[Contribution of cardiac CT in the management of congenital heart disease : experience of the Abidjan cardiology institute in 27 cases]. / Apport du scanner cardiaque dans la prise en charge des cardiopathies congénitales : expérience de l'institut de cardiologie d'Abidjan à propos de 27 cas.

INTRODUCTION AND OBJECTIVES: In contrast to developed countries, cardiac CT is not widely used in West Africa for the assessment of congenital heart disease, and has only recently been introduced in Côte d'Ivoire. The lack of data prompted this study, the aim of which was to describe our experience of the contribution of CT to the management of congenital heart disease in the Ivorian cardiology setting. PATIENTS AND METHOD: This was a prospective study which took place in the pediatric cardiology department over a period of 9 months (September 2022 to June 2023) which included all patients with congenital heart disease explored by echocardiography and cardiac scan. RESULTS: The average age was 5.7 ± 4.7 years with extremes of 5 months and 16 years. We noted a female predominance with a sex ratio of 0.52. The main heart diseases were: tetralogy of Fallot (37.1%), followed by pulmonary atresia with open septum (18.52%). The assessment of the anatomy of the pulmonary arteries (81.48%), the search for aortopulmonary collaterals (59.63%) and finally the assessment of the anatomy of the aorta (18.52%) were the main indications. The practice of cardiac CT scanning has enabled better accuracy in the diagnosis and management of congenital heart disease in several cases: an interruption of the aortic arch was found in 1 case initially suspected of hypoplasia of the aortic arch, three cases of partial abnormal pulmonary venous return and one case of total abnormal pulmonary venous return. Also, agenesis of the left pulmonary artery with birth anomaly of the right coronary artery in the assessment of tetralogy of Fallot was found in 1 case. The CT scan made it possible to specify the exact topography of the aortopulmonary collaterals in four cases of pulmonary atresia with an open septum. CONCLUSION: CT has enabled better visualization of the arterial and venous pulmonary tree, identification and exact localization of aorto-pulmonary collaterals, precise description of the three-dimensional anatomy of the coronary arteries, and better precision of congenital anomalies of the aorta.

Catheter ablation in adults with congenital heart disease: A 15-year perspective from a tertiary centre.

BACKGROUND: With the growing adult congenital heart disease (ACHD) population, the number of catheter ablation procedures is expected to dramatically increase. Data reporting experience and evolution of catheter ablation in patients with ACHD, over a significant period of time, remain scarce. AIM: We aimed to describe temporal trends in volume and outcomes of catheter ablation in patients with ACHD. METHODS: This was a retrospective observational study including all consecutive patients with ACHD undergoing attempted catheter ablation in a large tertiary referral centre over a 15-year period. Acute procedural success rate and freedom from recurrence at 12 and 24 months were analysed. RESULTS: From November 2004 to November 2019, 302 catheter ablations were performed in 221 patients with ACHD (mean age 43.6±15.0 years; 58.9% male sex). The annual number of catheter ablations increased progressively from four to 60 cases per year (P<0.001). Intra-atrial reentrant tachycardia/focal atrial tachycardia was the most common arrhythmia (n=217, 71.9%). Over the study period, acute procedural success rate increased from 45.0% to 93.4% (P<0.001). Use of irrigated catheters (odds ratio [OR] 4.03, 95% confidence interval [CI] 1.86-8.55), a three-dimensional mapping system (OR 3.70, 95% CI 1.72-7.74), contact force catheters (OR 3.60, 95% CI 1.81-7.38) and high-density mapping (OR 3.69, 95% CI 1.82-8.14) were associated with acute procedural success. The rate of freedom from any recurrence at 12 months increased from 29.4% to 66.2% (P=0.001). Seven (2.3%) non-fatal complications occurred. CONCLUSIONS: The number of catheter ablation procedures in patients with ACHD has increased considerably over the past 15 years. Growing experience and advances in ablative technologies appear to be associated with a significant improvement in acute and mid-term outcomes.

Cardiac computed tomography angiography in the paediatric population: Expert consensus from the Filiale de cardiologie pédiatrique et congénitale (FCPC) and the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV).

This paper aims to provide a paediatric cardiac computed tomography angiography expert panel consensus based on the opinions of experts from the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV) and the Filiale de cardiologie pédiatrique congénitale (FCPC). This expert panel consensus includes recommendations for indications, patient preparation, computed tomography angiography radiation dose reduction techniques and postprocessing techniques. We think that to realize its full potential and to avoid pitfalls, cardiac computed tomography angiography in children with congenital heart disease requires training and experience. Moreover, paediatric cardiac computed tomography angiography protocols should be standardized to acquire optimal images in this population with the lowest radiation dose possible, to prevent unnecessary radiation exposure. We also provide a suggested structured report and a list of acquisition protocols and technical parameters in relation to specific vendors.

[Assessment of intracardiac flows by Magnetic Resonance Imaging: The 4D Flow imaging]. / Évaluation des flux en imagerie par résonnance magnétique cardiaque : l'imagerie de flux 4D.

2D flow cardiac MRI is a well-established technique but has some current limitations in routine practice. New 4D flow MRI may overcome these limitations, providing dramatic dynamic imaging, easily understandable, allowing robust quantification of flows. 4D flow imaging should become soon the reference technic for valvular regurgitations and congenital heart disease.

Cardiovascular anatomy in children with bidirectional Glenn anastomosis, regarding the transcatheter Fontan completion.

BACKGROUND: Transcatheter stent-secured completion of total cavopulmonary connection (TCPC) after surgical preparations during the Glenn anastomosis procedure has been reported, but complications from this approach have precluded its clinical acceptance. AIMS: To analyse cardiovascular morphology and dimensions in children with bidirectional Glenn anastomosis, regarding the optimal device design for transcatheter Fontan completion without special surgical "preconditionings". METHODS: We retrospectively analysed 60 thoracic computed tomography and magnetic resonance angiograms performed in patients with a median age of 4.1 years (range: 1.8-17.1 years). Additionally, we simulated TCPC completion using different intra-atrial stent-grafts in a three-dimensional model of the representative anatomy, and performed calculations to determine the optimal stent-graft dimensions, using measured distances. RESULTS: Two types of cardiovascular arrangement were identified: left atrium interposing between the right pulmonary artery (RPA) and inferior vena cava, with the right upper pulmonary vein (RUPV) orifice close to the intercaval axis (65%); and intercaval axis traversing only the right(-sided) atrial cavity, with the RUPV located posterior to the atrial wall (35%). In the total population, the shortest median RPA-to-atrial wall distance was 1.9mm (range: 0.6-13.8mm), while the mean intra-atrial distance along the intercaval axis was 50.1±11.2mm. Regardless of the arrangement, 83% of all patients required a deviation of at least 5.9±2.4mm (range: 1.2-12.7mm) of the stent-graft centre at the RUPV level anteriorly to the intercaval axis to avoid covering or compressing this vein. Fixing the anterior deviation of the curved stent-graft centre at 10mm significantly decreased the range of bend angle per every given RUPV-RPA distance. CONCLUSIONS: For both types of cardiovascular arrangement, after conventional bidirectional Glenn anastomosis, the intra-atrial curved stent-graft seemed most suitable for achieving uncomplicated TCPC completion percutaneously without previous surgical "preconditionings" in the majority of children. Experimental study is necessary to validate this conclusion.

[Congenital heart disease in adolescents and adults: Management in a general cardiology department in Senegal]. / Cardiopathies congénitales de l'adolescent et de l'adulte : prise en charge dans un service de cardiologie générale au Sénégal.

BACKGROUND: Congenital heart diseases in adults include malformations treated in childhood that decompensate secondarily and those asymptomatic at birth, appear later. This study aims to identify congenital heart diseases in adults in general cardiology department of Senegal and to assess clinical presentations, treatment and outcomes. METHODS: We conducted a cross-sectional and descriptive study based on the records of patients aged at least 16 years and followed for congenital heart disease in the cardiology department of the General Hospital of Grand-Yoff in Dakar between May 2003 and March 2015. Diagnosis of heart disease was based on echocardiography. RESULTS: We have registered 50 dossiers of patients equivalent to a prevalence of 0.75%, with a female predominance (64%). The average age of patients was 36.2±18.4 years (16-79 years), and mean age of diagnosis was 29.76±22.58 years. Dyspnea was the main sign (60%). Main malformations were the atrial septal defect (38%), pulmonary stenosis (14%), the ventricular septal defect (12%) and patent ductus arteriosus (10%). According to the classification of Bethesda, heart disease was simple complexity (42%), intermediate (58%) or severe (10%). The treatment was medical in 43 patients and 7 patients had surgical repair. Main complications were infective endocarditis (10%), atrial fibrillation (12%), heart failure (24%) and pulmonary arterial hypertension (50%). CONCLUSION: Congenital heart diseases in adults seem underestimated in our countries. Surgical repair is rare. It is necessary to ensure a good management of the transition between pediatric and adult age.

Percutaneous pulmonary Melody® valve implantation in small conduits.

BACKGROUND: The Melody® pulmonary valve has received approval for the treatment of dysfunctional right ventricular (RV) outflow tract conduits ≥16mm. AIMS: To investigate technical and clinical outcomes in patients who underwent percutaneous pulmonary valve implantation (PPVI) in conduits ≤16mm. METHODS: Eleven patients were enrolled retrospectively between 2000 and 2015 in a cardiac referral centre. RESULTS: The PPVI indications were obstruction (n=5); regurgitation (n=1); and mixed lesions (n=5). Pre-stenting was performed during the procedure in seven cases. Three of the remaining four patients had previous stent implantation. The mean maximal dilatation balloon diameter/surgical initial diameter ratio was 1.3. Procedural haemodynamics showed a decrease in peak RV to pulmonary artery gradient (mmHg) from 50 (14-86) to 11 (5-23) (P<0.001), in RV systolic pressure (mmHg) from 69 (35-110) to 40 (25-50) (P=0.006) and in RV/aortic systolic pressure ratio from 0.8 (0.4-1.0) to 0.4 (0.3-0.6) (P=0.004). Acute device-related complications occurred in four patients (36.4%): two confined conduit tears managed with placement of a covered stent; and two vascular access complications. Mean follow-up after PPVI was 3.9 years (41 days to 6.7 years). At last follow-up, one patient had undergone surgical conduit replacement for recurrent conduit stenosis, one had percutaneous pulmonary valve dilatation and two had developed bacterial endocarditis and undergone surgical conduit replacement. Freedom from valve dysfunction at 1, 2 and 3 years was 91%, 91% and 82%, respectively. CONCLUSIONS: PPVI is feasible in small conduits ≤ 16mm with good procedural and early haemodynamic results, postponing the need for surgery. These results are not sustainable over time. Larger studies are needed to determine if bare-metal stent implantation as a bridge to surgical replacement provides the same results in this specific population.

Heart transplantation in adults with congenital heart disease.

With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients. This population is deemed to be at high risk of mortality after heart transplantation, although their long-term survival is similar to that of patients transplanted for other reasons. Indeed, heart transplantation in adults with congenital heart disease is often challenging, because of several potential problems: complex cardiac and vascular anatomy, multiple previous palliative and corrective surgeries, and effects on other organs (kidney, liver, lungs) of long-standing cardiac dysfunction or cyanosis, with frequent elevation of pulmonary vascular resistance. In this review, we focus on the specific problems relating to heart and heart-lung transplantation in this population, revisit the indications/contraindications, and update the long-term outcomes.

Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study.

BACKGROUND: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial. AIMS: To investigate outcomes in patients with ES, and their relationship with PAH-SDT. METHODS: Retrospective, observational, nationwide, multicentre cohort study. RESULTS: We included 340 patients with ES: genetic syndrome (n=119; 35.3%); pretricuspid defect (n=75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE5I]) 46.7%; dual therapy (ERA+PDE5I) 40.9%; triple therapy (ERA+PDE5I+prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0-9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4-47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8-21.6%] and 46.4% [95% confidence interval 38.2-55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P=0.0001 and P=0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P<0.001 and P<0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P=0.002, P=0.01 and P=0.04, respectively), and one or two PAH-SDTs with a lower risk of events (P=0.009). CONCLUSIONS: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.

[Sex differences in congenital heart disease]. / Différences entre les sexes dans les cardiopathies congénitales.

Gender influences the clinical presentation and the management of some acquired cardiovascular diseases, such as coronary artery disease, resulting in different outcomes. Differences between women and men are also noticed in congenital heart disease. They are mainly related to the prevalence and severity of some congenital heart defects at birth, and in adulthood to the prognosis, incidence of Eisenmenger syndrome and risks of pregnancy. The role of gender on the risk of operative mortality of congenital heart surgery remains debated.

Computed tomography imaging in children with congenital heart disease: Indications and radiation dose optimization.

Computed tomography (CT) technology is acquiring a key role in the diagnostic process of complex cardiac congenital anomalies. Recent advances and improvements in spatial and temporal resolution and radiation dose are encouraging the use of CT scanning in children. Paediatric cardiologists should have a good knowledge of the potential of CT techniques and their limitations to plan and properly perform CT examinations without forgetting radiation concerns. In this paper, we will discuss the principal indications for CT scans in newborns and children in our clinical practice. We will also outline the most-used strategies for dose reduction. Basic knowledge about the various CT techniques is crucial, not only to perform, but also to interpret CT results, thus helping the medical and surgical management of patients.

[Segmental approach to congenital heart diseases: Principles and applications to prenatal imaging]. / Approche segmentaire des cardiopathies congénitales : principes et applications en imagerie prénatale.

This pictorial essay will initially present the origin, definitions, objectives and main principles of the segmental approach to congenital heart diseases. Then, through ultrasound scans iconography we will consider its practical applications to prenatal screening. Eventually, through both ultrasound and MRI cases, we will discuss its potential use in fetal diagnostic evaluation.

Recreational scuba diving in patients with congenital heart disease: Time for new guidelines.

The number of recreational scuba divers is steadily increasing. In its latest recommendations, the French Federation of Undersea Studies and Sports listed congenital heart disease as a formal and final contraindication to scuba diving. On the other hand, with the progress made in their management, the prognosis and quality of life of patients with congenital heart diseases have improved considerably, enabling them to engage in physical and sports endeavours, which are known to confer general health and psychological benefits. As a consequence, the ability of these patients to dive has become a regular and recurrent issue. We review the various types of scuba diving, the physical performance required for its practice, its effects on cardiovascular function and the elements that need to be considered before recommending whether it can be practiced safely at various levels of difficulty. Because of the diversity and broad heterogeneity of congenital heart diseases, a detailed evaluation of each patient's performance based on clinical criteria common to all congenital heart diseases is recommended.

Speckle-tracking imaging in patients with Eisenmenger syndrome.

BACKGROUND: Adults with Eisenmenger syndrome have a survival advantage over those with idiopathic pulmonary arterial hypertension. Improved survival may result from preservation of right ventricular (RV) function. AIMS: To assess left ventricular (LV) and RV remodelling in patients with Eisenmenger syndrome compared to a control population, using speckle-tracking imaging. METHODS: Adults with Eisenmenger syndrome and healthy controls were enrolled into this prospective two-centre study. Patients with Eisenmenger syndrome with low acoustic windows, irregular heart rhythm or complex congenital heart disease were excluded. Clinical assessment, B-type natriuretic peptide (BNP), 6-minute walk test and echocardiography (including dedicated views to perform offline two-dimensional-speckle-tracking analysis) were performed on inclusion. RESULTS: Our patient population (n=37; mean age 42.3 ± 17 years) was mostly composed of patients with ventricular septal defect (37.8%) or atrial septal defect (35.1%). Compared with the control population (n=30), patients with Eisenmenger syndrome had reduced global LV longitudinal strain (-17.4 ± 3.5 vs. -22.4 ± 2.3; P<0.001), RV free-wall longitudinal strain (-15.0 ± 4.7 vs. -29.9 ± 6.8; P<0.001) and RV transverse strain (25.8 ± 25.0 vs. 44.5 ± 15.1; P<0.001). Patients with Eisenmenger syndrome also more frequently presented a predominant apical longitudinal and transverse strain profile. Among patients with Eisenmenger syndrome, those with a post-tricuspid shunt presented with reduced global LV longitudinal strain but increased RV transverse strain, compared to patients with pre-tricuspid shunt. CONCLUSION: Patients with Eisenmenger syndrome had impaired longitudinal RV and LV strain, but present a relatively important apical deformation. RV and LV remodelling, as assessed by speckle-tracking imaging, differ between patients with pre- and post-tricuspid shunt.

Cardiac imaging of congenital heart diseases during interventional procedures continues to evolve: Pros and cons of the main techniques.

Cardiac catheterization has contributed to the progress made in the management of patients with congenital heart disease (CHD). First, it allowed clarification of the diagnostic assessment of CHD, by offering a better understanding of normal cardiac physiology and the pathophysiology and anatomy of complex malformations. Then, it became an alternative to surgery and a major component of the therapeutic approach for some CHD lesions. Nowadays, techniques have evolved and cardiac catheterization is widely used to percutaneously close intracardiac shunts, to relieve obstructive valvar or vessel lesions, and for transcatheter valve replacement. Accurate imaging is mandatory to guide these procedures. Cardiac imaging during catheterization of CHD must provide accurate images of lesions, surrounding cardiac structures, medical devices and tools used to deliver them. Cardiac imaging has to be 'real-time' with an excellent temporal resolution to ensure 'eyes-hands' synchronization and 'device-target area' accurate positioning. In this comprehensive review, we provide an overview of conventional cardiac imaging tools used in the catheterization laboratory in daily practice, as well as the effect of recent evolution and future imaging modalities.

Stenting in paediatric and adult congenital heart diseases: A French multicentre study in the current era.

BACKGROUND: Many stents are used "off-label" during the management of congenital heart diseases (CHD). AIMS: To describe indications for, results of, and adverse events associated with stenting in CHD in current practice. METHODS: Participation in this study was proposed to all catheterization laboratories that specialize in CHD in France (M3C network). All paediatric and adult CHD cases with stent implantation in 2013 were included retrospectively. RESULTS: Overall, 207 stents were implanted in 151 patients across 11 centres. Median age was 13.7 years (range, 5 days to 70.1 years). Main procedure indications were branch pulmonary artery angioplasty (n=46, 29.1%), aortic (re)coarctation stenting (n=43, 27.2%), percutaneous pulmonary valve implantation (n=32, 20.2%) and ductus arteriosus stenting (n=14, 8.9%). The main stents implanted were the CP Stent™ (n=61, 29.5%), the Max™ LD stent (n=43, 20.8%), the Valeo(®) stent (n=28, 13.5%) and valved stents (n=30, 14.5%). Procedures were considered successful in 96.8% of cases (95% confidence interval [CI] 92.8-99.0%). Adverse events were observed in 23 procedures (14.7%, 95% CI 9.5-21.0%). Ductus arteriosus stenting (odds ratio 12.4, 95% CI 2.0-77.5; P<0.01) and pulmonary revalvulation (odds ratio 5.9, 95% CI 1.1-32.3; P=0.04) were risk markers for stent-related adverse events. CONCLUSIONS: Stents are used in various CHD catheterization procedures, from infancy to adult age. The adverse events rate is significant and is related to the type of procedure.

Incidence and predictors of Melody® valve endocarditis: a prospective study.

BACKGROUND: Percutaneous pulmonary valve replacement (PPVR) has achieved standard of care for the management of dysfunctional right ventricular outflow tract. AIM: Because of increasing reports of Melody(®) valve (Medtronic, Inc., Minneapolis, MN, USA) infective endocarditis (IE), we aimed to evaluate its incidence and predictors of occurrence. METHODS: We collected data on all patients who received a Melody valve in the pulmonary position from 2009 to 2012. RESULTS: A total of 86 consecutive patients underwent PPVR from 2009 to 2012; of these, five developed IE (5.8%). Freedom from IE was 91% at 50 months. Baseline demographics, type of conduit, PPVR procedural success, residual gradients and in situ duration of Melody valve were similar in IE and non-IE patients. A significant number of IE patients had additional unprotected invasive procedures during follow-up and abruptly discontinued antiplatelet therapy (P=0.0139 and P=0.002, respectively). The cumulative probability of survival without cardiovascular events for IE patients was 20% at 20 months, compared with 98.1% for non-IE patients (P<0.0001). Death was statistically associated with IE. CONCLUSION: Early- and late-onset IE of the Melody valve is emerging as a catastrophic complication of PPVR. Abrupt aspirin discontinuation and additional unprotected invasive procedures during follow-up are significant predictors of Melody valve IE. Owing to its rapidly progressive nature, aggressive invasive management should not be delayed.

[Surgery of grown up congenital heart disease. About 540 cases]. / Chirurgie des cardiopathies congénitales à l'âge adulte. À propos de 540 cas.

INTRODUCTION: With advances in recent decades in the field of congenital heart disease both for imaging in medical therapy, a large number of heart disease is diagnosed before birth. Many of them benefit from surgery and reach adulthood, they do not require further action. Some of them develop later in their lives other problems requiring reoperation in adulthood. This sparked the birth of a subspecialty within the department of congenital heart disease: GUCH Unit "grown up congenital heart disease". In developing countries, little heart are detected in childhood, a minority of them are operated and very few reach adulthood or with minor heart disease or become advanced enough then inoperable. Only part may still take advantage of surgery at this age. The aim of our study is to describe the spectrum and characteristics of congenital heart disease in adulthood in Algiers a center of cardiovascular surgery. POPULATION AND METHODS: A retrospective descriptive study of patients aged 15 and above operated for congenital heart defects between 1995 and 2011. RESULTS: Five hundred and forty patients aged 15 to 76years (29±10 years), including 314 women and 226 men are operated congenital heart defects between 1995 and 2011. The left-right shunts represent two thirds of heart disease, represented mainly (50%) by the atrial septal defect. Barriers to the ejection of the left heart represent one forth of cases with a predominance of subvalvular aortic stenosis. We find the native heart whose survival is considered exceptional in adulthood in the absence of surgery, such as tetralogy of Fallot, aortopulmonary windows wide, double outlet right ventricle and atrioventricular canal that take advantage of always surgery. The results are encouraging with low perioperative mortality (2%). CONCLUSION: The approach of congenital heart disease in developing countries is different from that of developed countries. Efforts need to be made in early detection and monitoring of congenital heart disease and improve access to surgery centers in close collaboration with pediatricians, cardiologists and obstetricians.