Utilization of the Melody valve for mitral valve replacement has been previously reported; however, left ventricular outflow tract obstruction is a frequent concern. In this report, a technique for supra-annular placement of the Melody valve in the mitral position is described which will minimize the risk of left ventricular outflow tract obstruction.
Introduction: Right ventricular (RV) function is an important prognostic factor in congenital and acquired heart disease. Quantitative measures of RV lateral wall function obtained from the apical 4 chamber (A4C) view are used to extrapolate global RV function. We aimed to use non-standard views of the RV to establish normal global longitudinal strain (GLS) in additional walls of the RV in healthy children to allow a more comprehensive RV assessment. Methods: A total of 88 healthy children (age 5-17 years) were recruited in a prospective, multi-institutional study. Images were acquired with the Vivid E-9 with post-processing performed using EchoPac (GE Ultrasound). RV views similar to LV apical 2 chamber (A2C) and apical 3 chamber (A3C) views were performed with care taken to exclude septal myocardium. GLS in the RV lateral (A4C), inferior (A2C) and posterior (A3C) walls were averaged over 3 cardiac cycles and calculated if at least 2 of 3 wall segments tracked. A global RV strain assessment was calculated by averaging the 3 available walls. Intraobserver variability was assessed. Results: GLS was measurable in 67% of lateral, 75% of inferior and 45% of posterior walls analyzed. The normal ranges for GLS across the pediatric age group are shown in figure 1. Overall, we observed a decrease in lateral wall GLS with increasing age; inferior and posterior GLS changed less with age. Average global RV strain was -31.3% +/- 2.4%. Intraobserver reliability in 15 patients showed coefficient of variance of 5.2%, 3.9% and 4% with no significant bias in the lateral, inferior and posterior walls. Conclusions: This report establishes reference values for inferior and posterior RV wall strain in children. We show that speckle tracking strain analysis is feasible in alternate walls of the RV allowing for more global assessment of RV function. Establishment of normal values in a larger cohort including younger children may improve future assessment of dysfunction in RV pathology.
ABSTRACT Background Pulmonary vein stenosis (PVS) is a rare condition in which neointimal proliferation leads to venous and arterial hypertension. Little is known about PVS after heart transplant (HTx) in children. We sought to describe the characteristics and outcomes of children who develop PVS after HTx. Methods We performed a retrospective review of patients ≤18 years old who underwent HTx at two HTx centers between April 2012 and October 2023. Patients with PVS were identified via database queries. Cardiac diagnosis, PVS location and extent, and outcomes were recorded. Results Over 11.5 years, 422 patients underwent HTx across both centers. Nineteen patients with PVS (10 male) were identified, 15 with de novo PVS. Sixteen had underlying congenital heart disease (CHD), two with anomalous pulmonary venous return. PVS was diagnosed at a median of 2 months (range 2 weeks to 14 years) after HTx. At time of initial diagnosis, 13 patients had one‐vessel PVS. At final follow‐up, 7/19 (37%) had increases in the number of vessels involved. Six patients underwent surgery, and nine patients had stent or balloon angioplasty. Two patients were treated for pulmonary hypertension following PVS diagnosis. Three patients died from right heart failure secondary to PVS. Conclusions This is the largest study to describe the characteristics of post‐HTx PVS in children. PVS occurs in 4.5% of HTx, and underlying CHD is a strong risk factor. Multiple vessels can be involved and may require catheter‐based or surgical intervention. Clinicians must be vigilant in monitoring the development of PVS in this population.
Tricuspid annular plane systolic excursion (TAPSE) is a measure of right ventricular (RV) longitudinal function that correlates with functional status and mortality in adults with pulmonary hypertension (PH). The diagnostic and predictive value of TAPSE in children with PH has not been fully examined. We aimed to define TAPSE across aetiologies of paediatric PH and assess the correlation between TAPSE and measures of disease severity. TAPSE measurements were obtained in 84 children and young adults undergoing treatment for PH and 315 healthy children to establish z-scores at moderate altitude for comparison. The relationships between TAPSE and echocardiographic, biomarker, and functional measures of disease severity between aetiologies were assessed. TAPSE z-scores in PH patients with congenital heart disease (CHD) repaired with open cardiac surgery (n = 20, mean −2.73) were significantly decreased compared with normal children and patients with other aetiologies of PH (P < 0.001) but did not reflect poorer clinical status. TAPSE z-scores in children with idiopathic PH (n = 29, −0.41), unrepaired CHD (n = 11, −0.1), and PH related to systemic disease (n = 14, −0.39) were not different from normal. TAPSE correlated modestly with brain natriuretic peptide, echocardiographic function parameters, and functional class except in patients with repaired CHD. Children with PH maintain normal TAPSE values early except when associated with repaired CHD. Superior RV adaptation to high afterload in children compared with adults may account for this finding. Reduced TAPSE after repair of CHD does not correlate with functional status and may reflect post-operative changes rather than poor function primarily due to PH.
Abstract Introduction : In adults with chronic kidney disease (CKD), abnormal left atrial reservoir strain (LASr) is an early, yet clinically significant, indicator of myocardial dysfunction. However, left atrial mechanics are understudied in youth with CKD. The objective of this study was to assess left atrial strain function in youth with CKD and similarly aged, healthy controls. Methods : We performed a single-center, retrospective, observational study of persons aged 12-21 years with stage 3-4 CKD and healthy controls. Exclusion criteria included a history of a kidney or other solid organ transplant, congenital heart disease, and/or dialysis requirement <3 months prior. We measured LAS (LASr, conduit, contractile), E/e’, E/A, left ventricular mass index (LVMI), and ejection fraction. Pearson correlations were performed between echocardiographic measures. Results: This study included 37 patients with CKD and 19 controls. Mean age was similar between groups and male sex was over-represented in both groups (CKD: 62%, Healthy: 63%). Mean ± standard deviation (SD) eGFR in the CKD group was 32 ±14mL/min/1.73m2. Mean absolute LASr was significantly lower in those with CKD (43.0 ±8.5%) compared to healthy controls (47.4 ±6.1%). Patients with CKD had significantly higher LVMI, and lower E/A and E’ compared to controls. There was poor correlation between LASr with E/A, E/e’, and LVMI. Conclusions : As observed in adults with CKD, LASr was significantly lower in youth with CKD compared to healthy controls. Moreover, LASr poorly correlated with traditional measures of diastolic dysfunction such as E/e’ and E/A.
