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J. Cardiovasc. Dev. Dis., Volume 9, Issue 7 (July 2022) – 34 articles

Cover Story (view full-size image): This investigation assessed a mixed adult cohort of 53 patients with mitochondrial diseases including chronic progressive external ophthalmoplegia (CPEO), Kearns–Sayre syndrome (KSS), neuropathy ataxia and retinitis pigmentosa (NARP), myoclonic epilepsy with ragged red fibers (MERRF), and Harel-Yoon syndrome (HYS). This cohort was characterized primarily by neurological manifestations and a low prevalence of cardiomyopathy in only six patients. These findings were supported by follow-up data that showed no indications of disease progression from a cardiac perspective. As patients age, a diagnosis of heart disease can likely be attributed to risk factors as opposed to an underlying diagnosis of mitochondrial disease. Traditional risk factors should be recognized to ensure efficient and effective care is provided with appropriate medical and device intervention when appropriate. View this paper
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6 pages, 2365 KiB  
Case Report
A Case Report of a Wenckebach Phenomenon Occurring during a His-Bundle Pacing Procedure: Is It Atrioventricular Node Pacing?
by Chengming Ma, Wenwen Li, Yongmei Cha, Yunlong Xia, Lianjun Gao and Yingxue Dong
J. Cardiovasc. Dev. Dis. 2022, 9(7), 231; https://doi.org/10.3390/jcdd9070231 - 19 Jul 2022
Viewed by 1841
Abstract
A 70-year-old man with severe valvular cardiomyopathy, permanent atrial fibrillation (AF) with a slow ventricular response, and transient atrioventricular (AV) block, was admitted to our center for severe heart failure and recurrent presyncope. While hospitalized, the coronary computed tomography angiography (CTA) showed huge [...] Read more.
A 70-year-old man with severe valvular cardiomyopathy, permanent atrial fibrillation (AF) with a slow ventricular response, and transient atrioventricular (AV) block, was admitted to our center for severe heart failure and recurrent presyncope. While hospitalized, the coronary computed tomography angiography (CTA) showed huge atriums. We tried His bundle pacing (HBP). HB potential was observed at site A, and the His-ventricular (HV) interval was 68 ms. The duration from the stimulus signal to the onset of paced QRS (S-QRSonset) at site A was 232 ms when pacing at 60 beats per minute (BPM) with the pacing threshold of 2.0 V/0.5 ms. The S-QRSonset was longer than the HV interval and had a notable and progressive prolongation from 252 ms to 456 ms during the pacing at 90 BPM. Then, we pushed another lead a little forward, and the S-QRSonset shortened back to 68 ms, and the paced QRS morphology was the same as the intrinsic QRS morphology with the pacing threshold of 1.5 V/0.5 ms. The progressively prolonged S-QRSonset demonstrated a Wenckebach phenomenon (WP), a well-known electrophysiological characteristic of the AV node (AVN). It is the first time to report an intraoperative AVN-pacing related-WP in a patient with persistent AF. The enlarged atrium might be convenient for capturing the AVN. There are some other potential explanations for this phenomenon. The diameters of atriums decreased significantly, and the symptoms improved after the procedure. This is the first reported case in which we might achieve AVN capture in a patient with persistent AF. Although we ultimately chose HBP for better long-term pacing thresholds, the result of this case suggested that AVN pacing may be possible. Full article
(This article belongs to the Section Electrophysiology and Cardiovascular Physiology)
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13 pages, 1548 KiB  
Article
Left Atrial Remodeling after Mitral Valve Repair for Primary Mitral Regurgitation: Evolution over Time and Prognostic Significance
by Jan Stassen, Aniek L. van Wijngaarden, Hoi W. Wu, Meindert Palmen, Anton Tomsic, Victoria Delgado, Jeroen J. Bax and Nina Ajmone Marsan
J. Cardiovasc. Dev. Dis. 2022, 9(7), 230; https://doi.org/10.3390/jcdd9070230 - 18 Jul 2022
Cited by 4 | Viewed by 3034
Abstract
Left atrial (LA) dilatation is associated with worse outcomes in primary mitral regurgitation (MR). However, the effects of mitral valve repair on LA size and its prognostic implications are not well known. In the current study, LA volume index (LAVi) and LA reservoir [...] Read more.
Left atrial (LA) dilatation is associated with worse outcomes in primary mitral regurgitation (MR). However, the effects of mitral valve repair on LA size and its prognostic implications are not well known. In the current study, LA volume index (LAVi) and LA reservoir strain (LASr) were evaluated immediately before and after surgery, and during long-term follow-up in 226 patients undergoing mitral valve repair for primary MR (age 62 ± 13 years, 66% male). Mean LAVi was reduced significantly after surgery and at long-term follow-up (from 56 ± 28 to 38 ± 21 to 32 ± 17 mL/m2; p < 0.001). LASr reduced significantly after surgery but increased again during the long-term (from 23.6 ± 9.4 to 11.5 ± 5.0 to 17.3 ± 7.5%; p < 0.001). Age, pre-operative LAVi, MR severity, and postoperative transmitral pressure gradient were associated with LA reverse remodeling by the long-term check-up. During a median follow-up of 72 (40–114) months, 43 (19%) patients died. Patients with LAVi ≥ 42 mL/m2 at long-term follow-up showed significant higher mortality rates compared to patients with LAVI < 42 mL/m2 (p < 0.001), even after adjusting for clinical covariates. In conclusion, significant LA reverse remodeling was observed both immediately and at long-term follow-up after mitral valve repair. LA dilatation at long term follow-up after surgery was still associated with all-cause mortality. Full article
(This article belongs to the Special Issue Mitral Valve Prolapse and Mitral Regurgitation)
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11 pages, 1355 KiB  
Article
Unipolar Electrogram-Guided versus Lesion Size Index-Guided Catheter Ablation in Patients with Paroxysmal Atrial Fibrillation
by Guohua Fu, Bin He, Binhao Wang, Mingjun Feng, Xianfeng Du, Jing Liu, Yibo Yu, Fang Gao, Weidong Zhuo, Yi Xu, Yingbo Qi and Huimin Chu
J. Cardiovasc. Dev. Dis. 2022, 9(7), 229; https://doi.org/10.3390/jcdd9070229 - 18 Jul 2022
Cited by 8 | Viewed by 2001
Abstract
Background: This research explores the relationship between the unipolar electrogram (UP-EGM) and lesion size index (LSI) in different regions of continuous circular lesions (CCLs) and to assess the safety and efficacy of UP-EGM-guided versus LSI-guided radiofrequency catheter ablation (RFCA) in patients with paroxysmal [...] Read more.
Background: This research explores the relationship between the unipolar electrogram (UP-EGM) and lesion size index (LSI) in different regions of continuous circular lesions (CCLs) and to assess the safety and efficacy of UP-EGM-guided versus LSI-guided radiofrequency catheter ablation (RFCA) in patients with paroxysmal atrial fibrillation (PAF). Methods: A total of 120 patients with drug-refractory PAF who underwent index RFCA were scheduled to be consecutively included from March 2020 to April 2021. All the patients were randomly divided 1:1 into two groups: the UP-EGM group and the LSI group. The first-pass PVI rate, acute PVI rate, and the sinus rhythm maintenance rate were compared. Results: A total of 120 patients with PAF were included in the study: the UP-EGM group (n = 60) and the LSI group (n = 60). All the LSI values in the UP-EGM group were less than those in the corresponding regions in the LSI group (all p < 0.001). There were no significant differences in the first-pass PVI rate and acute PVI rate between the two groups. After a mean follow-up period of 11.31 ± 1.70 months, the sinus rhythm maintenance rate in the UP-EGM group was comparable to that in the LSI group (90% vs. 91.7%, p = 0.752). Conclusion: UP-EGM-guided and LSI-guided RFCA are both effective and safe in patients with PAF. However, UP-EGM may be more suitable than LSI for guiding individual RFCA. Full article
(This article belongs to the Special Issue Catheter Ablation of Cardiac Arrhythmias: Practices and Outcomes)
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12 pages, 1516 KiB  
Article
Frailty Index and Cardiovascular Disease among Middle-Aged and Older Chinese Adults: A Nationally Representative Cross-Sectional and Follow-Up Study
by Xinyao Liu, Guolin Dai, Qile He, Hao Ma and Hongpu Hu
J. Cardiovasc. Dev. Dis. 2022, 9(7), 228; https://doi.org/10.3390/jcdd9070228 - 18 Jul 2022
Cited by 15 | Viewed by 3889
Abstract
Evidence for the association between the frailty index and cardiovascular disease (CVD) is inconclusive, and this association has not been evaluated in Chinese adults. We aim to examine the association between the frailty index and CVD among middle-aged and older Chinese adults. We [...] Read more.
Evidence for the association between the frailty index and cardiovascular disease (CVD) is inconclusive, and this association has not been evaluated in Chinese adults. We aim to examine the association between the frailty index and CVD among middle-aged and older Chinese adults. We conducted cross-sectional and cohort analyses using nationally representative data from the China Health and Retirement Longitudinal Study (CHARLS). From 2011 to 2018, 17,708 participants aged 45 years and older were included in the CHARLS. The primary outcome was CVD events (composite of heart disease and stroke). Multivariable adjusted logistic regression and Cox proportional hazards models were used to estimate the association between the frailty index and CVD in cross-sectional and follow-up studies, respectively. A restricted cubic spline model was used to characterize dose–response relationships. A total of 16,293 and 13,580 participants aged 45 years and older were included in the cross-sectional and cohort analyses, respectively. In the cross-sectional study, the prevalence of CVD in robust, pre-frailty and frailty was 7.83%, 18.70% and 32.39%, respectively. After multivariable adjustment, pre-frailty and frailty were associated with CVD; ORs were 2.54 (95% confidence interval [CI], 2.28–2.84) and 4.76 (95% CI, 4.10–5.52), respectively. During the 7 years of follow-up, 2122 participants without previous CVD developed incident CVD; pre-frailty and frailty were associated with increased risk of CVD events; HRs were 1.53 (95% CI, 1.39–1.68) and 2.17 (95% CI, 1.88–2.50), respectively. Furthermore, a stronger association of the frailty index with CVD was observed in participants aged <55, men, rural community-dwellers, BMI ≥ 25, without hypertension, diabetes or dyslipidemia. A clear nonlinear dose–response pattern between the frailty index and CVD was widely observed (p < 0.001 for nonlinearity), the frailty index was above 0.08, and the hazard ratio per standard deviation was 1.18 (95% CI 1.13–1.25). We observed the association between the frailty index and CVD among middle-aged and elderly adults in China, independent of chronological age and other CVD risk factors. Our findings are important for prevention strategies aimed at reducing the growing burden of CVD in older adults. Full article
(This article belongs to the Topic Biomarkers in Cardiovascular Disease—Chances and Risks)
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10 pages, 1408 KiB  
Article
Systolic Blood Pressure Response to Exercise in Endurance Athletes in Relation to Oxygen Uptake, Work Rate and Normative Values
by Anna Carlén, Gustaf Eklund, August Andersson, Carl-Johan Carlhäll, Magnus Ekström and Kristofer Hedman
J. Cardiovasc. Dev. Dis. 2022, 9(7), 227; https://doi.org/10.3390/jcdd9070227 - 15 Jul 2022
Cited by 5 | Viewed by 3483
Abstract
Work rate has a direct impact on the systolic blood pressure (SBP) during aerobic exercise, which may be challenging in the evaluation of the SBP response in athletes reaching high work rates. We aimed to investigate the exercise SBP response in endurance athletes [...] Read more.
Work rate has a direct impact on the systolic blood pressure (SBP) during aerobic exercise, which may be challenging in the evaluation of the SBP response in athletes reaching high work rates. We aimed to investigate the exercise SBP response in endurance athletes in relation to oxygen uptake (VO2), work rate and to recent reference equations for exercise SBP in the general population. Endurance athletes with a left-ventricular end-diastolic diameter above the reference one performed a maximal bicycle cardiopulmonary exercise test. The increase in SBP during exercise was divided by the increase in VO2 (SBP/VO2 slope) and in Watts, respectively (SBP/W slope). The maximum SBP (SBPmax) and the SBP/W slope were compared to the predicted values. In total, 27 athletes (59% men) were included; mean age, 40 ± 10 years; mean VO2max, 50 ± 5 mL/kg/min. The mean SBP/VO2 slope was 29.8 ± 10.2 mm Hg/L/min, and the mean SBP/W slope was 0.27 ± 0.08 mm Hg/W. Compared to the predicted normative values, athletes had, on average, a 12.2 ± 17.6 mm Hg higher SBPmax and a 0.12 ± 0.08 mm Hg/W less steep SBP/W slope (p < 0.01 and p < 0.001, respectively). In conclusion, the higher SBPmax values and the less steep SBP/W slope highlight the importance of considering work rate when interpreting the SBP response in endurance athletes and suggest a need for specific normative values in athletes to help clinicians distinguish physiologically high maximal blood pressure from a pathological blood pressure response. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Athletes)
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11 pages, 1151 KiB  
Review
Cardiotoxicity of Chemical Substances: An Emerging Hazard Class
by Nikolaos Georgiadis, Konstantinos Tsarouhas, Jean-Lou C. M. Dorne, George E. N. Kass, Petroula Laspa, Konstantinos Toutouzas, Elisabeth A. Koulaouzidou, Dimitrios Kouretas and Christina Tsitsimpikou
J. Cardiovasc. Dev. Dis. 2022, 9(7), 226; https://doi.org/10.3390/jcdd9070226 - 14 Jul 2022
Cited by 10 | Viewed by 2266
Abstract
(1) Background: Human health risks and hazards from chemical substances are well regulated internationally. However, cardiotoxicity, is not defined as a stand-alone hazard and therefore there are no defined criteria for the classification of substances as cardiotoxic. Identifying and regulating substances that cause [...] Read more.
(1) Background: Human health risks and hazards from chemical substances are well regulated internationally. However, cardiotoxicity, is not defined as a stand-alone hazard and therefore there are no defined criteria for the classification of substances as cardiotoxic. Identifying and regulating substances that cause cardiovascular adverse effects would undoubtedly strengthen the national health systems. (2) Methods: To overcome the aforementioned gap, a roadmap is proposed for identifying regulatory criteria from animal studies and endorse legislation in order to classify substances as cardiotoxic. The roadmap consists of: (i) the identification of the appropriate animal species and strains; (ii) the identification of the lines of scientific evidence (e.g., histopathological, biochemical and echocardiographic indices etc.) from animal studies with relevance to humans; (iii) the statistical analysis and meta-analysis for each line of scientific evidence after exposure to well-established cardiotoxicants to humans (e.g., anthracyclines) in order to identify threshold values or range of normal and/ or altered values due to exposure; (iv) validation of the above described lines of evidence in animals exposed to other alleged cardiotoxic substances (e.g., anabolic androgen steroids (AAS) and pesticides); (v) establishment of mechanisms of action based on information of either known or alleged cardiotoxicants; and (vi) introduction of novel indices and in silico methods. (3) Results: Preliminary results in rats indicate a clear distinction from normal values to values measured in rats exposed to anthracyclines regarding left ventricle (LV) fractional shortening (FS) and LV ejection fraction (EF). A distinctive pattern is similarly observed for Creatine Kinase-Myocardial Band isoenzyme (CK-MB) and cardiac tissue glutathione (GSH). These findings are encouraging and indicate that there is room for targeted research to this end, and that these specific indices and biochemical markers should be further investigated in order to be developed to regulatory criteria. (4) Conclusions: Further research should be conducted by both the scientific and regulatory community that aims to clearly define the cardiotoxicity hazard caused by chemicals and develop a full set of scientific criteria. Full article
(This article belongs to the Special Issue Cardiovascular Toxicity Related to Cancer Treatment)
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16 pages, 579 KiB  
Review
Sodium-Glucose Cotransporter-2 Inhibitors-from the Treatment of Diabetes to Therapy of Chronic Heart Failure
by Dominik Kurczyński, Bartosz Hudzik, Marta Jagosz, Jan Zabierowski, Jolanta Nowak, Andrzej Tomasik, Arkadiusz Badziński, Piotr Rozentryt and Mariusz Gąsior
J. Cardiovasc. Dev. Dis. 2022, 9(7), 225; https://doi.org/10.3390/jcdd9070225 - 14 Jul 2022
Cited by 3 | Viewed by 7981
Abstract
Sodium-glucose cotransporter-2 (SGLT2) inhibitors are currently the second-line pharmacotherapy in type 2 diabetes, particularly through their effectiveness in reducing glycemia, but also due to their cardioprotective and nephroprotective effects. In light of surprisingly satisfactory results from large, randomized trials on gliflozins, SGLT2 received [...] Read more.
Sodium-glucose cotransporter-2 (SGLT2) inhibitors are currently the second-line pharmacotherapy in type 2 diabetes, particularly through their effectiveness in reducing glycemia, but also due to their cardioprotective and nephroprotective effects. In light of surprisingly satisfactory results from large, randomized trials on gliflozins, SGLT2 received the highest recommendation (Class IA) with the highest level of evidence (A) in the treatment algorithm for HF with reduced LVEF in recent ESC HF guidelines. This great breakthrough in the treatment of HF is due to different mechanisms of action of gliflozins that are reported to be able to change the natural course of HF by reducing the risk of both hospitalization and death. They are recommended regardless of the patient’s diabetes status. This review summarizes the up-to-date literature on their beneficial and pleiotropic impact on the cardiovascular system. Full article
(This article belongs to the Special Issue Therapeutic Strategies for Heart Failure)
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10 pages, 734 KiB  
Article
30-Day Outcomes after Surgical or Transapical Aortic Valve Replacement in Symptomatic Aortic Regurgitation
by Minjian Kong, Ze Hong, Xianbao Liu, Xian Zhu, Jianan Wang and Aiqiang Dong
J. Cardiovasc. Dev. Dis. 2022, 9(7), 224; https://doi.org/10.3390/jcdd9070224 - 14 Jul 2022
Cited by 3 | Viewed by 1858
Abstract
Background: We aimed to analyze the short-term clinical outcomes of transapical aortic valve replacement (TA-TAVR) compared with surgical aortic valve replacement (SAVR) in symptomatic aortic regurgitation (AR) patients to draw preliminary conclusions about the advantages and disadvantages of TA-TAVR compared with SAVR [...] Read more.
Background: We aimed to analyze the short-term clinical outcomes of transapical aortic valve replacement (TA-TAVR) compared with surgical aortic valve replacement (SAVR) in symptomatic aortic regurgitation (AR) patients to draw preliminary conclusions about the advantages and disadvantages of TA-TAVR compared with SAVR and to provide evidence for future use of TA-TAVR in AR patients. Method: From September 2016 to September 2021, 69 patients undergoing TA-TAVR with J-valve implantation and 42 patients undergoing SAVR at the Second Hospital of Zhejiang University School of Medicine were analyzed for clinical data and 30-day follow-up outcomes to analyze and compare the differences in clinical endpoints between the two procedures. Results: At 30-day follow-up, there were no significant differences in mortality or neurological events between the two groups before and after the PSM. In secondary endpoints there were significant differences between the pre-match TAVR and SAVR groups, such as the incidence of paravalvular leaks (33.8% vs. 4.8%, p < 0.05), which also remained after the PSM (37.5% vs. 0, p < 0.05). In addition, the incidence of major bleeding was 7.4% in the TAVR group and 26.2% in the SAVR group before matching (p < 0.05). After matching, the statistical difference still remained. In longitudinal comparison, significant improvements in postoperative cardiac ultrasound indices and NYHA classification occurred in both groups. Conclusion: The TA-TAVR approach is safe and reliable, with similar clinical efficacy to SAVR, and has advantages in bleeding rate and speed of recovery. Full article
(This article belongs to the Special Issue Transcatheter Aortic Valve Implantation (TAVI))
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18 pages, 2122 KiB  
Review
TAVR for All? The Surgical Perspective
by Xiling Zhang, Thomas Puehler, Derk Frank, Janarthanan Sathananthan, Stephanie Sellers, David Meier, Marcus Both, Philipp Blanke, Hatim Seoudy, Mohammed Saad, Oliver J. Müller, Lars Sondergaard and Georg Lutter
J. Cardiovasc. Dev. Dis. 2022, 9(7), 223; https://doi.org/10.3390/jcdd9070223 - 12 Jul 2022
Cited by 3 | Viewed by 3252
Abstract
In spite of the noninferiority of transcatheter aortic valve replacement (TAVR) in high- and intermediate-risk patients, there are still obstacles that need to be overcome before the procedure is further expanded and clinically integrated. The lack of evidence on the long-term durability of [...] Read more.
In spite of the noninferiority of transcatheter aortic valve replacement (TAVR) in high- and intermediate-risk patients, there are still obstacles that need to be overcome before the procedure is further expanded and clinically integrated. The lack of evidence on the long-term durability of the bioprostheses used for TAVR remains of particular concern. In addition, surgery may be preferred over TAVR in patients with bicuspid aortic valve (BAV) or with concomitant pathologies such as other valve diseases (mitral regurgitation/tricuspid regurgitation), aortopathy, and coronary artery disease. In this review, we discuss and summarize relevant data from clinical trials, current trends, and remaining obstacles, and provide our perspective on the indications for the expansion of TAVR. Full article
(This article belongs to the Special Issue Transcatheter Aortic Valve Implantation (TAVI))
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21 pages, 1356 KiB  
Review
Exercise Training Effects on Circulating Endothelial and Progenitor Cells in Heart Failure
by Christos Kourek, Alexandros Briasoulis, Virginia Zouganeli, Eleftherios Karatzanos, Serafim Nanas and Stavros Dimopoulos
J. Cardiovasc. Dev. Dis. 2022, 9(7), 222; https://doi.org/10.3390/jcdd9070222 - 10 Jul 2022
Cited by 10 | Viewed by 2999
Abstract
Heart failure (HF) is a major public health issue worldwide with increased prevalence and a high number of hospitalizations. Patients with chronic HF and either reduced ejection fraction (HFrEF) or mildly reduced ejection fraction (HFmrEF) present vascular endothelial dysfunction and significantly decreased circulating [...] Read more.
Heart failure (HF) is a major public health issue worldwide with increased prevalence and a high number of hospitalizations. Patients with chronic HF and either reduced ejection fraction (HFrEF) or mildly reduced ejection fraction (HFmrEF) present vascular endothelial dysfunction and significantly decreased circulating levels of endothelial progenitor cells (EPCs). EPCs are bone marrow-derived cells involved in endothelium regeneration, homeostasis, and neovascularization. One of the unsolved issues in the field of EPCs is the lack of an established method of identification. The most widely approved method is the use of monoclonal antibodies and fluorescence-activated cell sorting (FACS) analysis via flow cytometry. The most frequently used markers are CD34, VEGFR-2, CD45, CD31, CD144, and CD146. Exercise training has demonstrated beneficial effects on EPCs by increasing their number in peripheral circulation and improving their functional capacities in patients with HFrEF or HFmrEF. There are two potential mechanisms of EPCs mobilization: shear stress and the hypoxic/ischemic stimulus. The combination of both leads to the release of EPCs in circulation promoting their repairment properties on the vascular endothelium barrier. EPCs are important therapeutic targets and one of the most promising fields in heart failure and, therefore, individualized exercise training programs should be developed in rehabilitation centers. Full article
(This article belongs to the Special Issue Sports Cardiology: From Diagnosis to Clinical Management)
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11 pages, 899 KiB  
Article
Low Prevalence of Cardiomyopathy in Patients with Mitochondrial Disease and Neurological Manifestations
by Anish Nikhanj, Jesi Bautista, Zaeem A. Siddiqi, Cecile L. Phan and Gavin Y. Oudit
J. Cardiovasc. Dev. Dis. 2022, 9(7), 221; https://doi.org/10.3390/jcdd9070221 - 9 Jul 2022
Cited by 2 | Viewed by 2578
Abstract
Patients with mitochondrial diseases can develop cardiomyopathy but with variable expressivity and penetrance. Our prospective study enrolled and evaluated a cohort of 53 patients diagnosed with chronic progressive ophthalmoplegia (CPEO, n = 34), Kearns–Sayre syndrome (KSS, n = 3), neuropathy ataxia and retinitis [...] Read more.
Patients with mitochondrial diseases can develop cardiomyopathy but with variable expressivity and penetrance. Our prospective study enrolled and evaluated a cohort of 53 patients diagnosed with chronic progressive ophthalmoplegia (CPEO, n = 34), Kearns–Sayre syndrome (KSS, n = 3), neuropathy ataxia and retinitis pigmentosa (NARP, n = 1), myoclonic epilepsy with ragged red fibers (MERRF, n = 1), Harel–Yoon Syndrome (HYS, n = 1) and 13 patients with undefined mitochondrial diseases, presenting primarily with neurological symptoms. Over a 4-year period, six patients in our study cohort were diagnosed with heart disease (11.3%), with only three patients having defined cardiomyopathy (5.7%). Cardiomyopathy was present in a 21-year-old patient with HYS and two CPEO patients having mild cardiomyopathy at an older age. Two CPEO patients had congenital heart disease, and a third CPEO had LV hypertrophy secondary to hypertension. In three patients, traditional risk factors for heart disease, including dyslipidemia, hypertension, and respiratory disease, were present. The majority of our adult cohort of patients have normal cardiac investigations with a median left ventricular (LV) ejection fraction of 59.0%, indexed LV mass of 67.0 g/m2, and normal diastolic and valvular function at baseline. A 12-lead electrocardiogram showed normal cardiac conduction across the study cohort. Importantly, follow-up assessments showed consistent cardiac structure and function. Our study shows a low prevalence of cardiomyopathy and highlights the breadth of phenotypic variability in patients with mitochondrial disorders. The presence of cardiovascular risk factors and aging are important comorbidities in our cohort. Full article
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6 pages, 243 KiB  
Editorial
Catalysts for Change: The Role of Nonprofits in Solving Single Ventricle Heart Disease
by Kaitlin A. Davis, Diane M. Pickles and Kirstie E. Keller
J. Cardiovasc. Dev. Dis. 2022, 9(7), 220; https://doi.org/10.3390/jcdd9070220 - 8 Jul 2022
Viewed by 2350
Abstract
Single ventricle (SV) heart disease comprises a spectrum of complex congenital heart defects (CHDs), including hypoplastic left heart syndrome (HLHS), one of the most common causes of death amongst infants with CHD. Despite its incompletely defined etiology and a dearth of curative solutions, [...] Read more.
Single ventricle (SV) heart disease comprises a spectrum of complex congenital heart defects (CHDs), including hypoplastic left heart syndrome (HLHS), one of the most common causes of death amongst infants with CHD. Despite its incompletely defined etiology and a dearth of curative solutions, SV is a solvable problem that can be addressed by unifying a nascent field that is ripe for investment, in part due to its high economic impact and growth potential. Here, we explore the landscape of SV and identify areas of opportunity that will yield an outsized impact through strategic investment that focuses on synchronization across disciplines, community involvement, and infrastructure development, and argue that nonprofits are the appropriate catalyst to spark transformative innovation and impact in the form of functional cures. Full article
7 pages, 4354 KiB  
Perspective
ETS1 and HLHS: Implications for the Role of the Endocardium
by Paul Grossfeld
J. Cardiovasc. Dev. Dis. 2022, 9(7), 219; https://doi.org/10.3390/jcdd9070219 - 8 Jul 2022
Cited by 3 | Viewed by 2194
Abstract
We have identified the ETS1 gene as the cause of congenital heart defects, including an unprecedented high frequency of HLHS, in the chromosomal disorder Jacobsen syndrome. Studies in Ciona intestinalis demonstrated a critical role for ETS1 in heart cell fate determination and cell [...] Read more.
We have identified the ETS1 gene as the cause of congenital heart defects, including an unprecedented high frequency of HLHS, in the chromosomal disorder Jacobsen syndrome. Studies in Ciona intestinalis demonstrated a critical role for ETS1 in heart cell fate determination and cell migration, suggesting that the impairment of one or both processes can underlie the pathogenesis of HLHS. Our studies determined that ETS1 is expressed in the cardiac neural crest and endocardium in the developing murine heart, implicating one or both lineages in the development of HLHS. Studies in Drosophila and Xenopus demonstrated a critical role for ETS1 in regulating cardiac cell fate determination, and results in Xenopus provided further evidence for the role of the endocardium in the evolution of the “hypoplastic” HLHS LV. Paradoxically, these studies suggest that the loss of ETS1 may cause a cell fate switch resulting in the loss of endocardial cells and a relative abundance of cardiac myocytes. These studies implicate an “HLHS transcriptional network” of genes conserved across species that are essential for early heart development. Finally, the evidence suggests that in a subset of HLHS patients, the HLHS LV cardiac myocytes are, intrinsically, developmentally and functionally normal, which has important implications for potential future therapies. Full article
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15 pages, 746 KiB  
Article
Procedure-Related Differences and Clinical Outcomes in Patients Treated with Percutaneous Coronary Intervention Assisted by Optical Coherence Tomography between New and Earlier Generation Software (Ultreon™ 1.0 Software vs. AptiVue™ Software)
by Rafał Januszek, Wojciech Siłka, Karol Sabatowski, Krzysztof Piotr Malinowski, Grzegorz Heba, Sławomir Surowiec, Michał Chyrchel, Łukasz Rzeszutko, Leszek Bryniarski, Andrzej Surdacki, Krzysztof Bartuś and Stanisław Bartuś
J. Cardiovasc. Dev. Dis. 2022, 9(7), 218; https://doi.org/10.3390/jcdd9070218 - 6 Jul 2022
Cited by 7 | Viewed by 2288
Abstract
(1) Introduction: Optical coherence tomography (OCT) intravascular imaging facilitates percutaneous coronary intervention (PCI). Software for OCT is being constantly improved, including the latest version Ultreon™ 1.0 Software (U) (Abbott Vascular, Santa Clara, CA, USA). In the current analysis, we aim to compare processing [...] Read more.
(1) Introduction: Optical coherence tomography (OCT) intravascular imaging facilitates percutaneous coronary intervention (PCI). Software for OCT is being constantly improved, including the latest version Ultreon™ 1.0 Software (U) (Abbott Vascular, Santa Clara, CA, USA). In the current analysis, we aim to compare processing results, procedural indices as well as clinical outcomes in patients treated via PCI. This was conducted using earlier generation OCT imaging software versions (non-U) and the newest available one on the market (U). (2) Methods: The study comprised 95 subsequent and not selected patients (55 processed with U and 40 non-U). The non-U processings were transferred for evaluation by U software, while the comparison of OCT parameters, selected clinical and procedural indices was performed between groups. We further assessed clinical outcomes during the follow-up period, i.e., major adverse cardiovascular events (MACE) and predictors of stent expansion. (3) Results: We did not detect any differences in general features between either of the assessed groups at baseline. Non-U software was more often used for bare-metal stenting (p = 0.004), while PCIs in the U group demanded a greater number of stents (p = 0.03). The distal reference of external elastic lamina (EEL) diameter was greater in the non-U group (p = 0.02) with no concurrent differences in minimal (p = 0.27) and maximal (p = 0.31) stent diameter. It was also observed that MACE was more frequently observed in the non-U group (p = 0.01). Neither univariable (estimate: 0.407, 95%CI: (−3.182) − 3.998, p = 0.82) nor multivariable (estimate: 2.29, 95%CI: (−4.207) − 8.788, p = 0.5) analyses demonstrated a relationship between the type of software and stent expansion. (4) Conclusions: Improvement in the software for image acquisition and processing of OCT is not related to stent expansion. The EEL diameter is preferably used to select the distal stent diameter in newer software. Full article
(This article belongs to the Section Imaging)
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22 pages, 3672 KiB  
Review
The Role of Epicardial Adipose Tissue in the Development of Atrial Fibrillation, Coronary Artery Disease and Chronic Heart Failure in the Context of Obesity and Type 2 Diabetes Mellitus: A Narrative Review
by Anirudh Krishnan, Harman Sharma, Daniel Yuan, Alexandra F. Trollope and Lisa Chilton
J. Cardiovasc. Dev. Dis. 2022, 9(7), 217; https://doi.org/10.3390/jcdd9070217 - 5 Jul 2022
Cited by 17 | Viewed by 8674
Abstract
Cardiovascular diseases (CVDs) are a significant burden globally and are especially prevalent in obese and/or diabetic populations. Epicardial adipose tissue (EAT) surrounding the heart has been implicated in the development of CVDs as EAT can shift from a protective to a maladaptive phenotype [...] Read more.
Cardiovascular diseases (CVDs) are a significant burden globally and are especially prevalent in obese and/or diabetic populations. Epicardial adipose tissue (EAT) surrounding the heart has been implicated in the development of CVDs as EAT can shift from a protective to a maladaptive phenotype in diseased states. In diabetic and obese patients, an elevated EAT mass both secretes pro-fibrotic/pro-inflammatory adipokines and forms intramyocardial fibrofatty infiltrates. This narrative review considers the proposed pathophysiological roles of EAT in CVDs. Diabetes is associated with a disordered energy utilization in the heart, which promotes intramyocardial fat and structural remodeling. Fibrofatty infiltrates are associated with abnormal cardiomyocyte calcium handling and repolarization, increasing the probability of afterdepolarizations. The inflammatory phenotype also promotes lateralization of connexin (Cx) proteins, undermining unidirectional conduction. These changes are associated with conduction heterogeneity, together creating a substrate for atrial fibrillation (AF). EAT is also strongly implicated in coronary artery disease (CAD); inflammatory adipokines from peri-vascular fat can modulate intra-luminal homeostasis through an “outside-to-inside” mechanism. EAT is also a significant source of sympathetic neurotransmitters, which promote progressive diastolic dysfunction with eventual cardiac failure. Further investigations on the behavior of EAT in diabetic/obese patients with CVD could help elucidate the pathogenesis and uncover potential therapeutic targets. Full article
(This article belongs to the Section Acquired Cardiovascular Disease)
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17 pages, 1551 KiB  
Article
Pathogenic Variants in Cardiomyopathy Disorder Genes Underlie Pediatric Myocarditis—Further Impact of Heterozygous Immune Disorder Gene Variants?
by Franziska Seidel, Kai Thorsten Laser, Karin Klingel, Josephine Dartsch, Simon Theisen, Thomas Pickardt, Manuel Holtgrewe, Anna Gärtner, Felix Berger, Dieter Beule, Hendrik Milting, Stephan Schubert, Sabine Klaassen and Jirko Kühnisch
J. Cardiovasc. Dev. Dis. 2022, 9(7), 216; https://doi.org/10.3390/jcdd9070216 - 5 Jul 2022
Cited by 7 | Viewed by 2980
Abstract
Myocarditis is an inflammatory disease of the heart. Pediatric myocarditis with the dilated cardiomyopathy (DCM) phenotype may be caused by likely pathogenic or pathogenic genetic variants [(L)P] in cardiomyopathy (CMP) genes. Systematic analysis of immune disorder gene defects has not been performed so [...] Read more.
Myocarditis is an inflammatory disease of the heart. Pediatric myocarditis with the dilated cardiomyopathy (DCM) phenotype may be caused by likely pathogenic or pathogenic genetic variants [(L)P] in cardiomyopathy (CMP) genes. Systematic analysis of immune disorder gene defects has not been performed so far. We analyzed 12 patients with biopsy-proven myocarditis and the DCM phenotype together with their parents using whole-exome sequencing (WES). The WES data were filtered for rare pathogenic variants in CMP (n = 89) and immune disorder genes (n = 631). Twelve children with a median age of 2.9 (1.0–6.8) years had a mean left ventricular ejection fraction of 28% (22–32%) and myocarditis was confirmed by endomyocardial biopsy. Patients with primary immunodeficiency were excluded from the study. Four patients underwent implantation of a ventricular assist device and subsequent heart transplantation. Genetic analysis of the 12 families revealed an (L)P variant in the CMP gene in 8/12 index patients explaining DCM. Screening of recessive immune disorder genes identified a heterozygous (L)P variant in 3/12 index patients. This study supports the genetic impact of CMP genes for pediatric myocarditis with the DCM phenotype. Piloting the idea that additional immune-related genetic defects promote myocarditis suggests that the presence of heterozygous variants in these genes needs further investigation. Altered cilium function might play an additional role in inducing inflammation in the context of CMP. Full article
(This article belongs to the Special Issue Pediatric Cardiomyopathies: From Genotype to Phenotype)
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11 pages, 864 KiB  
Article
Peak Oxygen Uptake on Cardiopulmonary Exercise Test Is a Predictor for Severe Arrhythmic Events during Three-Year Follow-Up in Patients with Complex Congenital Heart Disease
by Felix von Sanden, Svetlana Ptushkina, Julia Hock, Celina Fritz, Jürgen Hörer, Gabriele Hessling, Peter Ewert, Alfred Hager and Cordula M. Wolf
J. Cardiovasc. Dev. Dis. 2022, 9(7), 215; https://doi.org/10.3390/jcdd9070215 - 4 Jul 2022
Cited by 1 | Viewed by 1999
Abstract
Patients with congenital heart disease (CHD) are at increased risk for severe arrhythmia and sudden cardiac death (SCD). Although implantable cardioverter defibrillators (ICD) effectively prevent SCD, risk stratification for primary prophylaxis in patients with CHD remains challenging. Patients with complex CHD undergoing CPET [...] Read more.
Patients with congenital heart disease (CHD) are at increased risk for severe arrhythmia and sudden cardiac death (SCD). Although implantable cardioverter defibrillators (ICD) effectively prevent SCD, risk stratification for primary prophylaxis in patients with CHD remains challenging. Patients with complex CHD undergoing CPET were included in this single-center study. Univariable and backwards stepwise multivariable logistic regression models were used to identify variables associated with the endpoint of severe arrhythmic event during three years of follow-up. Cut-off values were established with receiver operating characteristic (ROC) curve analysis. Survival analysis was conducted via Kaplan–Meier plots. Severe Arrhythmia was documented in 97 of 1194 patients (8.1%/3 years). Independent risk factors for severe arrhythmia during follow-up were old age and a low peak oxygen uptake (V.O2peak) on multivariable analysis. Patients with more advanced age and with V.O2peak values of less than 24.9 mL/min/kg were at significantly increased risk for the occurrence of severe arrhythmias during follow-up. The combined analysis of both risk factors yielded an additional benefit for risk assessment. Age at CPET and V.O2peak predict the risk for severe arrhythmic events and should be considered for risk stratification of SCD in patients with complex CHD. Full article
(This article belongs to the Special Issue Congenital Heart Defects: Diagnosis, Management, and Treatment)
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14 pages, 1723 KiB  
Article
Neonatal Deletion of Hand1 and Hand2 within Murine Cardiac Conduction System Reveals a Novel Role for HAND2 in Rhythm Homeostasis
by Rajani M. George, Shuai Guo, Beth A. Firulli, Michael Rubart and Anthony B. Firulli
J. Cardiovasc. Dev. Dis. 2022, 9(7), 214; https://doi.org/10.3390/jcdd9070214 - 4 Jul 2022
Cited by 4 | Viewed by 2143
Abstract
The cardiac conduction system, a network of specialized cells, is required for the functioning of the heart. The basic helix loop helix factors Hand1 and Hand2 are required for cardiac morphogenesis and have been implicated in cardiac conduction system development and maintenance. Here [...] Read more.
The cardiac conduction system, a network of specialized cells, is required for the functioning of the heart. The basic helix loop helix factors Hand1 and Hand2 are required for cardiac morphogenesis and have been implicated in cardiac conduction system development and maintenance. Here we use embryonic and post-natal specific Cre lines to interrogate the role of Hand1 and Hand2 in the function of the murine cardiac conduction system. Results demonstrate that loss of HAND1 in the post-natal conduction system does not result in any change in electrocardiogram parameters or within the ventricular conduction system as determined by optical voltage mapping. Deletion of Hand2 within the post-natal conduction system results in sex-dependent reduction in PR interval duration in these mice, suggesting a novel role for HAND2 in regulating the atrioventricular conduction. Surprisingly, results show that loss of both HAND factors within the post-natal conduction system does not cause any consistent changes in cardiac conduction system function. Deletion of Hand2 in the embryonic left ventricle results in inconsistent prolongation of PR interval and susceptibility to atrial arrhythmias. Thus, these results suggest a novel role for HAND2 in homeostasis of the murine cardiac conduction system and that HAND1 loss potentially rescues the shortened HAND2 PR phenotype. Full article
(This article belongs to the Section Basic and Translational Cardiovascular Research)
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12 pages, 1126 KiB  
Article
Altered Heart Rate Turbulence and Variability Parameters Predict 1-Year Mortality in Heart Failure with Preserved Ejection Fraction
by Jus Ksela, Lea Rupert, Anze Djordjevic, Miha Antonic, Viktor Avbelj and Borut Jug
J. Cardiovasc. Dev. Dis. 2022, 9(7), 213; https://doi.org/10.3390/jcdd9070213 - 2 Jul 2022
Cited by 7 | Viewed by 2501
Abstract
Background: Heart failure with preserved ejection fraction (HFpEF) is a complex and heterogeneous clinical syndrome. In the absence of effective and potent treatment strategies, the main challenge in HFpEF management remains the availability of strong predictors of unfavourable outcomes. In our study, we [...] Read more.
Background: Heart failure with preserved ejection fraction (HFpEF) is a complex and heterogeneous clinical syndrome. In the absence of effective and potent treatment strategies, the main challenge in HFpEF management remains the availability of strong predictors of unfavourable outcomes. In our study, we sought to evaluate the potential prognostic value of heart rate turbulence (HRT) and variability (HRV) parameters on mortality in ambulatory HFpEF patients. Methods: This was a case–control study comparing HRT and HRV parameters in HFpEF survivors vs. non-survivors. Patients from the RESPOND Heart Failure Registry with HFpEF who underwent 24 h ECG monitoring (Holter) were included; HRT parameters (i.e., turbulence onset (TO) and turbulence slope (TS)) and HRV parameters (i.e., standard deviation of NN intervals (SDNN)) derived from 24 h Holter ECGs were calculated in patients who died within 12 months, and compared to their age-, gender-, LVEF-, ECHO-, aetiology-, and therapy-matched alive controls. Results: A total of 22 patients (mean age 80 ± 7 years, 18% female, mean LVEF 57 ± 9%) were included in the final analysis. In deceased patients, values of TO were significantly higher, and values of TS and SDNN were significantly lower as compared to survivors. Conclusions: HRT and HRV parameters have the ability to differentiate individuals with HFpEF who are at the greatest risk of unfavourable outcomes. The extent of autonomic disbalance as determined by HRT and HRV could potentially assist in the prognostic assessment and risk stratification of HFpEF patients. Full article
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9 pages, 1780 KiB  
Article
A Comparative Study on Surgical Treatment of Valvular Heart Disease between High-Volume Cardiac Centers in China and STS Data
by Hongyuan Lin, Jiamiao Gong, Yongjian Wu, Zhe Zheng and Jianfeng Hou
J. Cardiovasc. Dev. Dis. 2022, 9(7), 212; https://doi.org/10.3390/jcdd9070212 - 2 Jul 2022
Cited by 5 | Viewed by 2330
Abstract
The goal of this study is to summarize valvular surgery data from the Chinese Cardiac Surgery Registry (CCSR) and compare it to the most recent data from the Society of Thoracic Surgeons (STS). From 2016 to 2018, a total of 34,386 cases of [...] Read more.
The goal of this study is to summarize valvular surgery data from the Chinese Cardiac Surgery Registry (CCSR) and compare it to the most recent data from the Society of Thoracic Surgeons (STS). From 2016 to 2018, a total of 34,386 cases of the seven most common valvular surgical procedures was obtained from the CCSR. We calculated the proportions of different procedures in the CCSR cohort (n = 34,386) as well as the change in operation volume for each procedure. We also compiled rates of postoperative in-hospital mortality and five major complications across all procedures. All of the results were compared to the STS data. The CCSR and STS data showed divergent trends in valvular heart disease features and operation volume. Although the proportion of MV repair in the CCSR (13.7%) data was lower than in the STS data (23.2%), it demonstrated a substantial upward trend. In terms of operation volume, the CCSR data showed an upward trend, but the STS data showed a downward trend. CCSR procedures showed lower mortality (2% vs. 2.6%), reoperation (2.8% vs. 4.3%), and permanent stroke (0.5% vs. 1.6%) rates than STS procedures but higher rates of prolonged ventilation (22.4% vs. 10.4%) and renal failure (5.6% vs. 3.2%). Valvular surgery quality in China’s leading cardiac hospitals is roughly comparable to that in the United States. China, on the other hand, has some shortcomings that need improvement. Full article
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12 pages, 2014 KiB  
Article
Gene Expression Profiling of Markers of Inflammation, Angiogenesis, Coagulation and Fibrinolysis in Patients with Coronary Artery Disease with Very High Lipoprotein(a) Levels Treated with PCSK9 Inhibitors
by Katja Hrovat, Andreja Rehberger Likozar, Janja Zupan and Miran Šebeštjen
J. Cardiovasc. Dev. Dis. 2022, 9(7), 211; https://doi.org/10.3390/jcdd9070211 - 1 Jul 2022
Cited by 8 | Viewed by 2601
Abstract
Besides lipids, inflammation, angiogenesis, coagulation and fibrinolysis play very important roles in coronary artery disease (CAD). We measured gene expression of the inflammatory markers interleukin (IL)-1β (IL1B) and interferon (IFN)-γ (IFNG), vascular endothelial growth factor-A (VEGF-A) (VEGFA), [...] Read more.
Besides lipids, inflammation, angiogenesis, coagulation and fibrinolysis play very important roles in coronary artery disease (CAD). We measured gene expression of the inflammatory markers interleukin (IL)-1β (IL1B) and interferon (IFN)-γ (IFNG), vascular endothelial growth factor-A (VEGF-A) (VEGFA), and coagulation and fibrinolysis markers tissue factor (TF) (F3) and plasminogen activator inhibitor-1 (PAI-1) (SERPINE) in healthy controls and CAD patients with high lipoprotein(a) (Lp(a)). The aim of our study was to identify, first, if there is a difference in these markers between controls and patients; secondly, if these markers are associated with lipids; and third, what the influence of proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors is on these markers. We included 124 subjects, 27 controls and 97 patients with CAD (30 in placebo and 67 in the PCSK9 group). Blood samples were collected for lipid and gene measurement. The results showed higher expression of IL1B (p < 0.0001), VEGFA (p < 0.0001), and F3 (p = 0.018) in controls in comparison with patients. Significant correlations were observed between IL1B and lipids. Treatment with PCSK9 inhibitors increased VEGFA (p < 0.0001) and F3 (p = 0.001), and decreased SERPINE (p = 0.043). The results of our study underpin the importance of IL-1β, VEGF-A and TF in CAD as well as the effect of PCSK9 treatment on these markers. Full article
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10 pages, 799 KiB  
Article
Enhanced Detection of Cardiac Surgery-Associated Acute Kidney Injury by a Composite Biomarker Panel in Patients with Normal Preoperative Kidney Function
by Jurij Matija Kalisnik, Klemen Steblovnik, Eva Hrovat, Ales Jerin, Milan Skitek, Christian Dinges, Theodor Fischlein and Janez Zibert
J. Cardiovasc. Dev. Dis. 2022, 9(7), 210; https://doi.org/10.3390/jcdd9070210 - 1 Jul 2022
Cited by 4 | Viewed by 2199
Abstract
We have recently shown that minor subclinical creatinine dynamic changes enable the excellent detection of acute kidney injury (AKI) within 6–12 h after cardiac surgery. The aim of the present study was to examine a combination of neutrophil gelatinase-associated lipocalin (NGAL), cystatin C [...] Read more.
We have recently shown that minor subclinical creatinine dynamic changes enable the excellent detection of acute kidney injury (AKI) within 6–12 h after cardiac surgery. The aim of the present study was to examine a combination of neutrophil gelatinase-associated lipocalin (NGAL), cystatin C (CysC) and creatinine for enhanced AKI detection early after cardiac surgery. Elective patients with normal renal function undergoing cardiac surgery using cardiopulmonary bypass were enrolled. Concentrations of plasma NGAL, serum CysC and serum creatinine were determined after the induction of general anesthesia, at the termination of the cardiopulmonary bypass and 2 h thereafter. Out of 119 enrolled patients, 51 (43%) developed AKI. A model utilizing an NGAL, CysC and creatinine triple biomarker panel including sequential relative changes provides a better prediction of cardiac surgery-associated acute kidney injury than any biomarker alone already 2 h after the termination of the cardiopulmonary bypass. The area under the receiver-operator curve was 0.77, sensitivity 77% and specificity 68%. Full article
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13 pages, 2385 KiB  
Article
Biventricular versus Conduction System Pacing after Atrioventricular Node Ablation in Heart Failure Patients with Atrial Fibrillation
by Maja Ivanovski, Miha Mrak, Anja Zupan Mežnar and David Žižek
J. Cardiovasc. Dev. Dis. 2022, 9(7), 209; https://doi.org/10.3390/jcdd9070209 - 1 Jul 2022
Cited by 18 | Viewed by 3866
Abstract
Conduction system pacing (CSP) modalities, including His-bundle pacing (HBP) and left bundle branch pacing (LBBP), are increasingly used as alternatives to biventricular (BiV) pacing in heart failure (HF) patients scheduled for pace and ablate strategy. The aim of the study was to compare [...] Read more.
Conduction system pacing (CSP) modalities, including His-bundle pacing (HBP) and left bundle branch pacing (LBBP), are increasingly used as alternatives to biventricular (BiV) pacing in heart failure (HF) patients scheduled for pace and ablate strategy. The aim of the study was to compare clinical outcomes of HF patients with refractory AF who received either BiV pacing or CSP in conjunction with atrio-ventricular node ablation (AVNA). Fifty consecutive patients (male 48%, age 70 years (IQR 9), left ventricular ejection fraction (LVEF) 39% (IQR 12)) were retrospectively analysed. Thirteen patients (26%) received BiV pacing, 27 patients (54%) HBP and 10 patients (20%) LBBP. All groups had similar baseline characteristics and acute success rate. While New York Heart. Association (NYHA) class improved in both HBP (p < 0.001) and LBBP (p = 0.008), it did not improve in BiV group (p = 0.096). At follow-up, LVEF increased in HBP (form 39% (IQR 15) to 49% (IQR 16), p < 0.001) and LBBP (from 28% (IQR 13) to 40% (IQR 13), p = 0.041), but did not change in BiV group (p = 0.916). Conduction system pacing modalities showed superior symptomatic and echocardiographic improvement compared to BiV pacing after AVNA. With more stable pacing parameters, LBBP could present a more feasible pacing option compared to HBP. Full article
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4 pages, 663 KiB  
Case Report
Autopsy and Cardiac Magnetic Resonance Image Case of Bevacizumab-Related Cardiomyopathy
by Naoki Hashimoto, Daisuke Kitano, Takehiro Tamaki, Yutaka Koyama, Akimasa Yamada, Kinta Hatakeyama, Hiroyuki Hao and Yasuo Okumura
J. Cardiovasc. Dev. Dis. 2022, 9(7), 208; https://doi.org/10.3390/jcdd9070208 - 1 Jul 2022
Cited by 2 | Viewed by 2027
Abstract
We report an autopsy case of a 69-year-old female with cervical cancer. She was given bevacizumab-containing chemotherapy for 4 months. After two years of chemotherapy, she developed congestive heart failure (CHF) with left ventricular dysfunction. Cardiac magnetic resonance (CMR) imaging revealed late gadolinium [...] Read more.
We report an autopsy case of a 69-year-old female with cervical cancer. She was given bevacizumab-containing chemotherapy for 4 months. After two years of chemotherapy, she developed congestive heart failure (CHF) with left ventricular dysfunction. Cardiac magnetic resonance (CMR) imaging revealed late gadolinium enhancement (LGE) of linear mid-wall delayed enhancement located in the basal to the mid-septal wall, suggesting bevacizumab-related cardiotoxicity. Although she was treated with cardioprotective medications and discharged, she eventually died from worsening CHF a year later, and we conducted an autopsy. Histopathological examination revealed diffuse fibrosis in the myocardium, and the area where LGE was present on CMR showed thinning and wavy changes in cardiomyocytes with diffuse interstitial fibrosis and edema. Full article
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13 pages, 882 KiB  
Article
Clinical Outcomes after Endovascular Revascularisation of the Femoropopliteal Arterial Segment in Patients with Anticoagulant versus Antiplatelet Therapy: A Single-Centre Retrospective Cohort Study
by Kevin Pelicon, Klemen Petek, Anja Boc, Vinko Boc, Nataša Kejžar, Tjaša Vižintin Cuderman and Aleš Blinc
J. Cardiovasc. Dev. Dis. 2022, 9(7), 207; https://doi.org/10.3390/jcdd9070207 - 30 Jun 2022
Cited by 2 | Viewed by 2582
Abstract
To prevent atherothrombotic events, patients with peripheral arterial disease are typically prescribed antiplatelet therapy (APT). However, some of them receive anticoagulant therapy (ACT) due to comorbidities. Our aim was to determine possible differences in the effectiveness and safety of both treatments in patients [...] Read more.
To prevent atherothrombotic events, patients with peripheral arterial disease are typically prescribed antiplatelet therapy (APT). However, some of them receive anticoagulant therapy (ACT) due to comorbidities. Our aim was to determine possible differences in the effectiveness and safety of both treatments in patients after endovascular femoropopliteal revascularisation. We retrospectively analysed 1247 patients after successful femoropopliteal revascularisation performed in a single tertiary medical centre and classified them into the ACT or APT group, based on their prescribed treatment. The groups were characterised by descriptive statistics, and their characteristics were adjusted for confounders by propensity score matching. Effectiveness and safety outcomes were assessed within one year after revascularisation. The odds ratio for the composite outcome of all-cause death, PAD exacerbation, and major amputation due to vascular causes with ACT versus APT was 1.21 (95% CI 0.53–2.21; p = 0.484). The odds ratio for major bleeding as defined by the International Society on Thrombosis and Haemostasis with ACT versus APT was 0.77 (95% CI 0.13–3.84; p = 0.251). We found no statistically significant difference in the effectiveness and safety of ACT, when compared to APT in patients with similar cardiovascular risk factors and other baseline characteristics. Further prospective research is warranted. Full article
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14 pages, 896 KiB  
Review
Left Ventricular Noncompaction in Children: The Role of Genetics, Morphology, and Function for Outcome
by Sabine Klaassen, Jirko Kühnisch, Alina Schultze-Berndt and Franziska Seidel
J. Cardiovasc. Dev. Dis. 2022, 9(7), 206; https://doi.org/10.3390/jcdd9070206 - 30 Jun 2022
Cited by 2 | Viewed by 2995
Abstract
Left ventricular noncompaction (LVNC) is a ventricular wall anomaly morphologically characterized by numerous, excessively prominent trabeculations and deep intertrabecular recesses. Accumulating data now suggest that LVNC is a distinct phenotype but must not constitute a pathological phenotype. Some individuals fulfill the morphologic criteria [...] Read more.
Left ventricular noncompaction (LVNC) is a ventricular wall anomaly morphologically characterized by numerous, excessively prominent trabeculations and deep intertrabecular recesses. Accumulating data now suggest that LVNC is a distinct phenotype but must not constitute a pathological phenotype. Some individuals fulfill the morphologic criteria of LVNC and are without clinical manifestations. Most importantly, morphologic criteria for LVNC are insufficient to diagnose patients with an associated cardiomyopathy (CMP). Genetic testing has become relevant to establish a diagnosis associated with CMP, congenital heart disease, neuromuscular disease, inborn error of metabolism, or syndromic disorder. Genetic factors play a more decisive role in children than in adults and severe courses of LVNC tend to occur in childhood. We reviewed the current literature and highlight the difficulties in establishing the correct diagnosis for children with LVNC. Novel insights show that the interplay of genetics, morphology, and function determine the outcome in pediatric LVNC. Full article
(This article belongs to the Special Issue Pediatric Cardiomyopathies: From Genotype to Phenotype)
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8 pages, 470 KiB  
Article
Biological Variation in Rotational Thromboelastometry in Patients with Atrial Fibrillation Receiving Rivaroxaban
by Mojca Božič Mijovski, Jovan P. Antovic, Rickard E. Malmström and Alenka Mavri
J. Cardiovasc. Dev. Dis. 2022, 9(7), 205; https://doi.org/10.3390/jcdd9070205 - 29 Jun 2022
Cited by 1 | Viewed by 1624
Abstract
Rotational thromboelastometry (ROTEM) is a viscoelastic hemostasis test used primarily in the management of bleeding after trauma or in cardiac surgery. To allow safe and valid clinical interpretation of test results, objective specifications for analytical performance are needed, which are generally based on [...] Read more.
Rotational thromboelastometry (ROTEM) is a viscoelastic hemostasis test used primarily in the management of bleeding after trauma or in cardiac surgery. To allow safe and valid clinical interpretation of test results, objective specifications for analytical performance are needed, which are generally based on biological variation within (CVI) and between (CVG) individuals. The aim of this study was to evaluate biological variation in ROTEM in patients receiving rivaroxaban. Sixty patients with atrial fibrillation on stable rivaroxaban therapy were included, from whom blood was collected on six occasions: three times at trough and three at peak rivaroxaban concentrations. ROTEM® Extem and LowTF were measured as well as rivaroxaban concentration, PT, APTT, and anti-Xa. Within- (CVI) and between-subject (CVG) biological estimates were calculated. Knowledge of these biological variation components will help to establish the appropriate objective analytical performance specifications for ROTEM analysis. Full article
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11 pages, 756 KiB  
Article
Clinical Characteristics and Prognosis of Patients with Multi-Vessel Coronary Spasm in Comparison with Those in Patients with Single-Vessel Coronary Spasm
by Hiroki Teragawa, Chikage Oshita and Yuko Uchimura
J. Cardiovasc. Dev. Dis. 2022, 9(7), 204; https://doi.org/10.3390/jcdd9070204 - 28 Jun 2022
Cited by 10 | Viewed by 1848
Abstract
(1) Background: We have sometimes experienced patients with vasospastic angina (VSA) who presented multi-vessel spasm (MVS) on coronary angiography and spasm provocation test (SPT). However, the clinical characteristics of VSA patients with MVS and the prognosis of such patients in the clinical setting [...] Read more.
(1) Background: We have sometimes experienced patients with vasospastic angina (VSA) who presented multi-vessel spasm (MVS) on coronary angiography and spasm provocation test (SPT). However, the clinical characteristics of VSA patients with MVS and the prognosis of such patients in the clinical setting have not been clarified. Therefore, we compared the clinical characteristics and prognosis in VSA patients with MVS with those in VSA patients with single-vessel spasm (SVS). (2) Methods: A total of 152 patients (mean age, 67 years, 74 men and 78 women) with VSA, in which the presence of coronary spasm was assessed in both left coronary artery (LCA) and right coronary artery (RCA) on SPT, were enrolled. We defined VSA as the presence of >90% narrowing of the epicardial coronary artery on angiograms, accompanied by usual chest symptoms and/or ischaemic ST-T changes on the electrocardiogram. On SPT, MVS was defined as the presence of spasms on ≥2 major coronary arteries. Based on the presence of MVS, patients were divided into the MVS group and the SVS group. The frequencies of conventional coronary risk factors, blood chemical parameters, average times of anginal attack, SPT findings such as spasm provocation induced by a low dose of acetylcholine (L-ACh) and total occlusion due to coronary spasm (TOC), number of coronary vasodilators at discharge and major cardiovascular events (MACE, including cardiac death and readmission due to any cause of cardiovascular diseases) were compared between the two groups. (3) Results: The MVS and SVS groups were comprised of 98 (64%) and 54 (36%) patients, respectively. The level of fasting blood glucose (FBS) was lower (p < 0.01), and the level of cystatin-C (n = 89) tended to be higher (p = 0.07) in the MVS group than in the SVS group. The frequencies of L-ACh-induced coronary spasm (33% in MVS and 17% in SVS, p = 0.04) and TOC (12% in MVS, 0% in SVS, p < 0.01) were higher in the MVS group than in the SVS group. The average number of coronary vasodilators at discharge was higher in the MVS group (1.2 ± 0.4) than in the SVS group (0.9 ± 0.5, p < 0.01). The frequency of MACE was not different between the two groups. (4) Conclusions: Patients with MVS may have higher VSA activity on SPT and have more aggressive medications, leading to a comparable prognosis in VSA patients with SVS. MVS is an important indicator of at least VSA activity, and cardiologists should confirm this in SPT whenever possible. Further studies should confirm whether lower FBS levels and higher cystatin-C levels are any markers of MVS. Full article
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15 pages, 1070 KiB  
Review
ICIs-Related Cardiotoxicity in Different Types of Cancer
by Mei Dong, Ting Yu, Zhenzhen Zhang, Jing Zhang, Rujian Wang, Gary Tse, Tong Liu and Lin Zhong
J. Cardiovasc. Dev. Dis. 2022, 9(7), 203; https://doi.org/10.3390/jcdd9070203 - 28 Jun 2022
Cited by 18 | Viewed by 3249
Abstract
Immune checkpoint inhibitors (ICIs) are rapidly developing immunotherapy cancer drugs that have prolonged patient survival. However, ICIs-related cardiotoxicity has been recognized as a rare, but fatal, consequence. Although there has been extensive research based on different types of ICIs, these studies have not [...] Read more.
Immune checkpoint inhibitors (ICIs) are rapidly developing immunotherapy cancer drugs that have prolonged patient survival. However, ICIs-related cardiotoxicity has been recognized as a rare, but fatal, consequence. Although there has been extensive research based on different types of ICIs, these studies have not indicated whether cardiotoxicity is specific to a type of cancer. Therefore, we conducted a systematic review to analyze a variety of ICIs-related cardiotoxicity, focusing on different types of cancer. We found that the incidence of ICIs-related cardiac adverse events (CAEs) and common cardiotoxic manifestations vary with cancer type. This inspired us to explore the underlying mechanisms to formulate targeted clinical strategies for maintaining the cardiovascular health of cancer patients. Full article
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11 pages, 287 KiB  
Article
Medication Adherence and Belief about Medication among Vietnamese Patients with Chronic Cardiovascular Diseases within the Context of Implementing Measures to Prevent COVID-19
by Nguyet Kim Nguyen, Han Gia Diep, Hung Huynh Vinh Ly, Ngoc Le Minh Nguyen, Katja Taxis, Suol Thanh Pham, Trang Huynh Vo and Thang Nguyen
J. Cardiovasc. Dev. Dis. 2022, 9(7), 202; https://doi.org/10.3390/jcdd9070202 - 26 Jun 2022
Cited by 1 | Viewed by 2260
Abstract
Background: Long-term adherence is crucial for optimal treatment outcomes in chronic cardiovascular diseases (CVDs), especially throughout the COVID-19 wide-spreading periods, making patients with chronic CVDs vulnerable subjects. Aim: To investigate the relationship between the characteristics, beliefs about prescribed medication, COVID-19 prevention measures, and [...] Read more.
Background: Long-term adherence is crucial for optimal treatment outcomes in chronic cardiovascular diseases (CVDs), especially throughout the COVID-19 wide-spreading periods, making patients with chronic CVDs vulnerable subjects. Aim: To investigate the relationship between the characteristics, beliefs about prescribed medication, COVID-19 prevention measures, and medication adherence among patients with chronic CVDs. Methods: This is a cross-sectional study of outpatients with chronic CVDs in Southern Vietnam. The specific parts regarding the Beliefs about Medicines Questionnaires (BMQ—Specific) and the General Medication Adherence Scale (GMAS) were applied to assess the beliefs about and adherence to medication. The implementation measures to prevent COVID-19 in patients were evaluated according to the 5K message (facemask, disinfection, distance, no gathering, and health declaration) of the Vietnam Ministry of Health. A multivariable logistic regression with the Backward elimination (Wald) method was used to identify the associated factors of medication adherence. Results: A slightly higher score in BMQ-Necessity compared to BMQ-Concerns was observed. A total of 40.7% of patients were recorded as having not adhered to their medications. Patients’ behavior was most frequently self-reported by explaining their non-adherence (34.7%). Statistical associations were found between rural living place, unemployment status, no or only one measure(s) of COVID-19 prevention application, and medication adherence. Conclusion: During the COVID-19 spreading stage, patients generally showed a positive belief about medication when they rated the importance of taking it higher than its side effects. The data analysis suggested that rather than patients’ beliefs, the clinicians should consider the patient factors, including living place, employment, and the number of epidemic preventive measures applied for guiding the target patients for improving medication adherence. Full article
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