Search Results (81)

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

Mitral annular calcification (MAC) is usually considered an incidental, benign, age-related finding without serious complications in patients evaluated for cardiovascular or pulmonary disease with imaging studies that may result in mitral regurgitation or stenosis when severe. Therefore, it is usually not considered a significant alteration. However, there is accumulating evidence that it is associated with a higher risk of cardiovascular events, such as atherosclerotic coronary artery disease, aortic artery disease, carotid artery disease, peripheral artery disease, stroke, atrial fibrillation, atrioventricular and/or intraventricular conduction disturbance, systemic embolization, infective endocarditis, heart failure and mortality. The presence of MAC also significantly influences the outcome of mitral valve transcatheter and surgical interventions. Several conditions may predispose to MAC. MAC is strongly related to cardiovascular risk factors, such as hypertension, diabetes, smoking and cardiovascular atherosclerosis, and inflammation may also play a role in the pathogenesis of MAC. Also, conditions that increase mitral valve stress, such as hypertension, aortic stenosis and hypertrophic cardiomyopathy, predispose to accelerated degenerative calcification of the mitral annulus area. Congenital disorders, e.g., Marfan syndrome and Hurler syndrome, are also associated with MAC, due to an intrinsic abnormality of the connective tissue composing the annulus. Full article

Background and Clinical Significance: Scimitar Syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return, often requiring early surgical correction. It may coexist with other congenital or acquired cardiovascular anomalies, including valvular diseases such as mitral regurgitation. When surgical correction of Scimitar Syndrome is combined with mitral valve annuloplasty, the proximity to the atrioventricular node may potentially predispose patients to late-onset conduction disturbances, although causality remains speculative. Case Presentation: We describe the case of a 53-year-old male who developed paroxysmal atrial fibrillation, atrial flutter, and intermittent second-degree AV block decades after undergoing surgical correction of Scimitar Syndrome with concomitant mitral annuloplasty. Multimodal echocardiographic evaluation revealed preserved left atrial volume, normal intra-atrial conduction time, mildly reduced strain, and maintained atrial synchrony. The patient was treated with direct oral anticoagulants and beta-blockers and underwent the implantation of a ventricular leadless pacemaker. Conclusions: This case highlights the supportive role of atrial function imaging in assessing atrial health and informing rhythm management and procedural choices in surgically corrected congenital heart disease. Full article

Background: Aortic valve stenosis remains the most prevalent valvular pathology in Western countries. Rapid deployment bioprosthesis (RD) has emerged as a promising alternative to conventional valves for surgical aortic valve replacement (SAVR), particularly in elderly and high-risk patients. This study reports the short- and long-term outcomes of RD in patients with isolated aortic stenosis. Methods: A retrospective single-center analysis was conducted on 382 patients who underwent RD-AVR between 2014 and 2020. Data were collected from clinical files and national electronic databases. Primary outcomes included cardiopulmonary bypass (CPB) and cross-clamping (XC) times, postoperative complications, and long-term survival. Results: The mean age was 75.6 ± 5.9 years, with 29.8% of patients over 80 years old and a mean EuroSCORE II of 2.3 ± 1.5%. CPB and XC times were 36.7 ± 10.8 and 27.4 ± 8.1 min, respectively. Postoperative complications included acute kidney injury (AKI, 53.4%), de novo atrial fibrillation (31.9%), and high-grade/complete atrioventricular block with permanent pacemaker implantation (9.8%). In-hospital and 30-day mortality was 1.02% and 2.3%, respectively. The 5-year survival rate was 77%. At 6 months postoperatively, the mean transvalvular gradient was 11.1 ± 4.7 mmHg. At a median follow-up of 6.7 years, no cases of structural valve deterioration and only one case of endocarditis were reported. Conclusion: In this single-center study, RD in isolated AVR demonstrated favorable short- and long-term outcomes, including no structural valve deterioration at mid-term follow-up. These devices offer a safe and effective alternative to conventional SAVR, particularly in high-risk populations. Full article

In avian medicine, sonographic examination is an important diagnostic tool for heart diseases. Little is known about the diagnostic relevance of the Doppler blood flow profiles of parrots. In the present study, sonographic examinations of Congo grey parrots with atherosclerosis were evaluated retrospectively to gain more knowledge about their pulmonary and aortic systolic blood flow. The shapes of their Doppler sonographic blood flow profiles were quantified by the determination of the acceleration and deceleration phases. The investigations showed the differences between the aortic flow profile, with fast rising velocities, and the pulmonary flow profile, which has a round shape, in grey parrots. Diseased parrots with ultrasonographic signs of a right heart failure, such as an enlarged right ventricle and/or insufficiencies of the right atrioventricular and/or pulmonary valve, showed a significantly shorter acceleration and longer deceleration phase than parrots without heart failure or with sonographic signs of left heart failure only. The correlation of the shape of the pulmonary Doppler blood flow profile with the diameter of the left atrium, the systolic and diastolic diameter of the right ventricle, the fractional shortening of the left and right ventricle, and the mean aortic and pulmonary blood flow illustrates the importance of these sonographic parameters in the investigation of the function of the right ventricle in heart disease. Our examination showed that the shape of the pulmonary Doppler blood flow profile of Congo grey parrots provides important information about the pressure load on the right ventricle in heart diseases, which is comparable to that in small animal and human medicine. Full article

Adult congenital heart disease (ACHD) represents a significant portion of congenital anomalies, and with improved treatments leading to an increased life expectancy, its prevalence has been increasing over the past few decades. Nonetheless, a considerable number of patients with ACHD require cardiac rhythm management devices during their lifetime. Traditionally, transvenous pacemaker placement has been the standard mode of treatment for these patients. However, some patients with ACHD have anatomical barriers that obscure this mode of treatment. Leadless pacing systems (LPSs) have changed the field of pacing. Currently, two different LPSs are available. In a real-world setting, implanting an LPS in patients after tricuspid valve (TV) surgery seems to be a straightforward procedure with a low risk of complications, with patients showing no valvular dysfunction after the intervention. LPS implantation is an option to avoid device-related complications in patients with previous TV surgery. Moreover, it has been demonstrated that even the jugular approach seems as safe as the femoral approach and could be considered an alternative implantation method for LPSs. The Aveir VR leadless pacemaker is a helix LPS with unique features, such as its capacity as a dual-chamber leadless pacemaker, the ability to map electrical parameters before releasing the device, and its possibility of being retrievable. Hereby, we present the case of Ebstein’s anomaly, atrial septal defect closure, and previous TV surgery with symptomatic intermittent advanced atrioventricular block. This case illustrates that a transjugular approach for LPSs is also feasible in patients with ACHD. Full article

Jaguars play a crucial role in population control across multiple biomes. They are endangered and protected by in situ and ex situ conservation mechanisms to ensure their conservation. Cardiovascular diseases in wild mammals, including jaguars, often have unclear etiopathogenies, underscoring the need for research into novel hemodynamic parameters. This study evaluates the cardiovascular health of fifteen clinically healthy jaguars using conventional and Holter electrocardiography, non-invasive systemic blood pressure measurement, and echocardiography. Chemical restraint was achieved with medetomidine (0.08–0.1 mg/kg) and ketamine (5 mg/kg), with anesthesia reversed using atipamezole (0.25 mg/kg). The average heart rate was 72 ± 18 bpm, with sinus rhythm in ten animals and sinus arrhythmia in five. Six animals exhibited first and second-degree atrioventricular blocks, one had supraventricular complexes, and another had premature ventricular complexes. Non-invasive systolic blood pressure remained stable at 163 ± 29 mmHg during anesthesia. Echocardiographic examination revealed mitral, tricuspid, pulmonary, and aortic valve insufficiencies via color Doppler. The transmitral flow showed a normal E/A ratio and E` < A`, suggesting a pseudonormal ventricular filling pattern. No significant anesthetic complications were observed, affirming the protocol’s safety. This study provides valuable data, validating the anesthetic protocol and establishing reference cardiovascular values for jaguars, thus paving the way for future research in other veterinary species. Full article

Background: The Fontan procedure has provided pediatric patients suffering from severe congenital heart disease the opportunity to reach adulthood. Increasingly, we encounter the liver repercussions of Fontan circulation, alongside a decline in heart function and exercise performance. This study aims to identify the univentricular heart malformations that are most susceptible to liver dysfunction; assess which markers of liver injury are essential for multidisciplinary clinical follow-up of Fontan patients; determine the optimal approach for evaluating liver function in Fontan patients; and explore how a congenital cardiology team can interpret the data and respond effectively to signs of organ failure. Methods: Cross-sectional clinical study including patients who underwent a Fontan procedure at the University Hospital of Padua between 1982 and 2017. Patients were admitted for elective hospitalization between June 2021 and June 2022 and underwent clinical assessment, laboratory tests, and instrumental examinations. Results: Seventy patients were included in the study. On admission, 48 patients (72%) were in New York Heart Association (NYHA) functional class I, and the cardiopulmonary exercise test was normal for age and gender. At laboratory tests, 56% of patients showed changes in NTproBNP values, most of whom had right-sided ventricular morphology. Liver function tests showed abnormal Gamma-Glutamyl Transferase (GGT) blood levels in 68%. On cardiac imaging, at least moderate atrioventricular valve insufficiency was found in 9% of cases. Fibroscan showed altered hepatic stiffness values in 25% of cases. Statistical analysis showed that systemic atrioventricular valve (SAVV) dysfunction was significantly associated with a reduction of maximum oxygen consumption (VO₂ max) and hepatic stiffness. Conclusions: SAVV dysfunction is significantly responsible for worse functional outcomes and the development of hepatic fibrosis due to an increase in venous congestion. Setting up a careful multidisciplinary follow-up in these patients is mandatory for early detection of complications, prompt treatment, and better outcomes. Full article

Complete atrioventricular (AV) block is a severe conduction abnormality caused by intrinsic cardiac disease, ischemia, electrolyte imbalances, or drug interactions. Elderly patients on multiple medications are particularly vulnerable to polypharmacy-related interactions. This case report describes an 82-year-old female presenting to the emergency department with fatigue, syncope, and disorientation. Her medical history included atrial fibrillation, hypertension, and heart failure, with a medication regimen of digoxin 0.25 mg given daily 5 days out of 7, metoprolol 50 mg twice daily, lisinopril 10 mg daily, furosemide 40 mg daily, and spironolactone 50 mg daily. Clinical examination revealed bradycardia and a holosystolic murmur in the mitral valve area, while the electrocardiogram showed complete AV block at a ventricular rate of 35 bpm. Laboratory results indicated mild hyperkalemia (4.9 mmol/L). Suspecting a digoxin–beta-blocker interaction, antiarrhythmic therapy was discontinued. Within three days, the AV block resolved, transitioning to atrial fibrillation with a high ventricular rate. Bisoprolol was introduced for rate control, and hemodynamic stability was achieved. The patient was discharged with a revised medication regimen and showed no recurrence of AV block. This case emphasizes the importance of recognizing drug interactions as a reversible cause of AV block and using drug interaction checkers to manage polypharmacy, especially in elderly patients with multiple comorbidities. It also highlights the rare and paradoxical combination of atrial flutter and complete AV block. Full article

Trypanosoma cruzi, the etiological agent of Chagas disease, is a parasite known for its diverse genotypic variants, or Discrete Typing Units (DTUs), which have been associated with varying degrees of tissue involvement. However, aspects such as parasite attachment remain unclear. It has been suggested that the TcI genotype is associated with cardiac infection, the most common involved site in chronic human infection, while TcII is associated with digestive tract involvement. Traditional models for T. cruzi infection provide limited in vivo observation, making it challenging to observe the dynamics of parasite-host interactions. This study evaluates the cardiac attachment of trypomastigotes from TcI and TcII DTUs in zebrafish larvae. Labeled trypomastigotes were injected in the duct of Cuvier of zebrafish larvae and tracked by stereomicroscopy and light-sheet fluorescence microscopy (LSFM). Remarkably, it was possible to observe TcI parasites adhered to the atrium, atrioventricular valve, and circulatory system, while TcII trypomastigotes demonstrated adhesion to the atrium, atrioventricular valve, and yolk sac extension. When TcI and TcII were simultaneously injected, they both attached to the heart; however, more of the TcII trypomastigotes were observed attached to this organ. Although TcII DTU has previously been associated with digestive tissue infection, both parasite variants showed cardiac tissue attachment in this in vivo model. Full article

Pectus excavatum (PE) can be associated with either congenital or acquired heart disease. This study highlights the importance of PE surgical repair in cases of severe chest depression on the heart in underlying cardiac diseases exacerbating cardiopulmonary impairment. From January 2023 to March 2024, four male patients underwent PE repair, having heart disease including pericarditis, mitral valve prolapse, ventricular fibrillation arrest and type 1 second-degree atrioventricular block. PE severity was determined by the Haller index (HI). Preoperative assessment included a pulmonary function test, chest computed tomography and cardiac evaluation. The Nuss procedure was performed in three patients, whereas, in one patient, it was performed in combination with a modified Ravitch procedure. The median HI was five. The median time of chest tube removal was 6.5 days. Postoperative complications were prolonged air leak, atrial fibrillation and atelectasis. The median length of hospital stay was 19.5 days, and no 30-day postoperative mortality was recorded. In all patients, surgical repair helped to resolve the underlying cardiological issues, and surgical follow-ups were deemed regular. PE is generally an isolated congenital chest wall abnormality, and, when associated with a heart disease, it can have severe life-threatening hemodynamic consequences due to mechanical compression on the heart for which surgical corrections should be considered. Full article

Background: Pregnancy in women with biventricular circulation and a systemic right ventricle (sRV) is considered high risk, with limited data available on pregnancy outcomes. This study aimed to investigate pregnancy outcomes in this population. Materials and Methods: A systematic review was conducted using four major electronic databases. Pregnant women with a complete transposition of great arteries (d-TGA) after an atrial switch operation or a congenitally corrected transposition of the great arteries (ccTGA) were included. Results: In total, 15 studies including 632 pregnancies in 415 women with an sRV and biventricular circulation were identified, of whom 299 (72%) had d-TGA and 116 (28%) ccTGA. Maternal mortality or cardiac transplantation occurred in 0.8% of pregnancies. The most frequent maternal complications were the worsening of systemic atrioventricular valve regurgitation [pooled estimate (PE): 16%, 95% CI: 5;26], the deterioration of sRV function (PE: 15%, 95% CI: 2;27), the worsening of the NYHA class (PE: 13%, 95% CI: 6;20), all-cause hospitalization (PE): 10%, 95% CI: 7;12), arrhythmias (PE: 8%, 95% CI: 5;11), and symptomatic heart failure (PE: 6%, 95% CI: 3;10). Stillbirth occurred in 0.7% of pregnancies and neonatal death in 0.4%. Small-for-gestational-age neonates were encountered in 36% (95% CI: 21;52) of pregnancies and preterm delivery in 22% (95% CI: 14;30). A subgroup analysis showed no significant difference in outcomes between women with d-TGA and those with ccTGA, except for the worsening of the NYHA class, which occurred more often in d-TGA (18%, 95% CI: 12;27 vs. 6%, 95% CI: 3;15, respectively, p = 0.03). Conclusions: Maternal and fetal/neonatal mortality are low among pregnant women with biventricular circulation and an sRV. However, significant maternal morbidity and poor neonatal outcomes are frequently encountered, rendering management in specialized centers imperative. Full article

Congenitally corrected transposition of the great arteries (ccTGA) is an infrequent and complex congenital malformation, which accounts for approximately 0.5% of all congenital heart defects. This defect is characterized by both atrioventricular and ventriculoarterial discordance, with the right atrium connected to the morphological left ventricle (LV), ejecting blood into the pulmonary artery, while the left atrium is connected to the morphological right ventricle (RV), ejecting blood into the aorta. Due to this double discordance, the blood flow is physiologically normal. Most patients have coexisting cardiac abnormalities that require further treatment. Untreated natural course is often associated with progressive failure of the systemic right ventricle (RV), tricuspid valve (TV) regurgitation, arrhythmia, and sudden cardiac death, which occurs in approximately 50% of patients below the age of 40. Some patients do not require surgical intervention, but most undergo physiological repair leaving the right ventricle in the systemic position, anatomical surgery which restores the left ventricle as the systemic ventricle, or univentricular palliation. Various types of anatomic repair have been proposed for the correction of double discordance. They combine an atrial switch (Senning or Mustard procedure) with either an arterial switch operation (ASO) as a double-switch operation or, in the cases of relevant left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD), intra-ventricular rerouting by a Rastelli procedure. More recently implemented procedures, variations of aortic root translocations such as the Nikaidoh or the half-turned truncal switch/en bloc rotation, improve left ventricular outflow tract (LVOT) geometry and supposedly prevent the recurrence of LVOTO. Anatomic repair for congenitally corrected ccTGA has been shown to enable patients to survive into adulthood. Full article

Background: Valve-in-Valve (VIV) transcatheter aortic valve replacement (TAVR) is a potential solution for malfunctioning surgical aortic valve prostheses, though limited data exist for its use in Perceval valves. Methods: searches were performed on PubMed and Scopus up to 31 July 2023, focusing on case reports and series addressing VIV replacement for degenerated Perceval bioprostheses. Results: Our analysis included 57 patients from 27 case reports and 6 case series. Most patients (68.4%) were women, with a mean age of 76 ± 4.4 years and a mean STS score of 6.1 ± 4.3%. Follow-up averaged 9.8 ± 8.9 months, the mean gradient reduction was 15 ± 5.9 mmHg at discharge and 13 ± 4.2 mmHg at follow-up. Complications occurred in 15.7% of patients, including atrioventricular block III in four patients (7%), major bleeding or vascular complications in two patients (3.5%), an annular rupture in two patients (3.5%), and mortality in two patients (3.5%). No coronary obstruction was reported. Balloon-expanding valves were used in 61.4% of patients, predominantly the Sapien model. In the self-expanding group (38.6%), no valve migration occurred, with a permanent pacemaker implantation rate of 9%, compared to 5.7% for balloon-expanding valves. Conclusions: VIV-TAVR using both balloon-expanding and self-expanding technologies is feasible after the implantation of Perceval valves; however, it should be performed by experienced operators with experience both in TAVR and VIV procedures. Full article

Background/Objective: Transcatheter aortic valve replacement (TAVR) is indicated for severe aortic stenosis patients with a prohibitive surgical risk. However, its use has been expanding in recent years to include intermediate- and low-risk patients. Thus, registry data describing changes in patient characteristics and outcomes are needed. The aim of this study was to analyse the temporal changes in patient profiles and clinical outcomes of all-comer TAVR. Methods: Baseline characteristics and VARC-3 outcomes of 1632 consecutive patients undergoing TAVR between 2008 and 2021 were analysed. Results: The annual rate of TAVR increased from 30 procedures in 2008–2009 to 398 in 2020–2021. Over the follow-up period, patient age decreased from 85 ± 4 to 80 ± 6.8 (p < 0.001) and the STS score decreased from 5.9% to 2.8% (p < 0.001). Procedural characteristics significantly changed, representing a shift into a minimally invasive approach: adoption of local anaesthesia (none to 48%, p < 0.001) and preference of transfemoral access (74% in 2011–2012 vs. 94.5% in 2020–2021, p < 0.001). The rates of almost all procedural complications decreased, including major vascular and bleeding complications, acute kidney injury (AKI) and in-hospital heart failure. There was a striking decline in rates of complete atrioventricular block (CAVB) and the need for a permanent pacemaker (PPM). PPM rates, however, remain high (17.8%). Thirty-day and one-year mortality significantly declined to 1.8% and 8.3%, respectively. Multivariable analysis shows that AKI, bleeding and stroke are strong predictors of one-year mortality (p < 0.001). Conclusions: The TAVR procedure has changed dramatically during the last 14 years in terms of patient characteristics, procedural aspects and device maturity. These shifts have led to improved procedural safety, contributing to improved short- and long-term patient outcomes. Full article