Background: Despite the development of several imaging modalities for diagnosing Fontan-associated liver disease (FALD), there is no optimal protocol for the follow-up of FALD. We conducted a systematic review and meta-analysis to identify factors related to liver fibrosis using biopsy reports and to identify alternative noninvasive modalities that could better reflect liver histological changes in FALD. Methods: A systematic review and meta-analysis were conducted following the PRISMA guidelines Table S2. We searched Embase, PubMed, and Cochrane databases for studies on FALD, focusing on those assessing clinical factors associated with liver fibrosis severity through liver biopsy and noninvasive imaging techniques. Results: A total of 42 studies were identified, of which 12 conducted meta-analyses and subgroup analyses of the severity of liver fibrosis using liver biopsies. Liver biopsy results showed a weak positive correlation between Fontan duration and fibrosis severity (R = 0.36). Subgroup analyses revealed significant differences in hemodynamic parameters, such as Fontan pressure, between patients with mild and severe fibrosis. Platelet count, aspartate aminotransferase to platelet ratio index, and fibrosis-4 index were significantly associated with fibrosis severity, with severe fibrosis showing lower platelet counts and higher aspartate aminotransferase to platelet ratio index and fibrosis-4 index levels. Noninvasive imaging modalities, particularly magnetic resonance elastography and shear wave elastography, demonstrated strong correlations with biopsy-confirmed fibrosis severity. Conclusions: This study identifies key clinical factors, and noninvasive modalities accurately reflect liver fibrosis severity in patients with FALD. Clinical factors such as platelet count, aspartate aminotransferase to platelet ratio index, and fibrosis-4 index may aid in identifying patients at risk for severe fibrosis. In addition, magnetic resonance elastography and shear wave elastography are promising tools for noninvasive assessment in our study. Further research is needed to refine these diagnostic approaches and improve patient management.

Pulmonary arterial hypertension (PAH) related to an atrial septal defect (ASD) poses a challenge to transcatheter closure of an ASD (tcASD). We aimed to determine the predictors for remaining PAH (rPAH) post-tcASD. This retrospective study was conducted at a single tertiary university hospital. Adult patients with an ASD and PAH were divided into three groups according to pulmonary vascular resistance (PVR). Normalization of pulmonary atrial systolic pressure (PASP) was defined as an estimated right ventricular systolic pressure < 40 mmHg and was determined using transthoracic echocardiography. Among 119 patients, 80% showed PAH normalization post-tcASD. Normalization of PAH post-tcASD was observed in 100%, 56.2%, and 28.6% of patients in mild, moderate, and severe PVR groups, respectively. The patients’ New York Heart Association functional class improved. Multivariate logistic regression analysis showed that age and high PVR were significant risk factors for rPAH. A receiving operator curve analysis showed a PASP cutoff value > 67.5 mmHg to be predictive of rPAH post-tcASD, with an area under the curve value of 0.944 (sensitivity, 0.922; specificity 0.933). Most patients, including moderate-to-severe PAH patients, improved hemodynamically and clinically with tcASD. Since patients with severe PAH are at a risk of rPAH, tcASD should be performed by selecting the patient carefully based on pre-procedure medication, a vasoreactivity test, and a balloon occlusion test.

Choi HJ, et al. Korean Circ J. 2025 Apr;55:e63. https://doi.org/10.4070/kcj.2024.0381

We aimed to determine the feasibility, efficacy, success, and safety of intracardiac echocardiography (ICE) in transcatheter multiple atrial septal defect (ASD) closure. Of 185 patients with multiple ASDs who underwent transcatheter closure, 140 (76%) patients who weighed <30kg with a narrow distance between defects or in whom single device closure was anticipated were guided by ICE and 45 patients were guided by three-dimensional (3D) transesophageal echocardiography (TEE) with or without ICE. Patients in the ICE group were relatively younger and weighed less than those in the 3D TEE group (p < 0.0001). The ratio of the distance between defects >7 mm was high, and more cases required ≥2 devices in the 3D TEE group than those in the ICE group (p < 0.0001). All patients in the 3D TEE group and seven patients (5%) in the ICE group were operated on under general anesthesia (p < 0.0001). The fluoroscopic time was shorter in the ICE group (13.98 ± 6.24 min vs. 24.86 ± 16.47 min, p = 0.0005). No difference in the complete closure rate and complications was observed. ICE-guided transcatheter and 3D TEE were feasible, safe, and effective in successful multiple ASD device closures, especially for young children and patients at high risk under general anesthesia.

Glycogen storage disease (GSD) is a hereditary metabolic disorder caused by enzyme deficiency resulting in glycogen accumulation in the liver, muscle, heart, or kidney. GSD types II, III, IV, and IX are associated with cardiac involvement. However, cardiac manifestation in other GSD types is unclear. This study aimed to describe whether energy deprivation and the toxic effects of accumulated glycogen affect the heart of patients with GSD. We evaluated the left ventricle (LV) wall mass, LV systolic and diastolic function and myocardial strain with conventional echocardiography and two-dimensional speckle-tracking echocardiography (2D STE) in 62 patients with GSD type I, III, VI and IX who visited the Wonju Severance Hospital in 2021. Among the GSD patients, the echocardiographic parameters of 55 pediatrics were converted into z-scores and analyzed. Of the patients, 43 (62.3%), 7 (11.3%) and 12 (19.4%) patients were diagnosed with GSD type I, type III, and type IX, respectively. The median age was 9 years (range, 1–36 years), with 55 children under 18 years old and seven adults over 18 years. For the 55 pediatric patients, the echocardiographic parameters were converted into a z-score and analyzed. Multiple linear regression analysis showed that the BMI z-score (p = 0.022) and CK (p = 0.020) predicted increased LV mass z-score, regardless of GSD type. There was no difference in the diastolic and systolic functions according to myocardial thickness; however, 2D STE showed a negative correlation with the LV mass (r = −0.28, p = 0.041). Given that patients with GSD tend to be overweight, serial evaluation with echocardiography might be required for all types of GSD.

Most interventionalists use the Amplatzer Duct Occluder (ADO) or the Nit-Occlud^® Coils (NOC) to close patent ductus arteriosus (PDA). Data regarding the success and effect of NOCs in the occlusion of large PDAs are insufficient. We aimed to investigate whether the PDA occlusion of large PDAs using NOC is safe and efficient for all ages. This was a retrospective study involving 361 pediatric and adult patients who underwent the transcatheter closure of PDA using NOC over the past 21 years for all PDA sizes and ages. The sizes of PDA were classified as small, moderate, and large. A comparison of the aortic pressure before and after PDA occlusion using NOC showed significant differences in terms of systolic and pulse pressures for all age groups (p < 0.05). The rate of the residual shunts of NOC was 2%, while the rate of complete occlusions of NOC was 98% at 12 months after occlusion regardless of the shape of PDA. The complication rate with PDA occlusion using NOC was 5%. PDA occlusion using NOC is as effective and safe as ADO for the occlusion of PDA of all sizes. Therefore, PDA occlusion using NOC can be a safe and feasible procedure to close various sizes and types of PDA without complications.