Left ventricular non-compaction: diagnosis and clinical management

ESC CardioMed ◽  
2018 ◽  
pp. 1509-1512
Author(s):  
Jens Mogensen ◽  
Torsten B Rasmussen
Keyword(s):  
Systolic Function ◽  
Heart Diseases ◽  
Left Ventricular ◽  
Barth Syndrome ◽  
Left Ventricular Cavity ◽  
Healthy Individuals ◽  
Familial Disease ◽  
The Family ◽  
History Of ◽  
The Individual

Left ventricular non-compaction (LVNC) is characterized by a specific morphological appearance of the myocardium with an inner non-compacted hypertrabeculated layer and deep recesses communicating with the left ventricular cavity, and an outer compacted myocardium. LVNC is a specific morphological finding and may be present in healthy individuals with apparently normal hearts and in patients with various cardiac and systemic conditions including X-linked Barth syndrome, cardiomyopathies, congenital heart diseases, and non-cardiac systemic diseases. Recent investigations have revealed that LVNC may appear as the sole manifestation of disease in carriers of genetic mutations associated with dilated and hypertrophic cardiomyopathies. Therefore, it is important to consider the possibility of familial disease when diagnosing LVNC and explore the family history of the patient. Clinical screening of relatives should be offered when familial disease is suspected or when LVNC remains unexplained. Anticoagulation should be considered when LVNC appears in patients with impaired systolic function of the left ventricle to avoid formation of thrombi and cardiac embolization following an assessment of the entire risk profile of the individual patient.

Left ventricular non-compaction: diagnosis and clinical management

ESC CardioMed ◽  
2018 ◽  
pp. 1509-1512
Author(s):  
Jens Mogensen ◽  
Torsten B Rasmussen
Keyword(s):  
Systolic Function ◽  
Heart Diseases ◽  
Left Ventricular ◽  
Barth Syndrome ◽  
Left Ventricular Cavity ◽  
Healthy Individuals ◽  
Familial Disease ◽  
The Family ◽  
History Of ◽  
The Individual

Left ventricular non-compaction (LVNC) is characterized by a specific morphological appearance of the myocardium with an inner non-compacted hypertrabeculated layer and deep recesses communicating with the left ventricular cavity, and an outer compacted myocardium. LVNC is a specific morphological finding and may be present in healthy individuals with apparently normal hearts and in patients with various cardiac and systemic conditions including X-linked Barth syndrome, cardiomyopathies, congenital heart diseases, and non-cardiac systemic diseases. Recent investigations have revealed that LVNC may appear as the sole manifestation of disease in carriers of genetic mutations associated with dilated and hypertrophic cardiomyopathies. Therefore, it is important to consider the possibility of familial disease when diagnosing LVNC and explore the family history of the patient. Clinical screening of relatives should be offered when familial disease is suspected or when LVNC remains unexplained. Anticoagulation should be considered when LVNC appears in patients with impaired systolic function of the left ventricle to avoid formation of thrombi and cardiac embolization following an assessment of the entire risk profile of the individual patient.

scholarly journals Sub-clinical Detection of Left Ventricular Myocardial Dysfunction in Valvular Heart Diseases: A State-of-the-Art Review in a Speckle Tracking Echocardiography and Myocardial Performance

2020 ◽  
pp. 1-13
Author(s):  
Galaleldin Nagib Elkilany ◽  
◽  
Sherif Baath Allah ◽  
Petras Lohana ◽  
◽  
...  
Keyword(s):  
Mitral Valve ◽  
Maximum Rate ◽  
State Of The Art ◽  
Systolic Function ◽  
Heart Diseases ◽  
Myocardial Dysfunction ◽  
Pressure Rise ◽  
Left Ventricular ◽  
Myocardial Performance ◽  
Valve Diseases

Purpose of the state-of-the-art review: Left ventricular (LV) global longitudinal strain (GLS) is recently recognized as a more sensitive measure of LV myocardial systolic function compared with LV ejection fraction (LVEF). In addition, left ventricular GLS , myocardial performance index (MPI) and maximum rate of LV pressure rise during isovolumetric contraction (LV dP/dtmax) are more reproducible than traditional assessment of LV systolic function by two dimensional echocardiography (2DE) LVEF. These underutilized techniques can detect preclinical myocardial dysfunction in patients who are at risk of LV failure in valvular-induced heart disease . Current guidelines for diagnosis and treatment of valvular heart disease (VHD) include LVEF as one of the parameters to take into consideration in the clinical decision-making. However, a large body of evidence has shown that left ventricular GLS, MPI and LV dP/dtmax have been classically considered as a sensitive marker of LV contractility and inotropic state. In turn GLS and myocardial performance may be a better prognosticator than LVEF in aortic and mitral valve heart diseases. This timely state-of-the-art review, appraised the evidence and role of GLS, MPI and dP/dT as clinical tools in patients with aortic and mitral valve disease. Recent findings: Left ventricular GLS has been shown to be prognostic in low-flow, low-gradient severe aortic stenosis with preserved LVEF. The role of left ventricular GLS, Tei index (MPI) and maximum rate of LV pressure rise (LV dP/dtmax) in patients with aortic regurgitation and mitral valve diseases (regurgitation and stenosis) is less well established. Summary: Echocardiography is considered the primary non-invasive imaging tool for valvular heart disease assessment and the cornerstone method in diagnosing and evaluating the morphology and severity of aortic and mitral valve diseases. Currently, diagnostic-cardiac catheterization is no more recommended except in very rare cases when echocardiographic image quality is suboptimal, non-diagnostic and when the results of 2DE are discrepant with clinical data. Once clinical decision-making is based on the 2DE and three dimensional echocardiographic in assessment of the severity of mitral and aortic valve diseases, it is crucial that standards should be adopted to maintain accuracy and consistency across echocardiographic laboratories. This illustrative review article assesses left ventricular systolic function (LVEF) employing two and/or three dimensional echocardiography in comparison to GLS, MPI and LV dP/dtmax, especially applied for aortic valve (AV) and mitral valve (MV) diseases. It is noteworthy that this document only provides echocardiographic standards rather than making recommendations for clinical management. Conclusion: It is concluded that GLS, MPI and maximum rate of LV pressure rise during isovolumetric contraction (LV dP/dtmax) are recommended and more so, they should be increasingly used to identify subclinical LV myocardial dysfunction in patients with mitral and aortic valve heart diseases, to identify optimal timing for surgery and prognosticate outcomes after surgery

scholarly journals Diastolic Cardiac Function by MRI—Imaging Capabilities and Clinical Applications

Tomography ◽  
2021 ◽  
Vol 7 (4) ◽  
pp. 893-914
Author(s):  
El-Sayed H. Ibrahim ◽  
Jennifer Dennison ◽  
Luba Frank ◽  
Jadranka Stojanovska
Keyword(s):  
Cardiac Function ◽  
Diastolic Dysfunction ◽  
Systolic Function ◽  
Heart Diseases ◽  
Left Ventricular ◽  
Clinical Applications ◽  
Mri Imaging ◽  
Spatial And Temporal Patterns ◽  
Myocardial Relaxation ◽  
Diastolic Cardiac Function

Most cardiac studies focus on evaluating left ventricular (LV) systolic function. However, the assessment of diastolic cardiac function is becoming more appreciated, especially with the increasing prevalence of pathologies associated with diastolic dysfunction like heart failure with preserved ejection fraction (HFpEF). Diastolic dysfunction is an indication of abnormal mechanical properties of the myocardium, characterized by slow or delayed myocardial relaxation, abnormal LV distensibility, and/or impaired LV filling. Diastolic dysfunction has been shown to be associated with age and other cardiovascular risk factors such as hypertension and diabetes mellitus. In this context, cardiac magnetic resonance imaging (MRI) has the capability for differentiating between normal and abnormal myocardial relaxation patterns, and therefore offers the prospect of early detection of diastolic dysfunction. Although diastolic cardiac function can be assessed from the ratio between early and atrial filling peaks (E/A ratio), measuring different parameters of heart contractility during diastole allows for evaluating spatial and temporal patterns of cardiac function with the potential for illustrating subtle changes related to age, gender, or other differences among different patient populations. In this article, we review different MRI techniques for evaluating diastolic function along with clinical applications and findings in different heart diseases.

scholarly journals The Updating in Satir’s Family Therapy Model

2017 ◽  
Vol 5 (1) ◽  
pp. 425
Author(s):  
Neşide Yıldırım
Keyword(s):  
Family Therapy ◽  
The United States ◽  
Communication Patterns ◽  
Self Confidence ◽  
Perspective Change ◽  
The People ◽  
The Family ◽  
The Status ◽  
History Of ◽  
The Individual

Virginia Satir (1916-1988) is one of the first experts who has worked in the field of family therapy in the United States. In 1951, she was one of the first therapists who has worked all members of the family as a whole in the same session. She has concentrated her studies on issues such as to increase individual's self-esteem and to understand and change other people's perspectives. She has tried to make problematic people compatible in the family and in the society through change. From this perspective, change and adaptation are the two important concepts of her model. This is a state of being and a way to communicate with ourselves and others. High self-confidence and harmony are the first primary indicator of being a more functional human. She starts her studies with identifying the family. She uses two ways to do this; the first one is the chronology of the family that is history of the family, the second one is the communication patterns within the family. With this, she updates the status of the family. Updating is the detection of the current situation. The detection of the situation, in other words updating, constitutes the very essence of the model that she implements. In this study, communication patterns within the family are discussed for the updating, the chronological structure has not been studied. The characteristics of family communication patterns, the model of therapy that is applied by Satir for these patterns and the method which is followed in the model are discussed. According to her detection, the people who face with problems, use one of those four patterns or a combination of them. These communication patterns are Blamer, Sedative/Accepting, distracter/irrelevant and rational. Satir expresses that these four patterns are not solid and unchanging but all of them “can be converted”. For example, if one of the family members is usually using the soothing (sedative/accepting) pattern, in this case, it means that he/she wants to give the message that he/she is not very important in the inner world of the individual itself. However, if such a communication pattern is to be used repeatedly by an individual, he/she must know how to use it. According to Satir, this consciousness may be converted to a conscious gentleness and sensitivity that is automatically followed to please everyone. This study was carried out by using the copy of Satir’s book, which was originally called “The Conjoint Family Therapy” and translated into Turkish by Selim Ali Yeniçeri as “Basic Family Therapy” and published in Istanbul by Beyaz Yayınları in 2016. It is expected that the study will provide support to the education of the students and family therapists.

scholarly journals Semiotics of apical hypertrophic cardiomyopathy in magnetic resonance Imaging

2020 ◽  
Vol 19 (2) ◽  
pp. 18-23
Author(s):  
I. E. Obramenko
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Left Ventricle ◽  
Heart Diseases ◽  
Fibrous Tissue ◽  
Left Ventricular ◽  
Left Ventricular Cavity ◽  
Resonance Imaging ◽  
Apical Hypertrophic Cardiomyopathy

Introduction. About 0.2 % of the adult population all over the world suffers from hypertrophic cardiomyopathy. Early and timely diagnosis of the apical form of hypertrophic cardiomyopathy remains an urgent medical problem, since the disease has a wide variability of clinical manifestations and often occurs asymptomatic or with symptoms of other heart diseases. Magnetic resonance imaging is an informative method of radiation diagnosis of hypertrophic cardiomyopathy. The aim is improving of radiology diagnostics in applying to the apical form of hypertrophic cardiomyopathy. Materials and methods. 98 patients with apical hypertrophic cardiomyopathy aged 19 to 76 years were еxamined. There were 48 men and 50 women. All subjects were examined by a cardiologist, all patients underwent electrocardiography and echocardiography, 45 patients underwent magnetic resonance imaging (MRI) of the heart. Results. In our study 13 patients had MRI determined the isolated form of apical form of hypertrophic cardiomyopathy, 32 had combined one. 42.2% of the patients with symmetrical hypertrophy of all apical segments had sawtoothed configuration of the LV revealed by MRI. The symptom of left ventricular cavity obliteration was determined in 19 patients. The symptom of LV cavity sequestration was determined in 5 subjects. 5 patients had an aneurysm on the top of the left ventricle, 1 – on the top of the right ventricle. Signs of left ventricular outflow tract obstruction were visualized in 2 patients, intraventricular obstruction at the level of the middle segments of the left ventricle was determined in 5 cases. Akinesis and hypokinesis were detected in areas of fibrous changes (n=21) or in areas of cardiosclerosis (n=2). In 17.8 % of subjects identified non-compacted myocardium, in 3 cases it was combined with apical form of hypertrophic cardiomyopathy. With contrast enhancement in 29 patients, foci (n=22) or zones (n=7) of pathological accumulation of contrast agent were determined, which indicated the replacement of myocardium with fibrous tissue.

scholarly journals Prevalence of non-acute ischaemic cardiomyopathy in a Pakistani cohort

Heart Asia ◽  
2019 ◽  
Vol 11 (1) ◽  
pp. e011043
Author(s):  
Muhammad Salman Ghazni ◽  
Saba Aijaz ◽  
Rehan Malik ◽  
Asad Z Pathan
Keyword(s):  
Stress Testing ◽  
Systolic Function ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Rank Test ◽  
Ventricular Ejection Fraction ◽  
Primary Indication ◽  
History Of ◽  
Artery Disease

Heart failure with reduced left ventricular ejection fraction (HFrEF) is a frequently encountered clinical scenario. Coronary angiography (CAG) is usually performed to assess obstructive epicardial coronary artery disease (CAD) and the resultant ischaemia as causes of HFrEF.ObjectivesTo determine the frequency of obstructive CAD (OCAD) in patients with HFrEF and its independent predictors and outcomes.MethodsRetrospective observational study in Tabba Heart Institute on patients who underwent CAG during the past 4 years. Patients with prior known CAD or revascularisation were excluded. OCAD was defined as per the criteria from Felker et al. Regression modelling was performed to evaluate the predictors of OCAD. Survival was compared between the groups using the log rank test.ResultsOut of 2235 patients who underwent CAG, 260 had HFrEF as a primary indication for CAG and, of these, 119 (45.8%) had OCAD. Major predictors of OCAD were age >50 years at presentation (OR 2.0, 95% CI 1.1 to 3.7), presence of chest pain (OR 4.3, 95% CI 2.3 to 8.1), family history of premature CAD (OR 2.8, 95% CI 1.3 to 5.9) and utilisation of non-invasive (NIV) stress testing before CAG (OR 3.6, 95% CI 1.8 to 7.1). Survival was significantly lower (log rank p<0.001) in patients with OCAD with no revascularisation compared with OCAD with revascularisation or those who had non-obstructive CAD, and the latter two groups had comparable survival.ConclusionsOCAD is detected in nearly half of the patients with reduced left ventricular systolic function undergoing CAG. Clinical judgement based on thorough history and use of NIV stress testing can help in appropriate patient selection for this test.

scholarly journals A case of takotsubo cardiomyopathy in a patient with anaphylaxis treated with epinephrine

2017 ◽  
Vol 4 (3) ◽  
pp. 66
Author(s):  
Guramrinder S Thind ◽  
Prashant Patel ◽  
Sandeep Patri ◽  
Yashwant Agrawal
Keyword(s):  
Cardiac Catheterization ◽  
Takotsubo Cardiomyopathy ◽  
Systolic Function ◽  
Stress Test ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Epinephrine Infusion ◽  
Ventricular Ejection Fraction ◽  
History Of ◽  
Nuclear Stress Test

Takotsubo cardiomyopathy (TTC) is a recently identified transient cardiomyopathy that is usually associated with emotional or physical stress. Catecholamine surge appears to be central to the mechanism of TTC. TTC in the setting of anaphylaxis is rare. We present a case of a 58-year-old female was referred from an outside hospital after being diagnosed with anaphylaxis. She received 0.3 mg epinephrine intramuscularly and 1 mg intravenously. Upon admission to our hospital, she was complaining of chest pain. EKG done in the outside hospital showed ST elevations in the anterior leads but the EKG done at our hospital was normal. She had to be intubated in view of impending airway obstruction. She was subsequently started on epinephrine infusion in the intensive care unit for hypotension. She was found to have elevated troponins that trended up. An echocardiogram performed on day 3 revealed a left ventricular ejection fraction of 25% and apical hypokinesis suggestive of TTC. Cardiac catheterization was initially deferred in view of a history of dye allergy. A nuclear stress test was done instead that revealed reversible ischemia in anteroseptal regions. Cardiac catheterization was performed eventually with pre-medication that showed a near-normal coronary circulation. A repeat echocardiogram performed 6 weeks after discharge showed normal systolic function.

P4658Left ventricular non-compaction, trait or cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Kayvanpour ◽  
F Sedaghat-Hamedani ◽  
W T Gi ◽  
O F Tugrul ◽  
A Amr ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Heart Diseases ◽  
Pathological Condition ◽  
Meta Analysis ◽  
Positive Family History ◽  
Left Ventricular ◽  
Healthy Individuals ◽  
Comprehensive Overview ◽  
Ebstein Anomaly ◽  
Wide Range

Abstract Background The diagnosis of cardiac hypertrabecularization has increased considerably in recent years. Whether or not the non-compaction is a pathological condition or a physiological trait is still highly debated. We performed a meta-analysis and systematic review on more than 7,000 adult individuals with left ventricular non-compaction to provide a comprehensive overview on its clinical outcome as well as its genetic background. Methods We first retrieved PubMed/Medline literatures in English language between 2000 to 2018 on clinical outcome and genotype of patients with non-compaction. Altogether, 35 studies with non-compaction cardiomyopathy patients passed the selection criteria and were extensively reviewed and meta-analyzed. Furthermore, we summarized the results of 8 major studies, which investigated the non-compaction in athletes, pregnant women, patients with sickle cell disease, or in individuals from population-based cohorts. Results About 60% of the patients with left-ventricular non-compaction cardiomyopathy were male. The diagnosis was mostly made in the mid of patients' 5th decade. Seven percent of patients had congenital heart diseases (CHD) including atrial/ventricular septum defect or Ebstein anomaly. Around 25% of the patients had positive family history for cardiomyopathy. Frequent phenotypic manifestations were heart rhythm abnormalities including conduction disease (26%), supraventricular tachycardia (17%), and sustained or non-sustained ventricular tachycardia (18%). Neuromuscular disease was a reported comorbidity with a mean frequency of 5%. Three important outcome measures including systemic thromboembolic events (9%), heart transplantation (4%), and adequate ICD therapy (15%) were reported. The genetics of non-compaction cardiomyopathy showed TTN to be the most frequently mutated gene (11%), followed by MYH7 (9%), MYBPC3 (5%), and CASQ2, LDB3 (3% each). TPM1, MIB1, ACTC1, and LMNA mutations had an average frequency of 2% each followed by PLN, HCN4, TAZ, DTNA, TNNT2, and RBM20 (1% each). Eight studies that investigated the occurrence of non-compacted myocardial regions in apparently heart healthy individuals applied different, established imaging-based diagnostic criteria for non-compaction and could confirm its presence in a wide range of 1.3% to 37%. Conclusion This meta-analysis summarizes the clinical presentation of left ventricular non-compaction in a large dataset and indicates that its presence often leads to unfavourable outcome, but can also be observed in heart healthy individuals. Multimodal diagnostic workflows are needed for comprehensive understanding of these individuals and for distinguishing between benign morphological trait and manifest cardiomyopathy.

scholarly journals “De Criciúma para o mundo”: gênero, família e migração

2003 ◽  
Vol 3 ◽  
Author(s):  
Gláucia De Oliveira Assis
Keyword(s):  
Participant Observation ◽  
Individual Choice ◽  
Migration Literature ◽  
Worldwide Population ◽  
The Family ◽  
History Of ◽  
Migration Flows ◽  
And Gender ◽  
The Individual ◽  
Migratory Process

No final do século XX, a recente emigração de brasileiros para o exterior inseriu o Brasil nos novos fluxos da população mundial. Uma das características desses fluxos é o crescimento da participação feminina. Pesquisas recentes têm demonstrado a importância das mulheres nos fluxos migratórios contemporâneos como articuladoras de redes sociais na migração. Essas redes familiares e de parentesco são fundamentais tanto para aqueles que pretendem empreender a ‘aventura’ de migrar quanto para auxiliar nos momentos da chegada ao local de destino. Este artigo pretende demonstrar que a migração não é resultado apenas de uma escolha ‘racional’, mas de estratégias familiares nas quais homens e mulheres estão inseridos. Para percorrer a trajetória dos emigrantes o trabalho de campo se realizou em dois lugares: a cidade de Criciúma (SC) e a região de Boston, nos Estados Unidos. Os dados coletados a partir de entrevistas e de observação participante têm revelado que as mulheres não apenas esperam por seus maridos ou filhos, mas participam efetivamente do processo, integrando e articulando redes de migração. Os dados também sugerem que a migração provoca rearranjos familiares e de gênero ao longo do processo. Abstract The recent emigration of Brazilians, in late XXth century, has inserted Brazil into the new worldwide population flow. One characteristic of these flows is the growth of women in international migration. In the migration literature women participation in international flows has long been analyzed as subordinated to man, but recent research has illustrated the importance of women in migration flows. This paper intends to demonstrate that migratory process is resulted not only the individual choice, but also social networks (family, kingship, friendship), in which men and women are inserted. The work discusses data from fieldwork in Criciúma (SC) and in the Boston area, in United States. The data emerged from the interviews and participant observation showing that women not only wait for their husbands or children, but also participate in the process integrating and articulating migration networks. The data also made evident the changes in the family and gender relationships, suggesting that the migratory process rearticulate these relationships. This study therefore evidences that other factors, along with the ones of economics nature, contribute for the decision of migrating and make the history of this flow.

Assessing Families (Not Just Individuals) for Missionary Service

2016 ◽  
Vol 44 (4) ◽  
pp. 329-347 ◽  
Author(s):  
Fred C. Gingrich
Keyword(s):  
Family Relationships ◽  
Family Assessment ◽  
Family Unit ◽  
The Family ◽  
History Of ◽  
Assessment Techniques ◽  
Missionary Service ◽  
Mission Service ◽  
The One ◽  
The Individual

The assessment of missionaries tends to focus on the adult members of the family unit being approved for service. Yet, the family is the one consistent relational network that missionaries are connected to throughout the pre-field, on the field, and post-field phases of mission service. In addition, throughout the history of missions sending bodies have struggled to balance the needs of the missions context, the ministry gifts that the adult members of the family bring to the field, and the dynamics of their marital and family relationships. While the literature on missionary children has grown significantly, adopting a perspective that prioritizes the family unit as the unit being “sent” may result in helpful information regarding missionary attrition and longevity. Therefore, assessing missionary families, not only the individual members of the family, at the various stages of missionary service is warranted. Using concepts and techniques from systems theory, a model and logistical factors for assessing missionary families are presented, along with suggestions for whom to assess, what to assess, and how to conduct family assessment. Resources and possible assessment techniques are also provided.

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