Longitudinal changes in ventricular function and atrioventricular valve failure following cardiac morphology after Fontan procedure Ah Young Kim, Ji Eun Byun, Ji Eun Hwang, Wongi Woo, Chang Sin Kim, Se Yong Jung, Lucy Youngmin Eun, Jae Young Choi, Yu Rim Shin, Jo Won Jung, Han Ki Park Journal of Thoracic Disease, 2025 Background: Ventricular dysfunction and atrioventricular valve (AVV) failure in Fontan patients are associated with adverse long-term outcomes; however, data on longitudinal changes and their relationship with morphology are lacking. This study aimed to describe longitudinal changes in ventricular function and AVV regurgitation and to determine the risk factors in Fontan patients who develop ventricular dysfunction and AVV failure. Methods: We retrospectively reviewed echocardiographic images from patients who underwent Fontan procedure from 1984 to 2015. Mixed-effects model fits a unique linear regression line using serial ejection fraction (EF) and AVV regurgitation. Multivariate logistic regression was performed to find morphologic risk factors for ventricular dysfunction and AVV failure. Results: Out of 174 patients who underwent 3,203 echocardiograms, a significant 6.2% decrease in EF was observed (P<0.001) over a median follow-up of 17.6 years (interquartile range, 15.3-19.2 years) post Fontan procedure. Higher prevalence of ventricular dysfunction (EF <50%) was noted in dominant right ventricle (RV) and two-ventricular (2V) morphologies compared to left ventricle (LV) (P<0.001). AVV failure was more common in RV and 2V morphologies as well (P<0.001). Notably, patients with tricuspid valve (TV) and common AVV exhibited the most pronounced AVV failure (P<0.001). In multivariate analysis, RV, TV and common AVV were correlated with AVV failure (hazard ratio 5.37, 8.24 and 5.43, respectively). Conclusions: Fontan patients with long-term follow-up showed a progressive decline in ventricular and AVV function. Dominant RV, TV and common AVV were prognostic factors for predicting AVV failure. Further studies are warranted to explore and validate these findings.
Patient with Behçet Syndrome with Subaortic Pseudoaneurysm Who Underwent the Bentall Operation Showed Improvement after Medical Treatment Hanbyul Lee, Chang Sin Kim, Jo Won Jung, Han Ki Park, Jong Gyun Ahn Yonsei Medical Journal, 2025 Aortic regurgitation (AR) in children is usually caused by congenital valve anomalies, and Behçet syndrome (BS) can be suspected in cases of isolated AR. Patients with BS undergoing aortic valve surgery due to aortic valve invasion have a high risk of complications, such as leakage around the valve and dehiscence. Cardiovascular involvement occurs in 7%-46% of adult patients with BS and is the main cause of mortality; however, its prevalence is unclear and rare in children. A 12-year-old boy was diagnosed with severe AR associated with BS. A progressive subaortic pseudoaneurysm was observed after aortic valve replacement. The periaortic intracardiac pouch was at risk of rupture; therefore, the patient underwent a Bentall operation. After the Bentall procedure, a newly developed subaortic pseudoaneurysm was detected below the prosthetic valve. However, the risk of rupture reduced as the subaortic pseudoaneurysm regressed with anti-inflammatory drugs alone without reoperation. Repeated surgery is inevitable in patients with BS undergoing aortic valve surgery due to the progressive chronic inflammatory reactions that present with a pseudoaneurysm. Here, we report an 8-year follow-up of a pediatric case of BS with subaortic pseudoaneurysm, highlighting the importance of close follow-up, medical management, and early diagnosis in treating this condition.
Congenitally corrected transposition of the great arteries: One disease, diverse manifestations - A case series Maria Luisa Benesch Vidal, Han Ki Park, Kouichi Toda, Geetha Kandavello, Lucy Youngmin Eun, Dominica Zentner, Motoki Komori, Christoph Sinning European Heart Journal Case Reports, 2025 Background Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect with heterogenous clinical manifestations that can pose both diagnostic and management challenges throughout life. Case summary We describe four patients with ccTGA and different presentations including sudden cardiac arrest, progressive heart failure, post-partum heart failure, and NSTEMI. Aims and Discussion This case series aims to illustrate the importance of multimodality imaging to assist the diagnosis and support treatment strategies in patients with ccTGA. Direct long-term sequalae, such as arrythmias and heart failure, and the associated management challenges are highlighted. Additionally, the challenges of managing pregnancy with a sRV and the development of acquired heart disease demonstrate the clinical care challenges in caring for this population across the life span.
2023 KASNet Guidelines on Atrial Fibrillation Surgery Hyung Gon Je, Jae Woong Choi, Ho Young Hwang, Ho Jin Kim, Joon Bum Kim, Hee-Jung Kim, Jae-Sung Choi, Dong Seop Jeong, Jae Gun Kwak, Han Ki Park, Seung Hyun Lee, Cheong Lim, Jae Won Lee, and Journal of Chest Surgery, 2024 Hyung Gon Je, Jae Woong Choi, Ho Young Hwang, Ho Jin Kim, Joon Bum Kim, Hee-Jung Kim, Jae-Sung Choi, Dong Seop Jeong, Jae Gun Kwak, Han Ki Park, Seung Hyun Lee, Cheong Lim, Jae Won Lee, for the KASNet Guideline Writing Committee. J Chest Surg 2024;57:1-24. https://doi.org/10.5090/jcs.23.127
Heart Transplantation in a Patient with Complex Congenital Heart Disease, Physiologic Single Lung, and Severe Pulmonary Hypertension Hae Won Lee, Jo Won Jung, Ah Young Kim, Han Ki Park Yonsei Medical Journal, 2022 Here we report a case of successful heart transplantation (HT) in a patient with high risk on HT due to her complex congenital heart disease and its consequent complications; physiologic single lung and severe pulmonary arterial hypertension. HT was successfully performed in our patient using meticulous perioperative management techniques, such as control of collateral vessels, appropriate donor selection, pulmonary vasodilator therapy, post-transplant extracorporeal membranous oxygenation support, and atrial septostomy for right ventricular unloading.
Incidence and Related Risk Factors of Junctional Ectopic Tachycardia in Infants after Cardiac Surgery for Congenital Heart Disease Jae Hee Seol, Se Yong Jung, Jae Young Choi, Han Ki Park, Young Hwan Park, Nam Kyun Kim Congenital Heart Disease, 2022 Objective: Junctional ectopic tachycardia is common after cardiac surgery for congenital heart disease. However, its incidence and related risk factors in infants after cardiac surgery are not well known. The objective of this study was to determine the overall incidence and related risk factors for junctional ectopic tachycardia in neonates and infants. Methods: We enrolled a total of 271 patients aged
Is it possible for children in duchenne muscular dystrophy to preserve cardiac function with medical assistance? Han Geul Kim, Lucy Youngmin Eun, Han Ki Park Children, 2020 In patients with Duchenne muscular dystrophy (DMD), death secondary to cardiac or respiratory failure typically occurs in the second or third decade without treatment. Although cardiac dysfunction is treated with standard heart-failure strategies, it remains insufficient in DMD children. The purpose of this study was to evaluate the efficiency of cardiac medication and noninvasive ventilator support in DMD cardiomyopathy children with analyzing echocardiographic data. Forty-eight DMD children patients were included and divided into 2 groups by left ventricular (LV) ejection fraction (EF) at the time of initial treatment. Group 1: LV EF ≥ 45% and Group 2: LV EF < 45%. p-values were calculated using a Linear mixed model to estimate the association between cardiac medications and echocardiographic measurements. Before and after cardiac medications, the change values were significantly different in interventricular septal thickness at end diastole (IVSd), interventricular septal thickness at end systole (IVSs), left ventricular internal diameter end systole (LVIDs), left ventricular posterior wall thickness end diastole (LVPWd), ejection fraction (EF), fractional shortening (FS), deceleration time (DT), DT slope, Lat A’ and Lat E/E’ (p < 0.05). Group 2 patients revealed to take more kinds of cardiac medications than Group 1 (p < 0.05) including ACEIs, beta-blocker, and inotropics, then LV EF was better preserved in Group 2 than Group 1. It is certainly helpful to take individualized medical combination therapy including inotropic agents for cardiomyopathy in DMD children patients with EF < 45%.
Degenerative Calcification of Pericardial Bioprostheses: Comparison of Five Implantation Methods in a Rabbit Model Journal of Heart Valve Disease, 2015
