Charlene Bredy

Pregnancy outcomes in patients with pulmonary arterial hypertension associated with congenital heart disease

Objective There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD. Methods Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes. Results 28 pregnancies in 20 women (26±6 years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20 weeks’ gestation (WG)), 8 abortions and 2 miscarriages. Six (33%, 95% CI (11.9 to 54.3)) patients experienced severe cardiac events. The concerned women had lower resting oxygen saturation (79.6±4.1% vs 89.3±3.8%, p<0.01). The most common cardiac complications during the complete pregnancies were heart failure (n=4) and severe hypoxaemia (n=5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality=5.0% 95% CI (0.1 to 24.9)). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred. Conclusions Outcome of pregnancy in women with PAH-CHD is better than previously reported, with only 5% maternal mortality in our cohort. However, because of the severity of heart failure and the high rate of neonatal complications, patients should still be advised against pregnancy.

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% and 107%±17% of predicted values, respectively. VO2max was significantly lower in the CHD group, overall (37.8±0.3vs 42.6±0.4 mL/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±0.10 vs −0.19±0.14 mL/kg/min/year, P<0.01), for boys (−0.72±0.14vs 0.11±0.19 mL/kg/min/year, P<0.01) and for girls (−1.00±0.13 vs −0.55±0.21 mL/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.

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.

Pregnancy in adults with repaired/unrepaired atrial septal defect

Atrial septal defect (ASD) is the most common form of congenital heart disease. Left-to-right shunting leads to right ventricular (RV) volume overload with excessive pulmonary blood flow. Complications include exercise intolerance, pulmonary vascular disease, RV dysfunction, paradoxical thromboemboli, and atrial arrhythmias. Women with coexisting severe pulmonary hypertension should be counselled against pregnancy due to high incidence of maternal and fetal morbidity and mortality. In the absence of pulmonary hypertension, pregnancy is generally well tolerated in the setting of an ASD. Nevertheless, hemodynamic changes throughout gestation may increase the risk for complications, particularly in those with unrepaired ASDs. Arrhythmias are the most common cardiac event and occur in 4-5%, followed by paradoxical emboli in 2-5%. Obstetrical and neonatal complications include preeclampsia, a higher incidence of infants born small for gestational age, and higher fetal/perinatal mortality. Although there is no definitive evidence demonstrating superiority of an aggressive approach to ASD closure prior to pregnancy, it is currently common practice to electively close asymptomatic but large and/or hemodynamically significant ASDs prior to childbearing. Cardiology follow up during pregnancy should be adapted to clinical circumstances and includes transthoracic echocardiography during the second trimester and arrhythmia monitoring in the event of symptoms.

Feasibility of clinical hypnosis for transesophageal echocardiography in children and adolescents

Background: The use of hypnosis in children has been described more than two centuries ago, with a more recent research and clinical application. There is currently a good level of evidence for the efficacy of clinical hypnosis in children for minor surgery, medical procedures or pain management. The use of clinical hypnosis, in paediatric cardiology, for invasive procedures such as transesophageal echocardiography, has not been reported. Aims: This study evaluated the feasibility of clinical hypnosis in children undergoing transesophageal echocardiography. Method: This prospective, non-randomised, cross-sectional study was carried out over 24 months in a paediatric cardiology referral centre. All children aged 10–18 years requiring a transesophageal echocardiography examination, outside the operating room and the catheterisation laboratory, were eligible for the study. Children and families could choose between transesophageal echocardiography under clinical hypnosis or under general anaesthesia (<15 years) or sedation (⩾15 years). Results: We included 16 children aged 11–18 years (seven girls, mean age 14.1±2.5 years). The hypnotic state was achieved for 15 out of the 16 participating children (94%). The transesophageal echocardiography examination could be completely achieved with a full diagnosis for 15 out of 16 children (94%). In all cases, a transesophageal echocardiography examination under clinical hypnosis provided a complete diagnosis. Conclusion: This study demonstrated that hypnosis was feasible and effective for transesophageal echocardiography in adolescents and might be a good alternative to general anaesthesia. Further study with larger numbers of subjects and more diverse congenital cardiac conditions are needed to confirm the results in a more diverse sample.

Cardiopulmonary exercise test in children with congenital heart diseases: correlation between ventilatory parameters and maximum oxygen uptake

Gavotto A. (1,2), Matecki S. (2, 5), Guillaumont S. (3), Picot M.C. (4), Amedro P. (1,5) Pediatric and Congenital Cardiology Department, University Hospital, Montpellier, France (1); Pediatric Functional Exploration Laboratory, Physiology Department, University Hospital, Montpellier, France (2); Pediatric Cardiology and Rehabilitation Unit, Saint-Pierre Institute, Palavas-Les-Flots, France (3); Epidemiology and Clinical Research Department, University Hospital, Montpellier, France (4);Physiology and Experimental Biology of Heart and Muscles Laboratory PHYMEDEXP, UMR CNRS 9214 – INSERM U1046, University of Montpellier, Montpellier, France (5)

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).