J. Cardiovasc. Dev. Dis., Volume 10, Issue 1 (January 2023) – 32 articles

Background: Hemodynamic instability (HDI) is common during liver transplantation (LT); veno-venous bypass (VVB) is a tool used in selected cases to ensure hemodynamic stability and for surgical needs. Transesophageal echocardiography (TEE) allows the transplant team to identify the causes of HDI and to guide therapies. We present a case series of four patients showing the valuable role of TEE during LT in VVB. Methods: We report four explicative cases of TEE use in LT with VVB performed at IRCCS Azienda Ospedaliero–Universitaria di Bologna. Four transplants were performed between 2016 and 2022. Results: Many authors have highlighted the diagnostic value of TEE during LT in the case of HDI. However, its specific role during LT with VVB is poorly described. This paper illustrates multiple potential uses of TEE in LT with VVB: TEE as a guide for catheterization and optimal cannula positioning, TEE as a tool for intraoperative Patent Foramen Ovale management, TEE as help for anticoagulation therapy and finally, TEE as support when evaluating bypass efficiency and correcting hypovolemia. Conclusion: TEE is a useful instrument during LT with VVB. However, further studies are needed to assess the suitable applications of TEE during LT in patients with HDI requiring VVB. TEE should be part of the anesthetist’s cultural background. Full article

Pulmonary stenosis (PS) affects cardiopulmonary function and exercise performance. Cardiopulmonary exercise testing (CPET) together with transthoracic echocardiography (TTE) can measure exercise performance, PS progression, and treatment effects. We assessed exercise capacity in PS patients using these methods. We enrolled 28 PS patients aged 6–35 years who received surgery, balloon pulmonary valvuloplasty, and follow-up care. The control population was selected by a 1:1 matching on age, sex, and body mass index. Baseline and follow-up peak pulmonary artery pulse wave velocity (PAV) were compared using TTE. Initial CPET revealed no significant differences in anaerobic metabolic equivalent (MET), peak oxygen consumption (VO₂), and heart rate recovery between the two groups, nor were significant differences in pulmonary function identified. Within the PS group, there were no significant differences in MET, peak VO₂, and heart rate recovery between the baseline and final CPET. Similarly, no significant differences were observed between the baseline and final PAV. The exercise capacity of patients with properly managed PS was comparable to that of healthy individuals. However, during the follow-up, declining trends in pulmonary function, aerobic metabolism, and peak exercise load capacity were observed among adolescents with PS. This study provides long-term data suggesting that PS patients should be encouraged to perform physical activity. Regular reevaluation should also be encouraged to limit performance deterioration. Full article

Due to distressing statistics concerning cardiovascular diseases, remote monitoring of cardiac implantable electronic devices (CIED) has received a priority recommendation in daily patient care. However, most bedside systems available so far are not optimal due to limited patient adherence. We report that smartphone app technology communicating with CIED improved the patient’s engagement and adherence, as well as the accuracy of atrial and ventricular arrhythmias diagnosis, thus offering more efficient treatment and, consequently, better patient clinical outcomes. Our findings are in concordance with previously published results for implantable loop recorders and pacemakers, and provide new insight for heart failure patients with an implanted cardiac resynchronization therapy defibrillator. Full article

Background: The incidence of acute myocardial infarction (AMI) in the younger population has been increasing gradually in recent years. The objective of the present study is to investigate the safety and effectiveness of drug-eluting balloons (DEBs) in young patients with AMI. Methods: All consecutive patients with AMI aged ≤ 45 years were retrospectively enrolled. The primary endpoint was a device-oriented composite endpoint (DOCE) of cardiac death, target vessel myocardial infarction (MI), or target lesion revascularization (TLR). The secondary study endpoints included heart failure and major bleeding events. Results: A total of 276 young patients presenting with AMI were finally included. The median follow-up period was 1155 days. Patients treated with DEBs had a trend toward a lower incidence of DOCEs (3.0% vs. 11.0%, p = 0.12) mainly driven by the need for TLR (3.0% vs. 9.1%, p = 0.19) than those treated with DESs. No significant differences between the two groups were detected in the occurrence of cardiac death (0.0% vs. 0.5%, p = 0.69), MI (0.0% vs. 1.4%, p = 0.40), heart failure (0.0% vs. 1.9%, p = 0.39), or major bleeding events (1.5% vs 4.8%, p = 0.30). Multivariate regression analysis showed that DEBs were associated with a trend toward a lower risk of DOCEs (HR 0.13, 95% CI [0.02, 1.05], p = 0.06). Conclusions: The findings of the present study suggested that DEBs might be a potential treatment option in young patients with AMI. A larger scale, randomized, multicenter study is required to investigate the safety and effectiveness of DEBs in this setting. Full article

Background: For mitral valve surgery (MVS) in elderly, frail patients with increasing life expectancy, finding the least harmful means of access is a challenge. In the complexity of MVS approach evolution, using three different approaches (mini-thoracotomy (MT), partial upper-sternotomy (PS), full-sternotomy (FS), we developed a personalized, minimized-invasiveness algorithm for MVS. Methods: In this retrospective analysis, 517 elderly patients (≥70 years) were identified who had undergone MVS ± TV repair. MVS was performed via MT (n = 274), FS (n = 128) and PS (n = 115). The appropriate access type was defined according to several clinical patient conditions. Using uni- and multivariate regression models, we analyzed combined operative success (residual MV regurgitation, conversion to MV replacement or larger thoracic incisions); perioperative success (30-days mortality, thoracotomy, ECMO, pacemaker implantation, dialysis, longer ventilation); and reoperation-free long-term survival. An additional EuroSCORE2 adjustment was performed to reduce the bias of clinical conditions between all access types. Results: The EuroSCORE2-adjusted Cox regression analysis showed significantly increased reoperation-free survival in the MT cohort compared to FS (HR 0.640; 95% CI 0.442–0.926; p = 0.018). Mortality was additionally reduced after the implementation of PS (p = 0.023). Combined operative success was comparable between the three access types. The perioperative success was higher in the MT cohort compared to FS (OR 2.19, 95% CI 1.32–3.63; p = 0.002). Conclusion: Less-invasive approaches in elderly patients improve perioperative success and reoperation-free survival in those undergoing MVS procedures. Full article

Increased mechanical forces on developing cardiac valves drive formation of the highly organized extracellular matrix (ECM) providing tissue integrity and promoting cell behavior and signaling. However, the ability to investigate the response of cardiac valve cells to increased mechanical forces is challenging and remains poorly understood. The developmental window from birth (P0) to postnatal day 7 (P7) when biomechanical forces on the pulmonary valve (PV) are altered due to the initiation of blood flow to the lungs was evaluated in this study. Grossly enlarged PV, in mice deficient in the proteoglycan protease ADAMTS5, exhibited a transient phenotypic rescue from postnatal day 0 (P0) to P7; the Adamts5^−/− aortic valves (AV) did not exhibit a phenotypic correction. We hypothesized that blood flow, initiated to the lungs at birth, alters mechanical load on the PV and promotes ECM maturation. In the Adamts5^−/− PV, there was an increase in localization of the proteoglycan proteases ADAMTS1, MMP2, and MMP9 that correlated with reduced Versican (VCAN). At birth, Decorin (DCN), a Collagen I binding, small leucine-rich proteoglycan, exhibited complementary stratified localization to VCAN in the wild type at P0 but colocalized with VCAN in Adamts5^−/− PV; concomitant with the phenotypic rescue at P7, the PVs in Adamts5^−/− mice exhibited stratification of VCAN and DCN similar to wild type. This study indicates that increased mechanical forces on the PV at birth may activate ECM proteases to organize specialized ECM layers during cardiac valve maturation. Full article

Both particular myocardial locations in the human heart and the canonical transient receptor potential 6 (TRPC6) cation channel have been linked with cardiac pathophysiologies. Thus, the present study mapped TRPC6-protein distribution in select anatomic locations associated with cardiac disease in the context of an orienting pathological assessment. Specimens were obtained from 5 body donors (4 formalin fixation, 1 nitrite pickling salt-ethanol-polyethylene glycol (NEP) fixation; median age 81 years; 2 females) and procured for basic histological stains and TRPC6-immunohistochemistry. The latter was analyzed descriptively regarding distribution and intensity of positive signals. The percentage of positively labelled myocardium was also determined (optical threshold method). Exclusively exploratory statistical analyses were performed. TRPC6-protein was distributed widespread and homogenously within each analyzed sample. TRPC6-immunoreactive myocardial area was comparable regarding the different anatomic regions and sex. A significantly larger area of TRPC6-immunoreactive myocardium was found in the NEP-fixed donor compared to the formalin fixed donors. Two donors with more severe heart disease showed smaller areas of myocardial TRPC6-immunoreactivity overall compared to the other 3 donors. In summary, in the elderly, TRPC6-protein is widely and homogenously distributed, and severe cardiac disease might be associated with less TRPC6-immunoreactive myocardial area. The tissue fixation method represents a potential confounder. Full article

Given the high global disease burden of coronary artery disease (CAD), a major problem facing healthcare economic policy is identifying the most cost-effective diagnostic strategy for patients with suspected CAD. The aim of this review is to assess the long-term cost-effectiveness of coronary computed tomography angiography (CCTA) when compared with other diagnostic modalities and to define the cost and effective diagnostic utilization of computed tomography-fractional flow reserve (CT-FFR). A search was conducted through the MEDLINE database using PubMed with 16 of 119 manuscripts fitting the inclusion and exclusion criteria for review. An analysis of the data included in this review suggests that CCTA is a cost-effective strategy for both low risk acute chest pain patients presenting to the emergency department (ED) and low-to-intermediate risk stable chest pain outpatients. For patients with intermediate-to-high risk, CT-FFR is superior to CCTA in identifying clinically significant stenosis. In low-to-intermediate risk patients, CCTA provides a cost-effective diagnostic strategy with the potential to reduce economic burden and improve long-term health outcomes. CT-FFR should be utilized in intermediate-to-high risk patients with stenosis of uncertain clinical significance. Long-term analysis of cost-effectiveness and diagnostic utility is needed to determine the optimal balance between the cost-effectiveness and diagnostic utility of CT-FFR. Full article

Pulmonary regurgitation (PR) is a risk factor for sudden cardiac death in adult patients with repaired tetralogy of Fallot (TOF). However, transthoracic echocardiography (TTE) cannot fully visualize the pulmonary valve (PV) and PR. We investigated whether intracardiac echocardiography (ICE) could visualize the PV and PR better than TTE. Thirty adult patients with TOF (mean age 33 ± 15 years) scheduled for cardiac catheterization underwent ICE. The visualization of PV and the severity of PR were classified into three grades. ICE depicted the PV better than TTE (ICE vs. TTE: not visualized, partially visualized, and fully visualized: n = 1 [3%], n = 13 [43%], and n = 16 [53%] vs. n = 14 [47%], n = 13 [43%], and n = 3 [10%], p < 0.001). Especially in patients after pulmonary valve replacement (PVR), the PV was more fully visualized by ICE. The assessment of PR by TTE underestimated the severity of PR in comparison to cardiac magnetic resonance imaging (MRI) (severe PR: 8 [28%] vs. 22 [76%], p = 0.004), while there was no discrepancy between the results of ICE and MRI (21 [72%] vs. 22 [76%], p = 1.000). In comparison to TTE, ICE can safely provide better visualization of the PV and PR in adults with TOF, especially in patients who have undergone PVR. Full article

Aim: COVID-19 pandemic had a big impact on our life, it has revolutionized the practice of cardiology and the organization of hospital and outpatient activities. Thus the aim of our study was to assess the impact of the COVID-19 pandemic on the development of cancer therapy-related cardiac dysfunction (CTRCD). Methods and results: A single center retrospective study was carried out evaluating 96 cancer patients treated with anthracyclines and admitted to our Cardio-Oncology unit from June to August 2019 and 60 patients from June to August 2021. The incidence of CTRCD was assessed performing an echocardiogram at the time of the enrollment. We found a significantly higher incidence of CTRCD in the second period compared to first period (13% vs. 2%, p value 0.0058). In addition we found that fewer yearly visits were performed in our Cardio-oncology unit in 2021 compared to 2019 (300 patients/year in 2019 vs. 144 patients/year in the COVID era). Conclusion: COVID-19 pandemic seems to influence the onset of CTRCD in cancer patients by indirectly reducing hospital access of cancer patients and cardiological checks. In addition our data reflect the impact of the COVID-19 pandemic in the late diagnosis of cancer, in the reduction of hospital admissions and regular medical checks, in the increase of comorbidities and cardiovascular complications. Full article

(1) Background: Currently, echocardiography is the primary non-invasive diagnostic method used to screen patients with severe aortic valve stenosis (AS) for pulmonary hypertension (PH) by estimating systolic pulmonary artery pressure (sPAP). Other radiological methods have been a focus of research in the past couple of years, as it was shown that by determining the pulmonary artery (PA) diameter, prognostic statements concerning overall mortality could be made in these patients. This study compared established and novel cardiovascular biomarkers with the PA/BSA value to detect PH in patients with severe AS. (2) Methods: The study cohort comprised 188 patients with severe AS undergoing transcatheter aortic valve replacement (TAVR), who were then divided into two groups based on PA/BSA values obtained through CT-angiography. The presence of PH was defined as a PA/BSA ≥ 16.6 mm/m² (n = 81), and absence as a PA/BSA < 16.6 mm/m² (n = 107). Blood samples were taken before TAVR to assess cardiovascular biomarkers used in this study, namely brain natriuretic peptide (BNP), cardiac troponin I (cTnI), high-sensitive troponin (hsTN), soluble suppression of tumorigenesis-2 (sST2), growth/differentiation factor 15 (GDF-15), heart-type fatty acid-binding protein (H-FABP), insulin-like growth factor binding protein 2 (IGF-BP2), and soluble urokinase-type plasminogen activator receptor (suPAR). (3) Results: Patients with a PA/BSA ≥ 16.6 mm/m² showed significantly higher levels of BNP (p = <0.001), GDF-15 (p = 0.040), and H-FABP (p = 0.007). The other investigated cardiovascular biomarkers did not significantly differ between the two groups. To predict a PA/BSA ≥ 16.6 mm/m², cut-off values for the biomarkers were calculated. Here, GDF-15 (p = 0.029; cut-off 1172.0 pg/mL) and BNP (p < 0.001; cut-off 2194.0 pg/mL) showed significant results. Consequently, analyses of combined biomarkers were performed, which yielded IGF-BP2 + BNP (AUC = 0.721; 95%CI = 0.585–0.857; p = 0.004) as the best result of the two-way analyses and GDF-15 + IGF-BP2 + BNP (AUC = 0.727; 95%CI = 0.590–0.864; p = 0.004) as the best result of the three-way analyses. No significant difference regarding the 1-year survival between patients with PA/BSA < 16.6 mm/m² and patients with PA/BSA ≥ 16.6 mm/m² was found (log-rank test: p = 0.452). (4) Conclusions: Although PA/BSA aims to reduce the bias of the PA value caused by different body compositions and sizes, it is still a controversial parameter for diagnosing PH. Combining the parameter with different cardiovascular biomarkers did not lead to a significant increase in the diagnostic precision for detecting PH in patients with severe AS. Full article

Mechanical circulatory support (MCS) allows for functional left and right heart ventricle replacement. MCS induces a systemic inflammatory reaction and prothrombotic state leading to an increased risk of thrombus formation. The extracellular vesicles (EVs) are nanoparticles released from active/injured cells characterized by prothrombotic properties. Simple inflammatory parameters from whole blood count analysis have established a clinical role in everyday practice to describe immune-inflammatory activation. We hypothesized that increased plasma concentrations of EVs might be associated with the proinflammatory and pro-thrombotic characteristics of left ventricle assist device (LVAD) and right ventricle assist device (RVAD) devices. We presented a pilot study showing the concentration of peripheral blood serum, right and left ventricle mechanical assist device extracellular concentration in relation to thrombotic complication in patients treated with a biventricular pulsatile assist device (BIVAD). The observation was based on 12 replacements of pulsatile pumps during 175 days of observation. The proinflammatory characteristics of LVAD were noted. The proinflammatory and procoagulant activation by RVAD was observed. The results may provide possible explanations for the worse results of right-sided mechanical supports observed in clinical practice. Full article

Conventionally, scuba diving has been discouraged for adult patients with congenital heart disease (ACHD). This restrictive sports advice is based on expert opinion in the absence of high-quality diving-specific studies. However, as survival and quality of life in congenital heart disease (CHD) patients have dramatically improved in the last decades, a critical appraisal whether such restrictive sports advice is still applicable is warranted. In this review, the cardiovascular effects of diving are described and a framework for the work-up for ACHD patients wishing to engage in scuba diving is provided. In addition, diving recommendations for specific CHD diagnostic groups are proposed. Full article

The pivotal role of inflammation in the pathophysiology of heart-failure (HF) development and progression has long been recognized. High blood levels of pro-inflammatory and inflammatory markers are present and associated with adverse outcomes in patients with HF. In addition, there seems to be an interrelation between inflammation and neurohormonal activation, the cornerstone of HF pathophysiology and management. However, clinical trials involving anti-inflammatory agents have shown inconclusive or even contradictory results in improving HF outcomes. In the present review, we try to shed some light on the reciprocal relationship between inflammation and HF in an attempt to identify the central regulating factors, such as inflammatory cells and soluble mediators and the related inflammatory pathways as potential therapeutic targets. Full article

Background. This study aimed to investigate the association of preoperative right heart filling indicators with outcomes after coronary artery bypass grafting (CABG) at an 18 month follow up. Methods. Patients who underwent CABG at a single center were included in this study. In addition to the baseline preoperative indicators and perioperative data, initial parameters of the right ventricle (RV) systolic and diastolic function were assessed. Results. Among the 189 patients, a total of 19 (10.0%) MACE (cardiovascular death, nonfatal myocardial infarction and stroke) were recorded during an 18 month follow up. In patients with the development of MACE during the initial examination, the following changes in RV function were revealed compared with the group without MACE: a decrease in the e’t index (8.2 versus 9.6 cm/s, p = 0.029), an increase in the Et/e’t ratio (5.25 vs. 4.42, p = 0.049) and more frequent presence of RV pseudonormal filling (p = 0.03). In the binary logistic regression analysis, the development of MACE 18 months after CABG was associated with the nonconduction of PCI before surgery, the presence of peripheral atherosclerosis, an increase in IVST and Et/e’t and a decrease in LVEF. Conclusions. RV diastolic dysfunction in the preoperative period was associated with the development of MACE within 18 months after CABG, and the ratio Et/e’t was one of the independent predictors of MACE in a multiple regression analysis. This makes it expedient to include an assessment of not only systolic but also diastolic RV function in the preoperative examination. The inclusion of an assessment of RV diastolic function in the pre-CABG evaluation of patients deserves further study. Full article

The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease’s rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease. Full article

Background: Multi-vessel spasm (MVS) has a prognostic impact in patients with vasospastic angina (VSA). Thus, the presence of coronary spasm in both the left coronary artery (LCA) and right coronary artery (RCA) should be assessed through the spasm provocation test (SPT). Nitroglycerin (NTG) is used to avoid SPT-related complications; however, this unavoidable use of NTG may decrease the detection of MVS. Therefore, we investigated the frequency of the unavoidable use of NTG during SPT and clarified the clinical characteristics in patients with VSA who underwent the unavoidable use of NTG during STP. Methods: A total of 141 patients with positive SPT were evaluated. A positive SPT was defined as > 90% constriction in epicardial coronary arteries in response to acetylcholine, accompanied by the usual chest symptoms and/or ischaemic ST-T changes on electrocardiography. When a coronary spasm occurred, we usually wait for the spontaneous relief of the coronary spasm. However, if a prolonged coronary spasm or unstable haemodynamics occurred, 0.3 mg NTG was administered intracoronarily to promptly relieve the coronary spasm and this was defined as the unavoidable use of NTG. Even when the unavoidable use of NTG was administered in one coronary artery, an additional SPT was performed on another coronary artery. If a coronary spasm occurred in another coronary artery, a positive SPT was diagnosed. In contrast, if a coronary spasm was not induced after the unavoidable use of NTG, the judgement was classified as undiagnosed. The patients were divided into two groups according to the unavoidable use of NTG: U-NTG (n = 42) and the final use of NTG: F-NTG (n = 99). The clinical characteristics and frequencies of MVS (≥2 major coronary arteries in which a coronary spasm was provoked) and complications (malignant arrhythmia and unstable haemodynamics requiring catecholamines) during the SPT were compared between the groups. Results: Except for smoking status, all other clinical characteristics did not differ significantly between the groups. More current smokers were observed in the U-NTG group (29%) than in the F-NTG group (12%, p = 0.02). The frequency of MVS did not vary significantly between the groups (p = 0.28), with 64% for U-NTG and 55% for F-NTG. No significant difference was found between the groups in the frequency of severe complications during SPT (p = 0.83), with 2% for U-NTG and 3% for F-NTG. In the U-NTG group, the positive induction rate of coronary spasm in another coronary artery was 40% (17/42). Conclusions: The unavoidable use of NTG occurred in ~30% of patients with VSA, most of whom were current smokers. It did not decrease the detection of MVS and potentially prevented severe complications during SPT. Therefore, the unavoidable use of NTG is acceptable during SPT. However, an additional test may need to be performed to assess the presence of MVS. Full article

Objectives: Insertable cardiac monitors (ICM) allow continuous long-term electrocardiogram monitoring and the detection of paroxysmal atrial fibrillation (PAF) in patients with cryptogenic stroke (CS). Several years have passed since ICM was indicated for CS, and many stroke neurologists will experience cases in which ICM removal is required. As a standard protocol, reincision of the wound at the time of implantation has been proposed by ICM brands. However, it may be difficult due to adhesions of subcutaneous tissue, migration of the device from its original position, and the capsule formed around the device. Our objective is to describe simple alternative techniques for successful ICM removal. Materials and Methods: From December 2016 to September 2021, 37 patients with CS underwent ICM removal at our institution. The device was removed through an incision directly above the proximal end of the device, perpendicular to the wound at the time of ICM implantation. The subcutaneous tissue was removed bluntly using forceps along the edges of the proximal end of the device. When a capsule was attached to the device, we cut the capsule with the blade to release the device. Once the device was visible, the proximal end of the device was grasped with forceps, and the device was pulled from the pocket with gentle traction. All patients undergoing ICM removal received a systematic check for wound dehiscence, wound infection, bleeding, and tissue ischemia at an outpatient examination of 1 week. The 37 patients who underwent removal of ICM were retrospectively reviewed in the medical record and analyzed for procedural success, intraoperative complications, and wound course at one week. Results: All patients achieved procedural success. There were no intraoperative complications, wound dehiscence, bleeding, or skin ischemia at one week postoperatively. The reasons for removal were battery depletion in 65%, early removal before battery life after PAF detection in 32%, and exposure to the body surface in 3%. The devices removed were 62% Reveal LINQ (Medtronic, Minneapolis), 30% Confirm Rx (Abbott, Illinois), and 8% BioMonitor 2 (BIOTRONIK, Berlin), indicating that our method is effective regardless of model. Conclusion: We describe a simple technique for ICM removal for CS that is safe, reliable, and potentially effective in wound healing. Full article

Background: Transthyretin cardiac amyloidosis (ATTR) is a life-threatening, debilitating disease caused by abnormal formation and deposit of transthyretin (TTR) protein in multiple tissues, including myocardial extracellular matrix. It can be challenging to diagnose due to the myriad of presenting signs and symptoms. Additionally, numerous TTR mutations exist in certain ethnicities. Interestingly, our patient was discovered to have a very rare Gly67Ala TTR mutation typically not found in individuals of Asian descent. Recent advances in cardiovascular imaging techniques have allowed for earlier recognition and, therefore, management of this disease. Although incurable, there are now new, emerging treatments that are available for symptom control of cardiac amyloidosis, making early diagnosis vital for these patients, specifically their quality of life. Case summary: We outline a case of a 50-year-old Asian female who was initially hospitalized for nausea and vomiting and persistent orthostatic hypotension. She underwent a multitude of laboratory and imaging tests, resulting in a diagnosis of cardiac amyloidosis, which was confirmed to be due to a rare TTR mutation via genetic testing. Conclusions: Our objective is to describe various TTR mutations, existing diagnostic imaging modalities, and available treatments, as well as highlight the importance of early screening and awareness of cardiac amyloidosis, allowing for quicker diagnosis and treatment of this disease. Full article

Limited data are available on athlete’s heart for rugby athletes. This study aimed to investigate cardiac structure and its relationship with cardiorespiratory fitness in young Japanese rugby athletes. A prospective cross-sectional study using echocardiography and cardiopulmonary exercise testing (CPET) was conducted on 114 male collegiate rugby players. There was a higher prevalence of increased left ventricular (LV), atrial, and aortic dimensions in the young athletes than that in previously published reports, whereas the wall thickness was within the normal range. Anthropometry and CPET analyses indicated that the forwards and backs presented muscular and endurance phenotypes, respectively. Indexed LV and aortic dimensions were significantly larger in the backs than in the forwards, and the dimensions significantly correlated with oxygen uptake measured by CPET. On the four-tiered classification for LV hypertrophy, abnormal LV geometry was found in 16% of the athletes. Notably, the resting systolic blood pressure was significantly higher in athletes with concentric abnormal geometry than in the other geometry groups, regardless of their field positions. Japanese young athletes may exhibit unique phenotypes of cardiac remodeling in association with their fitness characteristics. The four-tiered LV geometry classification potentially offers information regarding the subclinical cardiovascular risks of young athletes. Full article

People living with CHD do less moderate-to-vigorous activity than their peers. This study sought to examine the impact of a community-based physical activity intervention for individuals with CHD. Individuals with CHD and family members participated in a 3 h, one-day Fearless event consisting of a variety of physical activity and education sessions. Consenting participants completed self-administered questionnaires pre-/post-event and completed a post-event feedback form. Descriptive statistics and paired t-tests were calculated across subgroups for each outcome/questionnaire. Written feedback was analyzed using a six-phase framework of reflexive thematic analysis. A total of 32 participants (six children, six adolescents, five youth, five all ages, and ten adults) with CHD completed this study. Following Fearless, youth with CHD reported spending less time being ‘inactive’ and more time being ‘somewhat active’. Adults with CHD reported spending more time walking and partaking in moderate activity and less time partaking in vigorous activity. Fearless successfully engaged individuals with CHD who were more sedentary, less active, and older. Fearless is a fun, family-friendly, physical activity intervention for individuals with CHD. Attending a Fearless event helped children, adolescents, and adults with CHD make incremental improvements to their physical activity levels and provided a framework for sport and recreation leaders who aim to promote physical activity amongst individuals with CHD. Full article

Objective: Global longitudinal strain (GLS) is a sensitive and reproducible predictive factor in patients with ischemic heart disease (IHD), although its correlation with exercise tolerance is unknown. We aimed to identify the correlation between global longitudinal strain (GLS) and cardiopulmonary exercise testing (CPX) parameters and assess the prognostic implications and accuracy of GLS in predicting exercise intolerance in populations with ischemic heart disease (IHD) using CPET criteria. Methods: Prospectively, 108 patients with IHD underwent CPX and 2D speckle-tracking echocardiography. Correlation between GLS and multiple CPX variables was assessed using Spearman’s correlation analysis and univariate regression analysis. A receiver operating characteristic (ROC) curve analysis was performed on GLS to detect exercise intolerance. Results: GLS was correlated with peak oxygen uptake (peak VO₂; r = −0.438, p = 0.000), %PPeak VO₂ (−0.369, p = 0.000), peak metabolic equivalents (METs@peak; r = −0.438, p < 0.01), and the minute ventilation–carbon dioxide production (VE/VCO₂) slope (r = 0.257, p < 0.01). Weak-to-moderate correlations were also identified for the respiratory exchange rate at the anaerobic threshold (RER@AT), end-tidal carbon dioxide at the anaerobic threshold (PETCO₂@AT), oxygen consumption at the anaerobic threshold (VO₂@AT), carbon dioxide production at the anaerobic threshold (VCO₂@AT), and metabolic equivalents at the anaerobic threshold (METs@AT; p < 0.01). On multivariate analysis, the results showed that age, the BMI, and GLS are independent predictors for reduced exercise capacity in patients with IHD (p < 0.01). The area under the ROC curve value of GLS for identifying patients with a peak VO₂ of <14 mL/kg/min was 0.73 (p = 0.000). Conclusion: As a sensitive echocardiographic assessment of patients with ischemic heart disease, global longitudinal strain is an independent predictor of reduced exercise capacity and has a sensitivity of 74.2% and a specificity of 66.7% to detect exercise intolerance. Full article

Maximal oxygen consumption (VO_2max) is an important health indicator that is often estimated using a multiple regression model (MRM) or linear extrapolation method (LEM) with the heart rate (HR) during a step test. Nonetheless, both methods have inherent problems. This study investigated a VO_2max estimation method that mitigates the weaknesses of these two methods. A total of 128 adults completed anthropometric measurements, a physical activity questionnaire, a step test with HR measurements, and a VO_2max treadmill test. The MRM included step-test HR, age, sex, body mass index, and questionnaire scores, whereas the LEM included step-test HR, predetermined constant VO₂ values, and age-predicted maximal HR. Systematic differences between estimated and measured VO_2max values were detected using Bland–Altman plots. The standard errors of the estimates of the MRM and LEM were 4.15 and 5.08 mL·kg⁻¹·min⁻¹, respectively. The range of 95% limits of agreement for the LEM was wider than that for the MRM. Fixed biases were not significant for both methods, and a significant proportional bias was observed only in the MRM. MRM bias was eliminated using the LEM application when the MRM-estimated VO_2max was ≥45 mL·kg⁻¹·min⁻¹. In conclusion, substantial proportional bias in the MRM may be mitigated using the LEM within a limited range. Full article

Jacobsen syndrome (JS) is a rare syndrome caused by a deletion of chromosome 11q. We report a patient with JS and hypoplastic left heart syndrome (HLHS) who required cardiac transplantation. She had many of the recognized morphological features in addition to immunological (lymphopenia) and hematological (thrombocytopenia) issues. The patient underwent a Norwood procedure with a modified Blalock–Taussig shunt (MBTS) and subsequently a Glenn procedure at six months of age. She developed desaturation, with severe tricuspid regurgitation and right ventricular dysfunction, and underwent heart transplantation at 7 months of age. After the transplant, she was hospitalized several times for severe infections. The diagnosis of Jacobsen syndrome came 2 months after transplant. Now, 5 years post-transplant, she is in relatively good health—her heart is functioning normally, her hospitalization rate is getting lower, and her immunological profile is stable. Full article

Diabetes, hypertension, and comorbidity are still crucial public health challenges that Bangladeshis face. Nonetheless, very few studies have been conducted to examine the associated factors, especially the socioeconomic inequalities in diabetes, hypertension, and comorbidity in Bangladesh. This study explored the prevalence of, factors connected with, and socioeconomic inequalities in diabetes, hypertension, and comorbidity among Bangladeshi adults. We used the Bangladesh Demographic and Health Survey (BDHS) data set of 2017–2018. A total of 12,136 (weighted) Bangladeshi adults with a mean age of 39.5 years (±16.2) participated in this study. Multilevel (mixed-effect) logistic regression analysis was employed to ascertain the determinants of diabetes, hypertension, and comorbidity, where clusters were considered as a level-2 factor. The concentration curve (CC) and concentration index (CIX) were utilized to investigate the inequalities in diabetes, hypertension, and comorbidity. The weighted prevalence of diabetes, hypertension, and comorbidity was 10.04%, 25.70%, and 4.47%, respectively. Age, body mass index, physical activity, household wealth status, and diverse administrative divisions were significantly associated with diabetes, hypertension, and comorbidity among the participants. Moreover, participants’ smoking statuses were associated with hypertension. Women were more prone to hypertension and comorbidity than men. Diabetes (CIX: 0.251, p < 0.001), hypertension (CIX: 0.071, p < 0.001), and comorbidity (CIX: 0.340, p < 0.001) were higher among high household wealth groups. A pro-wealth disparity in diabetes, hypertension, and comorbidity was found. These inequalities in diabetes, hypertension, and comorbidity emphasize the necessity of designing intervention schemes geared towards addressing the rising burden of these diseases. Full article

Thoracic aortic aneurysms (TAA) may be associated with complications such as rupture and dissection, which can lead to a fatal outcome. Increased central arterial stiffness has been proposed to be present in patients with TAA compared to unmatched controls. We aimed to assess whether wall properties in patients with TAA are also altered when compared to a matched control group. Applanation tonometry was performed in 74 adults with TAA and 74 sex, age, weight, height, and left ventricular ejection fraction matched controls. Subsequently analysis of the pulse wave was done using the SphygmoCor System. For comparing the two groups, AIx was adjusted to a heart rate of 75/min (AIx@75). 148 1-to-1 matched participants were included in the final model. There was no significant difference in the Alx@75 between the TAA group and the matched control group [mean (SD) of 24.7 (11.2) % and 22.8 (11.2) %, p = 0.240]. Adjusted for known cardiovascular risk factors, there was no association between TAA and AIx@75. Patients with TAA showed comparable arterial wall properties to cardiovascular risk factor matched controls. Since higher arterial stiffness is associated with TAA progression, it remains to be investigated if increased central arterial stiffness is a relevant factor of TAA emergence. Full article

The early repolarization pattern (ERP) is an electrocardiographic phenomenon characterized by the appearance of a distinct J-wave or J-point elevation at the terminal part of the QRS complex. ERP is associated with an increased risk of ventricular arrhythmias in susceptible individuals. The cardiac morphofunctional parameters in subjects with ERP have been characterized mainly by imaging techniques, which suggests that certain changes could be identified in the background of the electrical pathomechanism: however, in this regard, current data are often contradictory or insufficiently detailed. For clarification, a more comprehensive cardiac imaging evaluation of a large patient population is necessary. This review summarizes and analyses the data from the literature related to cardiac morphofunctional characteristics in individuals with ERP. Full article

(1) Background: Arteriovenous fistulas (AVFs) are the preferred access for hemodialysis. Unfortunately, about 60% of patients, especially female patients, fail to receive normal dialysis within one year after surgery because of AVF failure. However, the underlying mechanisms caused by sex differences in AVF failure remain unclear. (2) Methods: We performed analysis of DEGs and functional analysis with the dataset GSE119296 to reveal the biology underlying AVF failure. Immune responses were calculated using CIBERSORT. A protein–protein interaction network and hub gene were constructed using STRING and stepwise identification of potential drugs was performed online. (3) Results: Functional analysis showed that extracellular matrix reprogramming and PI3K-AKT pathway enrichment were significant in both male and female patients. COL1A1 was the hub gene in male patients, whereas CDK1 was the hub gene in female patients. Immune responses including γδ-T cells and mast cells are activated in female patients while no significant differences were noted in the male group. (4) Conclusions: In this study, we used a series of mature and recognized bioinformatic strategies to determine the following items: (1) Reveal the pathogenesis of AVF failure through HUB genes and signaling pathways between the different sexes. (2) Determine the relationship between sex differences in AVF failure and immune abnormalities. (3) Search for relevant sex-specific drugs targeting AVF failure. Full article