Dysregulation of mitochondrial dynamics mediated aortic perivascular adipose tissue-associated vascular reactivity impairment under excessive fructose intake.

Excessive fructose intake presents the major risk factor for metabolic cardiovascular disease. Perivascular adipose tissue (PVAT) is a metabolic tissue and possesses a paracrine function in regulating aortic reactivity. However, whether and how PVAT alters vascular function under fructose overconsumption remains largely unknown. In this study, male Sprague-Dawley rats (8 weeks old) were fed a 60% high fructose diet (HFD) for 12 weeks. Fasting blood sugar, insulin, and triglycerides were significantly increased by HFD intake. Plasma adiponectin was significantly enhanced in the HFD group. The expression of uncoupling protein 1 (UCP1) and mitochondrial mass were reduced in the aortic PVAT of the HFD group. Concurrently, the expression of peroxisome proliferator-activated receptor-γ coactivator 1α (PGC-1α) and mitochondrial transcription factor A (TFAM) were suppressed. Furthermore, decreased fusion proteins (OPA1, MFN1, and MFN2) were accompanied by increased fission proteins (FIS1 and phospho-DRP1). Notably, the upregulated α-smooth muscle actin (α-SMA) and osteocalcin in the PVAT were concurrent with the impaired reactivity of aortic contraction and relaxation. Coenzyme Q10 (Q, 10 mg/100 mL, 4 weeks) effectively reversed the aforementioned events induced by HFD. Together, these results suggested that the dysregulation of mitochondrial dynamics mediated HFD-triggered PVAT whitening to impair aortic reactivity. Fortunately, coenzyme Q10 treatment reversed HFD-induced PVAT whitening and aortic reactivity.

Outcomes and Hemodynamic Performances of Transcatheter Aortic Valve Replacement with Two Generations of Self-Expanding Transcatheter Aortic Valves.

Background: The superiority of the new-generation self-expanding Evolut R compared with the first-generation CoreValve with regards to outcomes after transcatheter aortic valve replacement (TAVR) is unclear. The aim of this study was to investigate the hemodynamic and clinical performance of Evolut R compared with its direct predecessor, CoreValve, in a Taiwanese population. Methods: This study included all consecutive patients who underwent TAVR with either CoreValve or Evolut R between March 2013 and December 2020. Thirty-day Valve Academic Research Consortium-2 (VARC-2)-defined outcomes and hemodynamic performances were investigated. Results: There were no significant differences in baseline demographic characteristics between the patients receiving CoreValve (n = 117) or Evolut R (n = 117). Aortic valve-in-valve procedures for failed surgical bioprosthesis and procedures under conscious sedation were performed significantly more often with Evolut R. Pre-dilatation was performed significantly more often and contrast media volume was significantly higher with CoreValve. Stroke (0% vs. 4.3%, p = 0.024) and the need for emergent conversion to open surgery (0% vs. 5.1%, p = 0.012) were significantly lower in Evolut R than in CoreValve recipients. Evolut R significantly reduced 30-day composite safety endpoint (4.3% vs. 15.4%, p = 0.004). Conclusions: Advancements in transcatheter valve technologies have resulted in improved outcomes for patients undergoing TAVR with self-expanding valves. With the new-generation Evolut R, device success was high and the 30-day composite safety endpoint was significantly reduced after TAVR compared with CoreValve.

Left ventricular assist device exchange from HeartMate II to HeartMate 3 in an Asian patient-a case report and literature review.

BACKGROUND: Pump exchange surgery of left ventricular assist device (LVAD) has been demonstrated in several studies; however, information for Asian patients was limited. CASE PRESENTATION: A 63-year-old man underwent a pump upgrade from HeartMate II to HeartMate 3 for driveline damage through limited left anterior thoracotomy and lower partial sternotomy. He did not experience any hemodynamic adverse events or device malfunction during postoperative follow-ups of 12 months. We also reviewed all published cases with HeartMate II exchange to HeartMate 3. CONCLUSIONS: The case demonstrated that it was safe and feasible to perform HMII LVAD exchange to HM3 through a limited approach for Asian patients.

Correlation of N-terminal-pro-brain natriuretic peptide with postoperative outcomes of older patients undergoing transcatheter aortic valve replacement.

BACKGROUND: Patients undergoing transcatheter aortic valve replacement surgery (TAVR) are typically older adults with multiple chronic diseases and therefore have a high surgical risk. The N-terminal of brain natriuretic peptide (BNP) and pro-BNP, referred to as NT-pro-BNP, is an easily measurable biomarker of heart failure. Studies on correlation between higher NT-pro-BNP levels and adverse prognoses after TAVR have yielded inconsistent results. Here, we investigated whether preoperative NT-pro-BNP levels are correlated with outcomes among older adults undergoing TAVR. METHODS: This retrospective study included older adults with severe aortic stenosis (AS) who received TAVR from a medical center between January 2013 and June 2017. The patients' demographics, preoperative laboratory data, postoperative complications, and 1-year mortality were recorded. They were divided into two groups based on their preoperative NT-pro-BNP levels. The post-TAVR outcomes in the two groups were analyzed using a multivariate logistic regression analysis of the binary results. RESULTS: Of the 132 patients included (mean age: 81.5 ± 8.1 years; 47% men), 96 (72.7%) had preoperative NT-pro-BNP levels ≤ 4853 ng/L, and 36 (27.3%) had preoperative NT-pro-BNP levels > 4853 ng/L. The postoperative outcomes were significantly better in the NT-pro-BNP≤4853 group than in the NT-pro-BNP>4853 group: postoperative extracorporeal membrane oxygenation fittings (4.2% vs 16.7%, p = 0.025), number of days in hospital (17.5 ± 21.0 vs 27 ± 17.0, p = 0.009), in-hospital mortality (4.2% vs 16.7%, p = 0.025), and 1-year mortality (11.5% vs 38.9%, p = 0.001); the significant differences persisted after controlling for other variables. CONCLUSION: For older patients undergoing TAVR with NT-pro-BNP levels > 4853 ng/L, their postoperative outcomes and 1-year mortality were correlated. Thus, NT-pro-BNP is useful for the risk assessment of patients undergoing TAVR and should be regarded as a biomarker in future risk assessments.

Continual Learning of a Transformer-Based Deep Learning Classifier Using an Initial Model from Action Observation EEG Data to Online Motor Imagery Classification.

The motor imagery (MI)-based brain computer interface (BCI) is an intuitive interface that enables users to communicate with external environments through their minds. However, current MI-BCI systems ask naïve subjects to perform unfamiliar MI tasks with simple textual instruction or a visual/auditory cue. The unclear instruction for MI execution not only results in large inter-subject variability in the measured EEG patterns but also causes the difficulty of grouping cross-subject data for big-data training. In this study, we designed an BCI training method in a virtual reality (VR) environment. Subjects wore a head-mounted device (HMD) and executed action observation (AO) concurrently with MI (i.e., AO + MI) in VR environments. EEG signals recorded in AO + MI task were used to train an initial model, and the initial model was continually improved by the provision of EEG data in the following BCI training sessions. We recruited five healthy subjects, and each subject was requested to participate in three kinds of tasks, including an AO + MI task, an MI task, and the task of MI with visual feedback (MI-FB) three times. This study adopted a transformer- based spatial-temporal network (TSTN) to decode the user's MI intentions. In contrast to other convolutional neural network (CNN) or recurrent neural network (RNN) approaches, the TSTN extracts spatial and temporal features, and applies attention mechanisms along spatial and temporal dimensions to perceive the global dependencies. The mean detection accuracies of TSTN were 0.63, 0.68, 0.75, and 0.77 in the MI, first MI-FB, second MI-FB, and third MI-FB sessions, respectively. This study demonstrated the AO + MI gave an easier way for subjects to conform their imagery actions, and the BCI performance was improved with the continual learning of the MI-FB training process.

Using Signal Features of Functional Near-Infrared Spectroscopy for Acute Physiological Score Estimation in ECMO Patients.

Extracorporeal membrane oxygenation (ECMO) is a vital emergency procedure providing respiratory and circulatory support to critically ill patients, especially those with compromised cardiopulmonary function. Its use has grown due to technological advances and clinical demand. Prolonged ECMO usage can lead to complications, necessitating the timely assessment of peripheral microcirculation for an accurate physiological evaluation. This study utilizes non-invasive near-infrared spectroscopy (NIRS) to monitor knee-level microcirculation in ECMO patients. After processing oxygenation data, machine learning distinguishes high and low disease severity in the veno-venous (VV-ECMO) and veno-arterial (VA-ECMO) groups, with two clinical parameters enhancing the model performance. Both ECMO modes show promise in the clinical severity diagnosis. The research further explores statistical correlations between the oxygenation data and disease severity in diverse physiological conditions, revealing moderate correlations with the acute physiologic and chronic health evaluation (APACHE II) scores in the VV-ECMO and VA-ECMO groups. NIRS holds the potential for assessing patient condition improvements.

The Full Informational Spectral Analysis for Auditory Steady-State Responses in Human Brain Using the Combination of Canonical Correlation Analysis and Holo-Hilbert Spectral Analysis.

Auditory steady-state response (ASSR) is a translational biomarker for several neurological and psychiatric disorders, such as hearing loss, schizophrenia, bipolar disorder, autism, etc. The ASSR is sinusoidal electroencephalography (EEG)/magnetoencephalography (MEG) responses induced by periodically presented auditory stimuli. Traditional frequency analysis assumes ASSR is a stationary response, which can be analyzed using linear analysis approaches, such as Fourier analysis or Wavelet. However, recent studies have reported that the human steady-state responses are dynamic and can be modulated by the subject's attention, wakefulness state, mental load, and mental fatigue. The amplitude modulations on the measured oscillatory responses can result in the spectral broadening or frequency splitting on the Fourier spectrum, owing to the trigonometric product-to-sum formula. Accordingly, in this study, we analyzed the human ASSR by the combination of canonical correlation analysis (CCA) and Holo-Hilbert spectral analysis (HHSA). The CCA was used to extract ASSR-related signal features, and the HHSA was used to decompose the extracted ASSR responses into amplitude modulation (AM) components and frequency modulation (FM) components, in which the FM frequency represents the fast-changing intra-mode frequency and the AM frequency represents the slow-changing inter-mode frequency. In this paper, we aimed to study the AM and FM spectra of ASSR responses in a 37 Hz steady-state auditory stimulation. Twenty-five healthy subjects were recruited for this study, and each subject was requested to participate in two auditory stimulation sessions, including one right-ear and one left-ear monaural steady-state auditory stimulation. With the HHSA, both the 37 Hz (fundamental frequency) and the 74 Hz (first harmonic frequency) auditory responses were successfully extracted. Examining the AM spectra, the 37 Hz and the 74 Hz auditory responses were modulated by distinct AM spectra, each with at least three composite frequencies. In contrast to the results of traditional Fourier spectra, frequency splitting was seen at 37 Hz, and a spectral peak was obscured at 74 Hz in Fourier spectra. The proposed method effectively corrects the frequency splitting problem resulting from time-varying amplitude changes. Our results have validated the HHSA as a useful tool for steady-state response (SSR) studies so that the misleading or wrong interpretation caused by amplitude modulation in the traditional Fourier spectrum can be avoided.

Impact of preprocedural mesenteric artery stenosis and mesenteric ischemia in patients undergoing transcatheter aortic valve replacement.

OBJECTIVES: We aimed to examine the incidence, etiologies, and consequences of acute mesenteric ischemia as well as the impact of preprocedural subclinical mesenteric artery stenosis in patients undergoing transcatheter aortic valve replacement. METHODS: Among prospective follow-up of 269 consecutive patients undergoing transcatheter aortic valve replacement, diagnosis of acute mesenteric ischemia was confirmed by abdominal computed tomography. Cumulative hazard of 1-year all-cause and cardiovascular mortality according to the absence or presence of mesenteric artery stenosis 70% or greater from preprocedural computed tomography angiography was analyzed. RESULTS: Acute mesenteric ischemia was confirmed in 7 patients (2.6%) during mid-term (median, 33.3 months, interquartile range, 15.0-61.0 months) follow-up. Thrombotic occlusions of previously stenotic mesenteric arteries account for 4 cases (57.1%), and embolic acute mesenteric ischemia constitute the rest (42.9%) of the cases. The mortality rate of acute mesenteric ischemia was 100%. At 30 days, death from acute mesenteric ischemia accounts for 40% of all-cause mortality and 67% of cardiovascular death. By multivariable analysis, higher Society of Thoracic Surgeons score and mesenteric artery stenosis 70% or greater were independently associated with acute mesenteric ischemia. Thirty-two patients (11.9%) with preprocedural mesenteric artery stenosis 70% or greater had an increased risk of all-cause mortality (adjusted hazard ratio, 3.78; 95% confidence interval, 1.74-8.19; P = .001) at 1 year after transcatheter aortic valve replacement. CONCLUSIONS: Acute mesenteric ischemia, an important cause of 30-day mortality, should be considered in patients who become clinically unstable after transcatheter aortic valve replacement, particularly but not exclusively in those with preexisting mesenteric artery stenosis. Mesenteric artery stenosis should be routinely assessed in all patients who are indicated for transcatheter aortic valve replacement considering the dismal prognosis of acute mesenteric ischemia.

Application of Whole Exome Sequencing and Functional Annotations to Identify Genetic Variants Associated with Marfan Syndrome.

Marfan syndrome (MFS) is a rare disease that affects connective tissue, which causes abnormalities in several organ systems including the heart, eyes, bones, and joints. The autosomal dominant disorder was found to be strongly associated with FBN1, TGFBR1, and TGFBR2 mutations. Although multiple genetic mutations have been reported, data from Asian populations are still limited. As a result, we utilized the whole exome sequencing (WES) technique to identify potential pathogenic variants of MFS in a Taiwan cohort. In addition, a variety of annotation databases were applied to identify the biological functions as well as the potential mechanisms of candidate genes. In this study, we confirmed the pathogenicity of FBN1 to MFS. Our results indicated that TTN and POMT1 may be likely related to MFS phenotypes. Furthermore, we found nine unique variants highly shared in a MFS family cohort, of which eight are novel variants worthy of further investigation.

Technical Characteristics and Feasibility of Coronary Angiography and Percutaneous Coronary Interventions Performed after Transcatheter Aortic Valve Replacement with Self-Expanding Valves.

BACKGROUND: Coronary angiography (CA) or percutaneous coronary intervention (PCI) after transcatheter aortic valve replacement (TAVR) may become technically challenging after implantation of the self-expanding Medtronic CoreValve (MCV) device, which extends above the coronary ostia. The aim of this study was to investigate the incidence and feasibility of CA or PCI and the outcomes of PCI after TAVR with the MCV device. METHODS: From July 2014 to April 2020, among 209 patients treated with TAVR with a MCV device, 14 (7%) underwent CA or PCI after the procedure at a mean duration of 28 ± 15 months at our institution. RESULTS: The mean age of the patients was 83 ± 6 years. Thirteen (93%) patients underwent CA due to angina symptoms with a positive noninvasive test, and 1 underwent CA for acute coronary syndrome. Most of the CA and PCI procedures were performed through a radial approach: 11 patients (79%) via the right radial artery, 1 (7%) the left radial artery, and 2 (14%) through the right femoral artery. CA of the left and right coronary arteries was successfully achieved in 13 patients (93%) with Judkin left (3.5 to 5) diagnostic catheters and in 11 patients (79%) with Judkin right (4) diagnostic catheters. The second-line catheter of choice was the Amplatz left (AL) 1 catheter for the right coronary artery and AL 2 for the left coronary artery. Procedural success was achieved in all 5 patients who underwent post-TAVR PCI without procedural or in-hospital complications. The use of a Guideliner microcatheter facilitated stent delivery in one patient. CONCLUSIONS: Coronary angiography or PCI following TAVR with a MCV device is feasible and safe, but requires understanding of the three-dimensional geometry of the prosthetic valve and its relationship to the coronary ostia.

Membranous septum length predicts conduction disturbances following transcatheter aortic valve replacement.

OBJECTIVES: Insufficient distance between membranous septum (MS) length and implant depth (ID) may aggravate mechanical compression of the conduction tissue by transcatheter aortic valve replacement (TAVR) prosthesis. We investigated the implication of MS length measured in the coronal view (coronal MS length) compared with infra-annular MS length from stretched vessel image to predict conduction disturbances following TAVR with CoreValve/Evolut R valves (Medtronic, Minneapolis, Minn). METHODS: Among 195 consecutive patients undergoing TAVR with CoreValve/Evolut R valves, we evaluated coronal, infra-annular MS lengths and ID, as well as MS length minus ID (ΔMSID) using pre-TAVR computed tomography and postprocedural angiography. RESULTS: Within 30 days, 6 (3.1%) required permanent pacemaker implantation and 31 (16.4%) developed left bundle branch block. When taking into account pre- and postprocedural parameters, multivariable logistic regression analysis revealed either coronal ΔMSID (odds ratio, 0.80; 95% confidence interval, 0.72-0.89; P < .001; cutoff point, 3.2 mm) or infra-annular ΔMSID (odds ratio, 0.84; 95% confidence interval, 0.76-0.92; P < .001; cutoff point, -0.2 mm) emerged as the only modifiable predictor of conduction disturbances. The area under the curve of coronal ΔMSID and infra-annular ΔMSID for predicting the occurrence of conduction disturbances were comparable (0.717 in coronal ΔMSID vs 0.708 in infra-annular ΔMSID; P = .761), but more patients could be guided by coronal MS length than infra-annular MS length (95.9% vs 87.2%; P = .002). CONCLUSIONS: Preprocedural assessment of MS length should be routinely adopted to determine the optimal ID to mitigate individual patient susceptibility to conduction disturbances after TAVR with self-expanding valves.

Impact of Prosthesis-Patient Mismatch After Transcatheter Aortic Valve Replacement in Asian Patients.

BACKGROUND: Little is known about the incidence of prosthesis-patient mismatch (PPM) and its impact after transcatheter aortic valve replacement with self-expanding valves in an Asian population. We aimed to assess postprocedural effective orifice area with standardized methods and the impact of PPM on midterm outcomes after CoreValve or Evolut R (Medtronic) implantation in an Asian population. METHODS: Among 201 consecutive patients undergoing CoreValve or Evolut R implantation, PPM was assessed at 30 days and defined based on core laboratory measured indexed effective orifice area as severe (less than 0.65 cm2/m2) or moderate (0.65 to 0.85 cm2/m2). Multivariable regression models were utilized to examine predictors of PPM as well as mortality and rehospitalization for heart failure at midterm follow-up. RESULTS: Moderate and severe PPM were observed after self-expanding valves in 37 patients (18.4%) and 3 patients (1.5%), respectively. These 40 patients were included in the PPM group. Predictors of PPM included female sex, larger body surface area, and lower left ventricular ejection fraction. At midterm (median 30.4 months; interquartile range, 17 to 57.8) follow-up, patients with PPM had an increased risk of all-cause death (adjusted hazard ratio 1.95; 95% confidence interval, 1.08 to 3.53; P = .027), cardiovascular mortality (adjusted hazard ratio 3.38; 95% confidence interval, 1.04 to 10.99; P = .043), and rehospitalization for heart failure (adjusted hazard ratio 2.40; 95% confidence interval, 1.11 to 5.17; P = .025). CONCLUSIONS: Patient-prosthesis mismatch was associated with higher midterm mortality and rehospitalization for heart failure in an Asian population. The expected postprocedural effective orifice area for any given valve size may be helpful in preprocedural decision making to avoid PPM.

High fructose induced osteogenic differentiation of human valve interstitial cells via activating PI3K/AKT/mitochondria signaling.

BACKGROUND: Aortic valve stenosis (AS) is a common, lethal cardiovascular disease. There is no cure except the valve replacement at last stage. Therefore, an understanding of the detail mechanism is imperative to prevent and intervene AS. Metabolic syndrome (MetS) is one of the major risk factors of AS whereas fructose overconsuming tops the list of MetS risk factors. However, whether the fructose under physiological level induces AS is currently unknown. METHODS: The human valve interstitial cells (hVICs), a crucial source to develop calcification, were co-incubated with fructose at 2 or 20 mM to mimic the serum fructose at fasting or post-fructose consumption, respectively, for 24 h. The cell proliferation was evaluated by WST-1 assays. The expressions of osteogenic and fibrotic proteins, PI3K/AKT signaling, insulin receptor substrate 1 and mitochondrial dynamic proteins were detected by Western blot analyses. The mitochondrial oxidative phosphorylation (OXPHOS) was examined by Seahorse analyzer. RESULTS: hVICs proliferation was significantly suppressed by 20 mM fructose. The expressions of alkaline phosphatase (ALP) and osteocalcin were enhanced concurrent with the upregulated PI3K p85, AKT, phospho(p)S473-AKT, and pS636-insulin receptor substrate 1 (p-IRS-1) by high fructose. Moreover, ATP production capacity and maximal respiratory capacity were enhanced in the high fructose groups. Synchronically, the expressions of mitochondrial fission 1 and optic atrophy type 1 were increased. CONCLUSIONS: These results suggested that high fructose stimulated the osteogenic differentiation of hVICs via the activation of PI3K/AKT/mitochondria signaling at the early stage. These results implied that high fructose at physiological level might have a direct, hazard effect on the progression of AS.

Diabetes Is the Most Critical Risk Factor of Adverse Complications After Peritoneal Dialysis Catheter Placement.

Introduction: Peritoneal dialysis (PD) is a kind of renal replacement therapy for end-stage renal disease (ESRD). While PD has many advantages, various complications may arise. Methods: This retrospective study analyzed the complications of ESRD patients who received PD catheter implantation in a single medical center within 15 years. Results: This study collected 707 patients. In the first 14 days after PD implantation, 54 patients experienced bleeding complications, while 47 patients experienced wound infection. Among all complications, catheter-related infections were the most common complication 14 days after PD implantation (incidence: 38.8%). A total of 323 patients experienced PD catheter removal, of which 162 patients were due to infection, while 96 were intentional due to kidney transplantation. Excluding those whose catheters were removed due to transplantation, the median survival of the PD catheter was 4.1 years; among them, patients without diabetes mellitus (DM) were 7.4 years and patients with DM were 2.5 years (p < 0.001). Further, 50% probability of surviving was beyond 3.5 years in DM patients with HbA1CC < 7 and 1.6 years in DM patients with HbA1C <7 (p ≥ 0.001). Conclusions: Catheter-related infections were the most common complications following PD catheter implantation. DM, especially with HbA1C ≥7, significantly impacted on the catheter-related infection and the survival probability of the PD catheter.

Vitamin B Mitigates Thoracic Aortic Dilation in Marfan Syndrome Mice by Restoring the Canonical TGF-ß Pathway.

Thoracic aortic aneurysm (TAA) formation is a multifactorial process that results in diverse clinical manifestations and drug responses. Identifying the critical factors and their functions in Marfan syndrome (MFS) pathogenesis is important for exploring personalized medicine for MFS. Methylenetetrahydrofolate reductase (MTHFR), methionine synthase (MTR), and methionine synthase reductase (MTRR) polymorphisms have been correlated with TAA severity in MFS patients. However, the detailed relationship between the folate-methionine cycle and MFS pathogenesis remains unclear. Fbn1C1039G/+ mice were reported to be a disease model of MFS. To study the role of the folate-methionine cycle in MFS, Fbn1C1039G/+ mice were treated orally with methionine or vitamin B mixture (VITB), including vitamins B6, B9, and B12, for 20 weeks. VITB reduced the heart rate and circumference of the ascending aorta in Fbn1C1039G/+ mice. Our data showed that the Mtr and Smad4 genes were suppressed in Fbn1C1039G/+ mice, while VITB treatment restored the expression of these genes to normal levels. Additionally, VITB restored canonical transforming-growth factor ß (TGF-ß) signaling and promoted Loxl1-mediated collagen maturation in aortic media. This study provides a potential method to attenuate the pathogenesis of MFS that may have a synergistic effect with drug treatments for MFS patients.

Intraoperative ECMO assistance during TAVI for aortic insufficiency in an Asian patient with LVAD support.

A 69-year-old male underwent concomitant left ventricular assist device (LVAD) implantation and aortic valve repair procedure for moderate aortic insufficiency (AI). Due to recurrence of symptomatic heart failure and post-repair AI deterioration from trivial to moderate-to-severe insufficiency 2 months later, transcatheter aortic valve implantation (TAVI) was arranged. During TAVI, intraoperative extracorporeal membrane oxygenation was used to maintain stable haemodynamics and to ensure a safe environment for prosthesis deployment. Postoperative 5 month follow-up revealed no AI and no paravalvular leakage.

Somatic SDHA mutations in paragangliomas in siblings: Case report of 2 cases.

RATIONALE: Paragangliomas (PGLs) are rare neuroendocrine tumors that are strongly influenced by genetics, and succinate dehydrogenase-deficient PGLs appear to constitute one of the most important categories. Interestingly, somatic PGLs only possess genomic alterations involving the SDHB and SDHD subunits, and no SDHA alterations have been described. Here, we are presenting the clinical and genetic analyses of 2 cases with the first somatic SDHA variant identified in PGLs. PATIENT CONCERNS: Here, we reported 2 family members with the diagnosis of PGL. Patient 1 is a 55-year-old woman with a functionally perigastric PGL that co-occurred with a gastric gastrointestinal stromal tumor (GIST), and patient 2 is a 43-year-old woman with a nonfunctionally pericardial PGL, who was the younger sister of the first patient. DIAGNOSES: Imaging surveys of the 2 cases depicted the presence of a perigastric and a pericardial mass, respectively. A diagnosis of paragangliomas was established by immunohistochemistry (IHC). INTERVENTIONS: Both patients underwent single-stage resection of the lesion after preoperative oral α-adrenoceptor therapy for 2 weeks. We later performed comprehensive genomic profiling on the tumor samples, including PGL and GIST from patient 1 and PGL from patient 2, and searched for novel actionable mutations, including in all succinate dehydrogenase subunits, as the IHC results were negative for SDHB. OUTCOMES: Both patients had an uneventful recovery after surgery and the sequencing showed a novel somatic variant in the SDHA gene on chromosome 5q11 (c.1945_1946delTT). Regular follow-up with biochemical testing and image studies showed no evidence of recurrence after a year for patient 1 and 6 years for patient 2. LESSONS: PGLs often lead to considerable diagnostic difficulty due to their multiple anatomical locations and variable symptoms, as presented by our cases. The comprehensive use of images and plasma/urine catecholamine measurement can aid the diagnosis of PGLs. In addition, our findings also demonstrate the usefulness and importance of genetic analysis of SDHA mutations in patients exhibiting SDHB IHC-negative PGL. Additional studies utilizing comprehensive genomic profiling are needed to identify the group of PGLs harboring this SDHA genomic alteration.

Optimization of extracorporeal membrane oxygenation therapy using near-infrared spectroscopy to assess changes in peripheral circulation: A pilot study.

Near-infrared spectroscopy (NIRS) has been proposed as a noninvasive modality for detecting complications in patients undergoing extracorporeal membrane oxygenation (ECMO), and it can simultaneously reveal the global circulatory status of these patients. We optimized ECMO therapy on the basis of real-time peripheral NIRS probing. Three patients underwent venoarterial (VA) ECMO and one patient underwent venovenous (VV) ECMO. All patients received peripheral ECMO cannulation with routine distal perfusion catheter placement. We designed an experimental protocol to adjust ECMO blood flow over 1 hour. Hemodynamic responses were measured using NIRS devices attached to the calf at approximately 60% of the distance from the ankle to the knee. HbO2 levels change substantially with adjustments in ECMO flow, and they are more sensitive than HHb levels and the tissue saturation index (TSI) are. NIRS for optimizing ECMO therapy may be reliable for monitoring global circulatory status.

Sex difference in the prognostic role of body composition parameters in Taiwanese patients undergoing transcatheter aortic valve implantation.

BACKGROUND: Evidence on association between body composition and outcomes of transcatheter aortic valve implantation (TAVI) is limited for Asian patients. This study investigated the prognostic role of body composition parameters in Taiwanese patients undergoing TAVI. MATERIALS AND METHODS: Data of consecutive patients undergoing TAVI for severe aortic stenosis between May 1, 2010 and August 31, 2019 were prospectively collected in this observational study. The association between body composition parameters (body mass index [BMI], body surface area [BSA], lean body mass [LBM], and LBM index) and cumulative mortality was analyzed using Cox proportional hazard regression model. RESULTS: A total of 221 patients (mean age 81.4 years), including 125 (56.6%) males, were included with median follow-up duration of 23.8 months. In males, multivariate analysis revealed that higher BMI (P = 0.035), BMI ≥ 20 kg/m2 (P = 0.026), and higher LBM index (P = 0.023) significantly predicted lower overall all-cause cumulative mortality. In females, none of the body composition parameters was significantly associated with all-cause cumulative mortality. Paradoxical association between BMI and estimated all-cause cumulative mortality was only significant among male patients. CONCLUSION: In Taiwanese TAVI patients, the prognostic effects of BMI and LBM index on cumulative mortality were only observed in males, not in females. Sex differences must be considered when stratifying risk among patients undergoing TAVI.