Accuracy of Artificial Intelligence-Based Automated Quantitative Coronary Angiography Compared to Intravascular Ultrasound: Retrospective Cohort Study.

BACKGROUND: An accurate quantitative analysis of coronary artery stenotic lesions is essential to make optimal clinical decisions. Recent advances in computer vision and machine learning technology have enabled the automated analysis of coronary angiography. OBJECTIVE: The aim of this paper is to validate the performance of artificial intelligence-based quantitative coronary angiography (AI-QCA) in comparison with that of intravascular ultrasound (IVUS). METHODS: This retrospective study included patients who underwent IVUS-guided coronary intervention at a single tertiary center in Korea. Proximal and distal reference areas, minimal luminal area, percent plaque burden, and lesion length were measured by AI-QCA and human experts using IVUS. First, fully automated QCA analysis was compared with IVUS analysis. Next, we adjusted the proximal and distal margins of AI-QCA to avoid geographic mismatch. Scatter plots, Pearson correlation coefficients, and Bland-Altman were used to analyze the data. RESULTS: A total of 54 significant lesions were analyzed in 47 patients. The proximal and distal reference areas, as well as the minimal luminal area, showed moderate to strong correlation between the 2 modalities (correlation coefficients of 0.57, 0.80, and 0.52, respectively; P<.001). The correlation was weaker for percent area stenosis and lesion length, although statistically significant (correlation coefficients of 0.29 and 0.33, respectively). AI-QCA tended to measure reference vessel areas smaller and lesion lengths shorter than IVUS did. Systemic proportional bias was not observed in Bland-Altman plots. The biggest cause of bias originated from the geographic mismatch of AI-QCA with IVUS. Discrepancies in the proximal or distal lesion margins were observed between the 2 modalities, which were more frequent at the distal margins. After the adjustment of proximal or distal margins, there was a stronger correlation of proximal and distal reference areas between AI-QCA and IVUS (correlation coefficients of 0.70 and 0.83, respectively). CONCLUSIONS: AI-QCA showed a moderate to strong correlation compared with IVUS in analyzing coronary lesions with significant stenosis. The main discrepancy was in the perception of the distal margins by AI-QCA, and the correction of margins improved the correlation coefficients. We believe that this novel tool could provide confidence to treating physicians and help in making optimal clinical decisions.

Real-World Safety and Clinical Outcomes of Macitentan in Asian Patients with Pulmonary Arterial Hypertension: A Prospective Multicenter Study.

BACKGROUND AND OBJECTIVE: Macitentan is approved for treating pulmonary arterial hypertension. However, the real-world evidence of macitentan use is limited. Therefore, we evaluated the safety and clinical outcomes of macitentan use in clinical practice under a post-marketing surveillance. METHODS: Patients with pulmonary arterial hypertension receiving macitentan treatment were prospectively and consecutively enrolled from 2014 to 2020 at 50 medical centers in Korea. Safety and clinical outcomes were monitored from baseline to the nearest timepoint of 24 weeks after macitentan initiation. The adverse events and adverse drug reactions were identified. Changes in the World Health Organization functional class were assessed as the primary clinical outcome, which was used to estimate the final effectiveness (both improved and maintained). Factors associated with safety and final effectiveness were identified. RESULTS: Among 474 patients enrolled in the study, 467 and 440 were included in the safety and clinical outcome analyses, respectively. Dyspnea, nasopharyngitis, and worsening pulmonary arterial hypertension were the most frequent adverse events with incidences of 5%, 3%, and 3%, respectively. The final effectiveness rate was 93%. Older age (adjusted odds ratio [aOR] = 1.021, p = 0.003) and higher level (III vs II) of baseline World Health Organization functional class (aOR = 1.784; p = 0.022) were significantly associated with a higher adverse event occurrence. Younger age (aOR = 0.947; p = 0.001) and shorter disease duration (aOR = 0.991; p = 0.010) were significantly associated with positive final effectiveness. CONCLUSIONS: This real-world study demonstrated the safety and clinical outcomes of macitentan use in Korean patients with pulmonary arterial hypertension. Macitentan was well tolerated and significantly effective with no new safety concerns during the 24 weeks.

Pre-implant global longitudinal strain as an early sign of pacing-induced cardiomyopathy in patients with complete atrioventricular block.

INTRODUCTION: Long-term right ventricular pacing is the only treatment for patients with a complete atrioventricular block (CAVB); however, it frequently triggers ventricular dys-synchrony with left ventricular (LV) dysfunction. Previous studies showed that an early decline of LV global longitudinal strain (GLS) predicts pacing-induced LV dysfunction. We aimed to investigate the potential ability of the initial LV strain to predict pacing-induced cardiomyopathy (PICM) through long-term follow-ups. METHODS: We retrospectively enrolled 80 patients with CAVB with normal LV function who were implanted with dual-chamber pacemakers between 2008 and 2018. Echocardiographic data and parameters (including longitudinal, radial, and circumferential strain based on speckle-tracking) were analyzed for the pre-implant (≤6 months) and post-implant periods. PICM was defined as a ≥10% reduction in the left ventricular ejection fraction (LVEF) resulting in an LVEF of <50% during the post-implant period. Predictors of PICM were identified using Cox proportional hazard models. RESULTS: Patients who developed PICM were more likely to exhibit lower baseline LV GLS, as well as wider native and pacing QRS durations, than those who did not develop PICM (P = .016, P = .011, and P = .026, respectively). In the multivariate analysis, pre-implant LV GLS (hazard ratio: 1.27; 95% confidence interval 1.009-1.492; P = .004) was independently associated with the development of PICM. CONCLUSION: A lower baseline LV GLS predicts an increased risk of PICM. Patients with CAVB exhibiting low GLS are at increased risk of PICM. More frequent follow-up visits are warranted in these patients, who may also require de novo His-bundle pacing or an upgrade to biventricular pacing.

Comparison of Clinical and Echocardiographic Characteristics between Cardiac Myxomas and Masses Mimicking Myxoma.

BACKGROUND AND OBJECTIVES: Cardiac myxoma is the most frequent benign cardiac tumor that can result in cardiac and systemic symptoms. We investigated clinical and echocardiographic characteristics of patients with cardiac masses suggesting myxoma. METHODS: We investigated 265 consecutive patients with an echocardiographic diagnosis of cardiac myxomas in 4 teaching hospitals in Korea. RESULTS: The mean age was 61±16 years and 169 patients (63.8%) were female. The most frequent referral reason for echocardiography was an evaluation of cardiac symptoms (43.4%). Tumors were incidentally detected in 82 patients (30.9%). Left atrium (LA) was the most frequently involved site (84.5%) and 19 patients (7.2%) had non-atrial tumors. The mean tumor size was 38.7×26.0 mm (range, 4-96 mm). Of 186 patients (70.2%) who had pathological diagnosis, 174 (93.5%) were confirmed with myxoma, 8 (4.3%) with other tumors and 4 (2.2%) with thrombi. Compared to myxoma, smaller size (20.4×12.6 mm vs. 41.4×27.6 mm, p<0.01) and non-LA location (87.5% vs. 10.5%, p<0.001) were associated with non-myxoma tumors, and more frequent atrial fibrillation (AF, 75.0% vs. 7.0%, p<0.001) and larger LA diameter (55.0±14.6 mm vs. 41.3±7.7 mm, p=0.001) were related to thrombi. CONCLUSIONS: Of 265 patients with an echocardiographic diagnosis with cardiac myxomas, 174 (65.7%) were surgically confirmed with myxomas. Compared with cardiac myxoma, other tumors were smaller and more frequently found in non-atrial sites. Thrombi were associated with AF and larger LA diameter.

New-onset atrial fibrillation predicting for complicating cardiac adverse outcome in scrub typhus infection.

BACKGROUND: Scrub typhus is a well-known infectious disorder of the Asia-Pacific region. However, adverse cardiac outcomes are an under-recognized complication of scrub typhus infection, and new-onset AF has been reported to be a prognostic factor in other, more common infectious diseases. The present study investigated whether new-onset atrial fibrillation (AF) is significantly associated with 3-month mortality and adverse cardiac complications in scrub typhus infection. METHODS: We examined data from the National Health Information Database (NHID) which covers nearly the entire population of South Korea, from 2006 to 2016. In total, 233 473 patients diagnosed with scrub typhus infection were selected as study participants. New-onset AF, acute heart failure (AHF), ischemic heart disease (IHD), and 3-month mortality were analyzed using a generalized estimating equation model with a Poisson distribution. RESULTS: Of these, 2402 patients (1%) were diagnosed with new-onset AF (87.2% were over 60 years of age, 43.3% were male). Those with new-onset AF were more likely to have underlying cardiovascular disease compared to those without new-onset AF. After being adjusted for demographic factors and comorbidities, those with new-onset AF had a higher incidence risk of concurrent AHF (4.1-fold) and IHD (1.9-fold) compared with those without new-onset AF. In particular, the 3-month mortality was also significantly associated with new-onset AF (1.3-fold), concurrent AHF (2.4-fold), and IHD (13.7-fold). CONCLUSIONS: New-onset AF was significantly associated with 3-month mortality and concurrent AHF and IHD. Therefore, new-onset AF could be a poor prognostic factor for 3-month mortality and cardiac complications in scrub typhus infection.

Major determinant of the occurrence of pacing-induced cardiomyopathy in complete atrioventricular block: a multicentre, retrospective analysis over a 15-year period in South Korea.

OBJECTIVES: The predictors of pacing-induced cardiomyopathy (PICM) for complete atrioventricular block (CAVB) have not yet been defined. The aim of this study was to investigate the major determinant of the occurrence of PICM. SETTING: This is a multicentre, retrospective analysis of CAVB from tertiary referral centres in Daejeon, South Korea. PARTICIPANTS: A cohort of 900 consecutive patients with an implanted pacemaker was collected from December 2001 to August 2015. Of these, a total of 130 patients with CAVB with pacing-dependent rhythm who underwent ECG and echocardiogram before and after implantation were analysed for the occurrence of PICM. OUTCOME MEASURES: Cox proportional hazards models evaluated the determinant of PICM by ECG, device parameters and echocardiogram over a mean of 4.5 years. RESULTS: PICM was observed in 16.1% (n=21) of all patients with CAVB (age, 64±11 years; male, 36.2%). The preimplant left ventricular (LV) ejection fraction (66%±9% vs 66%±8%) and non-apical pacing (40.4% vs 33.3%) were similar; however, the native QRS duration (124±34 ms vs 149±32 ms) and the paced QRS duration (pQRSd) (139±29 ms vs 167±28 ms) were significantly different between the two groups. The postimplant LV ejection fraction (61%±7% vs 31%±8%) was also significantly different at the end of follow-up. A pQRSd significantly correlated with PICM (HR 1.05, 95% CI 1.02 to 1.09, P=0.001). A pQRSd with a cut-off value of above 140 ms had a sensitivity of 95% while a pQRSd with a cut-off value of above 167 ms had a specificity of 90% for PICM. CONCLUSION: In patients with CAVB with pacing-dependent rhythm, regardless of the pacing site, the pQRSd is a major determinant of the occurrence of PICM.

Predictors of acute myocarditis in complicated scrub typhus: an endemic province in the Republic of Korea.

BACKGROUND/AIMS: Scrub typhus is known as a self-limited infectious disease. Cardiac complication is uncommon and usually not life-threatening. Until now, few cases of fulminant myocarditis by scrub typhus have been reported. So, we investigated incidence and predictors of acute myocarditis in severe scrub typhus. METHODS: We retrospectively reviewed 89 patients among 91 scrub typhus confirmed patients who examined an echocardiogram and cardiac biomarkers from 2005 to 2015 in the intensive care unit at our hospital. We excluded two patients who didn't have electrocardiography. Patients were divided into two groups and compared between scrub typhus with (n = 13) and without (n = 76) acute myocarditis. RESULTS: Age, sex, and underlying diseases were similar between the groups. The existence of eschar and duration of general ache and fever were similar between the groups. However, patients with acute myocarditis had more elevated total bilirubin, high incidence of ST elevations and paroxysmal atrial fibrillation (PAF) than those without acute myocarditis. Receiver operating characteristic analysis showed that the PAF was a predictor of myocarditis with a sensitivity of 70% and specificity of 84%. Predictive power of combination of ST-segment elevation and PAF was significantly associated with myocarditis in the multivariate analysis (odds ratio, 1.57; 95% confidence interval [CI], 1.21 to 11.7; p = 0.041) and area under the curve was 0.947 (95% CI, 0.878 to 0.983; p < 0.001). CONCLUSIONS: Acute myocarditis with scrub typhus may be more common than previously reported. Patients with high bilirubin and PAF are at increased risk of acute myocarditis with scrub typhus. These patients warrant closer follow-up and echocardiogram would be needed.

Current Awareness and Use of the Strain Echocardiography in Routine Clinical Practices: Result of a Nationwide Survey in Korea.

BACKGROUND: Because conventional echocardiographic parameters have several limitations, strain echocardiography has often been introduced in clinical practice. However, there are also obstacles in using it in clinical practice. Therefore, we wanted to find the current status of awareness on using strain echocardiography in Korea. METHODS: We conducted a nationwide survey to evaluate current use and awareness of strain echocardiography from the members of the Korean Society of Echocardiography. RESULTS: We gathered total 321 questionnaires from 25 cardiology centers in Korea. All participants were able to perform or interpret echocardiographic examinations. All participating institutions performed strain echocardiography. Most of our study participants (97%) were aware of speckle tracking echocardiography and 185 (58%) performed it for clinical and research purposes. Two-dimensional strain echocardiography was the most commonly used modality and left ventricle (LV) was the most commonly used cardiac chamber (99%) for clinical purposes. Most of the participants (89%) did not think LV strain can replace LV ejection fraction (LVEF) in their clinical practice. The common reasons for not performing routine use of strain echocardiography was diversity of strain measurements and lack of normal reference value. Many participants had a favorable view of the future of strain echocardiography. CONCLUSION: Most of our study participants were aware of strain echocardiography, and all institutions performed strain echocardiography for clinical and research purposes. However, they did not think the LV strain values could replace LVEF. The diversity of strain measurements and lack of normal reference values were common reasons for not using strain echocardiography in clinical practice.

Focal ablation for atrial tachycardia from the double-exit of the Marshall bundle inducing atrial fibrillation.

Atrial fibrillation (AF) from the ligament/vein of Marshall (LOM/VOM) has previously been described. We report the case of a 23-year-old woman with an antiarrhythmic drug-resistant AF induced by two distinct atrial tachycardias (ATs). Focal ablation of these ATs from the double-exit of the Marshall bundle using a three-dimensional map eliminated AF triggering, even though pulmonary vein electrical isolation is the cornerstone for paroxysmal AF. Such mechanisms are important as triggering factors to plan ablation for paroxysmal AF. Focal ablation for triggering and inducing AF, originating from the double-exit of the Marshall bundle may be effective in eliminating AF in young patients.

Treadmill exercise-induced E/e' elevation as a predictor of cardiovascular event in end-stage renal disease on peritoneal dialysis.

BACKGROUND/AIMS: Diastolic dysfunction is associated with cardiovascular (CV) events in end-stage renal disease (ESRD) on continuous ambulatory peritoneal dialysis (CAPD). However, conventional measurement of LA volume and E/e' using Doppler echocardiography has been limited to predict CV events in patients with ESRD on CAPD. METHODS: From September 2007 to September 2008, 30 consecutive patients with exertional dyspnea in ESRD on CAPD and normal systolic function was prospectively enrolled and underwent laboratory testing, coronary angiography, and treadmill exercise stress echocardiography (TESE). We divided the patients according to the presence of exercise-induced change of E/e' tissue Doppler and investigated whether this factor predicted CV events in ESRD on CAPD. RESULTS: Mean CAPD duration of all patients (70% male; mean age, 49 years) was 12 months. Patients with exercise-induced elevated E/e' (n = 12, 40%) and non-elevated E/e' (n = 18, 60%) demonstrated similar baseline characteristics. Exercise-induced elevated E/e' was predictable (cut-off value 14%, sensitivity 63%, and specificity 95%), with a hazard ratio of 1.13 (confidence interval, 1.03 to 1.24; p = 0.005), and significantly associated with CV events compared to the non-elevated E/e' group (log-rank, p = 0.007). CONCLUSIONS: Exercise-induced elevated E/e' determined using TESE might be feasible to predict CV events in patients with ESRD on CAPD.

Diastolic Dysfunction Induced by a High-Fat Diet Is Associated with Mitochondrial Abnormality and Adenosine Triphosphate Levels in Rats.

BACKGROUND: Obesity is well-known as a risk factor for heart failure, including diastolic dysfunction. However, this mechanism in high-fat diet (HFD)-induced obese rats remain controversial. The purpose of this study was to investigate whether cardiac dysfunction develops when rats are fed with a HFD for 10 weeks; additionally, we sought to investigate the association between mitochondrial abnormalities, adenosine triphosphate (ATP) levels and cardiac dysfunction. METHODS: We examined myocardia in Wistar rats after 10 weeks of HFD (45 kcal% fat, n=6) or standard diet (SD, n=6). Echocardiography, histomorphologic analysis, and electron microscopy were performed. The expression levels of mitochondrial oxidative phosphorylation (OXPHOS) subunit genes, peroxisome-proliferator-activated receptor γ co-activator-1α (PGC1α) and anti-oxidant enzymes were assessed. Markers of oxidative stress damage, mitochondrial DNA copy number and myocardial ATP level were also examined. RESULTS: After 10 weeks, the body weight of the HFD group (349.6±22.7 g) was significantly higher than that of the SD group (286.8±14.9 g), and the perigonadal and epicardial fat weights of the HFD group were significantly higher than that of the SD group. Histomorphologic and electron microscopic images were similar between the two groups. However, in the myocardium of the HFD group, the expression levels of OXPHOS subunit NDUFB5 in complex I and PGC1α, and the mitochondrial DNA copy number were decreased and the oxidative stress damage marker 8-hydroxydeoxyguanosine was increased, accompanied by reduced ATP levels. CONCLUSION: Diastolic dysfunction was accompanied by the mitochondrial abnormality and reduced ATP levels in the myocardium of 10 weeks-HFD-induced rats.

Extracorporeal membrane oxygenation saved a mother and her son from fulminant peripartum cardiomyopathy.

A 34-year-old full-term pregnant woman presented with abruptly aggravating dyspnea. A chest X-ray showed pulmonary edema, and an echocardiogram revealed a left ventricular ejection fraction of 39%. Despite conventional medical treatment for acute heart failure and mechanical ventilation, hypoxia and metabolic acidosis were aggravated, and the fetal heart rate decreased to 90 b.p.m., suggestive of fetal distress. We decided to initiate extracorporeal membrane oxygenation (ECMO) and perform a cesarean section. The infant was successfully delivered without hypoxic brain damage. The patient was weaned from ECMO 6 days after delivery and was extubated 1 day after discontinuation of ECMO. Left ventricular systolic function had completely recovered at this time. This is the first report of a patient with peripartum cardiomyopathy who had a successful delivery with the support of ECMO, demonstrating that ECMO can serve as a rescue therapy, not only treating peripartum cardiomyopathy but also permitting a safe delivery.

The effect of ablation for paroxysmal atrial fibrillation on left atrial volume and function: a one-year follow-up study.

PURPOSE: The effect of radiofrequency catheter ablation (RFCA) on left atrial (LA) volume and function in patients with paroxysmal atrial fibrillation (PAF) has not been extensively studied. The aim of this study was to evaluate the long-term impact of RFCA on LA volume and function in patients with PAF. MATERIALS AND METHODS: A total of 90 patients with drug-refractory PAF who had sinus rhythm on the initial echocardiogram were examined at baseline, 3 months and 1 year after ablation. We measured LA volume index, LA ejection fraction (LAEF; maximal-minimal LA volume/maximal LA volume), and LA active emptying fraction (LAAEF; mid-diastolic-minimal LA volume/mid-diastolic LA volume). RESULTS: After 12±1 months, 78 patients returned, and 61 patients (78%) had sinus rhythm. After 3 months, the LA maximal volume indices decreased (from 33±13 to 28±12 mL/m²; p<0.001). But, LAEF and LAAEF also decreased (from 48±13 to 39±12; p<0.001, from 27±13 to 19±11; p<0.001). After 1 year, LA volumes, LAEF, and LAAEF remained similar at 3 months. In patients without atrial fibrillation (AF) recurrence, LAEF and LAAEF decreased after 3 months (from 50±12 to 40±11; p<0.001, from 29±13 to 22±11; p<0.001) and did not change after 1 year. However, in patients with AF recurrence, those who did not have decreased levels after 3 months had significantly decreased after 1 year (from 43±14 to 34±11; p=0.026, from 22±12 to 15±10; p=0.012). CONCLUSION: Successful RFCA of PAF decreased LA volume and function at 3 months. At one year, LA volume and function was remained unchanged in successfully ablated patients whereas LA function in patients with AF recurrence worsened.

The feasibility of the initial postsystolic to systolic strain rate ratio as a predictor of the viability of ischemic myocardium with acute myocardial infarction.

BACKGROUND: Investigations of a strain index for the viability of ischemic myocardium with acute myocardial infarction (AMI) have been challenging. Therefore, the aim of this study was to evaluate patients with AMI to determine an optimal strain index for predicting the viability of ischemic myocardium. METHODS AND RESULTS: A total of 57 patients with AMI were assessed according to two-dimensional (2D) speckle tracking imaging strain and strain rate (SR), measured during the acute phase before urgent revascularization and at a 1-year follow-up postrevascularization. During the acute phase, all the myocardial segments were classified according to the acute end-systolic strain (Ses) values as normal (Ses ≤ -13%), hypocontractile (-13% < Ses ≤ -7%), or having a severe contractile abnormality (Ses > -7%). At the 1-year follow-up, we reassessed the recovery of the segments with a severe contractile abnormality. The viability of these segments was defined as an improved Ses (≤ -7%) at follow-up postrevascularization. The Ses values, postsystolic strain index (PSI), and SR values were significantly better in the viable segments than in the nonviable segments at both the acute phase and at follow-up (P < 0.001). The initial postsystolic to systolic SR ratio (SRps/SRs) had the best area under the curve (AUC = 0.897). In addition, a cutoff value of 0.6 predicted recovery from a severe contractile abnormality with a sensitivity of 75% and a specificity of 88%. CONCLUSIONS: The initial SRps/SRs ratio identified the viability of ischemic myocardium with AMI; therefore, this novel index may be clinically useful in the treatment of patients with AMI.

Gender difference in bone loss and vascular calcification associated with age.

BACKGROUND AND OBJECTIVES: It is widely known that both bone loss and vascular calcification are age-related processes. The purpose of this study was to investigate the relationship between coronary artery calcium (CAC) score or bone mineral density (BMD) with age and whether there is a gender difference factoring in the two conditions among healthy subjects. SUBJECTS AND METHODS: Between March 2009 and August 2011, participants included 1727 subjects (mean age: 55±10 years, M : F=914 : 813) with routine health check-ups. After being categorized into three groups (normal, osteopenia, and osteoporosis) according to the World Health Organization (WHO) diagnostic classification, we estimated BMD by dual energy X-ray absorptiometry (DEXA) and CAC score by dual-source CT (DSCT). RESULTS: There was a significant gender difference among the risk factors, including total-lumbar spine (1.213±0.176 g/cm(2) : 1.087±0.168 g/cm(2), p<0.001) and femur (1.024±0.131 g/cm(2) : 0.910±0.127 g/cm(2), p<0.001) in BMD by DEXA, and CAC score (68±227 : 27±116, p<0.001) in coronary artery calcification by DSCT. Age in male [odds ratio (OR): 1.138 {95% confidence interval (CI): 1.088-1.190}, p<0.001] and menopause in female subjects {OR: 12.370 (95% CI: 3.120-49.047), p<0.001} were, respectively, independently associated with osteopenia. CONCLUSION: Although our results do not demonstrate a direct association between CAC score and BMD in both genders, there is a gender difference of CAC score in normal and osteopenia groups according to the WHO diagnostic classification. Additionally, we suggest that more specific therapeutic strategies be considered during any early bone loss period, especially in female subjects.

Churg-Strauss syndrome associated with rapid deterioration of left ventricular diastolic dysfunction and conduction disturbance.

Cardiac involvement in Churg-Strauss syndrome (CSS) is a major cause of mortality. Here we report a case of a 75-year-old woman with eosinophilic endomyocarditis due to CSS. An electrocardiogram showed intraventricular conduction delay, and echocardiography showed an impaired relaxation pattern and biventricular apical thickening. Magnetic resonance imaging revealed subendocardial delayed enhancement with biventricular apical thrombi. Endomyocardial biopsy showed perivascular eosinophilic infiltration. Despite resolution of the hypereosinophilia after steroid therapy, her left ventricular (LV) diastolic function worsened into a restrictive pattern and she died with a ventricular escape rhythm on her 14th day in the hospital. This case is unusual in that there was rapid progression of the LV diastolic dysfunction and conduction disturbance due to CSS.

Hydroxychloroquine-induced cardiomyopathy that presented as pulmonary hypertension: a newly noted complication.

We examined a 63-year-old woman with progressive dyspnea. Two years prior to admission to our hospital, she had been diagnosed with rheumatoid arthritis and treated with hydroxychloroquine (HCQ) with a cumulative dose of 164 g. In addition, 2 months earlier, she had been diagnosed with connective tissue disease-related pulmonary artery hypertension. We performed an electrocardiogram and noted complete atrioventricular block. A transthoracic echocardiogram showed pulmonary hypertension. Due to the unclear nature of the pulmonary hypertension, we performed cardiac catheterization and right ventricular endomyocardial biopsy. Cardiac catheterization revealed that pulmonary hypertension was due to left ventricular dysfunction. Electron microscopy of the cardiac biopsy demonstrated a curvilinear body, diagnostic of HCQ toxicity. Thus, we diagnosed pulmonary hypertension owing to left heart disease and complete atrioventricular block that resulted from HCQ toxicity. Insertion of a permanent pacemaker and discontinuation of HCQ dramatically improved the disease state. This is the first report of this type of cardiac complication with HCQ; it raises the awareness that HCQ may cause cardiac complications despite a small cumulative dose relative to doses reported in other cases. Furthermore, we emphasize that cardiac catheterization played a critical role in the differential diagnosis from pulmonary hypertension associated with connective tissue disease.

A primary left atrial leiomyosarcoma mimicking myxoma: echocardiographic findings.

Echocardiography is the initial imaging modality to delineate the cardiac mass. Clinically echocardiographic information about morphology, location, motion and hemodynamic consequences of the tumor is very important for decision making in terms of additional imaging studies and treatment. We present a case of left atrial leiomyosarcoma masquerading as myxoma preoperatively and discuss the echocardiographic features for distinguishing leiomyosarcoma from a benign tumor, such as myxoma.

Complete atrioventricular block-induced Torsade de pointes, manifested by epilepsy.

Complete atrioventricular (AV) block is frequently regarded as a cause of informed syncopal attacks, even though the escape rhythm is maintained. Torsade de pointes (TdP) may be a significant complication of AV block associated with QT prolongation. Here, we report the case of a 42-year-old female who was referred to our hospital due to recurrent seizure-like attacks while taking anti-convulsant drugs at a psychiatric hospital. TdP with a long QT interval (corrected QT = 0.591 seconds) was observed on an electrocardiogram (ECG) taken in the emergency department. The patient's drug history revealed olanzapine as the suspicious agent. Even after the medication was stopped, however, the QT interval remained within an abnormal range and multiple episodes of TdP and related seizure-like symptoms were found via ECG monitoring. A permanent pacemaker was thus implanted, and the ventricular rate was set at over 80 beats/min. There was no recurrence of tachyarrhythmia or other symptoms.

Isolated left ventricular noncompaction cardiomyopathy accompanied by severe mitral regurgitation.

Isolated left ventricular noncompaction cardiomyopathy (IVNC) is a cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. This abnormality is often associated with other congenital cardiac defects. A 21-year-old man presented to the emergency department with worsening exertional dyspnea during the previous 2 months. Two-dimensional and Doppler echocardiography revealed an enlarged left atrium (LA) and a markedly dilated left ventricle (LV) with preserved LV systolic function, severe mitral valve regurgitation, and prolapse due to chordae rupture. The myocardium of the LV and right ventricle (RV) had excessively prominent trabeculations and deep intertrabecular recesses. He is the first patient in Korea who has undergone mitral valve replacement surgery because of severe mitral valve regurgitation and prolapse due to chordae rupture accompanied by IVNC.