• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1041-1048
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• 2007

Not a few patients in children and adolescents are suffering from right ventricular (RV) dysfunction resulting from various conditions such as chronic lung disease, left ventricular dysfunction, pulmonary hypertension, or congenital heart defect. The RV is different from the left ventricle in terms of ventricular morphology, myocardial contractile pattern and special vulnerability to the pressure overload. Right ventricular failure (RVF) can be evaluated in terms of decreased RV contractility, RV volume overload, and/or RV pressure overload. The management for RVF starts from clear understanding of the pathophysiology of RVF. In addition to correction of the underlying disease, management of RVF per se is very important. Meticulous control of volume status, inotropic agents, vasopressors, and pulmonary selective vasodilators are the main tools in the management of RVF. The relative importance of each tool depends on the individual clinical status. Medical assist device and surgery can be considered selectively in case of refractory RVF to optimal medical treatment.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1055-1060
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• 2007

Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes the systemic circulation (systemic RV) in congenital heart defects. Despite major progress being made, assessing the RV remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and cineangiography). Evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. While there is extensive literature on the pathophysiology and treatment of left heart failure, the data for right-sided heart failure is scarce. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. An understanding of RV physiology and hemodynamics will lead to a better understanding of current and future treatment strategies for right heart failure. This will review right-sided heart failure with the implications of volume and pressure loading of the RV in congenital heart diseases.

• Journal of Chest Surgery
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• v.57 no.1
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• pp.70-78
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• 2024

Background: This study investigated the outcomes of biventricular repair using right ventricle to pulmonary artery (RV-PA) conduit placement in patients aged <1 year. Methods: Patients aged <1 year who underwent biventricular repair using an RV-PA conduit between 2011 and 2020 were included in this study. The outcomes of interest were death from any cause, conduit reintervention, and conduit dysfunction (peak velocity of ≥3.5 m/sec or moderate or severe regurgitation). Results: In total, 141 patients were enrolled. The median age at initial conduit implantation was 6 months. The median conduit diameter z-score was 1.3. The overall 5-year survival rate was 89.6%. In the multivariable analysis, younger age (p=0.006) and longer cardiopulmonary bypass time (p=0.001) were risk factors for overall mortality. During follow-up, 61 patients required conduit reintervention, and conduit dysfunction occurred in 68 patients. The 5-year freedom from conduit reintervention and dysfunction rates were 52.9% and 45.9%, respectively. In the multivariable analysis, a smaller conduit z-score (p<0.001) was a shared risk factor for both conduit reintervention and dysfunction. Analysis of variance demonstrated a nonlinear relationship between the conduit z-score and conduit reintervention or dysfunction. The hazard ratio was lowest in patients with a conduit z-score of 1.3 for reintervention and a conduit z-score of 1.4 for dysfunction. Conclusion: RV-PA conduit placement can be safely performed in infants. A significant number of patients required conduit reintervention and had conduit dysfunction. A slightly oversized conduit with a z-score of 1.3 may reduce the risk of conduit reintervention or dysfunction.

• Clinical and Experimental Pediatrics
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• v.57 no.11
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• pp.484-488
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• 2014

Purpose: Chronic upper airway obstruction causes hypoxemic pulmonary vasoconstriction, which may lead to right ventricle (RV) dysfunction. Adenotonsillar hypertrophy (ATH) is the most common cause of upper airway obstruction in children. Therefore, we aimed to evaluate RV function in children with ATH. Methods: Twenty-one children (male/female, 15/6; mean age, $92.3{\pm}39.0$ months; age range, 4-15 years) with ATH and 21 healthy age- and gender-matched controls were included in this study. Tricuspid annular plane systolic excursion and RV myocardial performance index were measured by transthoracic echocardiography. Further, the plasma level of N-terminal of probrain natriuretic peptide (NT-proBNP), an indicator of RV function, was determined. Results: The snoring-tiredness during daytime-observed apnea-high blood pressure (STOP) questionnaire was completed by the patients' parents, and loud snoring was noted in the ATH group. The plasma NT-proBNP level was significantly higher in the ATH group than that in the controls ($66.44{\pm}37.63pg/mL$ vs. $27.85{\pm}8.89pg/mL$, P=0.001). The echocardiographic parameters were not significantly different between the groups. Conclusion: We were unable to confirm the significance of echocardiographic evidence of RV dysfunction in the management of children with ATH. However, the plasma NT-proBNP level was significantly higher in the ATH group than that in the control, suggesting that chronic airway obstruction in children may carry a risk for cardiac dysfunction. Therefore, more patients should be examined using transthoracic echocardiography. In addition, pediatricians and otolaryngologists should consider cardiologic aspects during the management of children with severe ATH.

• Journal of Cardiovascular Imaging
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• v.26 no.4
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• pp.201-213
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• 2018

BACKGROUND: Children with significant adenotonsillar hypertrophy (ATH) may show right ventricular (RV) dysfunction. We aimed to evaluate RV dysfunction in such children before adenotonsillectomy by evaluating peak longitudinal right atrial (RA) strain (PLRAS) in systole. PLRAS, electrocardiogram (ECG) and conventional echocardiographic parameters were compared to distinguish children with significant ATH with sleep-related breathing disorder (ATH-SRBD) from controls. METHODS: Fifty-six children (23 controls and 33 children with ATH-SRBD without symptoms of heart failure) were retrospectively studied. Preoperative echocardiograms and ECGs of children with ATH-SRBD who underwent adenotonsillectomy were compared to those of controls. Available postoperative ECGs and echocardiograms were also analyzed. RESULTS: Preoperatively, prolonged maximum P-wave duration (Pmax) and P-wave dispersion (PWD), decreased PLRAS, and increased tricuspid annulus E/E' were found in children with ATH-SRBD compared to those of controls. From the receiver operating characteristic curves, PLRAS was not inferior compared to tricuspid annulus E/E', Pmax, and PWD in differentiating children with ATH-SRBD from controls; however, the discriminative abilities of all four parameters were poor. In children who underwent adenotonsillectomy, echocardiograms $1.2{\pm}0.4$ years after adenotonsillectomy showed no difference in postoperative PLRAS and tricuspid annulus E/E' when compared with those of the preoperative period. CONCLUSIONS: Impaired RA deformation was reflected as decreased PLRAS in children with ATH-SRBD before adenotonsillectomy. Decreased PLRAS in these children may indicate subtle RV dysfunction and increased proarrhythmic risk. However, usefulness of PLRAS as an individual parameter in differentiating preoperative children with ATH-SRBD from controls was limited, similar to those of tricuspid annulus E/E', Pmax, and PWD.

• Korean Circulation Journal
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• v.48 no.12
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• pp.1135-1144
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• 2018

Background and Objectives: Mitochondria play a key role in the pathophysiology of heart failure and mitochondrial permeability transition pore (MPTP) play a critical role in cell death and a critical target for cardioprotection. The aim of this study was to evaluate the protective effects of cyclosporine A (CsA), one of MPTP blockers, and morphological changes of mitochondria and MPTP related proteins in monocrotaline (MCT) induced pulmonary arterial hypertension (PAH). Methods: Eight weeks old Sprague-Dawley rats were randomized to control, MCT (60 mg/kg) and MCT plus CsA (10 mg/kg/day) treatment groups. Four weeks later, right ventricular hypertrophy (RVH) and morphological changes of right ventricle (RV) were done. Western blot and reverse transcription polymerase chain reaction (RT-PCR) for MPTP related protein were performed. Results: In electron microscopy, CsA treatment prevented MCT-induced mitochondrial disruption of RV. RVH was significantly increased in MCT group compared to that of the controls but RVH was more increased with CsA treatment. Thickened medial wall thickness of pulmonary arteriole in PAH was not changed after CsA treatment. In western blot, caspase-3 was significantly increased in MCT group, and was attenuated in CsA treatment. There were no significant differences in voltage-dependent anion channel, adenine nucleotide translocator 1 and cyclophilin D expression in western blot and RT-PCR between the 3 groups. Conclusions: CsA reduces MCT induced RV mitochondrial damage. Although, MPTP blocking does not reverse pulmonary pathology, it may reduce RV dysfunction in PAH. The results suggest that it could serve as an adjunctive therapy to PAH treatment.

• Journal of Yeungnam Medical Science
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• v.31 no.1
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• pp.9-12
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• 2014

Postinfarction ventricular septal rupture (VSR) is a rare but fatal complication of acute myocardial infarction. In many cases, postinfarction VSR leads to hemodynamic instability and urgent surgical treatment is necessary. Here we describe a case of a patient with right ventricular (RV) dysfunction caused by acute RV infarction and with cardiogenic shock, whose condition improved after development of postinfarction VSR, but the patient died after corrective surgery.

• Radiation Oncology Journal
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• v.15 no.2
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• pp.137-143
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• 1997

Purpose : Radiation pneumonitis is one of the complications caused by radiation therapy that includes a Portion of the lung tissue. The severity of radiation induced pulmonary dysfunction depends on the irradiated lung volume, total dose, dose rate and underlying Pulmonary function. It also depends on whether chemotherapy is done or not. The irradiated lung volume is the most important factor to predict the pulmonary dysfunction in breast cancer Patients following radiation therapy. There are some data that show the irradiated lung volume measured from CT scans as a part of treatment Planning with the tangential beams. But such data have not been reported in Korea. We planned to evaluate the irradiated lung volume quantitatively using CT scans for the breast tangential field and search for useful factors that could Predict the irradiated lung volume Materials and Methods : The lung volume was measured for 25 patients with breast cancer irradiated with tangential field from Jan.1995 to Aug.1996. Parameters that can predict the irradiated lung volume included; (1) the peruendicular distance from the Posterior tangential edge to the posterior part of the anterior chest wall at the center of the field (CLD) ; (2) the maximum perpendicular distance from the posterior tangential field edge to the posterior Part of the anterior chest wall (MLD) ; (3) the greatest perpendicular distance from the Posterior tangential edge to the posterior part of anterior chest wall on CT image at the center of the longitudinal field (GPD) ; (4) the length of the longitudinal field (L). The irradiated lung volume(RV), the entire both lung volume(EV) and the ipsilateral lung volume(IV) were measured using dose volume histogram. The relationship between the irradiated lung volume and predictors was evaluated by regression analysis. Results :The RV is 61-279cc (mean 170cc), the RV/EV is $2.9-13.0\%\;(mean\;5.8\%)$ and the RV/IV is $4.9-29.0\%\;(mean\;12.2\%)$. The CLD, the MLD and the GPD ave 1.9-3.3cm, 1.9-3.3cm and 1.4-3.1cm respectively. The significant relations between the irradiated lung volume such as RV. RV/EV, RV/IV and parameters such as CLD, MLD, GPO, L. $CLD\timesL,\;MLD\timesL\;and\;GPD\timesL$ are not found with little variances in parameters. The RV/IV of the left breast irradiation is significantly larger than that of the right but the RV/EVS do not show the differences. There is no symptomatic radiation pneumonitis at least during 6 months follow up. Conclusion : The significant relationship between the irradiated lung volume and predictors is not found with little variation on parameters. The irradiated lung volume in the tangential held is liss than $10\%$ of entire lung volume when CLO is less than 3cm. The RV/IV of the left tangential field is larger than that of the right but there was no significant differences in RV/EVS. Symptomatic radiation pneumonitis has not occurred during minimum 6 months follow up.

• Tuberculosis and Respiratory Diseases
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• v.59 no.5
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• pp.487-496
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• 2005

Background : 'Major pulmonary thromboembolism' is defined as right ventricular (RV) dysfunction, with or without shock, accompanied by significant morbidity and mortality. In this study, those with major pulmonary thromboembolism were divided into the shock and RV dysfunction only groups, and then investigated the mortality and complications in thrombolysis or anticoagulation, respectively. Methods : In a retrospective study, between January 1995 and December 2004, 60 eligible patients with a major pulmonary thromboembolism, admitted in Asan Medical Center, were included. Results : A total of 57 patients were treated with medical therapy. Thrombolysis was performed in 13 patients (23%) and anticoagulation in 44 (77%). There were no differences in the APACHEII and SOFA scores between the two groups. 6 (46%) and 11 (25%) patients died in the thrombolysis and anticoagulation groups, respectively (p=0.176). In the 19 patients (33%) showing shock, thrombolysis was performed in 9 (47%) and anticoagulation in 10 (53%). 4 (44%) of the 9 patients treated with thrombolytic agents and 3 (30%) of the 10 treated with anticoagulants died (p=0.650). In the 38 patients (67%) showing RV dysfunction only, thrombolysis was performed in 4 (11%) and anticoagulation in 34 (89%). 2 (50%) of the 4 patients treated with thrombolytics and 8 (24%) of the 34 treated with anticoagulants died (p=0.279). Three patients (23%) who underwent thrombolysis had a major bleeding episode, compared with 2 (5%) who were treated with anticoagulants (p=0.072). Conclusion: The results of our study showed that thrombolysis did not lower mortality and tended to increase major bleeding compared with anticoagulation in both the shock and RV dysfunction only groups. Further evaluation of the efficacy and safety of thrombolytic therapy for major thromboembolism appears warranted in Korea.

• Journal of Chest Surgery
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• v.56 no.2
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• pp.75-86
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• 2023

Background: We investigated the long-term outcomes of truncus arteriosus repair at a single institution with a 30-year study period. Methods: Patients who underwent repair of truncus arteriosus between 1993 and 2022 were reviewed retrospectively. Factors associated with early mortality, overall attrition, and reintervention were identified using appropriate statistical methods. Results: In total, 42 patients were enrolled in this study. The median age and weight at repair were 26 days and 3.5 kg, respectively. Thirty patients (71.4%) underwent 1-stage repair. There were 8 early deaths (19%). In the univariable analysis, undergoing surgery before 2011 was associated with early mortality (p=0.031). The overall survival rate at 10 years was 73.8%. In the multivariable analysis, significant truncal valve (TrV) dysfunction (p=0.010), longer cardiopulmonary bypass time (p=0.018), and the earlier era of surgery (p=0.004) were identified as risk factors for overall mortality. During follow-up, 47 reinterventions were required in 27 patients (64.3%). The freedom from all-cause reintervention rate at 10 years was 23.6%. In the multivariable analysis, associated arch obstruction (p<0.001) and significant TrV dysfunction (p=0.011) were identified as risk factors for all-cause reintervention. Arch obstruction (p=0.027) and a number of TrV cusps other than 3 (p=0.014) were identified as risk factors for right ventricle to pulmonary artery (RV-PA) reintervention, and significant TrV dysfunction was identified as a risk factor for TrV reintervention (p=0.002). Conclusion: Despite recent improvements in survival outcomes after repair of truncus arteriosus, RV-PA or TrV reinterventions were required in a significant number of patients during follow-up.