Archives of Cardiovascular Diseases Supplements最新文献

Introduction

Nowadays, the fraction of ejection (FE) is no longer considered as the unique way of expression of the efficiency of cardiac labor. The development of strain imaging (SI), in echocardiography and in MRI, has led to a better understanding of ventricular contraction, especially in cases of cardiac dysfunction with preserved FE. But SI still under-exploited in clinical practice despite the fact that it is a proved prognostic factor in many cardiomyopathies. Recently, a new framework was developed to associate the FE and the deformation parameters, the global longitudinal (GLS) and circular strain (GCS), to give the opportunity of a global assessment of the cardiac function. This mathematical relationship was validated on the adult population. This work intends to determine the more accurate evaluation of the GCS and GLS values in pediatric population and its use in order to validate the mathematic model described.

Methods

Retrospective monocentric study carried out in the cardiopediatric unit of Florence University Hospital. Inclusion of all pediatric patients who completed cardiac MRI between November 2021 and December 2022. An assessment of the left ventricle FE, GCS and GLS and a double reading was made by two specialized cardiologists.

Results/Expected results

In total, 134 CMR were studied. A good reproducibility between readers was observed (GCS: r = 0,94, GLS: r = 0,83). The correlation between isolated strain parameters and FE (GCS: r = 0,84 and GLS: r = 0,68) and between theorical FE (GCS + GLS) and FE was high (FE/FE(th): r = 0,88). The use of endocardial strain and the assessment of GCS with short axis slice assured the best correlation with the FE.

Conclusion/Perspectives

The evaluation of GCS and GLS with CMR are a safe way to evaluate the mechanical contraction of the left ventricle in pediatric population. The use of an integrated mathematical formula gives a summarized information of SI with a very good correlation to the FE.

Introduction

The course after complete cure of tetralogy of Fallot is fraught with complications including Right ventricle (RV) systolic dysfunction. MRI has long been considered the gold standard for the study of RV systolic function. However, it remains an expensive and not always available method. The new ultrasound techniques such as the strain of the RV can be of an important contribution.

Objective

Calculate global and segmental RV strain.

Compare the RV strain with conventional measurements of RV systolic function.

Study the factors favoring the alteration of the longitudinal strain of the RV.

Methods

This is a prospective study of 30 patients older than 18 years followed for operated tetralogy of fallot. We measured the fractional area change, the TAPSE, the S’ wave of the RV and the longitudinal strain of RV. We then compared the strain with the other measurements and looked for echographic abnormalities associated with altered longitudinal strain of the RV.

Results/Expected results

The measurement of the longitudinal strain of the RV in its free wall has a mean of –18. The strain measurement showed a significant gradient between the base of the RV and the apex with a basal mean of –20.7 and an apical mean of –15.6. This can be explained by a widening of the apex and a bulging of the base.

Alteration in longitudinal strain of the VD showed a significant correlation with FAC, whereas there was no correlation with TAPSE and S’ wave.

Right atrium dilatation, LV diastolic dysfunction, and residual pulmonary obstruction are the echocardiographic parameters associated with altered longitudinal strain of the LV.

Conclusion/Perspectives

In conclusion, the follow-up of patients operated on for tetralogy of Fallot is fraught with complications, including RV systolic dysfunction. The measurement of the longitudinal strain of the RV is a promising tool for the study of the RV.

Introduction

Coarctation of the aorta (CoA) is often associated with bicuspid aortic valve (BAV). Both count as the most frequent congenital heart diseases and predispose to progressive valvular and aortic damage, leading to a higher morbimortality in undiagnosed patients. Rare cases of adult aortic coarctation lead to severely left ventricular impairment function so early diagnosis is crucial for prognosis improvement.

Objective

The objective of this case report is to highlight a rare form of late diagnosis of aortic coarctation discovered during heart failure with reduced ejection fraction (HFrEF) assessment.

Results/Expected results

A 60-year-old male presented to us with New York Heart Association functional class III dyspnea and atypical chest pain. On physical examination, he was found to have lower limbs pulse delay associated with asymmetrical blood pressure measurement between the upper and lower limbs. The echocardiography revealed severe heart failure with reduced ejection fraction (HFrEF), a type 1 left right bicuspid aortic valve (BaV) with moderate aortic stenosis (Figure 1), and severe coarctation of the aorta located in the aortic isthmus. The diagnosis was confirmed using computed tomography angiography, which allowed for a precise evaluation of the location and anatomy of the aorta. A pre-operative coronary angiography revealed a smooth epicardial coronary artery tree. HFrEF secondary to long-term high afterload was diagnosed and treated with optimal medical therapy, and scheduled for percutaneous balloon angioplasty of the aortic stenosis.

Conclusion/Perspectives

Despite the low prevalence of aortic coarctation among patients with adult congenital heart diseases, it should be screened in patients presenting with refractory heart failure symptoms or persistent high blood pressure despite optimal medical treatment.

Introduction

Thanks to the progresses made in the management of congenital heart diseases (CHD), life expectancy of these patients has greatly improved, including those of patients with complex CHD. Epidemiology of this growing population is rapidly evolving. French data are needed to prepare each regional healthcare plan.

Objective

We aim to analyse national PMSI database to assess trends and projections.

Methods

We investigated the number of hospitalizations stay of children and adults with CHD over 10 years from 2013 to 2022 in France. Complexity of CHD was defined according to M3C classification.

Results/Expected results

Number of hospital stays of patients with CHD varied from 37,339 to 45,310 in total and from 9,320 to 11,006 for patients with complex CHD. Over 10 years, the latter represent 26.2% of all hospitalizations.

In children, it varied from 19,787 to 23,023 in total and from 5,714 to 6,987 for children with complex CHD. Over 10 years, the latter represent 30.6% of all hospitalizations. The number of neonates with CHD hospitalized is very stable over time ranging from 1380 to 1596. A similar trend is observed for children.

In adults with CHD, it increased continuously by 55.9% from 15,056 to 23,475 and from 3,564 to 4,294 for adults with complex CHD. Over 10 years, the latter represent 21.0% of all hospitalizations. There is an increase in hospitalizations in adults with CHD with a 5-year projection suggesting an increase up to 35%. In particular number of ACHD hospitalized for management of arrhythmia or for catheterization has almost doubled over 10 years (Figure 1).

Conclusion/Perspectives

Number of children with complex CHD seems to be very stable over time. On the other hand, there is a growing number of hospitalizations in adults with CHD. Next healthcare plan should contribute to develop facilities for the specific management of adults with CHD in these centres.

Introduction

Newborns with complex congenital heart disease (CCCHD) are at high risk of neurodevelopmental disorders of multifactorial aetiology, including brain immaturity comparable to moderately pre-term infants. Nearly one third of children with CCCHD have a language delay, which negatively impacts their academic performance, communication, and social interactions.

Objective

Our team has shown that moderate preterm newborns have impaired cortical encoding of vowels compared to term newborns. Newborns with CCCHD may have a similar early encoding profile to preterm infants, which might serve as an early functional biomarker of language delay in this population.

Methods

We recruited 15 newborns with CCHD, between March and December 2022 within the department of Paediatric Cardiology in La Timone Children's Hospital. These neonates were passively exposed to a sequence of syllables using an earpiece, while cortical and subcortical event-related potentials were simultaneously recorded.

Results/Expected results

CCCHD newborns had a significantly longer neural conduction time between the cochlea and the first brainstem relay (inferior colliculus), compared with term newborns. We hypothesize that the amplitude of cortical event-related potentials will be lower in newborns with CCCHD compared to term newborns.

Conclusion/Perspectives

This altered encoding pattern is likely to be caused by immature auditory pathways. Thus, the detection of early alteration of perception could help to identify newborns at risk of language delay, allowing the implementation of targeted rehabilitation before the onset of speech disorder.

Introduction

Congenital heart disease is a condition in which the heart and blood vessels are affected at the time of birth antenatal diagnosis of these malformations allows the organization of neonatal care.

Objective

The objective of our study is to evaluate the value of ultrasound in the diagnosis of congenital heart disease and the contribution of ultrasound in the management of this disease.

Methods

We conducted a retrospective, descriptive study carried out at the department of gynecology-obstetrics D. We included in this study pregnant women aged 18 years and older and whose gestational age (GA) was ≥ 14 weeks of amenorrhea. Clear and informed consent was sought from the patients to participate in the study.

Results/Expected results

During our study, 96 observations were included. The average age of the patients was 32.4 years, ranging from 20 to 45 years. The most represented age group (36%) was between 30 and 35 years old. The majority of marriages were non-consanguineous at 70%. 7% of the heart diseases were detected before 18 weeks of amenorrhea and 49% between 18 and 24 weeks of amenorrhea. The majority of the heart diseases were detected after 24 weeks (43%). In total, 62% of the cardiopathies corresponds to abnormalities of the 4 chambers. 35% of the malformations are conotronic disorders. 4% of the congenital heart diseases are abnormalities of the heart rhythm. 15% of the 4 cavities anomalies are related to chromosomal aberrations. 38% of CAV are related to T21. 18% of patients had a medical termination of pregnancy (MTP), against 81% who continued their pregnancies.

Conclusion/Perspectives

In spite of the progress made in the prenatal diagnosis of Congenital Heart Disease and the increasing competence of referral ultrasound physicians in the detection of these anomalies, the rate of detection in the general population is still low as well as the term of detection.

Introduction

Heart failure (HF) with preserved ejection fraction (HFpEF) is a complex cardiac condition that results from a structural or functional impairment of filling of the heart. It has a high prevalence in elderly but has rarely been analyzed in children.

Objective

To identify HFpEF in children with biventricular physiology without shunt or outflow tract obstruction, through a dedicated algorithm applied to a large tertiary hospital database, and to describe the causes, hemodynamic profiles, treatment, and hard outcomes of this population.

Methods

We applied a combination of clinical, echocardiographic, and hemodynamic measurements to confirm the diagnosis of HFpEF to identify children with a confirmed HFpEF phenotype extracted from a pediatric hospital data warehouse.

Results/Expected results

In total, 137 HFpEF cases were included. Mean age was 7 ± 5.7 years. The cardiovascular causes of HFpEF were hypertrophic cardiomyopathy (44%), restrictive cardiomyopathy (RCM) (32%), cardiomyopathy due to repeated cardiac surgeries (10%), dilated cardiomyopathy (7%), and constrictive pericarditis (7%) (Figure 1). Children with RCM had the worst prognosis (27% ten years transplant-free survival, P < 0.005). Mean patient follow-up was 5.3 years. 43% of the patients were hospitalized for acute HF, 12% were transplanted and 31% died. NT-proBNP increase was found in all groups and was associated with mortality and transplantation, HR 1.91 (95% CI 1.31, 2.76, p-value < 0.001). Based on echocardiographic evaluation, 56% of the patients were diagnosed with pulmonary hypertension (PH), associated with mortality and transplantation, HR 2.84 (95% CI 1.58, 5.10, P < 0.001). In the 48 patients who underwent right heart catheterization, post-capillary or combined PH were the most frequent but the 15% with isolated pre-capillary PH had poorer outcomes, HR 4.60 (95% CI, 1.46, 14.5, P = 0.009).

Conclusion/Perspectives

HFpEF is a rare and concerning condition in children. Younger age, RCM, acute HF episodes, increased NT-proBNP are associated with hard outcomes. A precapillary component in patients with PH is associated with poorer outcomes.

Introduction

Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by a mutation on the alpha-galactosidase-A gene. The prognosis lies in the severity of the cardiac involvement which is the leading cause of mortality. Usually, it presents with hypertrophic cardiomyopathy, valvular disease, or conduction abnormalities. This process of storage is also present in every other organ and begins before birth. Enzyme replacement therapy (ERT) has shown a spectacular effect on extra-cardiac symptoms especially if started early in life. The effect of ERT on cardiac lesions is still a research question. Cardiac magnetic resonance (CMR) is a key exam in FD with its capacity of tissular characterization. But to date, there is a lack of data in the pediatric population to understand how to integrate CMR in early therapeutic decisions.

Methods

Monocentric cross-sectional study carried out in Montpellier University hospital from 2016 to 2022. All pediatric patients with FD were evaluated over time with clinical, biological and cardiac imaging (CMR, TEE). The objective was to determine the place of CMR in the therapeutic decisions.

Results/Expected results

In total, 6 patients were included, 3 boys/3 girls. Five patients were treated with ERT during the study. Low T1 values were observed in 4 patients. The normalization of T1 values was observed in the 4 years after ERT introduction for 3 patients (Figure 1).

Conclusion/Perspectives

CMR has to take an important role in the evaluation of FD. In the pediatric population, a recurrent assessment can be useful to detect the pejorative evolution of T1, especially in partial forms of Fabry disease. Obvious need of strong data is required to help the clinician in his daily practice.

Introduction

Percutaneous closure by interventional catheterization of atrial septal defect is currently considered to be the technique of first-choice closure subject to meeting the recommended criteria, anatomical and hemodynamic.

Objective

The objective of this work is to assess the effectiveness and safety of this technique.

Methods

Our retrospective study is carried out in the cardiology department of Ibn Rochd hospital, including 24 patients who benefit from percutaneous closure by catheterization intervention of their CIA.

Results/Expected results

Our series notes a female predominance (17 F and 7 M), the average age at the time of the intervention was 7 years (3 years is 17), the average weight is 27 kg (10–80 kg), the discovery of a heart murmur and pneumonia has repetitions are the 2 most frequent reasons for consultation. The TTE is the key examination that allowed the diagnosis positive of the CIA, to define its denials, the signs of repercussion and especially to establish with precision the anatomical edges of the defect and establish the indication for closure by interventional catheterization.

Transesophageal and transthoracic doppler echocardiography are a useful complement during the intervention which was of great help for the success of the installation of the prosthesis. The choice of the prosthesis is made according to the size of the CIA. All our patients are followed, their clinical examination finds a good evolution with improvement of the functional signs and good weight gain. Echocardiography is satisfactory (prosthesis in place and absence of shunt) in 23 cases of CIA or 95.8%, and showed a minimal residual shunt in only 1 case or 4.1%. No late complications are occurred over a 36-month follow-up.

Conclusion/Perspectives

Percutaneous closure of CIA is a safe and effective method; the selection of candidate patients is essential according to the type of CIA and its anatomy...

Introduction

Mitral annulus disjunction (MAD) and Pickelhaube sign are increasingly identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP). Their prevalence and consequences in children have never been studied.

Objective

To determine MAD prevalence in children with MVP and its potential link with Vas.

Methods

A cohort of 49 consecutive children from a single institution (age 12.8 ± 3.0, 33 females) with MVP and comprehensive clinical, arrhythmia (24-h Holter monitoring and exercise test) and doppler echocardiographic characterization including pulsed wave tissue doppler (PWTD) of the lateral mitral annulus was identified. The relationship between clinical and echocardiographic data and the presence of VAs was studied.

Results/Expected results

The presence of MAD was common (n = 25, 51%). Only 5 patients had significant VAs (lown > 2) characterized by polymorphic premature ventricular contractions or couplets. The presence of MAD was not associated with significant VAs on 24h-Holter monitoring but an association was found between significant VAs and spiked high velocity midsystolic signal > 16 cm/s on PWTD of the lateral mitral annulus (Pickelhaube sign, Figure 1) (P = 0.004), myxomatous mitral valve (P = 0.004) and left ventricular dilatation (P = 0.01). No patient had severe VAs characterized by ventricular tachycardia nor symptoms of dysrhythmia. Four out of 7 patients (57%) with coexisting Pickelhaube sign and myxomatous mitral valve had significant VAs. No difference was found between patients with or without MAD for gender, history of palpitation, severity of mitral regurgitation, incidence of connective tissue disorders and medications with beta-blockers or ACE inhibitors.

Conclusion/Perspectives

MAD is a common finding in children with MVP. Its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and the presence of myxomatous mitral valve may help to detect patients prone to significant VAs.