Infective endocarditis in patients after percutaneous pulmonary valve implantation with the stent-mounted bovine jugular vein valve: Clinical experience and evaluation of the modified Duke criteria.

AIMS: Percutaneous pulmonary valve implantation (PPVI) has proven good hemodynamic results. As infective endocarditis (IE) remains a potential complication with limited available clinical data, we reviewed our patient records to improve future strategies of IE prevention, diagnosis and treatment. METHODS: Medical records of all patients diagnosed with Melody® valve IE according to the modified Duke criteria were retrospectively analyzed in three Belgian tertiary centers. RESULTS: 23 IE episodes in 22 out of 240 patients were identified (incidence 2.4% / patient year) with a clear male predominance (86%). Median age at IE was 17.9 years (range 8.2-45.9 years) and median time from PPVI to IE was 2.4 years (range 0.7-8 years). Streptococcal species caused 10 infections (43%), followed by Staphylococcus aureus (n = 5, 22%). In 13/23 IE episodes a possible entry-point was identified (57%). IE was classified as definite in 15 (65%) and as possible in 8 (35%) cases due to limitations of imaging. Echocardiography visualized vegetations in only 10 patients. PET-CT showed positive FDG signals in 5/7 patients (71%) and intracardiac echocardiography a vegetation in 1/1 patient (100%). Eleven cases (48%) had a hemodynamically relevant pulmonary stenosis at IE presentation. Nine early and 6 late percutaneous or surgical re-interventions were performed. No IE related deaths occurred. CONCLUSIONS: IE after Melody® valve PPVI is associated with a relevant need of re-interventions. Communication to patients and physicians about risk factors is essential in prevention. The modified Duke criteria underperformed in diagnosing definite IE, but inclusion of new imaging modalities might improve diagnostic performance.

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.

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.

[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.

Changes in perceived health of children with congenital heart disease after attending a special sports camp.

Sports camps for children with cardiac anomalies have existed for many years. However, no formal evaluation of the benefits of attending such camps has been undertaken heretofore. We assessed potential changes in the self-perceived health of children with congenital heart disease who attended a special sports camp. Thirty-one children with cardiac anomalies attended a 3-day multisports camp. Sixteen children, all of whom were 10 years or older, literate, and Dutch- or French-speaking, completed the Child Health Questionnaire (CHQ-CF87) before and after attending the camp. The scores of the children were compared with those of healthy peers by calculating mean standardized differences. After attendance at the sports camp, the children achieved significant improvements in the self-perception of their physical functioning, role functioning due to emotional problems, role functioning due to behavioral problems, mental health, and general behavior. The children's self-esteem and general behavior after the camp were significantly better than that of their healthy counterparts. We conclude that children with congenital heart disease who participate in activities at special sports camps may reap benefits in terms of their subjective health status. Although further research is needed, we recommend the participation in sport activities by children with heart defects, and more specifically their participation in sports camps.

[Risks and benefits of cardiac surgery in Down's syndrome with congenital heart disease]. / Avantages et risques de la chirurgie cardiaque dans la trisomie 21.

UNLABELLED: Down's syndrome is the most frequent chromosomic anomaly. Fifty percent are associated with a congenital heart disease. Life expectancy and quality of life are increasing since 15 years with improving surgical techniques. PATIENTS AND METHODS: This study presents 73 patients with Down's syndrome who underwent a surgical repair of congenital heart disease between 1992 and 2002. Among them, 37 (50.6%) had a complete atrio-ventricular septal defect (AVSD) and 36 an other anomaly. RESULTS: Five children died in the post operative period: all had an AVSD. Two late deaths occurred due to extra cardiac causes during the follow up. Two children who underwent a repair of AVSD had to be reoperated. The majority of the children are doing well, without any cardiac symptom. Thirty-eight percent of patients with AVSD repair have a middle mitral or tricuspid regurgitation and three had a massive mitral or tricuspid regurgitation. One child presents neurological sequelae related to surgery. CONCLUSION: Congenital heart diseases in Down's syndrome can be repaired with a limited risk of death. Final results are good for many children with significant improvement of the quality of life and life expectancy.

Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium.

OBJECTIVE: To assess long term outcome of patients who underwent Mustard or Senning repair for transposition of the great arteries up to 30 years earlier. DESIGN: Retrospective review of medical records. SETTING: The six university hospitals in Belgium with paediatric cardiology departments. PATIENTS: 339 patients were reviewed, of whom 124 underwent the Mustard procedure and 215 the Senning procedure. This represents almost the entire population of patients in Belgium with either simple or complex transposition. MAIN OUTCOME MEASURES: Mortality, morbidity, functional abilities, social integration. RESULTS: Overall mortality was 24.2%. Early mortality (< or = 30 days after surgery) accounted for 16.5%, late mortality for 7.7%. Actuarial survival of early survivors at 10, 20, and 30 years after surgery was 91.7%, 88.6%, and 79.3%, respectively. Patients in the Senning cohort had a slightly better survival rate than those in the Mustard cohort (NS). Baffle obstruction occurred more often after Mustard repair (15.3%) than after the Senning procedure (1.4%). Arrhythmia-free survival did not differ between the two cohorts, but was determined by the complexity of the transposition. Survivors of the Senning cohort had better functional status, and tended to engage in more sports activities. CONCLUSIONS: The long term outcome for patients surviving the Mustard or Senning operation was favourable in terms of late mortality, morbidity, functional, and social status. Overall mortality in the Senning cohort did not differ from the Mustard group, but Senning patients had better functional status, greater participation in sports activities, and fewer baffle related problems.

[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.

The midwall stress-velocity relationship during manipulation of afterload.

Despite providing a physiological correction, measurements of contractility using the midwall stress-velocity relationship still show evidence of an unexplained hypercontractile state in some children. We investigated if by using midwall shortening indexes, the known overestimation of contractility at low afterload could be prevented. In 12 piglets (5 or 6 weeks old), afterload was manipulated by balloon occlusion of the descending aorta and infusion of sodium nitroprusside up to 5 mg/kg/min, and left ventricular function was measured using multiple variables. The regression line between the echocardiographically derived midwall velocity of circumferential fiber shortening and end systolic wall stress differed from the regression line of the endocardial stress-velocity relationship. Although the midwall regression line was almost horizontal (or afterload independent) for end systolic wall stress values of more than 30 g/cm2, the slope was still steeper below a certain point of afterload. The increased midwall velocity of fiber shortening at low afterload is comparable to the endocardial stress-velocity relationship and could account for the pseudo-hypercontractile state found in some children.

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.

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.

Hemodynamic changes in patients with Alagille's syndrome during orthotopic liver transplantation.

UNLABELLED: Children with Alagille's syndrome are at increased perioperative risk during orthotopic liver transplantation due to the cardiopulmonary abnormalities and the hemodynamic changes associated with this procedure. We studied 16 children with Alagille's syndrome who underwent 21 orthotopic liver transplantations. Peripheral pulmonary stenosis was present in all subjects. Right ventricular pressures were increased in 15 cases. Caval clamping resulted in a mean decrease of 15 +/-9 mm Hg in systolic blood pressure, 5 +/- 3 mm Hg in mean pulmonary artery pressure, and 4 +/- 3 mm Hg in central venous pressure. Systolic blood pressure decreased by 16 +/- 13 mm Hg, whereas mean pulmonary artery pressure and central venous pressure increased by 3 +/- 4 mm Hg and 1 +/- 4 mm Hg, respectively, at portal vein unclamping. There was no correlation between severity of pulmonary artery stenosis and hemodynamic changes. Veno-venous bypass used in four cases resulted in smaller hemodynamic changes. Time to extubation and duration of intensive care unit stay were unrelated to severity of pulmonary artery stenosis. IMPLICATIONS: Some children with Alagille's syndrome require liver transplantation. In our study, associated pulmonary artery stenosis did not dramatically increase perioperative risk. Veno-venous bypass decreased intraoperative hemodynamic changes in these patients.

The Ross operation: mid-term results.

BACKGROUND: The Ross operation, although more demanding, is now widely accepted as an alternative solution for aortic valve replacement in young adults and children. A review of our experience to assess the mid-term results with the Ross operation is presented. METHODS: From June 1991 through October 1997, 80 patients (mean age, 31 years) underwent aortic valve or root replacement with pulmonary autografts. Indications for operation were predominant aortic stenosis in 38 patients, aortic incompetence in 42 patients including endocarditis in 3 patients. Congenital lesions were present in 57 patients, either at pediatric (27 patients) or adult age (30 patients). Transthoracic echocardiography was performed preoperatively in all patients and serially after operation with the aims of measuring aortic and pulmonary annuli, evaluating transvalvular gradients and incompetence, and studying the left ventricular function. Intraoperative transesophageal echocardiography was used routinely. Complete root replacement was performed in 52 patients, intraluminal cylinder in 25 patients, and subcoronary implantation in 3 patients. RESULTS: One patient died in the early postoperative period (1.2%). There was no late death. The actuarial survival at 5 years was 98%+/-1%. All survivors remained in New York Heart Association functional class I and were free of complications and medications. No gradient or significant aortic incompetence could be demonstrated in 73 patients. One patient developed late aortic incompetence grade 3 and reoperation is considered. On the pulmonary outflow tract, 6 patients had gradients between 20 and 40 mm Hg as calculated on echocardiography. CONCLUSIONS: The pulmonary autograft gives excellent mid-term results with low mortality and no morbidity. It completely relieves the abnormal loading conditions of the left ventricle, resulting in a complete recovery of left ventricular function in most patients.

Influence of age and low afterload on the stress-velocity relation of the left ventricle.

The relation between systolic meridional wall stress (WS) and velocity of circumferential fiber shortening (VcFc) is widely accepted as a preload-independent index of contractility, with a linear relation in most subjects older than 2 y. However, this relation seems to become different in infants and after administration of inotropic agents. We decided to study the nature of the stress-velocity relation by a cross-sectional assessment of the influence of age, low afterload, and increased contractility. Study subjects were 30 healthy infants, 32 healthy older children, and 35 asymptomatic older children after completion of anthracycline chemotherapy. WS and VcFc at rest were studied in these infants and children. WS and VcFc were also studied after dobutamine infusion in both groups of older children. Linear regression analysis of the stress-velocity relation showed parallel slopes between the older children at rest and the post anthracycline children after dobutamine. The regression lines between the infants at rest and the healthy older children after dobutamine were also parallel, but with a different and steeper slope compared with the former groups. When comparing the stress-velocity relation of the overall population at rest with the overall population after dobutamine, the resulting regression lines are curvilinear and parallel, with a steeper slope at low afterload. The stress-velocity relation in infants and after dobutamine, resulting in low afterload is different compared with the stress- velocity relation in older children at rest and at higher afterload. Data of the overall population at rest and after dobutamine suggest a curvilinear relation.

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.