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Objective@#To investigate the prognostic value of preoperative cardiac magnetic resonance imaging (MRI) for long-term major adverse cardiac and cerebrovascular events (MACCEs) in patients undergoing tricuspid valve (TV) surgery for functional tricuspid regurgitation (TR). @*Materials and Methods@#The preoperative cardiac MR images, New York Heart Association functional class, comorbidities, and clinical events of 78 patients (median [interquartile range], 59 [51–66.3] years, 28.2% male) who underwent TV surgery for functional TR were comprehensively reviewed. Cox proportional hazards analyses were performed to assess the associations of clinical and imaging parameters with MACCEs and all-cause mortality. @*Results@#For the median follow-up duration of 5.4 years (interquartile range, 1.2–6.6), MACCEs and all-cause mortality were 51.3% and 23.1%, respectively. The right ventricular (RV) end-systolic volume index (ESVI) and the systolic RV mass index (RVMI) were higher in patients with MACCEs than those without them (77 vs. 68 mL/m2 , p = 0.048; 23.5 vs. 18.0%, p = 0.011, respectively). A high RV ESVI was associated with all-cause mortality (hazard ratio [HR] per value of 10 higher ESVI = 1.10, p = 0.03). A high RVMI was also associated with all-cause mortality (HR per increase of 5 mL/m2 RVMI = 1.75, p < 0.001).After adjusting for age and sex, only RVMI remained a significant predictor of MACCEs and all-cause mortality (p < 0.05 for both). After adjusting for multiple clinical variables, RVMI remained significantly associated with all-cause mortality (p = 0.005). @*Conclusion@#RVMI measured on preoperative cardiac MRI was an independent predictor of long-term outcomes in patients who underwent TV surgery for functional TR.

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Objective@#To investigate the prognostic value of preoperative cardiac magnetic resonance imaging (MRI) for long-term major adverse cardiac and cerebrovascular events (MACCEs) in patients undergoing tricuspid valve (TV) surgery for functional tricuspid regurgitation (TR). @*Materials and Methods@#The preoperative cardiac MR images, New York Heart Association functional class, comorbidities, and clinical events of 78 patients (median [interquartile range], 59 [51–66.3] years, 28.2% male) who underwent TV surgery for functional TR were comprehensively reviewed. Cox proportional hazards analyses were performed to assess the associations of clinical and imaging parameters with MACCEs and all-cause mortality. @*Results@#For the median follow-up duration of 5.4 years (interquartile range, 1.2–6.6), MACCEs and all-cause mortality were 51.3% and 23.1%, respectively. The right ventricular (RV) end-systolic volume index (ESVI) and the systolic RV mass index (RVMI) were higher in patients with MACCEs than those without them (77 vs. 68 mL/m2 , p = 0.048; 23.5 vs. 18.0%, p = 0.011, respectively). A high RV ESVI was associated with all-cause mortality (hazard ratio [HR] per value of 10 higher ESVI = 1.10, p = 0.03). A high RVMI was also associated with all-cause mortality (HR per increase of 5 mL/m2 RVMI = 1.75, p < 0.001).After adjusting for age and sex, only RVMI remained a significant predictor of MACCEs and all-cause mortality (p < 0.05 for both). After adjusting for multiple clinical variables, RVMI remained significantly associated with all-cause mortality (p = 0.005). @*Conclusion@#RVMI measured on preoperative cardiac MRI was an independent predictor of long-term outcomes in patients who underwent TV surgery for functional TR.

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24 mm² (sensitivity, 76.5%; specificity 64.8%), and the area under the ROC curve (AUC) was 0.72. For ASR(area), the cut-off value was > 1.58 (sensitivity, 76.5%; specificity, 58.0%) and the AUC was 0.64. Multivariable Cox regression showed that ARO > 24 mm² (hazard ratio = 3.79, p = 0.020) was a potential independent parameter for recurrent 3 + AR. ROC for the linear regression model showed that the AUC for both ARO and ASR(area) was 0.73 (95% confidence interval, 0.64–0.81, p < 0.001).CONCLUSION: ARO and ASR(area) detected on preoperative cardiac CT would be potentially helpful for identifying AR patients who may benefit from the David operation.

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BACKGROUND/AIMS@#Although the association between intrapulmonary shunt (IPS) and liver cirrhosis is clear, data of repeated contrast echocardiography (CE) before and after liver transplantation (LT) to evaluate factors associated with IPS are limited.@*METHODS@#Hand-agitated saline was used for CE and, by assessing left-chamber opacification, IPS was classified as grade 0 to 4. Grade 3/4 constituted significant IPS and hepatopulmonary syndrome (HPS) was defined as significant IPS with the arterial partial pressure of oxygen < 70 mmHg.@*RESULTS@#Before LT, 253 patients underwent CE and the frequency of significant IPS and HPS were 44% (n = 112) and 7% (n = 17), respectively. Child-Pugh score (odds ratio [OR], 1.345; 95% confidence interval [CI], 1.090 to 1.660; p = 0.006) and arterial oxygen content (OR, 0.838; 95% CI, 0.708 to 0.991; p = 0.039) were independent determinants of significant IPS, whereas direct bilirubin (OR, 1.076; 95% CI, 1.012 to 1.144; p = 0.019) was the only variable associated with HPS. Among 153 patients who underwent successful LT, repeated CE was performed in 97 (63%), which showed significant reductions in IPS grade (from 2.6 ± 1.0 to 1.2 ± 1.3, p < 0.001) and the prevalence of significant IPS (from 56% to 20%, p = 0.038). After adjustment for pre-LT IPS grade, time from LT to repeated CE presented negative linear relationship with post-LT IPS grade (r2 = 0.366, p < 0.001) and was the only determinant of post-LT IPS grade (OR, 1.009; 95% CI, 1.003 to 1.014; p = 0.004).@*CONCLUSIONS@#Repeated CE is useful to evaluate intrapulmonary vascular change before and after LT. Reversal of IPS after successful LT is time-dependent and follow-up duration should be considered for accurate assessment of IPS after LT.

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BACKGROUND AND OBJECTIVES@#The clinical characteristics of patients with diastolic dysfunction characterized by a relaxation abnormality with possible elevated filling pressure is remain to be determined. We sought to test whether diastolic stress echocardiography (DSE) is useful for characterization of these patients.@*METHODS@#A total of 120 patients (58 men, mean age of 64±7 years) with E/A ratio 15, hLVFP) and 40 (30%) developed exercise-induced pulmonary hypertension (systolic pulomary arterial pressure > 50 mmHg, EiPH) without hLVFP. The remaining 33 patients did not show hLVFP or EiPH. The incidence of EiPH with hLVFP was 21% (25/120). By multivariate analysis, age (odds ratio [OR], 1.07; 95% confidence interval [CI], 1.00–1.13; p=0.039) and systolic pulmonary artery pressure at rest (OR, 1.14; 95% CI, 1.02–1.27; p=0.02) were associated with EiPH, whereas late diastolic transmitral velocity (OR, 1.04; 95% CI, 1.00–1.08; p=0.03) and diastolic blood pressure (OR, 0.94; 95% CI, 0.90–0.99; p=0.02) were associated with hLVFP during exercise.@*CONCLUSIONS@#Patients with relaxation abnormality and possibly hLVFP showed markedly heterogeneous hemodynamic changes during low-level exercise and DSE was useful to characterize these patients.

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BACKGROUND AND OBJECTIVES@#Clinical data for Korean patients with bicuspid aortic valve (BAV) that underwent aortic valve (AV) surgery are currently limited.@*METHODS@#Data for 1,160 consecutive adult BAV patients who underwent AV surgery from 2000 to 2014 in 4 tertiary referral centers were retrospectively analyzed. A standard case report form was used for clinical and echocardiographic parameters.@*RESULTS@#Mean age at the time of AV surgery was 59±13 years. The most common cause of AV surgery was aortic stenosis (AS, 892 [77%]), followed by aortic regurgitation (AR, 199 [17%]), and infective endocarditis (69 [6%]). AS showed a skewed peak in the aged population and was the predominant cause of AV surgery (87%) in patients ≥50 years of age, whereas AR (46%) and active infective endocarditis (19%) were more common in younger patients (p < 0.001). Echocardiographic determination of the BAV phenotype revealed that fusion of the right coronary cusp (RCC) and left coronary cusp (LCC) was most common (622 [53%]), followed by fusion of RCC and non-coronary cusp (NCC) (313 [27%]), and fusion of LCC and NCC (42 [4%]); the BAV phenotype could not be determined in the remaining 183 patients (16%). Fusion of RCC and LCC was more commonly observed in patients with AR than in those with AS (74% vs. 49%; p < 0.001).@*CONCLUSION@#BAV patients were characterized by distinct surgical indications according to their age. Possible associations between BAV phenotypes and surgical indications with potential impacts of ethnicity need to be tested in further studies.

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Lung transplantation is the only treatment for end-stage lung disease, but the problem of donor shortage is unresolved issue. Herein, we report the first case of living-donor lobar lung transplantation (LDLLT) in Korea. A 19-year-old woman patient with idiopathic pulmonary artery hypertension received her father's right lower lobe and her mother's left lower lobe after pneumonectomy of both lungs in 2017. The patient has recovered well and is enjoying normal social activity. We think that LDLLT could be an alternative approach to deceased donor lung transplantation to overcome the shortage of lung donors.

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BACKGROUND: Overestimation of the severity of mitral regurgitation (MR) by the proximal isovelocity surface area (PISA) method has been reported. We sought to test whether angle correction (AC) of the constrained flow field is helpful to eliminate overestimation in patients with eccentric MR. METHODS: In a total of 33 patients with MR due to prolapse or flail mitral valve, both echocardiography and cardiac magnetic resonance image (CMR) were performed to calculate regurgitant volume (RV). In addition to RV by conventional PISA (RV(PISA)), convergence angle (α) was measured from 2-dimensional Doppler color flow maps and RV was corrected by multiplying by α/180 (RV(AC)). RV measured by CMR (RV(CMR)) was used as a gold standard, which was calculated by the difference between total stroke volume measured by planimetry of the short axis slices and aortic stroke volume by phase-contrast image. RESULTS: The correlation between RV(CMR) and RV by echocardiography was modest [RV(CMR) vs. RV(PISA) (r = 0.712, p < 0.001) and RV(CMR) vs. RV(AC) (r = 0.766, p < 0.001)]. However, RV(PISA) showed significant overestimation (RV(PISA) - RV(CMR) = 50.6 ± 40.6 mL vs. RV(AC) - RV(CMR) = 7.7 ± 23.4 mL, p < 0.001). The overall accuracy of RV(PISA) for diagnosis of severe MR, defined as RV ≥ 60 mL, was 57.6% (19/33), whereas it increased to 84.8% (28/33) by using RV(AC) (p = 0.028). CONCLUSION: Conventional PISA method tends to provide falsely large RV in patients with eccentric MR and a simple geometric AC of the proximal constraint flow largely eliminates overestimation.

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BACKGROUND AND OBJECTIVES: Clinical data for Korean patients with bicuspid aortic valve (BAV) that underwent aortic valve (AV) surgery are currently limited. METHODS: Data for 1,160 consecutive adult BAV patients who underwent AV surgery from 2000 to 2014 in 4 tertiary referral centers were retrospectively analyzed. A standard case report form was used for clinical and echocardiographic parameters. RESULTS: Mean age at the time of AV surgery was 59±13 years. The most common cause of AV surgery was aortic stenosis (AS, 892 [77%]), followed by aortic regurgitation (AR, 199 [17%]), and infective endocarditis (69 [6%]). AS showed a skewed peak in the aged population and was the predominant cause of AV surgery (87%) in patients ≥50 years of age, whereas AR (46%) and active infective endocarditis (19%) were more common in younger patients (p < 0.001). Echocardiographic determination of the BAV phenotype revealed that fusion of the right coronary cusp (RCC) and left coronary cusp (LCC) was most common (622 [53%]), followed by fusion of RCC and non-coronary cusp (NCC) (313 [27%]), and fusion of LCC and NCC (42 [4%]); the BAV phenotype could not be determined in the remaining 183 patients (16%). Fusion of RCC and LCC was more commonly observed in patients with AR than in those with AS (74% vs. 49%; p < 0.001). CONCLUSION: BAV patients were characterized by distinct surgical indications according to their age. Possible associations between BAV phenotypes and surgical indications with potential impacts of ethnicity need to be tested in further studies.

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No abstract available.

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BACKGROUND AND OBJECTIVES: The clinical characteristics of patients with diastolic dysfunction characterized by a relaxation abnormality with possible elevated filling pressure is remain to be determined. We sought to test whether diastolic stress echocardiography (DSE) is useful for characterization of these patients. METHODS: A total of 120 patients (58 men, mean age of 64±7 years) with E/A ratio 15, hLVFP) and 40 (30%) developed exercise-induced pulmonary hypertension (systolic pulomary arterial pressure > 50 mmHg, EiPH) without hLVFP. The remaining 33 patients did not show hLVFP or EiPH. The incidence of EiPH with hLVFP was 21% (25/120). By multivariate analysis, age (odds ratio [OR], 1.07; 95% confidence interval [CI], 1.00–1.13; p=0.039) and systolic pulmonary artery pressure at rest (OR, 1.14; 95% CI, 1.02–1.27; p=0.02) were associated with EiPH, whereas late diastolic transmitral velocity (OR, 1.04; 95% CI, 1.00–1.08; p=0.03) and diastolic blood pressure (OR, 0.94; 95% CI, 0.90–0.99; p=0.02) were associated with hLVFP during exercise. CONCLUSIONS: Patients with relaxation abnormality and possibly hLVFP showed markedly heterogeneous hemodynamic changes during low-level exercise and DSE was useful to characterize these patients.

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BACKGROUND/AIMS: Pulmonary endarterectomy (PEA) is the gold standard for treating chronic thromboembolic pulmonary hypertension (CTEPH) in Western countries. The aim of this study was to investigate the long-term outcomes of performing PEA on CTEPH patients in comparison with medical therapy at a single Korean center. METHODS: This retrospective study included 88 CTEPH patients. These patients were classified into the PEA group (n = 37) or non-PEA group (i.e., medical therapy; n = 51). The clinical characteristics, hemodynamic data, and long-term survival rates were compared. Independent prognostic factors for CTEPH were also investigated. RESULTS: CTEPH was not associated with either gender, and the mean age at diagnosis was 53.3 ± 13.7 years. Echocardiography revealed that the mean peak velocity of the tricuspid regurgitation jet was 4.2 ± 0.7 m/sec and the mean pulmonary arterial pressure was 51.7 ± 15.1 mmHg. The PEA and non-PEA groups demonstrated no significant differences, except in terms of the right ventricular end-diastolic diameter. The survival rates of the PEA group were significantly higher than the non-PEA group at 1, 3, 5, and 10 years (p = 0.032). Multivariate analyses indicated that World Health Organization class IV and PEA were significant predictors of poorer and better outcomes, respectively. CONCLUSIONS: PEA demonstrates more favorable effects on long-term survival than medical therapy in Korean CTEPH patients who were considered operable.

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BACKGROUND: Left ventricle (LV) in patients with aortic stenosis (AS) faces a double hemodynamic load incorporating both valvular stenosis and reduced systemic arterial compliance (SAC). This study aimed to evaluate the impact of global LV afterload on LV hypertrophy (LVH) before and after aortic valve replacement (AVR). METHODS: The study cohort included 453 patients (247 males; mean age, 64 ± 11 years) who underwent AVR. Pre- and post-AVR echocardiographic examinations were retrospectively analyzed including an index of valvuloarterial impedance (Z(VA)) and LV mass index/LV end-diastolic volume index (LVMI/LVEDVI) as a parameter of LVH. RESULTS: Pre-AVR LVMI/LVEDVI was 2.7 ± 0.9 g/mL with an aortic valve area (AVA) of 0.6 ± 0.2 cm². Z(VA) was 5.9 ± 1.9 mm Hg/mL/m² and showed a stronger correlation (β = 0.601, p 1 year after AVR. CONCLUSION: Z(VA) is a major determinant of concentric remodeling in AS before AVR and LVH regression after AVR, which should be incorporated in routine evaluation of AS.

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A 71-year-old female who was diagnosed with nonobstructive hypertrophic cardiomyopathy since 1999 presented with dyspnea and severe edema on both legs. For the management of her symptom, cardiac surgery including tricuspid annuloplasty, Maze operation and right atrial reduction plasty was performed. During follow-up after cardiac surgery, a plasma α-galactosidase activity was checked for the screening of Fabry disease and the result was around lower normal limit. DNA analysis was implemented for confirmation and it revealed a heterozygote α-galactosidase mutation at exon 6 [c.901C>T (p.Arg301Ter)]. This case suggests that Fabry disease might be easily undetected, and clinical suspicion is critical.

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BACKGROUND AND OBJECTIVES: Compared with conventional treatment, early surgery significantly reduced the composite end point of all-cause death and embolic events during hospitalization, but long-term data in this area are lacking. This study sought to compare long-term outcomes of early surgery with a conventional treatment strategy in patients with infective endocarditis (IE) and large vegetations. SUBJECTS AND METHODS: The Early Surgery versus Conventional Treatment in Infective Endocarditis (EASE) trial randomly assigned patients with left-sided IE, severe valve disease and large vegetation to early surgery (37 patients) or conventional treatment groups (39 patients). The pre-specified end points were all-cause death, embolic events, recurrence of IE and repeat hospitalizations due to the development of congestive heart failure occurring during follow-up. RESULTS: There were no significant differences between the early surgery and the conventional treatment group in all-cause mortality at 4 years (8.1% and 7.7%, respectively; hazard ratio [HR] 1.04; 95% CI, 0.21 to 5.15; p=0.96). The rate of the composite end point of death from any cause, embolic events or recurrence of IE at 4 years was 8.1% in the early surgery group and 30.8% in the conventional treatment group (HR, 0.22; 95% CI, 0.06-0.78; p=0.02). The estimated actuarial rate of end points at 7 years was significantly lower in the early surgery group than in the conventional treatment group (log-rank p=0.007). CONCLUSION: There was a substantial benefit in having early surgery for patients with IE and large vegetations whose health was sustained up to 7 years, and late clinical outcome after surgery was excellent in survivors of IE. (EASE clinicaltrials.gov identifier: NCT00750373)

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We reported a 48-year-old man with Behcet disease, who presented with right hemiparesis. His first brain MRI showed multiple enhanced lesions. During the recovery, he had an episode of left 6th nerve palsy without new lesions in a follow-up MRI. Third episode was cervical myelitis, resulting in respiratory difficulty and quadriplegia without any reflexes. The myelitis was not responsive to immunotherapy. He died of respiratory failure complicated with pneumonia. This is a rare case of full-blown neuro-Behcet disease.

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PURPOSE: Although several studies have reported the morphological and electrical characteristics in patients with hypertrophic cardiomyopathy (HCM), comparison between asymmetric and apical HCM has not been investigated in a reasonably sized cohort. MATERIALS AND METHODS: Echocardiography and electrocardiography were quantitatively analyzed in patients with HCM in a Korean tertiary referral center. RESULTS: Of 864 patients (mean age 55.4+/-14.2 years, 68.9% men), 255 (29.5%) patients had apical HCM, 553 (64.0%) patients asymmetric HCM, and 56 (6.4%) patients mixed type HCM. In echocardiographic evaluations, about three quarters of patients (75.8%) had left atrial enlargement. Left ventricular (LV) dilatations and systolic dysfunction were observed in 6.1% and 2.4%, respectively. QRS widening, PR prolongation, and pathologic Q wave are frequent in patients with asymmetric HCM, while LV strain is frequent in patient with apical HCM. The prevalence of J-point elevations (9.4% in inferior, 2.2% in lateral leads) were substantially higher than that in general population. Giant negative T wave was observed in 15.0% of total patients (32.2% in apical, 6.2% in asymmetric, 25% in mixed type). There was no significant correlation between the thickness of the apical wall and the amplitude of T wave inversion (r=-0.005, p=0.71). CONCLUSION: In a large cohort of HCM including apical type, repolarization abnormalities, including early repolarization and QT prolongation as well as LV strain, were significantly observed. T wave inversion was not appropriate for screening of HCM and not correlated with apical wall thickness.

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A 59-year old man was admitted for drowsiness and stiff neck. CSF examination showed lymphocytic pleocytosis and PCR for herpes simplex virus (HSV)-1 was positive in CSF. Brain MRI revealed enhanced lesions in left temporal lobe. His symptom improved with acyclovir. Follow-up studies showed red blood cells in CSF and a hematoma in the left temporal lobe. There was no additional symptom related to the hematoma. He was discharged after conservative care. Although rare, hematoma can develop in HSV-1 meningoencephalitis.

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We present a case of 48-year-old male who presented with coronary artery fistula draining into left ventricle. Transthoracic echocardiography showed abnormal blood flow draining into left ventricle, with enlarged coronary arteries and multiple vascular structures around ventricular myocardium. Coronary computed tomography revealed dilatation of entire left coronary artery which was wrapping around left ventricle, and draining into the posterior side of left ventricle. He did not undergo any invasive treatment, because he was not symptomatic.

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BACKGROUND AND OBJECTIVES: Transcatheter aortic valve implantation (TAVI) was recently introduced in Korea. The present report describes the experience of early TAVI cases. SUBJECTS AND METHODS: Between March, 2010 and October, 2011, 48 patients with severe symptomatic aortic stenosis (AS) were screened at the Asan Medical Center to determine their suitability for surgical intervention. Of these, 23 were considered unsuitable and underwent TAVI. Procedural success rates, procedure-related complications, and clinical outcomes were evaluated in the TAVI patients. RESULTS: Transfemoral (n=20) or transapical (n=3) TAVI was performed. The mean age of patients was 75.9+/-5.4 years and 57% were females. The mean logistic European System for Cardiac Operative Risk Evaluation was 25.6+/-5.1%. Implantation was successful in 22 patients (19 transfemoral, three transapical). After successful implantation, the mean aortic valve area increased from 0.68+/-0.14 cm2 to 1.45+/-0.33 cm2. There were no procedure-related complications or mortality. The patients showed no paravalvular aortic regurgitation with > or = moderate degree and remained stable without progression during follow-up. During follow-up (interquartile range, 1.1-12.9), all patients were alive without any occurrence of valve failure. CONCLUSION: TAVI procedure is feasible in patients with inoperative symptomatic AS leading to hemodynamic and clinical improvement. With accumulation of experience, proper patient selection and development of device technologies, TAVI should decrease adverse events and expand the indications in the near future.

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