Self-Expanding Pulmonary Valves in 53 Patients With Native Repaired Right Ventricular Outflow Tracts.

BACKGROUND: Self-expanding pulmonary valve grafts have been designed for percutaneous pulmonary valve implantation (PPVI) in patients with native repaired right ventricular (RV) outflow tracts (RVOTs). However, their efficacy, in terms of RV function and graft remodelling remain unclear. METHODS: Patients with native RVOTs who received Venus P-valve (N = 15) or Pulsta valve (N = 38) implants between 2017 and 2022 were enrolled. We collected data on patient characteristics and cardiac catheterization parameters as well as imaging and laboratory data before, immediately after, and 6 to 12 months after PPVI and identified risk factors for RV dysfunction. RESULTS: Valve implantation was successful in 98.1% of patients. The median duration of follow-up was 27.5 months. In the first 6 months after PPVI, all patients exhibited resolution of paradoxical septal motion and a significant reduction (P < 0.05) in RV volume, N-terminal pro-B-type natriuretic peptide levels, and valve eccentricity indices (-3.9%). Normalization of the RV ejection fraction (≥ 50%) was detected in only 9 patients (17.3%) and was independently associated with the RV end-diastolic volume index before PPVI (P = 0.03). Nine patients had residual or recurrent pulmonary regurgitation or paravalvular leak (graded as ≥ mild), which was associated with a larger eccentricity index (> 8%) and subsided by 12 months postimplantation. CONCLUSIONS: We identified the risk factors likely to be associated with RV dysfunction and pulmonary regurgitation following PPVI in patients with native repaired RVOTs. RV volume-based patient selection is recommended for PPVI of a self-expanding pulmonary valve, along with monitoring of graft geometry.

Determining Biventricular Repair Feasibility in Children with Dominant Right Ventricle Using Left Ventricular Quality Measured on Cardiac Computed Tomography.

Background: Left-ventricular (LV) characteristic measurements are crucial for evaluating the feasibility of biventricular repair (BiVR). This study aimed to determine the threshold of LV quality on cardiac computed tomography (CCT) for BiVR in children with a dominant right ventricle (DRV). Methods: We retrospectively reviewed all children with a DRV who underwent either BiVR or single ventricle palliation (SVP) at our institution between 2003 and 2019 in a case-control study with healthy individuals. Measurements including LV end-diastolic volume (LVEDV, mL), LV myocardial mass (LVMM, gm), and mitral annulus area (MAA, cm 2 ) were quantified using CCT. The factor with the highest correlation with body size was used to adjust these three measurements to derive normal references in the control group. The LV quality of patients on each CCT measurement was represented as a percentage of the normal reference data that we established. The feasible LV quality for BiVR was defined as the lowest limit of all three LV measurements in one subject who survived BiVR among our patients with DRVs. Results: The cohort comprised 30 patients and 76 healthy controls. Height was the factor with the highest correlation with all three LV measurements. Height-adjusted normal reference curves and formulas were created. The mean LV quality in surviving patients who underwent BiVR was better than that in those who underwent SVP. The lowest limits for LV quality in one survivor of BiVR were 39.1% LVEDV, 49.0% LVMM, and 44.9% MAA. During follow up, the LV quality of patients who received BiVR shifted to the normal range. Conclusions: LV quality should be at least greater than 45% of normal values to promise survival in patients with DRVs who are being considered for a BiVR.

Dynamic cardiac computed tomography characteristics of double-chambered right ventricle.

To introduce image characteristics of double-chambered right ventricle on cardiac computed tomography and set a diagnostic criterion for the diagnosis. We retrospectively collected and measured the right ventricular constrictive ratio on computed tomography images in children who had simple ventricular septal defects in the past 10 years, because double-chambered right ventricle is often associated with ventricular septal defects. The right ventricular constrictive ratio was defined as the subinfundibular cross-sectional intraluminal area during end-systole divided by the area during end-diastole in the same patient. We compared the right ventricular constrictive ratio between subjects with concomitant double-chambered right ventricle and those without. 52 children were included, and 23 (44.2%) of them have concomitant double-chambered right ventricle. In most cases (n = 21; 91.3%), the hypertrophied muscular bundles occur just inferior to the level of the supraventricular crest in the right ventricle. Mean right ventricular constrictive ratio in patients with double-chambered right ventricle (15%) was significantly smaller than that without (29%). A cut-off value of a right ventricular constrictive ratio less than 20.1% was established to diagnose double-chambered right ventricle with an 89.7% sensitivity and 78. 3% specificity. Right ventricular constrictive ratio can be a valuable asset for the preoperative diagnosis of double-chambered right ventricle with cardiac computed tomography.

Diagnosis of Pulmonary Arterial Hypertension in Children by Using Cardiac Computed Tomography.

OBJECTIVE: To establish diagnostic criteria for pulmonary arterial hypertension (PAH) in children by using parameters obtained through noninvasive cardiac computed tomography (CCT). MATERIALS AND METHODS: We retrospectively measured parameters from CCT images of children from a single institution in a multiple stepwise process. A total of 208 children with mean age of 10.5 years (range: 4 days-18.9 years) were assessed. The variables were classified into three groups: the great arteries; the ventricular walls; and the bilateral ventricular cavities. The relationship between the parameters obtained from the CCT images and mean pulmonary arterial pressure (mPAP) was tested and adjusted by the children's body size. Reference curves for the pulmonary trunk diameter (PTD) and ratio of diameter of pulmonary trunk to ascending aorta (rPTAo) of children with CCT images of normal hearts, adjusted for height, were plotted. Threshold lines were established on the reference curves. RESULTS: PTD and rPTAo on the CCT images were significantly positively correlated with mPAP (r > 0.85, p < 0.01). Height was the body size parameter most correlated with PTD (r = 0.91, p < 0.01) and rPTAo (r = -0.69, p < 0.01). On the basis of the threshold lines on the reference curves, PTD and rPTAo both showed 88.9% sensitivity for PAH diagnosis, with negative predictive values of 93.3% and 92.9%, respectively. CONCLUSION: PTD and rPTAo measured from CCT images were significantly correlated with mPAP in children. Reference curves and the formula of PTD and rPTAo adjusted for height could be practical for diagnosing PAH in children.

Developing a clinical scoring system to differentiate deep-seated atypical lipomatous tumor from lipoma of soft tissue.

BACKGROUND: Atypical lipomatous tumor (ALT) is a low-grade malignancy that frequently occurs at a subfascial anatomical location. While marginal excision is adequate for lipomas, excision with a surgical margin is suggested for ALTs. However, ALTs and lipomas are difficult to differentiate preoperatively, even with the help of imaging studies. In this study, we aimed to formulate a scoring system based on selected clinical and imaging characteristics to enhance the accuracy of pre-operative diagnosis of deep-seated ALTs. METHODS: We enrolled 417 cases of deep-seated lipoma and 53 cases of ALTs from soft tissue treated between 2005 and 2016. Tumors arising from the bone, internal cavities, retroperitoneum, or nervous system were excluded. Clinical data were analyzed along with magnetic resonance image (MRI) features. We further developed a scoring formula to distinguish deep-seated ALTs from lipomas. RESULTS: Older age, tumor location at lower limbs, and the presence of MRI features (larger size, thick septa > 2 mm, contrast enhancement>1 cm, fat component <75%) are identified as risk factors of ALT and were utilized to develop a scoring system for distinguishing ALTs from lipomas. The formula exhibited 90% sensitivity and 92.5% specificity, and a score more than 0.214 suggested a diagnosis of ALT. CONCLUSIONS: The scoring system developed in this study can facilitate the pre-operative diagnosis of deep-seated ALTs and lipomas. If ALT is suspected, further tumor biopsy followed by molecular diagnosis can establish a definite diagnosis. Therefore, this scoring system can serve as a cost-effective tool for the clinical management of deep-seated lipomatous tumors.