Contegra versus pulmonary homograft for right ventricular outflow tract reconstruction in newborns.

OBJECTIVES: Pulmonary homografts are standard alternatives to right ventricular outflow tract reconstruction in congenital heart surgery. Unfortunately, shortage and conduit failure by early calcifications and shrinking are observed for small-sized homografts in younger patients. In neonates, Contegra® 12 mm (Medtronic Inc., Minneapolis, Minnesota, United States of America) could be a valuable alternative, but conflicting evidence exists. There is no published study considering only newborns with heterogeneous pathologies. We retrospectively compared the outcomes of these two conduits in this challenging population. METHODS: Patients who underwent a right ventricular outflow tract reconstruction between January 1992 and December 2014 at the Hôpital Universitaire des Enfants Reine Fabiola were included. We retrospectively collected and analysed demographic, echocardiographic, surgical, and follow-up data. RESULTS: Of the 53 newborns who benefited from a right ventricular outflow tract reconstruction during the considered period, 30 received a Contegra 12 mm (mean age 15 ± 8 days), and 23 a small (9-14 mm) pulmonary homograft (mean age 10 ± 7 days). Overall mortality was 16.6% with Contegra versus 17.4% in the pulmonary homograft group (p = 0.98 log-rank). Operative morbidity and early re-operation for conduit failure were not significantly different between the two groups. Mean follow-up in this study is 121 ± 74 months. Survival free from re-operation was not different between the two groups (p = 0.15). Multivariable analysis showed that weight and significant early gradient were factors associated with anticipated conduit failure. CONCLUSIONS: Contegra 12 mm is a valid alternative to small pulmonary homografts in a newborn patient population. TRIAL REGISTRATION: NCT03348397.

Pulmonary valve replacement after right ventricular outflow tract reconstruction with homograft vs Contegra®: a case control comparison of mortality and morbidity.

BACKGROUND: Repair of congenital heart defects involving the right ventricular outflow tract may require the implantation of a right ventricle to pulmonary artery conduit. This conduit is likely to be replaced during childhood. This study compares the operative outcomes of the replacement procedure of Contegra® and homografts in pulmonary position. METHODS: From 1999 to 2016, 82 children underwent 87 right ventricle to pulmonary artery conduit replacements (60 Contegra® and 27 homografts). Demographics, operative and clinical data were obtained through a retrospective review of the medical records. The two groups were matched for comparison using propensity score matching. All the procedures were performed by the same team of surgeons. RESULTS: No statistically significant difference was observed between the two groups when considering the operative data for anesthesia, surgery, cardiopulmonary bypass and aortic clamping durations. A peroperative complication rate of 13.47% and 15.36% in Contegra® and homograft replacement groups respectively (p value = 0.758) was observed. There was no difference regarding the blood loss and fluid input. No statistically significant difference was observed between the two groups for the post-operative morbidity. We considered the Pediatric Risk of Mortality (PRISM) score, the day of extubation, the day of withdrawal of inotropic drugs, the length of the intensive care unit stay and the length of hospital stay. The overall mortality is 2.3% but there is no statistically significant difference between the two groups. CONCLUSION: Right ventricle to pulmonary artery conduit replacement procedure can be achieved with a low surgical morbidity or mortality, not influenced by the type of conduit that is replaced. Therefore, the choice between homograft or Contegra® for right ventricle to pulmonary artery reconstruction should not be influenced by the future surgical risk during the replacement procedure. TRIAL REGISTRATION: NCT03048071 . Registered 9 February 2017 (retrospectively registered).

Use of a new low profile Occlutech PLD device for closure of Fontan fenestrations.

For some higher risk patients, fenestrations are still created in total cavopulmonary connection conduits completing the Fontan circulation for univentricular hearts. If these fenestrations remain patent and hemodynamics are favorable, they are closed to improve oxygenation and exercise tolerance and to decrease the risk of paradoxical emboli. Depending on the type of fenestration, different devices are used. Unfortunately, the search for the ideal device is still ongoing. We propose the first experience with the off-label use of the new Occlutech paravalvular leak device. This device offers the combination of a small delivery sheath size, high flexibility, less material, and a low profile. The low profile in particular could be an advantage in order to reduce the risk of thrombus formation in the low velocity flow environment of the Fontan circulation. © 2015 Wiley Periodicals, Inc.

Implantation of an Absorb bioresorbable vascular scaffold in the stenotic aortopulmonary collateral artery of a young child with Alagille syndrome.

Stent implantation in children can be problematic due to the possibility of growth incapacity and difficulties during later surgery. For these reasons, bioresorbable stents may be a good alternative to conventional stents. We report our experience with implantation of an Absorb bioresorbable stent in the stenotic major aortopulmonary collateral artery (MAPCA) of a 1-year-old girl born with pulmonary atresia with ventricular septal defect.

Does congenital heart disease severely jeopardise family life and pregnancies? Obstetrical history of women with congenital heart disease in a single tertiary centre.

AIM: Women with congenital heart disease are often considered to be restricted in their obstetrical life and even their marital life. Our single-centre study aimed to determine the real-life situation of these women with regard to successful family life and any pregnancy complications they may experience. METHODS: From our database of adults with congenital heart disease, 160 of 178 women completed a questionnaire and had their files reviewed. They were classified into three groups according to their pregnancy risk - "good condition" group, no pregnancy restriction; "at-risk" group, pregnancy allowed with close follow-up at a tertiary centre; and "contraindicated" group, pregnancy inadvisable. RESULTS: The proportion of women in a relationship was 46% with no difference between the three groups. In the groups where pregnancy was allowed, 55% of women conceived a child. The total incidence of spontaneous abortion was 21%. The rate of caesarean section was 15%. The incidence of cardiac failure was 4.7%, arrhythmia 1.2%, endocarditis 1.2%, hypertension 2.4%, and preeclampsia 1.2%. Foetal complications included prematurity and/or low birth weight (9.5%) and one foetal malformation (0.82%). CONCLUSION: Women with severe congenital heart disease are willing to start a family and are successful in this enterprise. Although the complication rate during pregnancy in congenital heart disease remains high, with good monitoring these pregnancies occur without severe complications and a low rate of medical abortion or caesarean section.

Long-term electrocardiographic follow-up after repair of tetralogy of Fallot.

BACKGROUND: Fallot patients with conduction disturbances are prone to sudden cardiac death. However, knowledge about long-term electrocardiographic changes after Fallot repair is limited. METHODS: Measurements were performed on electrocardiograms recorded preoperatively, postoperatively, and during annual follow-up in 35 Fallot patients included in three groups: G1 if they received no patch (n = 7), G2 if they received a transannular patch (n = 13), and G3 if they received a pulmonary homograft (n = 15). RESULTS: PR interval increased over the study period in all groups (Z-score: from 0.9 ± 1.1 to 1.3 ± 0.9 in G1, 0.9 ± 1.2 to 1.7 ± 1.6 in G2, and 0.7 ± 0.7 to 1.4 ± 1.3 in G3). The QRS duration increased during the follow-up at a rate of 1.78 msec/year in G1, 2.34 msec/year in G2 despite pulmonary valve replacement in 10 patients, and 1.81 msec/year in G3 despite conduit replacement in 9. At the later follow-up, the QRS duration was significantly increased (Z= 4.5 ± 3.6 in G1, 5.7 ± 1.4 in G2, and 4.6 ± 1.9 in G3). One patient in each group had QRS duration of 170 msec or longer and the one in G3 had a history of serious ventricular arrhythmia. Three patients had a QTc duration above 460 msec. CONCLUSIONS: Progressive conduction disorders are noted during long-term follow-up in Fallot patients who received transannular patch but also in those who received no patch or a pulmonary homograft. It suggests that volume overloading related to the transannular patch but also pressure overloading and myocardial injury related to surgery contribute to their development.

Pericarditis as a rare complication of pneumococcal pneumonia in a young infant.

Purulent pericarditis is an exceptionally rare complication of pneumococcal pneumonia in infants but a rapidly fatal disease if left untreated. A previously healthy 4-month-old boy presented at our emergency department with a 10-day history of fever and non-productive cough. No signs of heart failure or cardiac friction rub were evidenced. Chest radiography showed lobar pneumonia, right pleural effusion and cardiomegaly. Echocardiography revealed a massive pericardial effusion, and an emergency drainage was performed. Streptococcus pneumoniae grew up from purulent pericardial fluid and blood cultures. After intravenous antibiotherapy, the outcome was favourable. The introduction of the pneumococcal vaccine may favour an increase in the incidence of non-vaccine serotypes which most commonly cause empyaema and perhaps pericarditis. Therefore, pericarditis should always be considered a possible complication in patients with pneumococcal pneumonia and empyaema.

Prevalence of preoperative arrhythmias in children with delayed treatment of severe congenital heart disease.

AIM: Our aim was to determine the real importance of rhythm and conduction disorders in children with unoperated severe congenital heart disease. METHODS: Consecutive children with delayed treatment of severe congenital heart disease were prospectively studied for the occurrence of arrhythmias before any invasive investigation or surgical procedure was performed. RESULTS: All 168 children were in sinus rhythm. One hundred and fifty-eight patients (94%) had no significant preoperative findings. One child with double discordance had an intermittent complete atrioventricular block, and another one had a long QT syndrome. Children with severe ventricular dysfunction had paroxysmal atrioventricular re-entry tachycardia in 3 cases and abnormally frequent premature ventricular complexes in 3 other cases. Children with severe left atrial dilatation had periods of atrial ectopic tachycardia in one case and atrial fibrillation in another case. CONCLUSIONS: The prevalence of rhythm and conduction disorders is relatively low in children with delayed treatment of severe congenital heart disease. Only those with congenital heart disease classically combined with such disorders and those with prolonged severe ventricular dysfunction and/or atrial dilatation are at risk of developing significant arrhythmias and should undergo a preoperative assessment of arrhythmias.

The effect of bosentan in patients with a failing Fontan circulation.

OBJECTIVES: To investigate the effect of bosentan in patients with a failing Fontan circulation. DESIGN: A multicentric open label, non-controlled study. SETTING: 5 tertiary care centres for congenital cardiology. PATIENTS: We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded. RESULTS: Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued. CONCLUSIONS: Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

Chronotropic impairment after surgical or percutaneous closure of atrial septal defect.

BACKGROUND: In previous studies, an attenuated heart rate response to exercise has been noted in patients after surgical closure of atrial septal defect. The aim of this study was to compare the prevalence of chronotropic impairment after surgical and percutaneous closure of atrial septal defect. METHODS: Thirty-eight pediatric patients who underwent a surgical (group A, n = 18) or transcatheter closure (group B, n = 20) of atrial septal defect in our institution were prospectively included in the study. Treadmill exercise testing was performed using the Bruce walking treadmill protocol to voluntary exhaustion, with continuous monitoring of heart rate and oxygen consumption. RESULTS: All the children were in sinus rhythm, and had normal values for peak oxygen uptake and endurance time. Exercise heart rate was significantly lower than normal in group A at the end of stage 2 (Z-score = -0.71 +/- SD 1.02), and in both groups at the end of stage 3 (Z-score = -2.06 +/- 1.76 in A and -1.00 +/- 0.71 in B) and at peak exercise (Z-score = -2.78 +/- 2.14 in A and -0.81 +/- 0.75 in B). However, the heart rate response to exercise was significantly less attenuated in group B than in group A. Moreover, maximal heart rate was <-2 SD in 8/18 surgical patients but in no patient of the group B. CONCLUSION: The chronotropic impairment is significantly less important after transcatheter closure of atrial septal defect than after surgical closure. It is an additional argument in favor of the interventional catheterization.

Workload of ambulatory activities in a tertiary paediatric cardiac centre.

BACKGROUND: Few data are available on the evolution in the number of referrals and the spectrum and frequency of issues addressed in paediatric cardiac outpatient clinics. AIM: To assess the volume and range of symptoms and diagnoses in patients, referred to a paediatric cardiac outpatient clinic in an academic hospital setting. METHODS: Data were collected prospectively over 6 months. RESULTS: Historical comparison showed that the number of outpatient visits increased from 819 during the first semester of 2004 to 865, 1045 and 1391 during the first semesters of 2005, 2006 and 2007, respectively. During the 6-month study period in 2007, 854/1391 visits concerned patients with known heart disease; the reason for the visit was follow-up of congenital heart disease (n=616 children, 128 adults), arrhythmia (n=91) or acquired heart disease (n=19). During the visit, the decision to perform diagnostic or therapeutic cardiac catheterization or a surgical procedure was taken in 47 cases. Foetal echocardiography was performed in 60 foetuses and was abnormal in 21 cases. Among the other 477 visits, which concerned patients without known heart disease, the most frequent clinical concern was cardiac murmur (n=193) and there were new diagnoses of congenital heart disease (n=28), ventricular dysfunction (n=2) and arrhythmia (n=7). CONCLUSIONS: The ambulatory paediatric cardiology workload in tertiary academic hospital settings is increasing alarmingly. These data may be helpful in future planning of consultant manpower and in curriculum development for cardiac training of students and residents.

Epidemiology of heart failure in a tertiary pediatric center.

BACKGROUND: In contrast to the adult age group, epidemiologic studies on heart failure (HF) in the pediatric population are lacking. The aim of this prospective study was to analyze the epidemiology of HF during infancy and childhood. METHODS: Of the 1,196 children with congenital and acquired heart diseases, primarily diagnosed during a 10-y period in one hospital, we identified those patients who developed HF. RESULTS: Within 10 y, 1,196 children with heart disease were indexed. Heart failure occurred in 124 of these patients (10.4%): 64 out of 1,031 children with congenital heart diseases (6.2%), 13 out of 96 children with rhythm or conduction disturbances (13.5%), 23 out of 39 children with acquired heart diseases (59.0%), and 24 out of 30 children with cardiomyopathies (80.0%). Heart failure occurred in 72 cases (58.1%) during the first year of life. The incidence in infancy was much more pronounced for congenital heart diseases than for other cardiac conditions. The mortality associated with HF and its cause was also lower for children with congenital heart disease (4.7%) than for the other cardiac conditions (8.7%, 23.0%, and 25.0%, for acquired heart diseases, rhythm disturbances, and cardiomyopathies, respectively). CONCLUSION: Clear differences exist between HF in infants and HF in children. Our study supports the observation that congenital heart disease is the most common causative factor of HF during infancy. Older children with HF are more likely to have acquired heart diseases, cardiomyopathies, and arrhythmias, and these conditions have a considerable mortality.

Noncardiac comorbidities of congenital heart disease in children.

BACKGROUND: Noncardiac conditions may complicate heart disease care, contribute to the progression of the disease and alter the response to treatment. The aim of this prospective study was to evaluate the proportion of cardiac children with such significant conditions. METHODS: Of the 1058 children with congenital heart disease primarily diagnosed during a 10-year-period in one hospital, we identified those patients who had significant congenital and acquired comorbidities. RESULTS: Associated problems were diagnosed in 224 children (21.2%). Among them, 118 children (11.2%) had genetic or syndromic conditions, of which 38 had Trisomy 21. Six subspecialty areas accounted for the vast majority of the cases: neurology (n = 140), pulmonology (n = 36), orthopaedics (n = 26), nephro-urology (n = 19), gastroenterology (n = 14) and endocrinology (n = 13). The most frequent associated conditions were mental retardation, asthma, epilepsy and scoliosis. During the study period, death occurred in 46 of the children (4.3%) and was not related to the cardiac disease in 20 cases (1.9%). CONCLUSION: A substantial proportion of children with congenital heart disease have significant noncardiac comorbidities. Close collaboration between paediatric cardiologists and paediatricians of other subspecialties is imperative to optimize care for these children.

Early results of valved bovine jugular vein conduit versus bicuspid homograft for right ventricular outflow tract reconstruction.

BACKGROUND: Homograft conduits are preferable for right ventricular outflow tract reconstruction in children, but their limited availability remains a major concern. Recently, a valve-containing segment of bovine jugular vein (Contegra, Medtronic Inc, Minneapolis, MN) has been introduced as a potential alternative conduit. METHODS: Early clinical and echocardiographic results of right ventricular outflow tract reconstruction were retrospectively compared between 41 children (mean age, 1.9 years), receiving a Contegra conduit and 36 patients (mean age, 2.7 years) with a size-reduced pulmonary homograft. RESULTS: Clinical outcome was comparable with two early deaths in the homograft group and one in the Contegra group. There were no conduit-related complications in either population. Early echocardiographic assessment showed only trivial to mild regurgitation in 9 homografts versus 17 Contegra conduits. The peak gradient across the right ventricular outflow tract conduit was comparable for both groups, although a larger number of patients, treated with a downsized homograft, had a small gradient at the distal junction with the pulmonary arteries (12 versus 6 patients). None of the patients had a gradient at the valvar level. CONCLUSIONS: The valved bovine jugular vein conduit offers a promising substitute for right ventricular outflow tract reconstruction in infants and children, with an early hemodynamic performance that compares favorably with downsized, bicuspid homografts. Clinical advantages are greater shelf availability and the natural continuity between valve and conduit, which allows proximal infundibular shaping without additional material. However, durability must be determined, even though most of these children will require right ventricular outflow tract reoperation after outgrowing the conduit.