Pascal Amedro

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

BACKGROUNDnDespite 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.nnnMETHODSnThis 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.nnnRESULTSnThirty-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.nnnCONCLUSIONSnPre-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.

Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals

OBJECTIVEnThe study objective was to report the long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals.nnnMETHODSnSince 1991, 20 patients with profound pulmonary artery hypoplasia (mean Nakata index 26 ± 14 mm(2)/m(2)) have undergone a medico-surgical strategy of native pulmonary artery rehabilitation to achieve complete repair with satisfactory hemodynamics (right ventricle to aortic pressure ratioxa0<xa00.8).nnnRESULTSnThe first step, right ventricle to pulmonary artery connection, was performed at a median age of 4.1 (0.1-18.7) months with 1 operative death. After a median duration of 4.3 (1.1-26) months, the second step of interventional catheterizations followed (median, 2 (1-7)/patient), consisting of 36 pulmonary angioplasties, 11 stent implantations, and 20 collateral occlusions. Significant pulmonary artery growth was obtained in all cases with a Nakata index of 208 ± 85 mm(2)/m(2) before surgical correction (Pxa0<xa0.001). The third step of surgical repair was performed at a median age of 1.9 (0.6-10.7) years, with right ventricular outflow reconstruction and ventricular septal defect closure fenestrated in 3 cases. During a mean follow-up of 8.2 ± 4.5 years, pulmonary artery rehabilitation was pursued in most patients, with 47 pulmonary angioplasties, 15 stent implantations, and 11 collateral occlusions. Three patients with a poor hemodynamic result died. At last visit, the 16 survivors are in New York Heart Association class I (nxa0=xa012) or II (nxa0=xa04) with satisfactory hemodynamics in 13 cases.nnnCONCLUSIONSnPulmonary artery rehabilitation allows complete repair in the majority of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals. However, long-term management often requires pursuit of the rehabilitation process.

How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease

Survival into adulthood of patients with unrepaired cyanotic congenital heart defects (CHDs) is possible when cyanotic CHDs are deemed unsuitable for radical surgical repair but are compatible with survival. These situations include, for example, complex pulmonary atresia with aortopulmonary collaterals and single-ventricle hearts (with and without earlier palliation). This is also the case when patients with cyanotic CHDs reach adulthood without serious symptoms requiring surgery, such as those with mild tetralogy of Fallot, Ebstein’s anomaly, and some cases of corrected transposition of the great arteries with pulmonary stenosis and ventricular septal defect. Many women with these heart conditions wish to become pregnant, which creates a situation of high maternal and fetal risks of complications.1 Management of these patients before, during, and after pregnancy has improved, with an earlier recognition of the underlying disease, improved understanding of cardiopulmonary physiopathology, better prenatal and peri-partum obstetric/anesthetic management, and the introduction of a multidisciplinary approach.2nnWe retrospectively reviewed the charts of all pregnant women with cyanotic CHDs (n=51) who were followed in 11 adult CHD referral …

Cardiopulmonary fitness in children with congenital heart diseases versus healthy children

Objective We aimed to compare the cardiopulmonary fitness of children with congenital heart diseases (CHD) with that of age-adjusted and gender-adjusted controls. We also intended to identify clinical characteristics associated with maximum oxygen uptake (VO2max) in this population. Methods and results We included in a cross-sectional multicentre study a total of 798 children (496 CHD and 302 controls) who underwent a complete cardiopulmonary exercise test (CPET). The association of clinical characteristics with VO2max was studied using a multivariate analysis. Mean VO2max in the CHD group and control represented 93%±20%u2009and 107%±17% of predicted values, respectively. VO2max was significantly lower in the CHD group, overall (37.8±0.3vs 42.6±0.4u2009mL/kg/min, P<0.0001) and for each group (P<0.05). The mean VO2max decline per year was significantly higher in CHD than in the controls overall (−0.84±0.10u2009vs −0.19±0.14u2009mL/kg/min/year, P<0.01), for boys (−0.72±0.14vs 0.11±0.19u2009mL/kg/min/year, P<0.01) and for girls (−1.00±0.13u2009vs −0.55±0.21u2009mL/kg/min/year, P=0.05). VO2max was associated with body mass index, ventilatory anaerobic threshold, female gender, restrictive ventilatory disorder, right ventricle systolic hypertension, tricuspid regurgitation, the number of cardiac catheter or surgery procedures, and the presence of a genetic anomaly. Conclusions Although the magnitude of the difference was not large, VO2max among children with CHD was significantly lower than in normal children. We suggest performing CPET in routine follow-up of these patients. Trial registration number ClinicalTrials.gov NCT01202916; Post-results.

Health-related quality of life among children with Turner syndrome: controlled cross-sectional study

Abstract Background: The aim of the study was to assess health-related quality of life (HR-QoL) in children with Turner syndrome in comparison with controls. Methods: We prospectively recruited 16 female girls with Turner syndrome (mean age 15.2±2.6 years) and 78 female controls (mean age 12.7±2.8 years) in randomly selected schools. We used the PedsQL, a generic HR-QoL questionnaire (self and parents’ versions). Results: Global HR-QoL scores in Turner syndrome were lower than controls for self-reports (respectively, 74.3±3.0 vs. 82.8±1.3, p=0.01) and parents’ reports (62.7±3.8 vs. 80.1±1.7, p<0.0001). In Turner syndrome, self-reported HR-QoL was impaired in school functioning (70.6±4.0 vs. 80.71±1.7, p=0.02), social functioning (78.2±4.0 vs. 90.4±1.8, p<0.01) and physical functioning (78.5±3.2 vs. 87.1±1.4, p=0.02), but not in emotional functioning. Parents’ reported HR-QoL was impaired in all four dimensions. Conclusions: HR-QoL was impaired in this cohort of young females with Turner syndrome, as in previously reported adult studies. In addition to medical treatment and routine clinical follow-up, female girls and teenagers with Turner syndrome should also be supported psychologically by social, educational and psychotherapeutic interventions that aim to address their self-esteem and emotional difficulties.

Transcatheter perforation followed by pulmonary valvuloplasty in neonates with pulmonary atresia and ventricular septal defect.

BACKGROUNDnThe classic management of neonates with pulmonary atresia with ventricular septal defect (PAVSD) and moderately hypoplastic pulmonary arteries is usually a systemic to pulmonary artery shunt or ductus arteriosus stenting. We report our experience of transcatheter treatment of PAVSD by perforation followed by balloon dilation of the valve, as it is performed in pulmonary atresia - intact ventricular septum.nnnPATIENTS AND METHODnThree patients were treated at a mean age and weight of 7.5 days (range 7-8) and 2.9kg (range 2.5-3.3), respectively. Two newborns were prenatally diagnosed, with micro deletion 22q11 in one case. The three patients had no other pulmonary blood flow support than the ductus arteriosus and were on prostaglandin E1 infusion. The pulmonary atresia was predominantly valvular without significant muscular obstruction. By echocardiography, the mean size of the pulmonary annulus was 6.5mm (range 6-7). In all cases, the valvular perforation was performed with a 0.014 coronary guidewire, followed by balloon dilation when successfully.nnnRESULTSnThe procedure succeeded in two cases but failed in the third newborn in whom a long subvalvar muscular stenosis was found at surgery. In the two successful cases, the mean postprocedural transpulmonary doppler gradient was 33.5mmHg (range 17-50). One patient experienced a femoral venous thrombosis that was successfully treated by heparin and a transient right bundle branch block occurred in another one. In the two successful cases, the prostaglandin E1 infusion was weaned and the surgical repair was performed at 4 and 12 months, respectively.nnnCONCLUSIONnIn selected cases with PAVSD, perforation followed by balloon dilation of the pulmonary valve offers an interesting alternative to other surgical or transcatheter palliative therapies.

Atrial septal defect closure: indications and contra-indications

Transcatheter closure has become an accepted alternative to surgical repair for ostium secundum atrial septal defects (ASD). However, large ASDs (>38 mm) and defects with deficient rims are usually not offered transcatheter closure but are referred for surgical closure. Transcatheter closure also remains controversial for other complicated ASDs with comorbidities, additional cardiac features and in small children. This article not only provides a comprehensive, up-to-date description of the current indications and contra-indications for ASD device closure, but also further explores the current limits for transcatheter closure in controversial cases. With the devices and technology currently available, several cohort studies have reported successful percutaneous closure in the above-mentioned complex cases. However the feasibility and safety of transcatheter technique needs to be confirmed through larger studies and longer follow-up.

Atrial septal defect and exercise capacity: value of cardio- pulmonary exercise test in assessment and follow-up

Nearly four decades ago, the World Health Organization stated that functional capacity explorations best reflected the impact of a chronic disease on quality of life. Today, cardio-pulmonary exercise test (CPET) is recommended in the follow-up of patients with congenital heart diseases (CHDs). Indeed, the maximum oxygen uptake (VO2max) and the ventilatory efficiency (VE/VCO2 slope) correlate with both the prognosis and the quality of life in this population. Atrial septal defects (ASDs) represent the second most frequent CHD and are usually considered as simple CHDs. However, the exercise capacity of ASD patients may be impaired. Therefore, the CPET provides important information in assessment and follow-up of patients with ASDs, for both children and adults. Exercise capacity of patients with unrepaired ASDs depends on the importance of the shunt, the right ventricular (RV) function and volume overload, the level of pulmonary arterial pressure, and the occurrence of arrhythmias. For repaired ASDs, exercise capacity also depends on the delay before closure and the type of procedure (catheter or surgery). In most cases, the exercise capacity is nearly normal and CPET contributes to promote sports participation. In addition, a regular CPET follow-up is necessary to evaluate the occurrence, severity and physiological mechanisms of comorbidities, i.e., heart failure, pulmonary hypertension and arrhythmia. Furthermore, CPET follow-up in patients with ASDs may detect early onset of muscular deconditioning, for which cardiac rehabilitation may be considered.

How should we determine normal echocardiographic right ventricle function reference values in pediatrics

Several models have been used to establish pediatric Z-scores for echocardiographic right ventricle (RV) variables. This study aimed to determine the most appropriate mathematical model for RV function reference values in pediatrics. Prospective cross-sectional study among 314 healthy children ages 2xa0days to 18xa0years (46% female, 88 infants, 26 neonates, 226 children). RV parameters (S′, E′, A′ waves, TEI index, TAPSE) were modelized with four explanatory variables: age, height, weight and body surface area. Four mathematical models were applied: a linear model, a polynomial model of degree 2, a linear regression model by spline with free knot, and a polynomial regression model of degree 2 by spline with a free knot. The choice of the best method used the adjusted coefficient of determination (aR2). The modelization of RV variables did not follow a linear model. A single explanatory variable could not determine all Z-scores, as specific and independent variables were required for each parameter. The quadratic spline model best adjusted the modelization of RV variables. The S′ wave was best modelized by the spline model with the weight (knot at 6.86xa0kg). The E′ wave was best modelized by the spline model with the age (knot at 0.29xa0years). The A′ wave was best modelized by the model with the height (knot at 81xa0cm). The TAPSE was best modelized by the spline model with the weight (knot at 9.04xa0kg). The spline regression models best applies to echocardiographic RV reference values in pediatrics.

0350: Correlation between cardio-pulmonary exercise test variables and health-related quality of life among children with congenital heart diseases

Background peak oxygen uptake (VO2) correlates with health related quality of life (QoL) in adults with heart failure. Cardio-pulmonary exercise test (CPET) is recommended in the follow-up of adults with congenital heart diseases (CHD). Few data is available as regards correlation between CPET and QoL among children with CHD. Methods and results 202 CHD children aged 8 to 18 performed a CPET (treadmill n=96, cycle-ergometer n=106). CHD severity was stratified into 4 groups. All children and parents filled out the Kidscreen QoL questionnaire. Peak VO2, anaerobic threshold (AT), oxygen pulse followed a downward significant trend with increasing CHD severity and conversely for VE/VCO2 slope. Self-reported and parent-reported physical well-being QoL scores correlated with peak VO2 (respectively r=0.27, p Conclusions peak VO2 and anaerobic threshold are the two CPET variables which best correlated with self and parents-reported QoL in this large pediatric cohort. If QoL is involved as a “patient related outcome” in a clinical trial in pediatric cardiology, we suggest to use parents related QoL scores. Clinical Trial Registration ClinicalTrials.gov (number NCT01202916).