediatric subspecialties. However, little is known about the timing, reasons, and nature of PPC team interventions in advanced heart diseases (AHD). OBJECTIVES: Here we describe how, when, and why PPC teams interact with referred teams of children suffering from AHD. METHODS: We conducted a retrospective nationwide survey among PPC teams in France. All patients referred to participating PPC teams for a cardiologic disease in 2019 were studied. RESULTS: Among six PPC teams, 18 patients with AHD had a PPC consultation in 2019. Six of these patients had cardiomyopathy and 12 had congenital heart disease (CHD). The median age at referral was 0.9 months for CHD and 72 months for cardiomyopathy. An antenatal diagnosis had been made for six families with CHD, and two of them were referred to PPC before birth allowing for a prenatal palliative care plan. The main reason for referral was ethical considerations (50%) followed by organization for home-based palliative care (28%). PPC teams participated in ethical discussions when asked to but also provided family support (12/18), home-based PPC (9/18), coordination of care (5/18), support of the referred team (4/18), and symptoms management (3/18) CONCLUSION: The main reason for referral to PPC was ethical considerations, but PPC interventions followed a holistic model of care. Prospective outcomes measurement and partnerships should be further developed.

Midterm results of the Ross procedure in a pediatric population: bicuspid aortic valve is not a contraindication.

Our objective was to analyze retrospectively the short- and midterm results of the Ross operation in children and the impact of bicuspid aortic valve (BAV) disease on outcome. From 1991 to 2003, 41 patients (26 male, 15 female) underwent a Ross procedure. Aortic disease was congenital in all but one. Sixty-six percent had BAV. Mean age at operation was 10.13 +/- 5.6 years (range, 0.4-18.3 years). Root replacement technique was performed in all but two (inclusion technique). There were two early deaths. Mean follow-up was 6 +/- 3.8 years (range, 0.1-14 years). The autograft (neo-aorta) presented absent, trivial, mild, and moderate regurgitation in 42%, 46%, 10%, and 2%, respectively, at latest follow-up. Root dilation was seen in 64% of the patients (mean Z-score, +3.53 +/- 0.04). Four patients (9%) required allograft replacement, two for endocarditis and two for stenosis. Allograft stenosis (gradient >20 mmHg) was detected in 44% of the remaining patients, without symptoms or the need for reintervention. Estimated freedom from allograft replacement at 5 and 10 years was 97% and 89%. Left ventricular dimensions and function were normal in all patients. No difference was found between patients with BAV and those with tricuspid aortic valves in aortic regurgitation or root dilation. BAV was not identified as a risk factor for root dilation. In conclusion, the Ross operation remains an excellent option for aortic valve replacement in children. BAV is not associated with a worse outcome and is not a risk factor for aortic root dilation at medium-term follow-up. Long-term follow-up is, however, required.

[Evaluation of the efficiency of ERTL4/6 screening tests for language disorders for children with a 22q11 deletion]. / Etude de l'efficacité des epreuves de repérage de troubles de langage ERTL4/A6 sur des enfants porteurs d'une microdélétion 22q11.

OBJECTIVE: ERTL (Epreuves de Repérage de Troubles de Langage) tests have been designed as a screening test to detect rapidly language disorders. To evaluate the effectiveness of these tests, we have compared them to the form P (NEEL-P) tests for the examination of language in 6 children with a 22q11 microdeletion. MATERIALS AND METHOD: 6 children aged from 4 years and 4 months to 5 years and 4 months, 4 boys and 2 girls have been tested. These tests have been performed in two sessions lasting between 1 and 1 1/2 hour. RESULTS: They are consistent in both tests. ERTL has identified all the children with a disorder. CONCLUSION: These fast tests should be used more often to detect children requiring additional and more complex language and neuropsychological evaluations.

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

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

[Coarctation of the aorta presenting as a pulsatile occipital mass]. / Coarctation de l'aorte révélée par une masse occipitale pulsatile.

A healthy 18 months old boy, is referred to our hospital for a thrilling pulsatile mass behind the left ear. Aortography in the ascending aorta shows a severe coarctation with almost interruption of the aorta between the left carotid and left subclavian artery. Both external carotid arteries provide an important collateral pathway through occipital arteries to two dilated vertebral arteries. Descending aorta is feeding by a reverse blood flow into vertebral and subclavian arteries. The child has been operated. This is an original presentation of severe coarctation of the aorta with development of an important and vital collateral pathway.

Detection of pulmonary and coronary artery anomalies in tetralogy of Fallot using non-ECG-gated CT angiography.

OBJECTIVES: To evaluate the use of non-ECG-gated computed tomography (CT) angiography to describe pulmonary and coronary defects in patients with tetralogy of Fallot (TOF). PATIENTS AND METHODS: This retrospective study was carried out on TOF patients having undergone pre-operative non-ECG-gated CT angiography between February 2007 and September 2012. The following clinical parameters were recorded: mean age at CT angiography, sex, the existence of genetic disease and the need to sedate the patient prior to CT angiography. CT data were analyzed retrospectively to determine the site(s) of pulmonary stenosis (infundibular, valvular or arterial), the size of pulmonary arteries and the presence of anomalous coronary artery courses. CT findings were then compared to the anatomy observed during surgery. RESULTS: Thirty-five patients were included in the study. The mean age was 4.30±1.91months (boys/girls=17/18). Two patients had associated chromosome disorders (one 22q11 microdeletion and one CHARGE syndrome). Sixteen patients (45.71%) were sedated prior to CT. Pulmonary artery assessment revealed 24 patients (68.57%) with infundibular stenosis, 5 (17.5%) with infundibular and/or valvular stenosis, and 6 (21%) with anomalous pulmonary arteries. CT angiography also evidenced anomalous coronary arteries in 8 patients (22.85%). CONCLUSION: Due to its reduced scanning time and high spatial resolution, non-ECG-gated CT angiography is a non-invasive imaging modality that provides accurate information on pulmonary and coronary artery anatomy in patients with TOF.

Tetralogy of Fallot: transannular and right ventricular patching equally affect late functional status.

BACKGROUND: In tetralogy of Fallot, transannular patching is suspected to be responsible for late right ventricular dilatation. METHODS AND RESULTS: In our institution, 191 patients survived a tetralogy of Fallot repair between 1964 and 1984. Transannular patching was used in 99 patients (52%), patch closure of a right ventriculotomy in 35, and direct closure of a right ventriculotomy in 55. Two had a transatrial-transpulmonary approach. To identify predictive factors of adverse long-term outcome related to right ventricular dilatation, the following events were investigated: cardiac death, reoperation for symptomatic right ventricular dilatation, and NYHA class II or III by Cox regression analysis. Mean follow-up reached 22+/-5 years. The 30-year survival was 86+/-5%. Right ventricular patching, whether transannular or not, was the most significant independent predictor of late adverse event (improvement chi(2)=16.6, P:<0.001). In patients who had direct closure, the ratio between end-diastolic right and left ventricular dimensions on echocardiography was smaller (0.61+/-0.017 versus 0. 75+/-0.23, P:=0.007), with a smaller proportion presenting severe pulmonary insufficiency (9% versus 40%, P:=0.005). There was no difference between right ventricular and transannular patching concerning late outcome (log rank P: value=0.6), right ventricular size (0.70+/-0.28 versus 0.76+/-0.26, P:=0.4), or incidence of severe pulmonary insufficiency (30% versus 43%, P:=0.3). CONCLUSIONS: In tetralogy of Fallot, transannular patching does not result in a worse late functional outcome than patching of an incision limited to the right ventricle. Both are responsible for a similar degree of long-term pulmonary insufficiency and right ventricular dilatation.

Balloon angioplasty of native coarctation: clinical outcomes and predictors of success.

OBJECTIVES: We sought to investigate the clinical impact of balloon angioplasty for native coarctation of the aorta (CoA) and determine predictors of outcome. BACKGROUND: Balloon dilation of native CoA remains controversial and more information on its long-term impact is required. METHODS: Hemodynamic, angiographic and follow-up data on 69 children who underwent balloon angioplasty of native CoA between 1988 and 1996 were reviewed. Stretch, recoil and gain of CoA circumference and area were calculated and related to outcomes. RESULTS: Initial systolic gradients (mean +/- SD, 31+/-12 mm Hg) fell by -74+/-27% (p < 0.001), with an increase in mean CoA diameters of 128+/-128% in the left anterior oblique and 124+/-87% in the lateral views (p < 0.001). Two deaths occurred, one at the time of the procedure and one 23 months later, both as a result of an associated cardiomyopathy. Seven patients had residual gradients of >20 mm Hg. One patient developed an aneurysm, stable in follow-up, and four patients had mild dilation at the site of the angioplasty. Freedom from reintervention was 90% at one year and 87% at five years with follow-up ranging to 8.5 years. Factors significantly associated with decreased time to reintervention included: a higher gradient before dilation, a smaller percentage change in gradient after dilation, a small transverse arch and a greater stretch and gain, but not recoil. CONCLUSION: Balloon dilation is a safe and efficient treatment of native CoA in children. Greater stretch and gain are factors significantly associated with reintervention, possibly related to altered elastic properties and vessel scarring.

Elevated right ventricular pressures are not a contraindication to liver transplantation in Alagille syndrome.

BACKGROUND: Elevated right ventricle pressure resulting from pulmonary artery stenoses may affect outcome and survival after liver transplantation in patients with Alagille syndrome. METHODS AND RESULTS: Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for Alagille syndrome (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable pruritus, and failure to thrive. All patients had pulmonary artery stenosis. In 10 patients considered to have elevated RV pressure on ECG and/or Doppler-echocardiography, a cardiac catheterization was performed before liver transplantation. Mean RV systolic pressure was 55 mmHg (median 49.5 mmHg, range 35-98 mm Hg), mean RV to left ventricular systolic pressure ratio 0.53 (median 0.53, range 0.29-0.78) with a ratio above 0.5 in 6 patients (median 0.66, range 0.5-0.8). All patients underwent successful liver transplantation. Five patients died 1 to 9 months after transplantation from noncardiac causes. In two of them, cardiac catheterization before transplantation showed a RV to left ventricular pressure ratio of 0.51 in one and 0.37 in the second. In the three others, echocardiography before transplantation estimated RV pressures below 0.5 systemic pressures. At follow-up (median 6 years, range 1.5-15 years), liver tests were normal in all, none complained of pruritus and body weight was normalized in 70%. None of the patients presented cardiac symptoms, arrhythmias, or worsening of their cardiac status. CONCLUSIONS: Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pressure.

Comparison of two transcatheter closure methods of persistently patent arterial duct.

A randomized trial of arterial duct occlusion with a double umbrella (DU) or wire coil (WC) was undertaken for patients <18 years of age, weighing >10 kg with isolated ducts < or = 3 mm in diameter. Baseline, procedural, and outcome characteristics were compared in an intention-to-treat analysis according to randomization group. From 40 consecutively screened patients, 2 were not enrolled due to a ductal diameter of >3 mm on initial aortography, 38 patients were randomized to either the DU (n = 20) or WC (n = 18) groups. The groups did not differ significantly with respect to age, weight, gender, duct size, type, or branch pulmonary artery diameters. Crossover occurred only in the DU group, where 4 patients (20%) had a ductal diameter of < or = 1 mm and could not be entered for umbrella placement. All remaining DU group patients had ductal diameters of > or = 1.3 mm (p <0.0001). There were no embolizations or secondary implants in the DU group, but in the WC group there was 1 early and 1 late embolization, with 6 patients (33%) with > or = 2 coils. Mean times for the procedure (DU 68+/-19 minutes; WC 65+/-27 minutes; p = 0.70) and fluoroscopy (DU 14+/-4 minutes; WC 11+/-6 minutes; p = 0.22) did not differ significantly. Angiographic duct closure was documented in 4 of 13 patients (31%) of the DU group and 4 of 18 patients (22%) of the WC group (p = 0.69). Combined with an echocardiogram, closure in 11 of 17 patients with DU (65%) and 13 of 18 patients with WC (72%) (p = 0.64) was documented before hospital discharge. One WC group patient received thrombolytic therapy for a femoral artery thrombus. Follow-up at a median of 6.5 months (range 3.2 to 37) showed closure by Doppler echocardiography in 15 of 19 patients with DU (79%) versus 14 of 18 patients with WC (78%) (p = 1.0). Thus, with a tendency toward similar procedural characteristics and outcomes, the higher cost of the DU system compared with coil implants favors the use of coils for closure of the small arterial duct.

Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum.

OBJECTIVES: Since 1990, transcatheter pulmonary valvotomy has become an alternative to surgical valvotomy in the management of neonates and infants with pulmonary atresia and intact ventricular septum. We sought to determine whether right ventricular growth after transcatheter pulmonary valvotomy is commensurate with body growth. METHODS: Laser or radiofrequency-assisted balloon valvotomy was attempted in 12 neonates and infants with pulmonary atresia and intact ventricular septum. Tricuspid and mitral valve dimensions were measured retrospectively on the cross-sectional echocardiograms performed before the procedure and during follow-up. Z-values were used to standardize tricuspid valve dimensions with body size. RESULTS: The atretic pulmonary valve was successfully perforated and dilated in nine of 12 patients. Five of these nine patients required additional transcatheter or surgical procedures to augment the pulmonary blood flow. Of six survivors, five are regularly followed up with a median follow-up of 60 months (range 37 to 68 months). All five have two-ventricle circulation, two of the five patients requiring surgical enlargement of the right ventricular outflow tract with or without closure of the atrial septal defect. Echocardiographic tricuspid valve dimensions and Z-values before transcatheter valvotomy tended to be smaller in the patients who died than in the survivors. In the survivors, the absolute tricuspid valve dimensions increased after valvotomy but the Z-values tended to decrease or stayed constant. CONCLUSIONS: Transcatheter valvotomy is a good alternative to surgical valvotomy in patients with pulmonary atresia and intact ventricular septum. Two-ventricle circulation can be achieved despite subnormal right ventricular growth.

Endovascular stent implantation for the management of postoperative right ventricular outflow tract obstruction: clinical efficacy.

OBJECTIVE: Extracardiac conduits between the right ventricle and pulmonary arteries commit patients to multiple reoperations. We reviewed our experience with stent implantation in obstructed conduits. METHODS: Between 1990 and 1997, stents were implanted across 43 conduits. The median age at procedure was 6 years (0.5-17 years), and the median interval between conduit insertion and stent implantation was 2.4 years (0.3-14 years). RESULTS: Mean systolic right ventricular pressures and gradients, respectively, decreased from 71 +/- 18 mm Hg and 48 +/- 19 mm Hg before to 48 +/- 15 mm Hg and 19 +/- 13 mm Hg after stent placement. Mean percentage of predicted valve area for body surface area increased from 26% +/- 12% to 48% +/- 17% after stent placement. Fifteen patients underwent a second transcatheter intervention (dilation or additional stent), and 2 patients, a third, allowing further postponement of surgery in 8 patients. One sudden death occurred 2.8 years after stent placement. Surgical conduit replacement has occurred in 20 patients. Body growth was maintained during follow-up. Freedom from surgical reintervention was 86% at 1 year, 72% at 2 years, and 47% at 4 years. Higher right ventricular pressure and gradient before and after stent placement and lower percentage of predicted valve area for body surface area after stent placement were associated with shorter palliation. CONCLUSION: Endovascular stent placement across obstructed conduits is a safe and effective palliation that allows for normal body growth.

Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections.

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.

Late homograft valve insertion after transannular patch repair of tetralogy of Fallot.

BACKGROUND AND AIMS OF THE STUDY: Transannular patch repair of tetralogy of Fallot leads to pulmonary insufficiency and progressive right ventricular dilatation responsible for a decreased exercise capacity. We studied the impact of late homograft insertion on the regression of the right ventricular volumes in symptomatic patients. METHODS: Between July 1992 and August 1996, 15 consecutive patients (age range: 4 to 24 years) were operated on at a median of 13 years (range: 3 to 20 years) after transannular patch repair of tetralogy of Fallot. All patients complained of exertional dyspnea and fatigue. Syncopes were reported in six patients and four patients had sustained episodes of ventricular tachycardia. Fourteen had pulmonary regurgitation grade 3 or 4 and one had an associated stenosis and insufficiency. All patients had a dilated right ventricle. At reoperation, no patients presented with major aneurysm. The patch was resected and the right ventricular outflow tract reconstructed with a cryopreserved pulmonary homograft. Right ventricular volumes were studied before the procedures and at the last follow up consultation. RESULTS: There was no operative death. One patient who had a concomitant patch repair of a hypoplastic left pulmonary artery needed extracorporeal circulatory support for eight days. After a median follow up of 25 months (range: 3 to 54 months) all patients but one are in NYHA class I. There were no late deaths. The mean end-diastolic diameter of the right ventricle decreased from 36 +/- 9 mm before surgery to 31 +/- 6 mm (not significant). The mean ratio between the end-diastolic diameter of the right and left ventricles decreased from 0.94 +/- 0.3 to 0.74 +/- 0.2 (p < 0.01). CONCLUSION: An increasing number of patients who had transannular patch repair for tetralogy of Fallot will require reoperation for symptomatic long-term pulmonary regurgitation. Homograft reconstruction of the right ventricular outflow tract of these patients induces regression of their right ventricular dilatation and leads to their functional recovery.

Ross procedure in congenital patients: results and left ventricular function.

METHODS: From April 1990 to August 1995, 121 patients (median age 42 years) underwent aortic valve replacement with allografts (69 patients) or autografts (52 patients). In this latter group, 24 Ross procedures have been performed in congenital patients since November 1991 (median age 10 years, range five months to 27 years): aortic incompetence (n = 17), isolated aortic stenosis (n = 5), small stenotic prosthesis (n = 2). Transthoracic echocardiography was obtained preoperatively in all patients and serially after surgery with the aim of measuring aortic and pulmonary annuli and evaluate gradients and incompetence and to study the left ventricular function. Intraoperative transoesophageal echocardiography was routinely used. Complete root replacement was performed in all patients. RESULTS: One patient died in the early postoperative period (4%). There was no late death. All survivors remained in NYHA class I and were free of complications and medications. No gradient nor any significant aortic incompetence could be demonstrated. In 17 patients with predominant aortic incompetence before surgery, the left ventricular function was followed prospectively, end-diastolic left ventricular dimensions diminished drastically from 2 +/- 3.4 S.D. above normal to -0.63 +/- 2.4 S.D. at one week postoperatively (day 10) to reach a normal value one to three months after surgery. Left ventricular mass remained abnormal at day 10 (from 4.7 +/- 3.3 S.D. to 5.3 +/- 3.8 S.D.) and diminished more progressively to reach a normal value (0.14 +/- 1.4 S.D.) at three months. This resulted in a significant decrease of end-systolic wall stress (-3.6 +/- 2.1 S.D.) and in a hyperdynamic function in the immediate postoperative days except in two patients. These two patients were characterized preoperatively by more severely dilated left ventricle (end diastolic dimension 5.3 +/- 0.03 versus 1.6 +/- 3 S.D.) with decreased left ventricular wall thickness (1.19 +/- 0.7 versus 3.44 +/- 1.9 S.D.), decreased ratio between end diastolic wall thickness and end diastolic dimension (0.14 +/- 0.06 versus 0.2 +/- 0.06) and a decreased velocity of shortening. Unlike the other 15 patients, the left ventricular function did not recover completely at mid term follow-up in those two patients. CONCLUSION: The Ross operation is a safe procedure and allows us to suppress completely the abnormal loading conditions of the left ventricle, resulting in a complete recovery of left ventricular function in most patients.

Transcatheter management of residual shunts after initial transcatheter closure of a patent arterial duct.

OBJECTIVES: To assess the efficacy and safety of transcatheter reocclusion of persistent leaks following previously attempted transcatheter occlusion of persistent arterial duct. DESIGN: Retrospective study. SETTING: Tertiary pediatric cardiology centre. PATIENTS: From February 1987 through October 1996, trans-catheter occlusion of a residual ductal shunt was attempted in 42 consecutive patients at a median age of 5.0 years (range 1.6 years to 16.2 years). INTERVENTIONS: Fourty patients had successful placement of a double umbrella occluder (n=27) or coils (n=13) across residual shunts. Complications included device embolization in two patients and hemolysis in one patient. OUTCOME MEASURES AND RESULTS: Mean z-score for left ventricular end-diastolic dimension (LVEDD) at initial echocardiography was +2.55 +/- 1.89 (P<0.0001 versus normal); z-score for left pulmonary artery (LPA) diameter was +2.00 +/- 1.52 (P<0.0001). Mean LPA to right pulmonary artery (RPA) diameter ratio was 1.05 +/- 0.18. At follow-up echocardiogram, a median of two years (range six months to 7.7 years) after the second procedure, a shunt was persistent in 3% of the patients. Mean LVEDD and LPA diameter z-value, and mean LPA to RPA diameter had dropped significantly to +0.42 +/- 1.31, +0.07 +/- 1.15 and 0.86 +/- 0.14 (P<0.001), respectively. LPA flow acceleration was present in 25% of patients. Three of nine patients, in whom lung perfusion scan was performed, had left lung perfusion below 40%. Small weight and age at catheterization were significant risk factors for LPA flow disturbance. CONCLUSIONS: Repeat transcatheter occlusion is safe and successful in eliminating residual shunt across the arterial duct. Attention should, however, be addressed to the potential for LPA stenosis and growth, and flow should be regularly assessed.

[Retro-tracheal left pulmonary artery. Report of 2 cases]. / Artère pulmonaire gauche rétro-trachéale. A propos de 2 cas.

The authors report the cases of two neonates with retro-tracheal left pulmonary arteries (pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardiography showed the vascular anomaly associated with a large ventricular septal defect (VSD). After standard radiological investigation and bronchoscopy to exclude an associated tracheo-bronchial malformation, the VSD was repaired surgically with reimplantation of the left pulmonary artery. Unfortunately, the patient died of major airways obstruction in the postoperative period. Autopsy showed tracheo-bronchial anomalies which had not been diagnosed preoperatively. The second patient presented with hypoventilation of the right lung. After echocardiographic diagnosis of the anomaly, a thorough investigation (thoracic CT, helicoidal scan, bronchoscopy) was carried out and no associated bronchial malformations were observed. Reimplantation of the left pulmonary artery was successful and the postoperative course was uneventful. Retro-tracheal left pulmonary artery is a rare malformation Which is difficult to diagnose. It requires extensive pulmonary investigations and a multi-disciplinary approach. The prognosis is poor when there are associated tracheo-bronchial malformations.