Search Results (58)

Introduction: Giant sinus of Valsalva aneurysms (SVA) represent a rare cardiovascular pathology that may remain asymptomatic for an extended period. However, they are associated with a high risk of life-threatening complications, including compression of adjacent structures and aneurysm rupture. Case presentation: We report a clinical case of a 71-year-old female patient with a long-standing history of arterial hypertension and cardiac arrhythmias, in which echocardiography revealed aneurysmal dilatation of the right coronary sinus. Cardiac computed tomography (CT) confirmed the presence of a giant aneurysm of the right sinus of Valsalva measuring 70 × 51 × 49 mm, compressing the outflow tracts of both ventricles (right—up to 7 mm, left—up to 8 mm) and the left inferior pulmonary vein (up to 3 mm), which clinically manifested as dyspnoea, lower-extremity oedema, and rhythm disturbances. The patient successfully underwent complex reconstructive surgery, including aortic root replacement and valve repair. Despite the technical success of the operation, the patient died from pneumonia three months postoperatively. Discussion: This observation underscores the critical role of imaging modalities (echocardiography and CT) in verifying this pathology. The use of multimodal imaging facilitated both a timely diagnosis and a detailed three-dimensional evaluation of the aneurysm’s relationship with adjacent structures. This information, in turn, guided personalised surgical planning. Conclusions: This case highlights the necessity of considering giant SVA in the differential diagnostic workup of patients who present with unexplained symptoms of heart failure. Full article

Background/Objectives: Alcoholic cardiomyopathy (ACM) is a major preventable cause of non-ischemic dilated cardiomyopathy (DCM), yet its specific cardiac magnetic resonance (CMR) remains incompletely defined. We aimed to characterize the CMR features of ACM, focusing on late gadolinium enhancement (LGE) subpatterns and biventricular function and to compare them with idiopathic DCM. Methods: In total, 148 consecutive patients (ACM n = 20, idiopathic DCM n = 128) referred for CMR at a single center were retrospectively analyzed. Sequential logistic regression adjusted for age, sex, left ventricular ejection fraction (LVEF), and right ventricular ejection fraction (RVEF) was used to identify independent association with LGE presence. Results: LVEF did not differ between groups (32.5% vs. 35.0%, p = 0.293). ACM patients showed significantly worse RVEF (40.5% vs. 52.0%, p = 0.010) and larger indexed right ventricle (RV) volumes. Any LGE was present in 70% vs. 40% (p = 0.015); when the non-specific RV insertion point pattern (non-RV-IP) was excluded, non-RV-IP LGE was 45% vs. 22.7% (p = 0.051), with a specific midwall linear pattern (25% vs. 8%, p = 0.033). ACM was independently associated with LGE across all models with an adjusted odds ratio (OR) of 3.06 [95% CI 1.05–8.95], p = 0.041, and RV dysfunction (RVEF < 45%) (OR 4.79 [95% CI 1.60–14.32], p = 0.005). No differences in major adverse cardiovascular events (MACEs) were observed at 24 months (log-rank p = 0.697). Conclusions: ACM has a distinct CMR phenotype characterized by midwall linear LGE fibrosis and more severe RV involvement, independent of left ventricle (LV) systolic function. These exploratory findings suggest that CMR may provide clinically relevant phenotypic information in ACM beyond LVEF, warranting confirmation in prospective studies. Full article

Tricuspid regurgitation (TR) is a prevalent cardiovascular disorder with significant clinical impact. TR is frequently silent and underdiagnosed and is estimated to impact over 70 million people globally. Characterized by retrograde blood flow from the right ventricle into the right atrium due to incomplete valve closure, TR leads to right heart dilation, systemic congestion, and eventually right-sided heart failure. Importantly, TR may contribute to the onset of atrial fibrillation (AF), the most common sustained arrhythmia, affecting approximately 59 million individuals worldwide. Despite its growing clinical importance, the pathophysiology of TR remains incompletely understood, and current animal models of TR, based on direct valve manipulation, limit translational applicability. We present a novel, minimally invasive porcine model of TR established via femoral/jugular vein catheterization with deployment of an inferior vena cava (IVC) filter. The filter partially impedes tricuspid valve closure, inducing TR without valvular injury. Validation was achieved through multimodal imaging, including fluoroscopy, echocardiography, and electrocardiography, confirming hallmark features of TR, including right atrial and ventricular enlargement and arrhythmic activity. This model provides a reproducible, minimally invasive platform for studying selected features of TR progression. Its minimally invasive nature and preservation of native valvular structure make it a useful preclinical platform for mechanistic and translational research. Full article

Background: Right-ventricular (RV) involvement is starting to gain recognition in pediatric dilated cardiomyopathy (DCM), but its deformation characteristics and its relationship to functional status remain insufficiently defined. Methods: Twenty-nine children with primary DCM were compared with age- and sex-matched healthy controls. Conventional echocardiography and two-dimensional speckle-tracking echocardiography (2D-STE) were performed. Segmental deformation (basal, mid-ventricular and apical levels) was analyzed using a linear mixed-effects model. Associations between strain indices and advanced functional limitation (NYHA/Ross Class III-IV) were evaluated using exploratory logistic regression and ROC analysis. Results: Children with DCM showed significant impairment in both ventricles. Conventional parameters (TAPSE, tricuspid E/A ratio, RV E′ velocity, and LV ejection fraction) were reduced. Right ventricular strain was significantly lower in DCM compared with controls (p < 0.05). Segmental analysis demonstrated a basal-predominant reduction in RV strain. Right-ventricular free-wall longitudinal strain correlated with RV S′ (r = −0.49), RV E′ (r = −0.46), LVGLS (r = 0.79) and LV ejection fraction (r = −0.63, all p < 0.05). In univariable analysis, RVFWSL predicted advanced functional class (OR 1.13 per 1% decrease, p = 0.026), while LVGLS remained the independent predictor in the multivariable model. A combined model incorporating RVFWSL and LVGLS demonstrated excellent discriminative accuracy (AUC 0.95). Conclusions: Pediatric DCM is characterized by RV involvement with a distinct basal-dominant deformation pattern. Biventricular strain assessment improves the identification of children with advanced functional class and may enhance functional stratification beyond conventional indices. Full article

Background/Objectives: After pneumonectomy, the right ventricular stroke volume is pumped into pulmonary vessels whose volume has been reduced by approximately 50%. To sustain conditions for pulmonary flow, the flow reserve is increased in the remaining lung, which is conducive to the development of pulmonary hypertension symptoms. This study sought to examine pulmonary flow in one lung and the size of the right atrium (RA), right ventricle (RV) and pulmonary artery (PA) in patients who had undergone pneumonectomy and to establish the influence of time since pneumonectomy on these parameters, as well as their potential mutual dependencies. Methods: The retrospective analysis included 34 patients who had undergone pneumonectomy. Pulmonary flow was measured by means of perfusion scintigraphy. The diameters of the RA, RV and PA were evaluated based on computed tomography with contrast. Results: We observed complete or near-complete utilization of flow reserve in 38.2% (13/34) of patients, enlarged transversal and longitudinal dimensions of the RA in 17.6% (6/34) and 32.3% (11/34) of patients, respectively, and enlarged transversal and longitudinal dimension of the RV in 67.6% (23/34) and 44.1% (15/34) of patients, respectively. Dilatation of the PA was discovered in 23.5% (8/34) to 26.5% (9/34) of patients, as well as the presence of an extensive complex of radiographic features of pulmonary hypertension (PH) syndrome in 23.5% (8/34) of cases. Conclusions: Radiological features of PH were present in a significant number of patients. These features developed at varying rates but were present in all patients followed >10 years after the procedure. Full article

Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital coronary anomaly with an estimated incidence of 1 in 300,000 live births. While commonly diagnosed in infancy, adult presentations are exceedingly rare and pose significant diagnostic challenges. Delayed diagnosis may result in progressive myocardial ischemia, heart failure, arrhythmias, or sudden cardiac death. Surgical correction is the definitive treatment, with the goal of restoring a dual coronary artery system and preventing irreversible myocardial damage. We present the case of a 30-year-old male with a prior history of non–ST-elevation myocardial infarction who was referred for evaluation of exertional angina and symptoms of heart failure. Transthoracic echocardiography revealed a dilated left ventricle with an ejection fraction (LVEF) of 35%. Coronary angiography and cardiac MDCT identified an anomalous origin of the left circumflex artery (LCx) from the right pulmonary artery (RPA) and a coronary–pulmonary artery fistula involving the LAD. The patient underwent successful surgical correction with reimplantation of the LCx into the ascending aorta. Postoperative recovery was uneventful. At 3-month follow-up the patient was symptom-free, though echocardiography revealed persistent LV dilation and reduced LVEF, necessitating continued pharmacologic therapy and monitoring. This case highlights the importance of maintaining a high index of suspicion for ALCAPA in adult patients with unexplained cardiomyopathy or ischemic symptoms. Early diagnosis and surgical intervention remain crucial for improving long-term outcomes and preventing life-threatening complications. Full article

Background: Arteriovenous fistulas (AVFs) may contribute to cardiac remodeling and consequently to an increased risk of heart failure and cardiovascular mortality in patients with end-stage kidney disease (ESKD). We aimed to assess cardiac changes following AVF creation and identify potential parameters associated with cardiac remodeling. Methods: In our prospective, single-center study, ESKD patients without significant pre-existing cardiac disease underwent 2D and 3D echocardiographic evaluation before and after AVF creation, along with AVF flow measurement. Cardiac remodeling was assessed using 3D indexed left and right ventricular end-diastolic volumes (LVEDVi, RVEDVi), while systolic function was assessed using longitudinal strain and 3D ejection fraction. Results: We included 20 patients (18 men; median age 73.5 years [IQR: 67–77]) with a mean AVF flow of 1140 ± 345 mL/min. At a median of 8.2 months (IQR: 7.3–9.3) following AVF creation, significant biventricular dilatation was observed: LVEDVi increased from 89 ± 14 to 97 ± 21 mL/m² (p < 0.05) and RVEDVi from 80 ± 15 to 91 ± 18 mL/m² (p < 0.05), while the systolic function of both ventricles did not change significantly. The right ventricle showed the most pronounced remodeling and it was independently associated with volume overload (p = 0.003) and elevated left ventricular filling pressure (p = 0.030), but not with AVF flow. Conclusions: Moderate AVF flow was associated with cardiac remodeling, primarily affecting the right ventricle. Fluid overload and left ventricular filling pressure were key factors associated with right ventricular remodeling, underscoring the need for careful fluid management and vascular access planning in ESKD patients. Full article

The image represents the post-mortem heart of a 28-year-old female patient, diagnosed in childhood with complete common atrioventricular canal defect. At time of diagnosis, the family refused surgery, as did the patient during her adulthood. Despite being advised against pregnancy, she became pregnant. On presentation to hospital, she was cyanotic, with clubbed fingers, and hemodynamically unstable, in sinus rhythm, with Eisenmenger syndrome and respiratory failure partially responsive to oxygen. During pregnancy, owing to systemic vasodilatation, the right-to-left shunt is increased, with more severe cyanosis and low cardiac output. Echocardiography revealed the complete common atrioventricular canal defect, with a single atrioventricular valve with severe regurgitation, right ventricular hypertrophy, pulmonary artery dilatation, severe pulmonary hypertension and a hypoplastic left ventricle. The gestational age at delivery was 38 weeks. She gave birth to a healthy boy, with an Apgar score of 10. The vaginal delivery was chosen by an interdisciplinary team. The cesarean delivery and the anesthesia were considered too risky compared to vaginal delivery. Three days later, the patient died. The autopsy revealed hepatomegaly, a greatly hypertrophied right ventricle with a purplish clot ascending the dilated pulmonary arteries and a hypoplastic left ventricle with a narrowed chamber. A single valve was observed between the atria and ventricles, making all four heart chambers communicate, also insufficiently developed interventricular septum and its congenital absence in the cranial third. These morphological changes indicate the complete common atrioventricular canal defect, with right ventricular dominance, which is a rare and impressive malformation that requires mandatory treatment in early childhood in order for the condition to be solved. Full article

Background: Noonan syndrome (NS) is a relatively common RASopathy that can be associated with a variety of phenotypic and genotypic variations and potential long-term health consequences. Its most described prenatal ultrasound features in the first trimester are thickened nuchal translucency (NT) and dilated jugular sacs; while heart defects, polyhydramnios and facial dysmorphisms are its known manifestations in the second and third trimesters. Methods: We present two cases of NS with the prenatal ultrasound diagnosis of external hydrocephalus (EH) in the second trimester. Results: Case 1 had a normal first trimester scan and showed mild polyhydramnios, an echogenic intracardiac focus (EIF) in the left ventricle and pyelectasis in the second trimester in association with the EH. The whole exome sequencing (WES) confirmed a pathogenic variant in the SOS1 gene. Case 2 showed increased NT, agenesis of the ductus venosus (DV), single umbilical artery (SUA), an EIF in the right ventricle and an abnormal prefrontal space ratio (PSFR). By the 19th gestational week, EH appeared. The ambient and quadrigeminal cisterns were also slightly widened. The WES revealed a PTPN11 gene variant. Conclusions: The most reported sonographic features of NS are either non-specific or difficult to integrate into routine screening, requiring substantial experience. In our two cases, we detected EH in the second trimester, which is rarely described as a prenatal ultrasound diagnosis. To our current knowledge, this is the first case reported of EH in NS caused by an SOS1 gene variant and these are the first cases reported with the prenatal sonographic diagnosis of EH in NS. Full article

Morphometric cardiac reference values are reported for macropods and koalas (Phascolarctos cinereus). Body weight (BW), heart weight (HW), left ventricle (LV) wall, interventricular septum (S), right ventricle (RV) wall thickness, and LV+S and RV weights were measured at postmortem examination of 48 macropods and 32 koalas that had no evidence of cardiovascular disease. The HW/BW% (0.43–0.96%) and (LV+S)/RV (2.80–4.22) for macropods were comparable to domestic species. In koalas, the HW/BW% (0.25–0.51%) was lower, and the (LV+S)/RV (3.06–5.41) ranged higher than in macropods and domestic species. The LV:RV of koalas (1.0–10.8) was more variable than in macropods (1.17–4.27). Two macropods with cardiac disease were assessed on postmortem examination against the generated reference values. An adult male common wallaroo (Osphranter robustus) was found dead with copious serous peritoneal effusion, chronic passive hepatic congestion with centrilobular fibrosis, and dilation of the RV, while the LV:RV was elevated, supportive of RV thinning. A 21-year-old female zoo-housed Matschie’s tree kangaroo (Dendrolagus matschiei) had a flaccid thin-walled RV, LV cardiomyocyte hypertrophy, interstitial myocardial fibrosis and myofiber degeneration, pulmonary oedema, and serous pericardial effusion. The (LV+S)/RV and LV:RV were elevated and RV:S decreased, supporting left hypertrophic cardiomyopathy. Species-specific reference values presented in this study facilitate objective and improved postmortem cardiac assessment in macropods and koalas. Full article

Pulmonary hypertension associated with interstitial lung disease (ILD-PH) frequently complicates the course of patients with fibrotic ILD. In this narrative review, the authors assess current diagnostic tools and management considerations in ILD-PH patients. ILD-PH is associated with increased morbidity and mortality and may be suggested by the presence of symptoms out of proportion to the extent of the ILD. There are other clues to the presence of PH in the context of ILD including the need for supplemental oxygen, a reduced DLCO especially if accompanied by a disproportionately higher forced vital capacity, imaging demonstrating an enlarged pulmonary artery or a dilated right ventricle, or objective evidence of a reduced exercise capacity. While echocardiography is one screening tool, right heart catheterization remains the gold standard for the diagnosis of PH. When appropriate, treatment with inhaled treprostinil, or possibly other pulmonary vasodilators, may be indicated. Full article

A 1-year-old Maltese–Poodle mixed breed dog was referred to the cardiology service because of severe exercise intolerance and daily exercise-induced syncopal episodes. Physical examination revealed no abnormalities. Echocardiography showed intermittent underfilling of the heart. Abdominal ultrasound examination revealed a segmental aplasia of the caudal vena cava between the kidneys and the liver, and azygos continuation of the cava. The azygos vein dorsal to the right kidney showed a severe aneurysm with stasis of blood. Computed tomographic angiography showed that the right crus of the diaphragm was probably responsible for the intermittent compression of the dilated azygos vein, which was thought to have subsequently led to insufficient venous return to the heart. The underfilled ventricles could not produce sufficient cardiac output, which caused the assumed cerebral hypoperfusion due to presumed systemic arterial hypotension. Under general anesthesia a self-expanding nitinol stent was implanted into the azygos vein at the level of the diaphragm. All clinical signs resolved immediately after surgery. The dog remained free from clinical signs (6 months after surgery). This is the first report that describes the successful treatment of this congenital vascular anomaly. Ultrasonography of the caudal vena cava should be performed in dogs with unexplained syncope. Full article

Acute heart failure (AHF) is a complex clinical syndrome characterized by the rapid or gradual onset of symptoms and/or signs of heart failure (HF), leading to an unplanned hospital admission or an emergency department visit. AHF is the leading cause of hospitalization in patients over 65 years, thus significantly impacting public health care. However, its prognosis remains poor with high rates of mortality and rehospitalization. Many pre-existing cardiac conditions can lead to AHF, but it can also arise de novo due to acute events. Therefore, understanding AHF etiology could improve patient management and outcomes. Cardiomyopathies (CMPs) are a heterogeneous group of heart muscle diseases, including dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), non-dilated cardiomyopathy (NDLVC), and arrhythmogenic right ventricular cardiomyopathy (ARVC), that frequently present with HF. Patients with CMPs are under-represented in AHF studies compared to other etiologies, and therefore therapeutic responses and prognoses remain unknown. In DCM, AHF represents the most frequent cause of death despite treatment improvements. Additionally, DCM is the first indication for heart transplant (HT) among young and middle-aged adults. In HCM, the progression to AHF is rare and more frequent in patients with concomitant severe left ventricle (LV) obstruction and hypertrophy or severe LV systolic dysfunction. HF is the natural evolution of patients with RCM and HF is associated with poor outcomes irrespective of RCM etiology. Furthermore, while the occurrence of AHF is rare among patients with ARVC, this condition in NDLVC patients is currently unknown. In this manuscript, we assessed the available evidence on AHF in patients with CMPs. Data on clinical presentation, therapeutic management, and clinical outcomes according to specific CMPs are limited. Future HF studies assessing the clinical presentation, treatment, and prognosis of specific CMPs are warranted. Full article

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. Full article

Background: Dilated cardiomyopathy (DCM) involves myocardial remodeling, characterized by significant fibrosis and extracellular matrix expansion. These changes impair heart function, increasing the risk of heart failure and sudden cardiac death. This study investigates the prognostic value of circulating fibrosis biomarkers as a less invasive method in DCM patients. Methods: Plasma samples from 185 patients with confirmed DCM were analyzed to measure 13 circulating biomarkers using Luminex bead-based multiplex assays and ELISA. The prognostic value of these biomarkers was evaluated concerning heart failure-associated events and all-cause mortality. Results: Elevated MMP-2 levels (>1519.3 ng/mL) were linked to older age, higher diabetes prevalence, lower HDL, increased NT-proBNP and hs-TnT levels, and severe systolic dysfunction. High TIMP-1 levels (>124.9 ng/mL) correlated with elevated NT-proBNP, more atrial fibrillation, reduced exercise capacity, and larger right ventricles. Increased GDF-15 levels (>1213.9 ng/mL) were associated with older age, systemic inflammation, renal impairment, and poor exercise performance. Elevated OPN levels (>81.7 ng/mL) were linked to higher serum creatinine and NT-proBNP levels. Over a median follow-up of 32.4 months, higher levels of these biomarkers predicted worse outcomes, including increased risks of heart failure-related events and mortality. Conclusions: Circulating fibrosis biomarkers, particularly MMP-2, TIMP-1, GDF-15, and OPN, are valuable prognostic tools in DCM. They reflect the severity of myocardial remodeling and systemic disease burden, aiding in risk stratification and therapeutic intervention. Integrating these biomarkers into clinical practice could improve DCM management and patient prognosis. Full article