A case of drug-induced hypersensitivity syndrome complicated with fulminant type 1 diabetes and type 2 myocardial infarction.

Drug-induced hypersensitivity syndrome (DiHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) is a type of drug eruption that causes multiorgan disorders after the administration of certain drugs such as anticonvulsants. Herein, we report the case of a 66-year-old man with DiHS/DRESS complicated by fulminant type 1 diabetes (FT1D), shock, and cardiac involvement who was treated conservatively without systemic corticosteroid administration. He had taken carbamazepine for trigeminal neuralgia for 7 weeks until he noticed eruptions on his trunk. Two days after admission, he developed diabetic ketoacidosis, resulting in hypovolemic shock. The patient was diagnosed with FT1D, and insulin was administered. Additionally, the patient had a fever over 38°C, elevated white blood cells (>20 000/µL), liver dysfunction, atypical lymphocytes, and lymphadenopathy, but no evidence of viral reactivation. The lymphocyte transformation test for carbamazepine was positive, and human leukocyte antigen typing revealed the A31:01 haplotype, a risk factor for carbamazepine-induced cutaneous adverse drug reactions. Collectively, a diagnosis of atypical DiHS and a definitive case of DRESS was made. Moreover, myocardial dysfunction wall motion was observed. A close examination revealed mild coronary artery stenosis, leading to a diagnosis of type 2 myocardial infarction due to relative ischemia. The patient was carefully monitored without systemic corticosteroid administration because both clinical findings and laboratory data peaked on the same day. The patient's eruption and general condition improved, and he was discharged 4 weeks later. While most cases of DiHS/DRESS with cardiac involvement present with myocarditis, the possibility of ischemic heart disease should be considered in patients with cardiac involvement under shock.

Impact of preprocedural echocardiographic parameters on increased stroke volume after transcatheter aortic valve replacement.

Aims: Increased stroke volume (SV) is a prognosticator of severe aortic stenosis (AS) after transcatheter aortic valve replacement (TAVR). This study aimed to investigate preprocedural echocardiographic predictors of increased SV after TAVR. Methods and results: Clinical and echocardiographic data were retrospectively analysed in 129 patients with severe AS who underwent TAVR (2013-2015). We compared the echocardiographic data and cardiac events between the decreased SV group (n=28) and the increased SV group (n=101). Univariate and multivariate analyses were used to assess the predictors of increasing SV. AS severity significantly diminished, left and right ventricular function improved, and SV index (SVi) increased after TAVR: aortic valve area index (0.46±0.13 vs. 1.18±0.33 cm2, p<0.001); aortic regurgitation (AR) grade (1.85±0.55 vs. 1.60±0.54, p<0.001); left ventricular ejection fraction (59.9±12.7 vs. 64.1±12.0%, p<0.001); right ventricular fractional area change (RVFAC) (48.8±11.9 vs. 53.3±14.0%, p<0.001); SV index (SVi) (46.7±11.0 vs. 52.8±12.0 ml/m2, p<0.001). Kaplan-Meier survival estimates suggested that the SVi increase was associated with the decreased cardiovascular events one year after TAVR (hazard ratio 4.08, 95% confidence interval [CI]: 1.32-12.7, p=0.02). On multivariate analysis, preprocedural AR grade (odds ratio [OR] 7.00, 95% CI: 2.76-17.8, p<0.001) and preprocedural RVFAC (OR 1.05, 95% CI: 1.01-1.10, p=0.011) correlated with the SV increase. Conclusions: Preprocedurally, greater AR and higher RVFAC could predict an increased SVi and thus the occurrence of fewer cardiac events. Preserved preprocedural RV systolic function is crucial for an increased SV after TAVR.

Incidence, predictors, and midterm clinical outcomes of left ventricular obstruction after transcatheter aortic valve implantation.

OBJECTIVES: We aimed to evaluate the incidence and midterm clinical outcomes of left ventricular obstruction (LVO) after transcatheter aortic valve implantation (TAVI). BACKGROUNDS: LVO is occasionally unmasked following valve replacement for severe aortic stenosis. However, little is known about the prevalence and effects of LVO after TAVI. METHODS: A total of 158 patients who underwent TAVI in our center between October 2013 and November 2015 received echocardiographic evaluations at baseline; before hospital discharge; and at 3, 6, and 12 months after TAVI. LVO was defined as a peak pressure gradient >30 mm Hg. RESULTS: Over 1 year of follow-up after TAVI, 21 patients (13.3%) demonstrated postprocedural LVO. The incidence was highest at 3-months follow-up and decreased at 6 months or later. Of the 21 patients with LVO, 20 (95.2%) demonstrated midventricular obstruction (MVO), whereas only 1 (4.8%) showed obstruction of the outflow tract (LVOT) with systolic anterior motion (SAM) of the mitral leaflet. In a multivariate analysis, the LVOT diameter (odds ratio [OR], 0.45; 95% confidence interval [CI], 0.30-0.67; P < 0.001), transvalvular velocity (OR, 2.44; 95% CI, 1.13-5.26; P = 0.023), and the presence of accelerated intraventricular flow at baseline (OR, 6.13; 95% CI, 1.49-25.2; P = 0.012) were associated with the occurrence of LVO. Postprocedural LVO was not associated with midterm all-cause death or heart failure events. CONCLUSION: In patients who underwent TAVI, MVO occurred more often than LVOT obstruction. However, the occurrence of postprocedural LVO was not associated with worsened clinical outcomes in these patients.

Comparison of Clinical Outcomes After Transcatheter vs. Minimally Invasive Cardiac Surgery Closure for Atrial Septal Defect.

BACKGROUND: Percutaneous closure has replaced surgery for the majority of cases of secundum atrial septal defect (ASD). However, technological advances have made contemporary minimally invasive cardiac surgery (MICS) less invasive than conventional surgery. The aim of this study was to compare clinical outcomes of percutaneous closure of secundum ASD with those of contemporary MICS.Methods and Results:We conducted a single-center retrospective study of 354 patients with ASD treated either with the Amplatzer Septal Occluder (134 patients) or MICS (220 patients) between 2000 and 2013. Success rates and the incidence of complications were compared. The success rates were 98% for percutaneous closure and 100% for MICS. There were no deaths in either group. Major complications occurred in 2 patients (1.5%) who underwent percutaneous closure and in 8 patients (3.6%) treated with MICS (P=0.16). Minor complications occurred in 15 patients (11.2%) who underwent percutaneous closure and in 46 patients (20.9%) treated with MICS (P=0.02). On multivariate analysis, MICS (odds ratio [OR]: 2.91, 95% confidence interval [CI]: 1.46-5.81; P=0.002) and age >70 years (OR: 3.50, 95% CI: 1.40-8.75; P=0.008) were independent predictors of complications. CONCLUSIONS: Percutaneous closure and MICS had high success rates without deaths. For ASD patients with a suitable anatomy, percutaneous closure can be considered as the first therapeutic option.

Right ventricular dyssynchrony predicts clinical outcomes in patients with pulmonary hypertension.

BACKGROUND: RV dyssynchrony has been described in patients with pulmonary hypertension (PH) and is an independent predictor of clinical worsening; however, the optimal method for measuring RV dyssynchrony has not been fully established. This study aimed to investigate whether RV dyssynchrony is correlated with hemodynamics and prognosis in PH patients and to identify the best parameter for evaluating RV dyssynchrony. METHODS: This study assessed 100 PH patients, measuring mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), and cardiac index (CI) by right heart catheterization (RHC). RV strain curves were obtained using two-dimensional speckle-tracking echocardiography (2DSTE), and time from QRS onset to maximum peak longitudinal strain (Tmax) or time to first peak (Tfirst) was measured. Difference in time between the earliest and the latest segment (TD) and standard deviation (SD) of T in 6 segments with RV apex (-SD6) and 4 segments without RV apex (-SD4) were also assessed. RESULTS: Among all RV dyssynchrony parameters, Tmax-SD6 showed the strongest correlation with RV hemodynamics such as mPAP (R2=0.27, P<0.0001) and PVR (R2=0.22, P<0.0001). Univariate and multivariate Cox proportional hazard analyses showed that Tmax-SD6 significantly correlated with and independently predicted event-free survival in patients with PH. CONCLUSIONS: RV dyssynchrony is a useful index for RV function and an independent predictor of clinical outcomes in patients with PH. In addition, the inclusion of apical motion is essential for the optimal assessment of RV dyssynchrony.

Prognostic value of three-dimensional echocardiographic right ventricular ejection fraction in patients with pulmonary arterial hypertension.

BACKGROUND: Right ventricular (RV) function is an independent predictor of clinical outcomes in patients with pulmonary arterial hypertension (PAH). However, it remains controversial which RV parameter should be measured as an appropriate index for the treatment of PAH. The aim of this study was to identify the most useful parameter that correlates with hemodynamics and predicts clinical outcomes in PAH. RESULTS: Most of the clinical and echocardiographic RV parameters were significantly correlated with pulmonary vascular resistance (PVR) as well as mean pulmonary arterial pressure (mPAP). Among these, three dimensional right ventricular ejection fraction (3DRVEF) showed the strongest hemodynamic correlation, followed by 6-minute walk distance. Receiver operating characteristic analysis of association with cardiac events including death, hospitalization, and intervention revealed a greater area under the curve for 3DRVEF than for mPAP (0.78 vs. 0.74). Kaplan-Meier analysis showed that patients with 3DRVEF less than 38% had significantly shorter event-free survival than those with greater than 38% (P = 0.0007). Finally, the Cox proportional hazards analysis revealed that 3DRVEF, but not mPAP, was an independent predictor of clinical events in PAH. MATERIALS AND METHODS: Eighty-six consecutive patients were enrolled in this study. RV hemodynamic parameters were measured by right heart catheterization (RHC). RV function was assessed using two-dimensional speckle-tracking echocardiography and three-dimensional transthoracic echocardiography (3DTTE) to evaluate RV free wall global strain (RVFS) and RVEF. CONCLUSIONS: RVEF measured by 3DTTE could be a useful parameter for noninvasively assessing RV hemodynamics and predicting the clinical outcomes in PAH patients.

Changes in Right Ventricular Dysfunction After Balloon Pulmonary Angioplasty in Patients With Chronic Thromboembolic Pulmonary Hypertension.

The aim was to investigate the effect of balloon pulmonary angioplasty (BPA) on right ventricular (RV) function in chronic thromboembolic pulmonary hypertension. Twenty-six patients with chronic thromboembolic pulmonary hypertension were enrolled and were divided into 2 groups, group H with high (>30 mm Hg) mean pulmonary arterial pressure and group L with low (25 to 30 mm Hg) mean pulmonary arterial pressure. RV function was assessed using 2-dimensional speckle-tracking echocardiography as well as 3-dimensional echocardiography, and RV dyssynchrony was assessed by the RV strain curves. Exercise capacity was evaluated by the 6-minute walk distance. RV dilatation was significantly reduced after BPA. In group H, RV ejection fraction, RV free wall longitudinal strain and RV dyssynchrony were all impaired before BPA and were ameliorated after BPA. In group L, RV ejection fraction as well as RV dyssynchrony were impaired without the reduction of RV free wall longitudinal strain and were improved after BPA, indicating that RV dysfunction may be attributable to the RV dyssynchrony in group L. Furthermore, RV dyssynchrony at baseline was the only parameter that was correlated with improvement in the 6-minute walk distance after BPA. RV dyssynchrony may affect RV function and could be the useful parameter for clinical outcome after BPA.

Left atrial strain is a powerful predictor of atrial fibrillation recurrence after catheter ablation: study of a heterogeneous population with sinus rhythm or atrial fibrillation.

AIMS: Accumulating data show the efficacy of catheter ablation (CA) for atrial fibrillation (AF); however, postoperative recurrence is not uncommon. The aim of this study was to identify predictors of AF recurrence in patients undergoing CA. METHODS AND RESULTS: We studied 100 patients with symptomatic paroxysmal (68) or persistent (32) AF who underwent CA preceded by transthoracic echocardiographic examination. Of these, 50 had sinus rhythm during echocardiography (Group NSR) and 50 had AF rhythm (Group AF). The left atrial (LA) strain was measured by two-dimensional speckle tracking echocardiography. Echocardiographic parameters were compared between the patients with AF recurrence and no recurrence. During 12 months of follow-up, 26 of 100 patients (11 in Group NSR and 15 in Group AF) had AF recurrence; these patients had significantly longer AF duration, a lower LA global strain (LA-GS), lower LA lateral total strain (LA-LS), and larger maximum LA volume index (LAVImax) than those who maintained sinus rhythm. Multivariate logistic regression identified basal LA-LS and LAVImax as independent predictors of AF recurrence. Furthermore, receiver operating characteristic analyses revealed that basal LA-LS was the most useful parameter for predicting AF recurrence [area under the curve (AUC): 0.84 vs. 0.74 in LAVImax]. Subanalyses showed that LAVImax was another independent predictor of AF recurrence in Group AF, but not in Group NSR, while basal LA-LS was a significant predictor in both groups. CONCLUSION: LA myocardial function assessed by basal LA-LS could predict AF recurrence after CA. Notably, such an assessment could be applicable even during AF rhythm, suggesting its convenience in the clinical setting without defibrillation before analysis.

Significance of echocardiographic assessment for right ventricular function after balloon pulmonary angioplasty in patients with chronic thromboembolic induced pulmonary hypertension.

Balloon pulmonary angioplasty (BPA) may improve hemodynamics and exercise tolerance in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We studied consecutive 25 patients with CTEPH who underwent BPA and evaluated hemodynamics by right-sided heart catheterization. Right ventricular (RV) function was assessed before and after BPA by echocardiography including speckle-tracking echocardiography and 3-dimensional echocardiography. BPA improved the mean pulmonary artery pressure, pulmonary vascular resistance, and cardiac index. BPA also ameliorated the 3-dimentional RV volume, RV ejection fraction, and RV systolic peak strain, all of which were significantly correlated with hemodynamic parameters. The changes in cardiac index were significantly correlated with those in 3-dimentional RV volume index. Furthermore, RV dyssynchrony quantified by the RV strain analyses was ameliorated after BPA even in patients with mild pulmonary hypertension, implicating the merit of BPA in this patient population with CTEPH. BPA not only improved the hemodynamics in patients with CTEPH, but also ameliorated RV remodeling and dyssynchrony as assessed by 3-dimensional echocardiography or speckle-tracking echocardiography. Thus, the assessment of RV function may provide valuable information about the appropriate indication for BPA, its efficacy, and the therapeutic goal for patients with CTEPH.

A rare case of crowned dens syndrome mimicking aseptic meningitis.

BACKGROUND: Crowned dens syndrome (CDS), related to microcrystalline deposition in the periodontoid process, is the main cause of acute or chronic cervical pain. Microcrystal-line deposition most often consists of calcium pyrophosphate dehydrate crystals and/or hydroxyapatite crystals. CASE PRESENTATION: This report describes the case of an 89-year-old woman who presented with sudden onset, high fever, severe occipital headache, and neck stiffness. A laboratory examination revealed a markedly elevated white blood cell count (11,100/µl) and C-reactive protein level (23.8 mg/dl). These clinical findings suggested severe infection such as meningitis with sepsis. However, the results of blood culture, serum endotoxin, and procalcitonin were all negative, and cerebrospinal fluid studies revealed only a slight abnormality. The patient was first diagnosed with meningitis and treated with antiviral and antibiotic agents as well as non-steroidal anti-inflammatory drugs, but they only had limited effects. A cervical plain computed tomography (CT) scan and its three-dimensional (3D) reconstruction detected a remarkable crown-like calcification surrounding the odontoid process. On the basis of the CT findings, the patient was diagnosed as a severe case of CDS and was immediately treated with corticosteroids. The patient's condition drastically improved within a week after one course of corticosteroid therapy. CONCLUSION: Some atypical symptoms of CDS are misleading and may be misdiagnosed as meningitis, as happened in our case. A CT scan, especially a 3D-CT scan, is necessary and useful for a definitive diagnosis of CDS. CDS should be considered as a differential diagnosis of a possible etiology for fever, headache, and cervical pain of unknown origin.