François Sassolas

Current patterns of infective endocarditis in congenital heart disease

Objective: To assess the changing profile of infective endocarditis in patients with congenital heart disease. Methods: All cases diagnosed from 1966 to 2001 (revised Duke criteria) were retrospectively reviewed and categorised in periods I (< 1990) and II (⩾ 1990). Results: 153 episodes occurred, 81 in period I and 72 in period II. Mean age of affected patients was higher in period II. Non-operated ventricular septal defect, Rastelli correction and palliated cyanotic heart disease increased. Infective endocarditis in corrective surgery changed to patients with prosthetic material. Post-surgical cases decreased. Dental problems were the leading cause (period I 20% v II 33% of cases) with a large variety of pathological organisms (multiple species of Streptococcus). Cutaneous causative infections increased (5% to 17%) with different species of Staphylococcus. Negative blood cultures lessened (20% to 7%, p  =  0.03). Streptococci were the most common causative organisms in both periods. Severe heart failure and cardiac complications lessened (20% to 4% and 31% to 18% during periods I and II, respectively). Early surgery was more frequent in period II (32% v 18.5%, p  =  0.02). One- and 10-year survival was 91% v 97% in period I and 89% v 97% in period II, respectively (NS). Conclusion: Current targets include complex cyanotic disease, congenital heart disease corrected with prosthetic material and small ventricular septal defect. Postoperative cases lessened; dental and cutaneous causes increased. Survival was unchanged. Prophylactic measures targeted at dental and cutaneous sources should be emphasised.

Rabbit antithymocyte globulin as induction immunotherapy in pediatric heart transplantation.

Background. There is little published data on the use of antithymocyte globulins in children. This retrospective study describes the use of Thymoglobulin (Imtix, SangStat, Lyon, France) in pediatric cardiac transplantation over a 13-year period in a single center that adjusted the dose of Thymoglobulin according to platelet count monitoring and examines the short-term hematological effects as well as longer-term outcomes. Methods. Data for all children who received a heart transplant at the Hôpital Cardiologique at Lyon from 1984 to 2001 and who were given Thymoglobulin as part of their immunosuppressive protocol were extracted. The dose of Thymoglobulin given depended on baseline platelet count and was 2, 1.5, or 1 mg/kg per day over 5 days for the following platelet count groups: greater than 150,000/mm3 (normal group), 100 to 150,000/mm3 (mild thrombocytopenia group), and 50 to 100,000/mm3 (moderate thrombocytopenia group). Results. Thirty children of median age 14.2 years were given a median cumulative dose of Thymoglobulin of 8 mg/kg per patient; the moderate thrombocytopenia subgroup was given significantly less (6.4 mg/kg) (P =0.032). Immediate tolerability of Thymoglobulin was good, with no cases of first-dose syndrome, anaphylaxis, or serum sickness. The platelet count decreased at the start of therapy, but recovered after discontinuation, and did not give rise to clinical concern. Patients were followed up for a median of 6.3 years (7 days–15.5 years); actuarial survival was 90%, 86%, and 74.5%, respectively, at 1, 5, and 10 years. In the first year, 50% of patients suffered an episode of rejection. The overall incidence of infection in the month following transplantation was 40%. One lymphoma occurred at 5 months. Conclusions. The use of Thymoglobulin in pediatric heart-transplant patients as part of an immunosuppressive protocol, with dose adjustment according to platelet levels, has been shown to be effective in terms of rejection rate and patient survival and safe in terms of the incidence of infections and malignancy.

Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study

BACKGROUND Despite serious long-term sequel, women with Fontan palliation have reached childbearing age. However there is paucity of data on the pregnancy outcomes and management of this condition. We aimed to determine the maternal and fetal outcomes of pregnancy in women with Fontan palliation. METHODS This multicentric, retrospective study included women with Fontan circulation followed in 13 French specialized centers from January 2000 to June 2014. All pregnancies were reviewed, including miscarriages, abortions, premature and term births. We reviewed maternal and fetal outcomes. RESULTS Thirty-seven patients had 59 pregnancies. Mean age was 27 ± 5 years at first pregnancy. There were 16 miscarriages (27%) and 36 live births with 1 twin pregnancy. Cardiac events occurred in 6 (10%) pregnancies, with no maternal death. The most common cardiac complication was atrial arrhythmia, which occurred in 3 patients. Hematological complications including thromboembolic/hemorrhagic events (n=3/7) occurred in 5 women antepartum (n=2/3), and 4 women postpartum (n=1/4). Two of the 3 thromboembolic events occurred in patients without anticoagulation. There was a high incidence of prematurity (n=25/36, 69%). Anticoagulation was associated with adverse neonatal outcome (OR=10.0, 95% CI [1.5-91.4], p<0.01). After a median follow-up of 24 months, there was no significant worsening of clinical status and thromboembolic disease noted. CONCLUSIONS Pre-selected women can successfully complete pregnancy with Fontan circulation. There is an increase in cardiac and neonatal morbidity during pregnancy. Because thromboembolism could have a severe consequence on Fontan circulation, anticoagulation should be indicated during pregnancy and postpartum period.

Mechanical valve in aortic position is a valid option in children and adolescents.

OBJECTIVE The choice of a valve substitute remains a challenge in young patients, with numerous reports of early degeneration and calcification of biological valves in this age group. Therefore an assessment of the long-term results after mechanical aortic valve replacement in children was initiated. METHODS A retrospective study was conducted in 54 consecutive patients aged 1.1 to 17 years (mean 12.8 +/- 4 years) operated on between 1975 and 1993. Aetiology was congenital in 34 patients, rheumatic in 13, infectious in 5, and dystrophic in 2. Concomitant surgery included mitral valve replacement (10), aortic annulus enlargement (9), correction of truncus arteriosus (7), Bentall operation (2), coarctation repair (2), tricuspid valvuloplasty (2), correction of double outlet right ventricle (1), and replacement of a right ventricle to pulmonary artery conduit (1). A Bjork-Shiley valve was implanted in 14 patients, and a St Jude Medical valve in 40. All patients were given Warfarin with a monthly INR control. Follow-up was completed through questionnaires mailed to referring physicians and direct clinical examination. RESULTS Overall early mortality was 13% (7 cases), and 6% (2 cases) in the 32 patients operated on after 1984. Follow-up was complete in 45 survivors (2 lost to follow-up), with a total follow-up of 261 patient-years. There were 6 late deaths, 4 being cardiac and due to persistent LV dysfunction, and 2 valve-related, due respectively to major gastro-intestinal bleeding and massive thromboembolism. Linearized rates of valve thrombosis and anticoagulant-related hemorrhage were both 0.3% per patient-year. Actuarial survival rate was respectively 84.5% at 5 years and 70.2% at 10 years. Reoperation was necessary in 3 patients for recurrent LV outflow tract obstruction. One patient with severe LV dysfunction is awaiting a heart transplant. CONCLUSION We conclude that the longterm outcome after mechanical aortic valve replacement in children and adolescents is satisfactory and comparable to currently available reports on biological substitutes. The mandatory anticoagulant therapy is well tolerated in this age group.

Long-term clinical and hemodynamic evaluation of porcine valved conduits implanted from the right ventricle to the pulmonary artery.

OBJECTIVE This retrospective study was initiated to evaluate the long-term results of valved prosthetic conduits implanted in the right ventricular outflow tract in patients with complex ventricular-pulmonary discontinuity. METHODS A cohort of 103 patients out of 127 (24 early deaths, 19%) operated on between 1973 and 1996 with porcine valved conduits was available for evaluation, with a follow-up ranging from 1 to 21.6 years (mean follow-up 8.4 +/- 6 years). A total of 74 hemodynamic studies were performed after the operation, 50 patients having undergone at least 1 cardiac catheterization during the follow-up period. RESULTS There were 16 late deaths, and the actuarial survivals, including early mortality, were 72.9% +/- 4% at 5 years, 63.1% +/- 5% at 10 years, and 58.2% +/- 5% at 15 years, at which time 20 patients were still available for review and exposed to the risk of dying. The mean peak systolic gradient across the right ventricular outflow tract was plotted as a function of time, showing a gradual increase and a significant step-up after the eighth year, from 43 +/- 36 to 69 +/- 19 mm Hg (P < .005). Reoperation was required for progressive conduit obstruction between 1.1 and 17.7 years after implantation (mean 7.4 +/- 4.8 years) in 25 patients (24%, 70% CL 15%-33%), with generally very few symptoms, or for residual ventricular septal defect in 3 patients. Freedom from reoperation was 79.5% +/- 5% at 10 years and 65.8% +/- 7% at 15 years. CONCLUSIONS Porcine conduits may represent a valuable alternative to biologic substitutes with similar long-term results. Given the few symptoms, progressive conduit stenosis after the eighth postoperative year imposes a yearly noninvasive patient evaluation during the follow-up.

Non-invasive detection of coronary artery disease by dobutamine-stress echocardiography in children after heart transplantation

BACKGROUND Coronary vasculopathy is the main cause of cardiac graft failure. Because yearly coronary angiography is invasive in children, a non-invasive method for detecting graft vasculopathy is needed. The aim of this study was to test dobutamine-stress echocardiography in a pediatric population to determine its feasibility, safety and reliability in the detection of graft coronary artery disease. METHODS Eighteen patients, aged 2 days to 16.8 years at transplantation (mean 8.4 years), underwent 44 dobutamine-stress echocardiography (DSE) exams, at a follow-up of 1.1 to 11.8 years (mean 5.1 years). Selective coronary angiography was performed for comparison. Echocardiographic recordings were obtained in 4 standard views of the left ventricle and measurements carried out within the frames of a 16-segment model. Segmental scores of contractility were obtained for each segment and a total segmental contractility index was calculated at each stage. RESULTS All patients reached the maximum dose stage. Maximum heart rate was 57% to 90% of predicted maximum. Maximum systolic blood pressure reached 190 mmHg. Segmental scores were normal in 37 and abnormal in 7 cases. Echographic results were concordant with angiography in 82% and discordant in 18% of the cases (4 negative DSEs with minor angiographic lesions, 2 positive DSEs with normal angiography), but there was no significant angiographic lesion with normal DSE. CONCLUSIONS DSE is a safe and highly feasible non-invasive technique in transplanted children. A normal DSE study successfully predicts the absence of significant coronary artery disease in the post-transplant population.

La prévention de l'endocardite infectieuse chez l'enfant. Situation et protocoles actuels

Summary Infective endocarditis remains a severe, potentially lethal disease, which justifies a rigourous prevention schedule. Children with cyanotic congenital heart disease, mitroaortic valvulopathies, prosthetic valve and uncorrected ventricular septal defect are the most susceptible. Dental care is the main cause of bacterial graft, followed by upper respiratory tract and cutaneous infections. Prevention is mainly based upon antibiotic prophylaxis but patient education and good dental hygiene are also important.

Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus

BACKGROUND Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. CONCLUSION Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.

0532: Features and outcomes of acute myocarditis in children

This study was to assess features and outcomes of children with acute myocarditis. Methods Patients 10y). Results 72 patients were included (1983 to 2012), 30males, aged 4.1±5.1y (med1.5y): 43 in group I, 17 in II and 12 in III. Heart failure was present at onset in 57(78%): 8 cardiogenic shock (12%), 30 severeHF (44%) were more frequent in I (56%) and II (46%) than in III (17%, p in 3). Nine patients died (13%) within 2months post-diagnosis (2days to 8.6months), 1 was transplanted (3rdmonth), 19 have sequellae (27.5%), 40 recovered (58%), at FU= 5.5±5.6y. Inotrope was needed in 34(47%):51%, 59% and 16% of groups I, II and III respectively (p Download : Download full-size image Abstract 0254 - Figure: Increase in HMDP heart retention after 16months. Conclusion Acute myocarditis in children has favourable outcomes despite early mortality. Myocardial dysfunction and heart failure are less frequent in patients > 10years. Mechanical circulatory support lessens mortality. Myocardial contractility progressively improves within the first 6months after onset.

0450: Outcomes of pregnancy in women with Fontan palliation

Background Fontan palliation has improved long-term survival of (functional) single ventricles, allowing women to reach childbearing age. Outcomes of pregnancy are scarce and the management during pregnancy, as preventive anticoagulation, should be specified. We aimed to determine the outcomes of pregnancy in women with Fontan palliation. Method This retrospective multicentric study included women who had undergone Fontan palliation, followed in 12 French centers. All pregnancies were counted, including miscarriages and abortions. We observed maternal, obstetrical and neonatal outcomes, and we compared cardiovascular status before pregnancy and at the last follow-up. Results Twenty seven patients had 42 pregnancies (mean age=27±5 years old at first pregnancy). 15/42 (36%) were miscarriages and 24 were live births (62% of wanted pregnancies, 1 twin pregnancy). Cardiac complications occurred in 21% of pregnancies, there was no maternal death. They were essentially thrombo-embolic events (n=3) and supra-ventricular arrhythmias (n=2). Obstetrical complications occurred in 52% of pregnancies. They were premature rupture of membranes (n=5), preterm labour (n=3), placental abruption (n= 2). Hemorrhagic complications occurred in 3 women, 2 had curative anticoagulation. There were 78% of fetal/neonatal complications including one intrauterine death and one neonatal death. The main neonatal complication was the prematurity (n=17/24, 71%), and we observed 2 cases of congenital heart disease recurrence in fetuses (5%). Three severe complications occurred during postpartum period: 2 failing Fontan and 1 increase of NYHA functional class.Follow-up at mid-terms (median=5.6 months) showed no significant worsening of clinical status and cardiac function in patients. Conclusion Women can successfully complete pregnancy after a Fontan palliation, only if pre-partum cardiac condition has been completely evaluated and is satisfactory. Anticoagulation should be recommended to prevent severe thrombo-embolic complications during pregnancy.