Journal of Cardiovascular Development and Disease最新文献

Athletes with heart disease are at increased risk of malignant ventricular arrhythmias and sudden cardiac death compared to their sedentary counterparts. When athletes have symptoms or abnormal findings at preparticipation screenings, a precise diagnosis by differentiating physiological features of the athlete's heart from pathological signs of cardiac disease is as important as it is challenging. While traditional imaging methods such as echocardiography, cardiac magnetic resonance, and computed tomography are commonly employed, nuclear medicine offers unique advantages, especially in scenarios requiring stress-based functional evaluation. This article reviews the use of nuclear medicine techniques in the diagnostic work-up of athletes with suspected cardiac diseases by highlighting their ability to investigate myocardial perfusion, metabolism, and innervation. The article discusses the application of single photon emission computed tomography (SPECT) and positron emission tomography (PET) using radiotracers such as [99mTc]MIBI, [99mTc]HDP, [18F]FDG, and [123I]MIBG. Several clinical scenarios are explored, including athletes with coronary atherosclerosis, congenital coronary anomalies, ventricular arrhythmias, and non-ischemic myocardial scars. Radiation concerns are addressed, highlighting that modern SPECT and PET equipment significantly reduces radiation doses, making these techniques safer for young athletes. We conclude that, despite being underutilized, nuclear medicine provides unique opportunities for accurate diagnosis and effective management of cardiac diseases in athletes.

Background: The utility of synthetic ECV, which does not require hematocrit values, has been reported; however, high-quality CT images are essential for accurate quantification. Second-generation Deep Learning Reconstruction (DLR) enables low-noise and high-resolution cardiac CT images. The aim of this study is to compare the differences among four reconstruction methods (hybrid iterative reconstruction (HIR), model-based iterative reconstruction (MBIR), DLR, and second-generation DLR) in the quantification of synthetic ECV.

Methods: We retrospectively analyzed 80 patients who underwent cardiac CT scans, including late contrast-enhanced CT (derivation cohort: n = 40, age 71 ± 12 years, 24 males; validation cohort: n = 40, age 67 ± 11 years, 25 males). In the derivation cohort, a linear regression analysis was performed between the hematocrit values from blood tests and the CT values of the right atrial blood pool on non-contrast CT. In the validation cohort, synthetic hematocrit values were calculated using the linear regression equation and the right atrial CT values from non-contrast CT. The correlation and mean difference between synthetic ECV and laboratory ECV calculated from actual blood tests were assessed.

Results: Synthetic ECV and laboratory ECV showed a high correlation across all four reconstruction methods (R ≥ 0.95, p < 0.001). The bias and limit of agreement (LOA) in the Bland-Altman plot were lowest with the second-generation DLR (hybrid IR: bias = -0.21, LOA: 3.16; MBIR: bias = -0.79, LOA: 2.81; DLR: bias = -1.87, LOA: 2.90; second-generation DLR: bias = -0.20, LOA: 2.35).

Conclusions: Synthetic ECV using second-generation DLR demonstrated the lowest bias and LOA compared to laboratory ECV among the four reconstruction methods, suggesting that second-generation DLR enables more accurate quantification.

Aims: The goal of this study was to establish a simple model of 3D endothelial spheroids with mosaic gene expression using adeno-associated virus (AAV) transduction, with a future aim being to study the activity of post-zygotic mutations common to vascular malformations.

Methods: In this study, 96-well U-bottom plates coated with a commercial repellent were seeded with two immortalized human endothelial cell lines and aggregation monitored using standard microscopy or live-cell analysis. The eGFP expression was used to monitor the AAV transduction.

Results: HUVEC-TERT2 could not form spheroids spontaneously. The inclusion of collagen I in the growth medium could stimulate cell aggregation; however, these spheroids were not stable. In contrast, the hCMEC/D3 cells aggregated spontaneously and formed reproducible, robust 3D spheroids within 3 days, growing steadily for at least 4 weeks without the need for media refreshment. The hCMEC/D3 spheroids spontaneously developed a basement membrane, including collagen I, and expressed endothelial-specific CD31 at the spheroid surface. Serotypes AAV1 and AAV2^QUADYF transduced these spheroids without toxicity and established sustained, mosaic eGFP expression.

Conclusions: In the future, this simple approach to endothelial spheroid formation combined with live-cell imaging could be used to rapidly assess the 3D phenotypes and drug and radiation sensitivities arising from mosaic mutations common to brain vascular malformations.

Cardiovascular disease remains a significant cause of morbidity and mortality worldwide, and its manifestations continue to pose a challenge in clinical practice [...].

Electrocardiogram modifications in athletes are common and usually reflect structural and electrical heart adaptations to regular physical training, known as the athlete's heart. However, these electrical modifications sometimes overlap with electrocardiogram findings that are characteristic of various heart diseases. A missed or incorrect diagnosis can significantly impact a young athlete's life and potentially have fatal consequences during exercise, such as sudden cardiac death, which is the leading cause of death in athletes. Therefore, it is crucial to correctly distinguish between expected exercise-related electrocardiogram changes in an athlete and several electrocardiogram abnormalities that may indicate underlying heart disease. This review aims to serve as a practical guide for cardiologists and sports clinicians, helping to define normal and physiology-induced electrocardiogram findings from those borderlines or pathological, and indicating when further investigations are necessary. Therefore, the possible athlete's electrocardiogram findings, including rhythm or myocardial adaptation, will be analyzed here, focusing mainly on the differentiation from pathological findings.

Background: End-stage heart failure is associated with high mortality. Recent developments such as the left ventricular assist device (LVAD) have improved patient outcomes. The HeartMate 3 LVAD is a novel centrifugal pump that was developed to provide hemodynamic support in heart failure patients, either as a bridge-to-transplant (BTT), myocardial recovery, or destination therapy (DT). Our objective was to evaluate the survival rates and LVAD-related complications of the HeartMate 3 LVAD before and after hospital discharge in our center.

Methods: We retrospectively reviewed all patients implanted with the HeartMate 3 LVAD in our institute between September 2015 and June 2024. Patients who received a Heart Ware Ventricular Assist Device (HVAD) and HeartMate 2 LVAD devices were excluded. The primary endpoint was survival before and after hospital discharge. The secondary endpoints included an incidence of serious LVAD adverse events (bleeding, major infection, hemolysis, device thrombosis and malfunction, and neurological dysfunction) and the causes of re-admission along the follow-up period.

Results: A total of 48 consecutive HeartMate 3 LVAD patients were enrolled in this study. The mean age was 56.1 ± 10.6 years. A total of 72.9% of patients received LVAD therapy as a BTT, 14.6% as DT, 10.4% as a bridge-to-decision, and 2.1% as a bridge-to-recovery. A total of 85.4% of patients were discharged after implantation. The main cause for in-hospital mortality was right ventricular failure (8.3%), followed by stroke, abdominal bleeding, and multi-organ failure (2.1% each). One patient (2.1%) had successful heart transplantation, 26 patients (63.4%) are still on LVAD support, and 11 (26.8%) patients have died during follow-up. The main cause of mortality after hospital discharge was sepsis, which occurred in 9.8% of patients, followed by right ventricular failure, non-LVAD-related causes, unknown causes with two (4.9%) cases each, and one case of fatal stroke (2.4%). During the follow-up, there was no need for LVAD replacement.

Conclusions: HeartMate 3 LVAD is associated with excellent in-hospital survival rates in patients with end-stage heart failure. Right ventricular failure was the main cause of death before hospital discharge, whereas sepsis was the main cause of death after hospital discharge.

Primary aldosteronism (PA) is commonly associated with resistant hypertension. Biochemical tests can be clinically useful in the screening and diagnosis of primary aldosteronism. This study aimed to identify the cutoff values of aldosterone levels (A) and the aldosterone-renin ratio (ARR) for an accurate prediction of PA in patients with apparent resistant hypertension in a real-life scenario. This database-based study included a historical cohort of male and female patients with apparent resistant hypertension, aged 18 years or older and surveyed for PA in a specialized center from 2008 to 2018. Aldosterone and plasma renin activity (PRA) or the plasma renin concentration (PRC) were measured in the treated hypertensive patients. The patients with positive screening results were subsequently referred to the endocrinology department for confirmatory tests. The patients with confirmed PA were included in the case group, and the others remained as controls. Receiver-operating characteristic (ROC) curves were used to identify the cutoff points for aldosterone and the ARR, thereby analyzing their sensitivity and specificity for confirmed PA. Among the 3464 patients (59 ± 13 years old, 41% male) who had apparent resistance hypertension screened, PA was confirmed in 276 individuals (8%). A ≥ 16.95 ng/dL (95% CI: 0.908-0.933) had an odds ratio of 6.24 for PA, while A/PRA ≥ 29.88 (95% CI: 0.942-0.984) or an A/PRC ≥ 2.44 (95% CI: 0.978-0.990) had an odds ratio of 216.17 for PA diagnoses. Our findings suggest that a positive PA screening with aldosterone ≥ 17 ng/dL associated with A/PRA ≥ 29.88 or an A/PRC ratio of ≥2.44 should be sufficient to confirm the diagnosis of PA without confirmatory testing.

Objectives: Coronary artery fistulas (CAFs) are rare congenital anomalies with an occurrence rate of 0.002-0.3%. The right coronary artery (RCA) is reportedly the most common site of origin of CAFs, but fistulas draining to the left atrium (LA) are rare. We presented a three-year-old boy with a symptomatic congenital RCA-to-LA fistula, which was successfully percutaneously occluded with an Amplatzer vascular plug 4 (AVP4).

Case report: The diagnosis was made by echocardiography when he was two months old. During the follow-up period of 2 years, a progressive dilatation of the RCA and enlargement of the left ventricle was detected, so treatment for congestive heart failure was initiated. At the age of three, the patient presented with a history of occasional mild central chest pain and discomfort and mild dyspnea on exertion. On a 24 h ECG Holter monitor, the depression of ST segments was registered. CT angiography highlighted a large type B RCA fistula to the LA, which extended along the atrioventricular sulcus. The proximal RCA diameter was 7 mm. The fistula was tortuous, with segmental narrowing and three curves. Cardiac catheterization was performed across the right femoral artery on the three-year-old boy (body weight: 13 kg). Across the 4F Judkins right guiding catheter, an AVP4 of 5 mm was placed in the distal part of the CAF connected with the delivery cable. After 15 min, ECG changes were not registered, so the device was released. Immediate post-deployment angiography demonstrated complete CAF occlusion, with satisfying flow in the distal coronary artery. The patient was discharged after four days. In the short-term follow-up period, the boy was symptom-free.

Conclusions: In our experience, given the existence of the left-to-left shunt and the more pronounced exercise-induced coronary steal phenomenon that occurs in medium-sized and large CAFs, occlusion is necessary to prevent the further progression of clinical signs and symptoms.

Advances in cardiovascular imaging have expanded the scope and precision of rare diagnoses. Handling a patient with a giant left atrium, we focused on the existence and associated factors of "lone giant (left or right) atria" in our clinical setting. The aim of the current study was to establish reasonable cut-off values for the diagnosis of "giant atrium". Our analysis utilised echocardiography and cardiovascular magnetic resonance (CMR) imaging databases, with the original data re-assessed to ensure consistency and comparability. Four patients met the search criteria, with two cases requiring CMR to confirm the diagnosis of "giant atrium", correcting the initial echocardiographic assessment. Both echocardiography and CMR excel in the assessment of atrial anatomy, although the superior image quality and multiplanar capabilities of CMR support its preference. In assessing the atrial size, the use of 3D volumetric measurements should replace traditional biplane methods due to the complex anatomy of the atrium. We propose the use of an indexed volume threshold (>120 mL/m²) rather than simple diameter measurements for the diagnosis of "giant atria". Structural atrial abnormalities appear to correlate with an increased risk of atrial arrhythmias, while potential serious complications such as thromboembolism or compression symptoms require further observation in larger patient cohorts to establish definitive risks.