Archives of Cardiovascular Diseases最新文献

Introduction

Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO.

Objective

To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure.

Methods

Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children's Hospitals (Paris, France).

Results

Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10 years. Late reintervention consisted in LV-PA conduit replacement (n = 3), PA stenting (n = 1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, n = 1). At last follow-up, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation.

Conclusion

We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical.

Introduction

Disparities persist regarding the access to pediatric and congenital cardiac catheterization procedures (PCCC). Developing capacities in French overseas territories located far away from mainland France remains essential to reducing inequalities and mortality.

Objective

To describe the characteristics of patients, procedures type risk categories (PTRC) and outcomes for all PCCC performed on the French island of Martinique (Caribbean).

Methods

Single center observational study. We recorded prospectively all consecutives PCCC performed in the catheter laboratory of the university Hospital of Martinique from January 2007 to December 2023. Demographics, procedures characteristics, PTRC, adverse events occurrence, as well as 24-hours mortality were documented. All results were retrospectively analysed.

Results

We performed 869 PCCC in 770 consecutives patients (31.3% < 1-year-old; 9.4% aged < 1 month) living in French territories (90%) of Martinique, Guadeloupe, French Guiana, Saint-Martin, or natives from others Caribbean islands (10%). The median age and weight (IQR) were 4 years (0.6–13) and 16 kg (6.4–4.6). There were 126 urgent and 43 (4.9%) rescue procedures. PCCC were predominantly interventional (68.7%; success rate: 95.1%) covering all types of procedures, except revalvulation. The overall median PTRC was 2 (2–3) with a 24-h mortality of 1.1%. Complications occurred in 35 cases (4%), of which 18 were major, either procedure and anaesthesia-related (40%) or likely linked (40%) to the critical condition on admission at the cathlab. Overtime, the case-mix complexity continued to increase (PTRC > 3 in 25% of patients, with a median age and weight of 2.6 years and 6.5 kg, within the past 5 years).

Conclusion

Developing permanent PCCC capabilities in French Martinique is efficient and safe. This ongoing international program is sustainable with the inclusion of patients coming from all French territories and neighbouring Caribbean islands and the continuous support of French Health Authorities.

Background

Aims

Methods

Conclusion

Background

Most patients with Fontan circulation struggle to increase cardiac output during exercise affecting aerobic capacity, response to training and quality of life. This poor cardiac performance affects their aerobic capacity, their response to training and their quality of life. We sought to identify the pre-Fontan and managerial factors that influence their exercise cardiac performance.

Methods

We retrospectively collected pre and post-Fontan anatomical, anamnestic and hemodynamic data in 57 consecutive young Fontan patients (median age 14.7 years) who underwent cardiopulmonary exercise test. We studied their exercise cardiac performance using a thoracic bioelectrical impedance device that allows measurement of the peak cardiac index (pCI).

Results

The median age at Fontan was 6.4 ± 3 years. In multivariable models, pCI was negatively influenced by cyanosis duration (B = −0.240; P = 0.0002). Peak indexed stroke volume (pSVi) was negatively influenced by Norwood procedure (B = -8.415; P = 0.005) and cyanosis duration (B = −1.591; P = 0.0002) and peak heart rate (pHR) by pre-Fontan peripheral oxygen saturation (B = 0.602 P = 0.009). Cyanosis duration ≤ 6.9 years predicted a pSVi ≥ 45 mL/m2 (AUC = 0.747; P = 0.001). Furthermore, pSVi and pHR were correlated with post-Fontan ventricular filling pressure (post-VFP) (respectively rp = −0.539, p = 0.012 and rp = −0.552; P = 0.010) and post-VFP was correlated with bidirectional cavopulmonary shunt duration (rs = 0.498, P = 0.023).

Conclusion

In young Fontan patients, the duration of cyanosis and low peripheral oxygen saturation prior to the Fontan procedure affect both components of cardiac exercise performance probably through increased ventricular filling pressure. Shortening the duration of cyanosis could help preserve cardiac performance in the long term.

Introduction

Despite these advances in paediatric cardiology, the stratification of CHD severity using a simple and reproducible classification has not been established, as can be the NYHA functional class in adult heart failure. Various CHD classifications have been used in CHD, focusing on anatomical lesions, complexity of care, or physiological status, but their prognostic value has not been determined.

Objective

We aimed to compare the accuracy of the main existing CHD classifications (Uzark, Stout and Bethesda classifications), in the prediction of functional status in children with CHD, as determined by cardiopulmonary fitness.

Methods

Longitudinal cohort study.

Results

The CHD population having had 2 CPET included 296 subjects (n = 129 female). The time between the first (T1) and second CPET (T2) assessments was 4.1 ± 1.6 years. The performance of classifications according to VO₂max at T1 was better for Uzark classification. The VO₂max Z-score decreased significantly according to the severity group (groups 1 and 2 > group 3 > group 4) and group 4 had a significant VO₂max decrease of −6.68 [−10.69; −2.67] mL/kg/min compared to group 1. The prediction of classifications at T2 according to VO₂max was better for Uzark classification with AUC values of 0.62 [0.55–0.69], compared to 0.59 [0.51–0.66] for Stout and 0.55 [0.48–0.62] for Bethesda (Fig. 1).

Conclusion

Among the existing cardiovascular risk classifications for CHD, the Uzark classification appeared to be the most reliable for discriminating the severity of CHD according to exercise capacity and for predicting the VO₂max impairment than the other classifications tested. This longitudinal study also showed the continued decline in exercise capacity, whatever the CHD, and recalls the interest of regular monitoring to offer care adapted to these patients (such as rehabilitation programs) for primary prevention of the added cardiovascular risks of adulthood.

Introduction

Congenital heart defects in the context of heterotaxy are severe, with a complex anatomy. These defects are described using the binary concept of clinical loop (D-Loop or L-Loop), which implies that ventricle position is determined during heart looping. Recent work in the mouse has shown unexpected plasticity of ventricle position after heart looping. Based on a analysis of Nodal mutants with heterotaxy and right bronchus isomerism, 27% of D-Loop mutants at birth underwent leftward embryonic heart looping. These mice with a revertant loop, all display abnormal ventricle position.

Objective

Analysis of 3D ventricle position in human patients with heterotaxy and right bronchus isomerism, using the strategy developed in the mouse based on the 3D orientation of the interventricular septum.

Methods

Constitution of an heterotaxy cohort from the Necker database. Controls were selected as patients with transposition of the great arteries who had a systematic CT scan at 6 years. Three dimensions reconstructions of CT scans and quantitative analyses were performed using the Imaris software.

Expected results

Control patients had their right ventricle-left ventricle axis in the expected anteroposterior and left-right orientation. We collected 506 patients with heterotaxy syndrome, 186 of whom had CT scan. Forty patients had right bronchial isomerism. Among them, we found 29 (72%) patients who had an orientation of the interventricular septal axis similar to control, 7 (18%) patients with abnormal supero-inferior ventricles and 4 (10%) patients with abnormal left-right ventricles (Fig. 1). In total, 32% of patients in D-Loop have a malpositioned interventricular septum whereas 0 of patients in L-Loop.

Perspectives

There is a high proportion of malpositioned ventricles in patients with D-Loop (32%), similar to what is observed in Nodal mouse mutants. This result supports conserved mechanisms in the mouse and human, including plasticity of ventricle position after heart looping.

Introduction

Mean pulmonary artery pressure (mPAP) ≥ 20 mmHg defines pulmonary hypertension (PH).

Objective

Critically evaluate this cut-off in very young patients.

Methods

In total, 1129 consecutive patients had a reliable mPAP measurement between 1–12 months of life. These invasive measurements and their clinical history were reviewed.

Results

Age was mean ± SD 158 ± 100 days, weight 5.5 ± 1.9 kg. Patients with a palliated single ventricle anatomy 129/1129, peripheral PA-branch stenosis or MAPCAs 45/1129, PA-banding for open shunt 30/1129, or severe RVOTO 185/1129 were excluded. Of the remaining 726 patients 163/726 (22%) had mPAP < 20 mmHg while 563 (78%) had PH with mPAP ≥ 20 mmHg.

Overall, PH at TTE was the reason for invasive mPAP measurement in 236 patients and only 16/236 patients (7%) had a mPAP < 20 mmHg but 12/16 (75%) had a mPAP ≥ 18 mmHg. In the group with mPAP ≥ 20 mmHg PH persisted at 6-months in 109/236 (46%) (Fig. 1A) and in 68/236 (28%) at long-term follow-up (Fig. 1B). In the group with mPAP between 18 and 20 mmHg PH persisted at 6-months in 9/12 (75%) and in 3/12 (25%) at long-term follow-up. None of the patients with mPAP < 18 mmHg had PH at follow-up.

In patients with less than 3 months of age, PH at TTE was the reason for invasive mPAP measurement in 73/306 (23%) and only 3/73 patients (4%) had a mPAP < 20 mmHg, but 2/3 (75%) had a mPAP ≥ 18 mmHg. In the group with mPAP ≥ 20 mmHg PH persisted at 6-months in 34/70 (48%) (Fig. 2A) and in 34/70 (48%) at long-term follow-up (Fig. 2B). In the group with mPAP between 18 and 20 mmHg PH persisted at 6-months in 2/2 (100%) and in 2/2 (100%) at long-term follow-up. None of those with mPAP < 18 mmHg had PH at follow-up.

Conclusion

Pulmonary hypertension defined by mPAP ≥ 20 mmHg is very common in this cohort of young patients, especially in case of suspected PH at echo. A different cut-off defined as 18 mmHg should be discussed in patients with less than 12-months.

Introduction

Mitral valve replacement in infants is associated with high mortality and a significant rate of re-intervention. There is limited research examining the long-term consequences of valvuloplasty in this particular age group.

Objective

Evaluate patient outcomes with mitral disease (stenosis, regurgitation or both) who had mitral valvuloplasty or replacement in the first year of life.

Methods

Descriptive monocentric retrospective study including all children with mitral valve repair or replacement under 1 year of age over a period of 22 years (2001–2023). The outcomes assessed were: early mortality (at 30 days), late mortality, need for re-intervention.

Results

Fifty-six patients were identified. Median age at surgery was 147 days and median weight was 5.1 kg. Thirty-nine patients underwent mitral valve repair and 17 mitral valve replacement. Mean duration of ICU stay was 7 days. Overall median hospital stay was 11 days. Mortality rate was 17.9% (7.1% before discharge). Overall survival was 96% at 30 days, 86% at 1 year 82% at 3 years. Overall survival was significantly higher in patient with mitral valve repair compared to mitral valve replacement (P = 0.039). The only mortality risk factor identified was replacement of the mitral valve compare to repair, HR: 9 (1.3–94.8; P = 0.038). Twenty-three patients (41.1%) needed re-intervention. The re-intervention free survival rate after repair was 81% at 1 year; 65% at 5 years and 46% at 10 years. The re-intervention free survival rate after replacement was 74% at 1 year; 55% at 5 and 10 years. A log-rank test showed no difference in re-intervention free survival between mitral valve repair or replacement.

Conclusion

Mitral valve surgery in infants carries particularly high risks and is associated with a high rate of re-interventions. While mitral valvuloplasty demonstrates superior outcomes in term of mortality, it frequently serves as a temporary measure, postponing the need for valve replacement.