Magalie Ladouceur

Effects of cardiac resynchronization therapy on echocardiographic indices, functional capacity, and clinical outcomes of patients with a systemic right ventricle

AIMSnSurgically (SC) or congenitally corrected (CC) transposition of the great arteries (TGA), associated with a systemic right ventricle (RV), is often complicated by heart failure. This retrospective study assessed the functional and mechanical effects of cardiac resynchronization therapy (CRT) in patients presenting with TGA.nnnMETHODS AND RESULTSnSeven patients with SC (n = 5) or CC (n = 2) TGA (mean age 24.6 +/- 12 years), a failing systemic RV, and intraventricular dyssynchrony, underwent implantation of a CRT-P. Permanent pacemakers were previously implanted in five patients. The leads were implanted by a combined transvenous and epicardial approach in the five patients with SC TGA. Echocardiography, including tissue Doppler imaging and cardiopulmonary exercise testing were performed before and during CRT. Since, in all patients, ventricular dyssynchrony was due to delayed septal wall contraction, the interventricular septum and RV free wall were stimulated synchronously, with a view to resynchronize a maximum amount of myocardium. After 19.4 +/- 8.1 months of CRT, mean QRS duration decreased from 160 +/- 31 to 120 +/- 28 ms (P = 0.03), intraventricular delay from 104 +/- 27 to 14 +/- 15 ms (P = 0.01), New York Heart Association functional class from 3.0 to 1.57 (P = 0.01), and peak oxygen consumption increased from 13.8 +/- 2.5 to 22.8 +/- 6.7 mL/kg/min (P = 0.03). One patient died suddenly at 23 months of follow-up.nnnCONCLUSIONSnCRT was technically feasible and associated with improvements in cardiac mechanical function and clinical status in patients with TGA, failing systemic RV, and intraventricular dyssynchrony.

Key issues of daily life in adults with congenital heart disease

Increasing survival rates of patients with congenital heart disease have resulted in a new and growing patient population of adults with operated congenital heart disease. Medical professionals face the specific medical needs of these patients but must also deal with their daily life issues. Adult patients with congenital heart disease report difficulties in several areas of daily life, such as sport, employment, insurability and travel or driving. Moreover, they must have a healthy lifestyle to prevent cardiovascular complications. All these issues can be addressed in a specific educational program. In this review, we discuss the different daily life issues of adults with congenital heart disease and the preventive measures that can be proposed to improve their quality of life.

Genetic analyses in a cohort of children with pulmonary hypertension.

The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers. The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD). No mutations were found in the 23 children with APAH-CHD. In the 40 children with IPAH or FPAH, 12 mutations were found: five on BMPR2; four on ACVRL1; and three on TBX4. In the three PVOD cases, two carried the EIF2AK4 mutation. Mutation carriers had a more severe disease at diagnosis and more aggressive first-line therapy was required. The three patients with PVOD had a very severe disease at diagnosis and required a lung transplantation. The genetic architecture of paediatric PAH is enriched in ACVRL1 and TBX4 mutations compared to adult PAH, but further studies are required to confirm these results. Childhood-onset PAH in children carrying a mutation in one of the genes tested has a more severe presentation at diagnosis but a similar outcome to that observed in non-carriers. Paediatric pulmonary hypertension has a specific genetic architecture http://ow.ly/54yP301hCQi

Neonatal transcatheter closure of a large pulmonary arteriovenous fistula.

A congenital fistulous connection between the right pulmonary artery and the left atrium is a rare condition, resulting in early cyanosis and cardiac failure. These patients usually required urgent surgery. We present a neonate in whom we successfully closed such a large fistula via catheterization with an Amplatzer Duct Occluder, resulting in rapid clinical improvement, and obviating the need for surgical repair.

Longitudinal strain of systemic right ventricle correlates with exercise capacity in adult with transposition of the great arteries after atrial switch

BACKGROUNDnSystemic right ventricle (sRV) dysfunction in d-transposition of the great arteries following atrial switch (d-TGA) is associated with increased mortality. We aimed to characterize maladaptive sRV mechanisms in d-TGA patients, analyzing relation of echocardiographic parameters of sRV systolic function to objective measurements of exercise capacity.nnnMETHODSnForty-seven adult patients with d-TGA and atrial switch (mean age 31.6±4.2years) underwent conventional echocardiography, bidimensional strain (2D-strain), cardiac magnetic resonance (CMR) imaging and cardiopulmonary exercise evaluation on the same day. Those with median peak oxygen uptake (VO2)>64.5% (n=23) constituted group A, those with VO2≤64.5% (n=24) constituted group B and 23 healthy age and gender matched subjects constituted the control group.nnnRESULTSnIn group A, global longitudinal peak systolic 2D-strain (GLS) of sRV was significantly reduced compared to GLS of normal RV and LV in the healthy control group (p<0.01), however peak longitudinal 2D strain was similar at basal and mid-segment of sRV free wall than normal LV. In group B, GLS was significantly reduced compared to group A (-10.9±2.9% vs -13.1±2.3%, p<0.05), mostly due to significant decrease of interventricular septum longitudinal strain. Other echocardiographic systolic parameters were not significantly different between groups A and B. Only sRV GLS showed significant correlation with functional capacity as measured by VO2 (r=0.42, p<0.01), while CMR RVEF did not.nnnCONCLUSIONnGLS of sRV predicts functional capacity and may be more sensitive than CMR RVEF in detecting early myocardial damage of sRV in patients with d-TGA and atrial switch.

Associated genetic syndromes and extracardiac malformations strongly influence outcomes of fetuses with congenital heart diseases.

BACKGROUNDnCongenital heart disease (CHD) is often associated with extracardiac malformations (ECMs) and genetic syndromes.nnnAIMSnTo determine the effect of cytogenetic anomalies and/or ECMs associated with CHD on parental decision to choose termination of pregnancy (TOP) or compassionate care (CC), as well as on the outcome of children born alive.nnnMETHODSnThis 10-year retrospective study included all prenatally diagnosed cases of CHD in a single tertiary referral centre.nnnRESULTSnFrom January 2002 to December 2011, 2036 consecutive cases of fetal CHD (798xa0TOPs and 1238xa0live births, including 59 with postnatal CC) were included. CHD was associated with a known cytogenetic anomaly in 9.8% of cases and a major ECM in 11.7% of cases. The proportion of prenatally identified associated cytogenetic anomalies was significantly lower in the live-birth group than in the TOP plus CC group (4.2% vs 17.5%; P<0.001); this was also true for ECMs (8.1% vs 16.7%; P<0.001). The mortality rate was higher in the group with an associated cytogenetic anomaly or ECM (29.1%) than in cases with isolated CHD; a 2.4-fold increase in the death rate was observed (95% confidence interval 1.34-4.38; P=0.003). These associations remained significant after multivariable analysis, including the severity of the CHD (uni- or biventricular physiology).nnnCONCLUSIONnPrenatal diagnosis of a known cytogenetic anomaly or major ECM strongly influences parental decision to choose TOP or postnatal CC. Genetic syndromes and ECMs are associated with a higher mortality rate, independent of the complexity of the CHD.

Percutaneous valvulation of failing Fontan: Rationale, acute effects and follow-up

BACKGROUNDnFontan circulation is fragile and unfavourable evolution is frequent. Fontan physiology largely depends on respiration and gravity. The hypothesis for valvulation is that valvulation of the circuit reduces the effects of respiration and the proclive position, and increases anterograde flow towards the systemic circulation, with increasing exercise capacity and benefits for enteropathy. Because it originates from the bovine jugular vein, the Melody(®) valve (Medtronic, Minneapolis, MN, USA) is naturally designed to work in a low-pressure environment.nnnAIMSnTo report our experience of percutaneous valvulation of refractory failing Fontan circulation.nnnMETHODSnWe reviewed all patients who received a Melody valve in Fontan circulation in our unit.nnnRESULTSnFour patients were included: two had severe and refractory protein-losing enteropathy; one had severe oedema and ascites; and one had very severe lower limb venous insufficiency. The Melody valve was successfully implanted in all patients. Central venous pressure and inferior vena cava pressure did not change after valvulation. There were no early complications. At follow-up, no acute or mid-term thrombosis was noted. Two patients had intracardiac echocardiography 6 and 24 months after valvulation: the Melody valve was found not to be functioning in both cases. One patient died 3 months after valvulation; the cause was unrelated to the procedure.nnnCONCLUSIONnPercutaneous valvulation of Fontan circulation is technically feasible. More clinical studies are needed before considering this treatment as an option.

Impaired atrioventricular transport in patients with transposition of the great arteries palliated by atrial switch and preserved systolic right ventricular function: A magnetic resonance imaging study

OBJECTIVESnWe aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship of these diastolic parameters with exercise performance and BNP, in patients with preserved systolic systemic right ventricle function.nnnDESIGNnSingle-center, cross-sectional, prospective study.nnnSETTINGnIn patients with D-TGA palliated by atrial switch, diastolic dysfunction of the systemic right ventricle may precede systolic dysfunction.nnnMETHODSnForty-five patients with D-TGA and atrial switch and 45 age and sex-matched healthy subjects underwent cardiac magnetic resonance imaging and echocardiography. Filling flow-rates measured by phase-contrast cardiac magnetic resonance imaging were analyzed using customized software to estimate diastolic parameters and compared with exercise performance.nnnRESULTSnIn D-TGA, early filling of systemic right ventricle was impaired with a lower peak filling rate normalized by filling volume (Ef/FV measured by cardiac magnetic resonance imaging) and a higher early filling peak velocity normalized by early peak myocardial velocity (EUS /Ea measured by echocardiography) compared with controls (Pu2009≤u2009.04). Stroke volume of systemic right ventricle showed a direct and significant association with pulmonary venous pathway size (respectively ru2009=u20090.50, Pu2009<u2009.01). Systemic right atrial area and systemic right ventricle mass/volume index measured by cardiac magnetic resonance imaging, as well as Ef/FV were significantly correlated with exercise performances and BNP (Pu2009<u2009.01). All correlations were independent of age, gender, body mass index and blood pressure.nnnCONCLUSIONSnSystemic right ventricle pre-load and stroke volume depend mainly on intraatrial pathway function. Moreover, systemic right ventricle remodeling and right atrial dysfunction impair systemic right ventricle filling, leading to BNP increase and exercise limitation. Cardiac magnetic resonance imaging should assess systemic right ventricle filling abnormalities in D-TGA patients.

Reduction of radiation exposure in transcatheter atrial septal defect closure: How low must we go?

BACKGROUNDnCardiac catheterization relies on X-ray imaging. Most procedures are now standardized. Interventionists must strive to minimize radiation exposure to reduce the risk of induced cancers.nnnAIMSnTo describe the radiation level in our institution, and evaluate the components contributing to radiation exposure, during transcatheter atrial septal defect (ASD) closure.nnnMETHODSnRadiation doses for ASD closure performed between Januaryxa02009 and Novemberxa02015 were reviewed retrospectively. Data on fluoroscopic time, dose area product (DAP), DAP/kg of body weight and total air kerma were collected.nnnRESULTSnOne hundred and seventy-four consecutive patients were included. Procedural success was 98.3%. Median procedural and fluoroscopic times were 15minutes and 1.2minutes, respectively. Median total air kerma, DAP and DAP/kg were 9.2xa0mGy, 88.3μGy.m2 and 3.2μGy.m2/kg, respectively. Risk factors associated with higher DAP were older age, larger ASD and device, need for balloon calibration, occurrence of complications and use of higher frame rate. Reduction of frame rate to 7.5xa0frames/second alone reduced by a factor of 2 the median DAP, DAP/kg and air kerma (99 vs 43μGy.m2, 3.5 vs 1.7μGy.m2/kg and 11 vs 4.8xa0mGy, respectively; P<0.001).nnnCONCLUSIONSnA low dose of radiation can be achieved for transcatheter ASD closure, even in complex ASDs, by following these recommendations: reduction of frame rate; avoidance of lateral view and cine acquisition; and limitation of fluoroscopic time by avoiding unnecessary manoeuvres and using echocardiographic guidance as much as possible.

Educational needs of adolescents with congenital heart disease: Impact of a transition intervention programme

BACKGROUNDnAdolescents and young adults with congenital heart disease (CHD) have complex health needs and require lifelong follow-up. Interventions to facilitate the paediatric-to-adult healthcare transition are recommended, but outcomes remain largely under-investigated.nnnAIMSnTo identify the educational needs and the impact of a transition intervention on knowledge and self-management skills in adolescents and young adults with CHD.nnnMETHODSnFrom September 2014 to May 2015, 115 adolescents and young adults with CHD (mean age 17±2 years; 47 girls) were consecutively enrolled. Among these, 22 had participated in a structured educational programme in the previous 11±4 months (education group) and 93 had not (comparison group). Knowledge about their health status was assessed using a targeted CHD questionnaire.nnnRESULTSnThe mean overall health knowledge score (maximum of 20) in the education group was significantly higher than in the comparison group (11.7±3.5 vs. 8.6±3.2; P<0.001). We observed significant gaps in knowledge in the comparison group: e.g. 61.3% vs. 90.0% knew their condition name (P=0.01), 21.5% vs. 63.6% were aware of recommended follow-up (P=0.004), and 12.8% vs. 75.0% of girls knew to check their heart condition before pregnancy (P<0.01). In multivariable analysis, after adjustment for age, structured CHD education and higher academic attainment were significant determinants of health-related knowledge (P<0.01).nnnCONCLUSIONnEducation during adolescent-to-adult transition has a significant impact on health knowledge. Structured CHD educational programmes could improve understanding and prevent potential future complications.