ABSTRACT
Evidence suggests an association between obesity and the risk for cardiomyopathy development; however, robust evidence is still lacking. In this study we sought to explore the relationship of obesity with hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) and possible interactions with sex using large-scale epidemiological real-world data. We analysed data from the Nationwide Inpatient Sample of US hospitalisations for the years 2015-2019. There were a total of 46 934 admissions with diagnosis of HCM and 170 924 with DCM. There was a significant interaction between cardiomyopathies' diagnosis with sex and age subgroups; the rates of both DCM and HCM increased with age (p < .001 for both); DCM diagnosis was significantly higher in males compared with females (0.85% vs. 0.35%, p < .001). After adjustment for age, sex, race and presence of arterial hypertension there was a significant stepwise positive association between obesity and the population rates of both cardiomyopathy subtypes. For hospitalised patients with a body mass index (BMI) ≥30 kg/m2 there was an odds ratio (OR) of 1.68 (95% CI: 1.55-1.81, p < .001) for HCM and OR = 1.82 (95% CI: 1.79-1.84, p < .001) for DCM. More importantly, the positive relationship between a cardiomyopathy diagnosis (HCM or DCM) with increasing BMI was driven by the male sex (p < .001 for both) and it was non-significant in females. The findings from this nationwide observational analysis support a sexual dimorphism in the relationship between obesity and HCM or DCM, which should be further investigated.
ABSTRACT
Pericardial effusions, especially large ones, have traditionally been regarded with concern by clinicians due to the sometimes unpredictable development of life-threatening cardiac tamponade. In the European Society of Cardiology Guidelines on pericardial diseases, the simplified algorithm for pericardial effusion triage and management recommends pericardial drainage in cases of cardiac tamponade and/or suspicion of bacterial or neoplastic etiology. In the presence of acute pericarditis, empiric anti-inflammatory treatment should be given, while when a specific indication known to be associated with pericardial effusion is found, then treatment of the underlying cause is indicated. Notably, the most challenging subgroup of patients includes those with large, asymptomatic, C-reactive-protein-negative, idiopathic effusions. In the latter subjects, pericardial drainage is proposed in cases of chronic effusions (lasting more than three months). However, this recommendation is based on scant data stemming from small-sized non-randomized studies. Nevertheless, recent evidence in a larger cohort of patients pointed out that a watchful waiting strategy is a safe option in terms of complication-free survival. This review summarizes the contemporary evidence on this challenging topic and provides recommendations for tailoring individual patient treatments.
ABSTRACT
Acute myocardial infarction (AMI) usually represents the clinical manifestation of atherothrombotic coronary artery disease (CAD) resulting from atherosclerotic plaque rupture. However, there are cases in which coronary angiography or coronary computed tomography angiography reveals patients with acute coronary syndrome with non-obstructive CAD. This clinical entity is defined as myocardial infarction with non-obstructive coronary arteries (MINOCA) and often considered as a clinical dynamic working diagnosis that needs further investigations for the establishment of a final etiologic diagnosis. The main causes of a MINOCA working diagnosis include atherosclerotic, non-atherosclerotic (vessel-related and non-vessel-related), and thromboembolic causes This literature review aimed to investigate the major thromboembolic causes in patients presenting with MINOCA regarding their etiology and pathophysiologic mechanisms, as well as diagnostic and treatment methods.
ABSTRACT
Recurrent pericarditis is a problematic clinical condition that impairs the quality of life of the affected patients due to the need for repeated hospital admissions, emergency department visits, and complications from medications, especially glucocorticoids. Unfortunately, available treatments for recurrent pericarditis are very limited, including only a handful of medications such as aspirin/NSAIDs, glucocorticoids, colchicine, and immunosuppressants (such as interleukin-1 (IL-1) blockers, azathioprine, and intravenous human immunoglobulins). Until recently, the clinical experience with the latter class of medications was very limited. Nevertheless, in the last decade, experience with IL-1 blockers has consistently grown, and valid clinical data have emerged from randomized clinical trials. Accordingly, IL-1 blockers are a typical paradigm shift in the treatment of refractory recurrent pericarditis with a clearly positive cost/benefit ratio for those unfortunate patients with multiple recurrences. A drawback related to the above-mentioned medications is the absence of universally accepted and established treatment protocols regarding the full dose administration period and the need for a tapering protocol for individual medications. Another concern is the need for long-standing treatments, which should be discussed with the patients. The above-mentioned unmet needs are expected to be addressed in the near future, such as further insights into pathophysiology and an individualized approach to affected patients.
ABSTRACT
Background: Pericardial effusion is common in pregnancy, with causes similar to the general population. Usually, it is found in the third trimester and disappears spontaneously after labour; however, there is a risk of progression to tamponade. Management is based on expert opinion, since few studies have been published. Case summary: A woman with enlargement of a known, chronic, presumably idiopathic pericardial effusion, in the 17th gestation week, presented with mild dyspnoea, without specific echocardiographic signs of cardiac tamponade. She received double antithrombotic treatment with aspirin 100â mg, started before conception, and a prophylactic dose of tinzaparin 4500â IU, started at the beginning of the pregnancy due to obstetrical antiphospholipid syndrome. A multidisciplinary team consisting of the treating obstetrician-gynaecologist, haematologist, cardiothoracic surgeon, and cardiologist discussed the management, taking into account the large size of the effusion and the significant increase during pregnancy, the possibility of further increase during the third trimester, the antiplatelet and antithrombotic treatment, which increased the haemorrhagic risk, and the difficulty and risk to intervene later in pregnancy. A surgical pericardial window was proposed to the patient and family and was performed uneventfully. Discussion: This case demonstrates the importance of a multidisciplinary team approach and shared decision-making in the management of these complex cardio-obstetric patients in order to achieve optimal therapeutic results.
ABSTRACT
AIM: Anakinra, an anti IL-1 agent targeting IL-1 alfa and beta, is available for the treatment of recurrent pericarditis in cases with corticosteroid dependence and colchicine resistance after failure of conventional therapies. However, it is unclear if the combination with colchicine, a non-specific inhibitor of the inflammasome targeting the same inflammatory pathway of IL-1, could provide additional benefit to prevent further recurrences. The aim of the present observational study is to assess whether the addition of colchicine on top of anakinra could prolong the time to first recurrence and prevent recurrences better than anakinra alone. METHODS: International, all-comers, multicentre, retrospective observational cohort study analysing all consecutive patients treated with anakinra for corticosteroid-dependent and colchicine-resistant recurrent pericarditis. The efficacy endpoint was recurrence rate and the time to the first recurrence. RESULTS: A total of 256 patients (mean age 45.0±15.4 years, 65.6% females, 80.9% with idiopathic/viral aetiology) were included. 64 (25.0%) were treated with anakinra as monotherapy while 192 (75.0%) with both anakinra and colchicine. After a follow-up of 12 months, 56 (21.9%) patients had recurrences. Patients treated with colchicine added to anakinra had a lower incidence of recurrences (respectively, 18.8% vs 31.3%; p=0.036) and a longer event-free survival (p=0.025). In multivariable analysis, colchicine use prevented recurrences (HR 0.52, 95% CI 0.29 to 0.91; p=0.021). CONCLUSIONS: The addition of colchicine on top of anakinra treatment could be helpful to reduce recurrences and prolong the recurrence-free survival.
Subject(s)
Interleukin 1 Receptor Antagonist Protein , Pericarditis , Female , Humans , Adult , Middle Aged , Male , Interleukin 1 Receptor Antagonist Protein/adverse effects , Retrospective Studies , Colchicine/adverse effects , Adrenal Cortex Hormones , Pericarditis/diagnosis , Pericarditis/drug therapy , Pericarditis/chemically induced , Interleukin-1ABSTRACT
Dilated cardiomyopathy (DCM) is a common heart muscle disorder of nonischemic etiology associated with heart failure development and the risk of malignant ventricular arrhythmias and sudden cardiac death. A tailored approach to risk stratification and prevention of sudden cardiac death is required in genetic DCM given its variable presentation and phenotypic severity. Currently, advances in cardiogenetics have shed light on disease mechanisms, the complex genetic architecture of DCM, polygenic contributors to disease susceptibility and the role of environmental triggers. Parallel advances in imaging have also enhanced disease recognition and the identification of the wide spectrum of phenotypes falling under the DCM umbrella. Genotype-phenotype associations have been also established for specific subtypes of DCM, such as DSP (desmoplakin) or FLNC (filamin-C) cardiomyopathy but overall, they remain elusive and not readily identifiable. Also, despite the accumulated knowledge on disease mechanisms, certain aspects remain still unclear, such as which patients with DCM are at risk for disease progression or remission after treatment. Imagenetics, that is, the combination of imaging and genetics, is expected to further advance research in the field and contribute to precision medicine in DCM management and treatment. In the present article, we review the existing literature in the field, summarize the established knowledge and emerging data on the value of genetics and imaging in establishing genotype-phenotype associations in DCM and in clinical decision making for DCM patients.
Subject(s)
Cardiomyopathy, Dilated , Humans , Cardiomyopathy, Dilated/diagnosis , Cardiomyopathy, Dilated/genetics , Cardiomyopathy, Dilated/therapy , Precision Medicine/methods , Death, Sudden, Cardiac/etiology , Arrhythmias, Cardiac/genetics , Genetic Association StudiesABSTRACT
BACKGROUND: Hypertrophic Cardiomyopathy (HCM) is characterized by myocardial hypertrophy, fibrosis, and sarcomeric disarray. OBJECTIVE: To evaluate the expression levels of circulating miR-21 and -29 in patients with HCM and their association with clinical characteristics and myocardial fibrosis. METHODS: In this case-control study, 27 subjects with HCM, 13 subjects with hypertensive cardiomyopathy, and 10 control subjects were enrolled. Evaluation of patients' functional capacity was made by the six-minute walk test. Echocardiographic measurements of left ventricle systolic and diastolic function were conducted. Cardiac magnetic resonance late gadolinium enhancement (LGE) -through a semiquantitative evaluation- was used in the assessment of myocardial fibrosis extent in HCM patients. The expression of miR-21 and -29 in peripheral blood samples of all patients was measured via the method of quantitative reverse transcription polymerase chain reaction. RESULTS: Circulating levels of miR-21 were higher in both hypertensive and HCM (p<0.001) compared to controls, while expression of miR-29 did not differ between the three studied groups. In patients with HCM and LGE-detected myocardial fibrosis in more than 4 out of 17 myocardial segments, delta CT miR-21 values were lower than in patients with myocardial LGE in 3 or fewer myocardial segments (2.71 ± 1.06 deltaCT vs. 3.50 ± 0.55 deltaCT, p<0.04), indicating the higher expression of circulating miR-21 in patients with more extensive myocardial fibrosis. CONCLUSION: MiR-21 was overexpressed in patients with HCM and hypertensive cardiomyopathy. Importantly, in patients with HCM, more extensive myocardial fibrosis was associated with higher levels of miR-21.
Subject(s)
Cardiomyopathy, Hypertrophic , Fibrosis , MicroRNAs , Humans , MicroRNAs/blood , MicroRNAs/genetics , Cardiomyopathy, Hypertrophic/blood , Cardiomyopathy, Hypertrophic/pathology , Cardiomyopathy, Hypertrophic/genetics , Male , Middle Aged , Female , Case-Control Studies , Adult , Myocardium/pathology , Myocardium/metabolism , EchocardiographyABSTRACT
INTRODUCTION: The management of even large pericardial effusions in asymptomatic patients is still a matter of debate. Aim of the present study is to explore, in a multicenter setting, the rate of post-cardiac injury syndromes (PCIS) and pericardial effusion recurrence after pericardial effusion drainage procedure. MATERIAL AND METHODS: This is a multicenter international retrospective study including a consecutive cohort of patients diagnosed with large, chronic and idiopathic pericardial effusions, prospectively evaluated from January 2003 to December 2021 who underwent a clinically indicated pericardial drainage procedure. Two separate end-points were recorded: 1) recurrence of pericardial effusion after drainage without any sign of pericardial inflammation 2) occurrence of PCIS, defined as the new onset of pericarditis 1 to 6 weeks after pericardial intervention. RESULTS: 124 patients were enrolled (50 % female, mean age 64 years old). A mean follow-up of 29.6 ± 25.6 months was obtained in 110 patients (88 %). 110 patients were treated with pericardiocentesis (89 %), 25 with pleuro-pericardial windows (20 %), and 1 with pericardiectomy (1 %). PCIS occurred in 21 out of 124 patients followed for at least 6 weeks (16.9%). Recurrence of pericardial effusion after drainage without any sign of pericardial inflammation occurred in 68 out of 110 patients at a longer follow-up (61.8 %). At multivariate analysis only inflammatory cells in pericardial fluid was associated with PCIS and pericardiocentesis with pericardial effusion recurrency. CONCLUSION: Our data support the need of caution with the use of pericardiocentesis in asymptomatic patients with large pericardial effusion as it is often associated with pericardial effusion recurrence. Of interest the presence of inflammatory cells in the pericardial fluid is associated with PCIS after pericardial drainage procedures.
Subject(s)
Drainage , Pericardial Effusion , Pericardiocentesis , Recurrence , Humans , Pericardial Effusion/etiology , Female , Male , Middle Aged , Retrospective Studies , Aged , Pericarditis/etiology , Pericardial Window Techniques , Pericardiectomy , Heart Injuries/complicationsABSTRACT
Acute coronary syndromes (ACSs) encompass a spectrum of life-threatening cardiovascular conditions, including unstable angina (UA) and myocardial infarction. While significant progress has been made in the understanding and management of ACS over the years, it has become increasingly evident that sex-based differences play a pivotal role in the pathophysiology, presentation, and outcomes of these conditions. Despite this recognition, the majority of clinical research in the field has historically focused on male populations, leading to a significant knowledge gap in understanding the unique aspects of ACS in women. This review article aims to comprehensively explore and synthesize the current body of literature concerning the sex-specific characteristics of ACS, shedding light on the epidemiology, risk factors, clinical presentation, diagnostic challenges, treatment strategies, and prognosis in women. By elucidating the distinct aspects of ACS in women, this review intends to foster greater awareness and improved clinical management, ultimately contributing to enhanced cardiovascular care for female patients.
ABSTRACT
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy. The hallmark of HCM is myocardial fibrosis which contributes to heart failure, arrhythmias, and sudden cardiac death (SCD). OBJECTIVE: To identify the factors implicated in heart failure symptoms and functional capacity of patients with HCM. METHODS: In this cohort study, 43 patients with HCM were recruited. According to functional capacity and symptoms presentation, patients were categorized according to New York Heart Association (NYHA) classification, and echocardiographic measurements of left ventricle systolic and diastolic function were conducted. The echocardiographic assessment of right ventriculo-arterial coupling (RVAC) was made by calculating the tricuspid annular peak systolic tissue Doppler velocity (TASV)/estimated RV systolic pressure (RVSP) ratio. RESULTS: Almost half (51%) of our study population present symptoms of heart failure and were categorized as the symptomatic group-NYHA 2 or higher. Maximum LVOT gradient, RVSP, and the ratio of E/e' were higher in the symptomatic group compared with the asymptomatic group. TASV was lower in the symptomatic group compared with the asymptomatic group (11 ± 1 cm/s vs. 13 ± 2 cm/s, p = 0.04). However, there was no difference in other potentially influential factors, such as heart rate or systemic blood pressure. The SCD risk score does not differ between the two studied groups. The RVAC (estimated with the TASV/RVSP ratio) was lower in the symptomatic group compared with the asymptomatic group (0.32 ± 0.09 vs. 0.46 ± 0.11, p < 0.001). CONCLUSION: A low RVAC (as estimated with TASV/RVSP ratio) value could represent an echocardiographic marker of right ventricular-arterial uncoupling in patients with HCM and impaired functional status.
ABSTRACT
Endothelial dysfunction (ED) is characterized by imbalanced vasodilation and vasoconstriction, elevated reactive oxygen species (ROS), and inflammatory factors, as well as deficiency of nitric oxide (NO) bioavailability. It has been reported that the maintenance of endothelial cell integrity serves a significant role in human health and disease due to the involvement of the endothelium in several processes, such as regulation of vascular tone, regulation of hemostasis and thrombosis, cell adhesion, smooth muscle cell proliferation, and vascular inflammation. Inflammatory modulators/biomarkers, such as IL-1α, IL-1ß, IL-6, IL-12, IL-15, IL-18, and tumor necrosis factor α, or alternative anti-inflammatory cytokine IL-10, and adhesion molecules (ICAM-1, VCAM-1), involved in atherosclerosis progression have been shown to predict cardiovascular diseases. Furthermore, several signaling pathways, such as NLRP3 inflammasome, that are associated with the inflammatory response and the disrupted H2S bioavailability are postulated to be new indicators for endothelial cell inflammation and its associated endothelial dysfunction. In this review, we summarize the knowledge of a plethora of reviews, research articles, and clinical trials concerning the key inflammatory modulators and signaling pathways in atherosclerosis due to endothelial dysfunction.
ABSTRACT
OBJECTIVES: The impact of plasma biomarkers on diagnosis and prognosis of patients with acute pericarditis (AP) has been poorly investigated. This study aims to assess the diagnostic and prognostic role of d-dimer (DD), an easily obtainable biomarker, in patients with AP. PATIENTS AND METHODS: This is a prospective clinical study enrolling 265 consecutive patients hospitalized between September 2010 and May 2019 with a first episode of AP. At baseline, demographics, clinical features, laboratory and imaging findings were recorded. All patients were followed-up for a minimum of 18 months. Endpoints included cardiac tamponade, new-onset atrial fibrillation, pericardial drainage, recurrent/constrictive pericarditis and death. RESULTS: DD was measured in 165 out of 265 patients (62.3%, median levels 1456 ng/mL) Among them, 121 patients (73.3%) presented with elevated age-adjusted DD levels. Patients with elevated DD depicted a higher rate of pleural (69.4%, vs 38.6%, p<0.001) and pericardial effusions (89.3% vs 72.7%, p = 0.009). Elevated DD correlated with admission (rho=0.37) and peak (rho=0.36) C-reactive protein values. Patients with elevated DD depicted a trend towards a greater prevalence of pericardial tamponade vs those without (14.9% vs 4.5% respectively, p = 0.07). In the 43.8% of patients with elevated DD who underwent computed tomography pulmonary angiography (CTPA), no case of pulmonary embolism or aortic syndrome was unveiled. CONCLUSION: DD elevation is detected in the majority of AP cases at presentation and may herald cardiac tamponade. In patients with chest pain not attributable to alternative causes, elevated DD denotes an inflammatory condition and should not prompt unnecessary investigations, such as CTPA.
Subject(s)
Cardiac Tamponade , Pericarditis , Humans , Cardiac Tamponade/diagnosis , Cardiac Tamponade/etiology , Prognosis , Prospective Studies , Pericarditis/diagnosis , BiomarkersABSTRACT
Cardiac magnetic resonance (CMR) imaging has been established as a valuable diagnostic tool in the assessment of pericardial diseases by providing information on cardiac anatomy and function, surrounding extra-cardiac structures, pericardial thickening and effusion, characterization of pericardial effusion, and the presence of active pericardial inflammation from the same scan. In addition, CMR imaging has excellent diagnostic accuracy for the non-invasive detection of constrictive physiology evading the need for invasive catheterization in most instances. Growing evidence in the field suggests that pericardial enhancement on CMR is not only diagnostic of pericarditis but also has prognostic value for pericarditis recurrence, although such evidence is derived from small patient cohorts. CMR findings could also be used to guide treatment de-escalation or up-titration in recurrent pericarditis and selecting patients most likely to benefit from novel treatments such as anakinra and rilonacept. This article is an overview of the CMR applications in pericardial syndromes as a primer for reporting physicians. We sought to provide a summary of the clinical protocols used and an interpretation of the major CMR findings in the setting of pericardial diseases. We also discuss points that are less well clear and delineate the strengths and weak points of CMR in pericardial diseases.
Subject(s)
Pericardial Effusion , Pericarditis, Constrictive , Pericarditis , Humans , Magnetic Resonance Imaging/methods , Pericardial Effusion/diagnostic imaging , Pericarditis/diagnostic imaging , Pericarditis, Constrictive/diagnostic imaging , Pericardium/pathologyABSTRACT
BACKGROUND: Several investigations have highlighted the role of water quality in cardiovascular health. In the present study, we have investigated the effects of drinking water hardness on atherosclerotic burden in carotid arteries and arterial stiffness. METHODS: "Corinthia" study was conducted in the homonym region in Greece from 2015 to 2017. Carotid atherosclerosis was assessed by intima-media thickness (IMT). Pulse wave velocity (PWV) was used to evaluate arterial stiffness. Tap-water samples were collected from the study area and analyzed for a variety of elements, as well as pH and total hardness. RESULTS: Individuals living in lower drinking water hardness areas (Area 1) versus individuals living in higher water hardness areas (Area 2) had lower max IMT (p = 0.004) and were less susceptible to carotid plaque formation (p = 0.004). Interestingly, individuals over 65 years from Area 1 had lower mean IMT, max IMT, and less plaque formation (p < 0.001 for all). The mean value of PWV in the overall study population was below the 10 m/s cutoff, which defines arterial stiffness (9.15 ± 2.79 m/s). Nevertheless, a marginally higher rate of vascular stiffening was noted in Area 2 vs. Area 1 (16.2% and 12.8%, respectively, p = 0.048). CONCLUSIONS: According to this cross-sectional study's findings, a positive association between extra hard water and carotid atherosclerotic burden was found. However, the association with arterial stiffness is unclear and should be investigated further.
Subject(s)
Atherosclerosis , Carotid Artery Diseases , Drinking Water , Vascular Stiffness , Humans , Carotid Intima-Media Thickness , Pulse Wave Analysis , Cross-Sectional Studies , Hardness , Carotid Artery Diseases/epidemiology , Carotid Arteries/diagnostic imaging , Atherosclerosis/epidemiology , Atherosclerosis/etiology , Atherosclerosis/prevention & control , Risk FactorsABSTRACT
INTRODUCTION: Mining of electronic health record (EHRs) data is increasingly being implemented all over the world but mainly focuses on structured data. The capabilities of artificial intelligence (AI) could reverse the underusage of unstructured EHR data and enhance the quality of medical research and clinical care. This study aims to develop an AI-based model to transform unstructured EHR data into an organised, interpretable dataset and form a national dataset of cardiac patients. METHODS AND ANALYSIS: CardioMining is a retrospective, multicentre study based on large, longitudinal data obtained from unstructured EHRs of the largest tertiary hospitals in Greece. Demographics, hospital administrative data, medical history, medications, laboratory examinations, imaging reports, therapeutic interventions, in-hospital management and postdischarge instructions will be collected, coupled with structured prognostic data from the National Institute of Health. The target number of included patients is 100 000. Natural language processing techniques will facilitate data mining from the unstructured EHRs. The accuracy of the automated model will be compared with the manual data extraction by study investigators. Machine learning tools will provide data analytics. CardioMining aims to cultivate the digital transformation of the national cardiovascular system and fill the gap in medical recording and big data analysis using validated AI techniques. ETHICS AND DISSEMINATION: This study will be conducted in keeping with the International Conference on Harmonisation Good Clinical Practice guidelines, the Declaration of Helsinki, the Data Protection Code of the European Data Protection Authority and the European General Data Protection Regulation. The Research Ethics Committee of the Aristotle University of Thessaloniki and Scientific and Ethics Council of the AHEPA University Hospital have approved this study. Study findings will be disseminated through peer-reviewed medical journals and international conferences. International collaborations with other cardiovascular registries will be attempted. TRIAL REGISTRATION NUMBER: NCT05176769.
Subject(s)
Cardiovascular System , Electronic Health Records , Humans , Artificial Intelligence , Retrospective Studies , Research Design , Aftercare , Ecosystem , Patient Discharge , Multicenter Studies as TopicABSTRACT
Cardiovascular disease (CVD) is the leading cause of mortality worldwide [...].
Subject(s)
Cardiovascular Diseases , Humans , Nutritional Status , Nutrition Surveys , Risk FactorsABSTRACT
A few data exist on the differences of implantable aortic valve bio-prostheses. We investigate three generations of self-expandable aortic valves in terms of the outcomes. Patients undergoing transcatheter aortic valve implantation (TAVI) were allocated into three groups according to the valve type: group A (CoreValveTM), group B (EvolutTMR) and group C (EvolutTMPRO). The implantation depth, device success, electrocardiographic parameters, need for permanent pacemaker (PPM), and paravalvular leak (PVL) were assessed. In the study, 129 patients were included. The final implantation depth did not differ among the groups (p = 0.07). CoreValveTM presented greater upward jump of the valve at release (2.88 ± 2.33 mm vs. 1.48 ± 1.09 mm and 1.71 ± 1.35 mm, for groups A, B, and C, respectively, p = 0.011). The device success (at least 98% for all groups, p = 1.00) and PVL rates (67% vs. 58%, vs. 60% for groups A, B, and C, respectively, p = 0.64) did not differ. PPM implantation within 24 h (33% vs. 19% vs. 7% for groups A, B, and C, respectively, p = 0.006) and until discharge (group A: 38% vs. group B: 19% and group C: 9%, p = 0.005) was lower in the newer generation valves. Newer generation valves present better device positioning, more predictable deployment, and fewer rates of PPM implantation. No significant difference in PVL was observed.
ABSTRACT
Paediatric cardiomyopathies form a heterogeneous group of disorders characterized by structural and electrical abnormalities of the heart muscle, commonly due to a gene variant of the myocardial cell structure. Mostly inherited as a dominant or occasionally recessive trait, they might be part of a syndromic disorder of underlying metabolic or neuromuscular defects or combine early developing extracardiac abnormalities (i.e., Naxos disease). The annual incidence of 1 per 100,000 children appears higher during the first two years of life. Dilated and hypertrophic cardiomyopathy phenotypes share an incidence of 60% and 25%, respectively. Arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy, and left ventricular noncompaction are less commonly diagnosed. Adverse events such as severe heart failure, heart transplantation, or death usually appear early after the initial presentation. In ARVC patients, high-intensity aerobic exercise has been associated with worse clinical outcomes and increased penetrance in at-risk genotype-positive relatives. Acute myocarditis in children has an incidence of 1.4-2.1 cases/per 100,000 children per year, with a 6-14% mortality rate during the acute phase. A genetic defect is considered responsible for the progression to dilated cardiomyopathy phenotype. Similarly, a dilated or arrhythmogenic cardiomyopathy phenotype might emerge with an episode of acute myocarditis in childhood or adolescence. This review provides an overview of childhood cardiomyopathies focusing on clinical presentation, outcome, and pathology.