Cardiomyopathy in fetus

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#1 Cardiomyopathy in fetus

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Cardiomyopathy in fetus

Fetal cardiomyopathy refers to a very rare situation where Cardiomyopathy in fetus cardiomyopathy occurs in utero. It is often a diagnosis of exclusion where, by definition, there Why do economists use models an absence of an underlying congenital cardiac morphological anomaly. The estimated incidence is variable with the high end of the reported spectrum approaching 1 in births 6. It often results from a primary dysfunction in cardiac muscle functioning i. A fetal cardiomyopathy can have a broad spectrum of syndromic, as well non-syndromic, associations, which include:. Dedicated fetal Cardiomyopathy in fetus is often required. Radiographic features will depend on the subtype. Often the fetal heart is enlarged resulting in an increased cardiothoracic circumferential ratio. A poor outcome is observed in many affected fetuses. The presence of diastolic dysfunction in a fetal cardiomyopathy is considered to be associated with the highest risk of mortality 4. Treatment options vary depending on the presence of associated Beaker movie tracy e. You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Unable to process the form. Check for Cardiomyopathy in fetus and try again. Thank you for updating your details. Log in Sign up. Articles Cases Courses Quiz. About Blog Go ad-free. Epidemiology Pathology Radiographic assessment Cardiomyopathy in fetus and prognosis References. Edit article Share article View revision history. Synonyms or Alternate Spellings: Fetal cardiomyopathies In utero Cardiomyopathy in fetus In utero cardiomyopathies Foetal cardiomyopathy Foetal cardiomyopathies. Support Radiopaedia and see fewer ads. Loading Stack - 0 images remaining.

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Go to top of page. Objectives The purpose of this study was to determine the phenotypic presentation, causes, and outcome of fetal cardiomyopathy CM and to identify early predictors of outcome. Background Although prenatal diagnosis is possible, there is a paucity of information about fetal CM. Baseline echocardiographic variables associated with mortality in actively managed patients included ventricular septal thickness HR: Conclusions Fetal CM originates from a broad spectrum of etiologies and is associated with substantial mortality. Early echocardiographic findings appear useful in predicting adverse perinatal outcomes. Most children present with a dilated or hypertrophic phenotype 2—4 without an identifiable genetic, familial, infectious, or metabolic cause 5,6. CM is the most common indication for cardiac transplantation in infants 1,7. The condition is rarely diagnosed prenatally, and there is little knowledge of the disease spectrum and outcome when detected prenatally. Cardiac pathology secondary to these conditions is often reversible 9,10 , with a substantially better long-term prognosis compared with primary CM. Accordingly, the purpose of this single-center cohort study was to assess the disease pattern and outcome of disorders in which the primary pathology is the fetal myocardium and to determine epidemiological and hemodynamic markers associated with adverse outcomes. The Hospital for Sick Children is the exclusive tertiary perinatal cardiac care provider for a population with 80, live births per year. Of 8, pregnancy referrals to the Fetal Cardiac Program between January and June , 2, were affected by fetal heart disease. These included 61 fetuses 2. After echocardiographic diagnosis of fetal CM, a comprehensive evaluation by the High-Risk Pregnancy Program and, after birth, by the Heart Failure Program was arranged. The diagnostic workup included genetic counseling, virology polymerase chain reaction, TORCH [toxoplasmosis, other infections, rubella, cytomegalovirus, and herpes simplex virus] serology , metabolic screening, karyotype, pan-cardiomyopathy gene-panel screening, microarray, and,...

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Lydia and Julia A. A cardiomyopathy is a disorder of heart muscle that may be a primary disorder or may be associated with structural anomalies or pericardial disease. The congestive, or dilated, form is most common and presents with dilated, poorly contracting chambers, atrioventricular regurgitation, and, often, associated hydrops fetalis pleural and pericardial effusions, ascites, and skin thickening. The second most common type of cardiomyopathy is the hypertrophic form. Hypertrophic cardiomyopathies are recognized by markedly thickened ventricular walls and septum. The third form, a restrictive cardiomyopathy, is unusual in the fetus, manifesting primarily as endocardial fibroelastosis. Fetal cardiomyopathies are caused by a broad spectrum of underlying disorders Table 20—1. There is not always correlation between presentation i. In general, dilated cardiomyopathies can be divided into two categories. The first contains cardiomyopathies resulting from high-output failure caused by severe fetal anemias, or volume overload from massive arteriovenous shunting. Severe fetal anemias result in high-output failure when the fetal heart increases cardiac output by increasing heart rate or stroke volume, or both, to meet peripheral oxygen requirements. As a result, cardiac work and oxygen demand increase and eventually exceed myocardial oxygen supply. Myocardial ischemia leads to poor cardiac function and contractility with chamber dilation Fig. Anemia-associated cardiomyopathies include immune hydrops fetalis, homozygous alpha-thalassemia, glucosephosphate-dehydrogenase deficiency, and hemophilia A. Maternal serum antibodies are formed from the mixing of maternal and fetal blood during parturition of a prior pregnancy, placental abruption, amniocentesis, spontaneous or therapeutic abortion, blood transfusion, or placental intervillous hemorrhage. Maternal IgG crosses the placenta, enters the fetal circulation, and binds to fetal red blood cell antigens. Fetal hemolysis and hemolytic anemia may then occur. This hemoglobin irreversibly binds to oxygen, resulting in the inability of red blood cells to deliver oxygen to fetal tissues. Homozygous alpha-thalassemia is uniformly fatal to the...

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Cardiomyopathies CM are a very rare disease in fetuses with a very poor outcome. Only isolated case reports and small case series were reported. According with published studies we will describe the fetal CM starting from their echocardiographic presentation: The term of the "noncompaction" of the left ventricular myocardium, is used in cases with DCM with evidence of numerous prominent trabeculations with deep myocardial recesses. The high intrauterine loss, occurring in one third of affected fetuses, likely accounts for these differences. Fetal echocardiography, B and M-mode, is the main diagnostic tool and it is useful for the therapeutic orientation and to determine the neonatal outcome. A haemodynamic evaluation can be performed by Doppler mode. Systolic and diastolic fetal cardiac function have become part of the routine evaluation of the fetal heart. Cardiomyopathies can be isolated or associated with other cardiac and non cardiac malformations. All the studies confirm a great variability of DCM in the fetal age as for the anatomical and functional forms, etiology and hemodynamic impact with different final outcome. Genetic, metabolic, infective, and cardiac diseases may present with DCM. Ventricular dysfunction may be progressive in utero and after birth, but possibility of improvement or even normalization of the left ventricular dysfunction is known in all forms of DCM, "idiopathic", post infective or in noncompaction of left ventricle. The outcome is worse in presence of fetal hydrops, significant atrioventricular valve regurgitation, for the earlier age at presentation and when diastolic dysfunction is associated with systolic dysfunction. Etiologically primary fetal HCM is a heterogeneous condition that can be the result of intrinsic fetal pathology as well as of extrinsic factors. It can be concentric or asymmetric. Prognosis of infants with HCM associated with maternal diabetes is good while a bad prognosis has been reported in fetuses without diabetic mother....

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METHODS A total of fetuses between 16 and 41 gestational weeks, referred to paediatric cardiologists for detailed echocardiography, were included in this study. Echocardiography is the only imaging modality available for diagnosis of structural heart defect, arrhythmias, and abnormal cardiac haemodynamics in the fetus. Some defects can also evolve or progress during fetal life, making a definitive diagnosis early in gestation difficult. Echocardiographic assessment of fetal arrhythmias is usually accomplished using M-mode and Doppler techniques. Echocardiography not only establishes the kind of arrhythmia present but can also identify associated structural and functional heart disease as well. Fetal congestive heart failure is diagnosed when tissue perfusion of the fetus is inadequate, characterised by cardiomegaly, atrioventricular valve regurgitation, and abnormal venous velocities. The indications for referral were: Two dimensional imaging was obtained from five approaches including four-chamber, five-chamber, long axis, short axis and arch views. The studies were judged to be of adequate diagnostic quality if the following were defined: Colour and pulsed or continuous wave Doppler echocardiography were carried out when a clinically significant structural defect was suspected by imaging. The type of arrhythmia was analysed using an M-mode echocardiogram at the atrial and ventricular levels. The non-structural cardiac abnormalities were identified as dilatated cardiomyopathy, hypertrophic cardiomyopathy, aneurysm of the foramen ovale, and isolated pericardial effusion or echogenic foci. All studies were performed using the standard transabdominal approach. The abnormal studies were recorded on videotape for subsequent playback and analysis. Personal data were recorded for each mother. The progress and outcome of each pregnancy including those where no abnormality was detected were followed up, and where appropriate, post mortem examinations were performed. Follow up lasted 12 months. The reasons for the referral and the findings are shown in table 1. All cases were siblings. Five mothers also had fetuses with arrhythmia...

Cardiomyopathy in fetus

JACC: Heart Failure

These findings led to the diagnosis of severely dilated cardiomyopathy (DCM) with poor systolic function and early signs of fetal decompensation. The mild fetal tachycardia appeared to be secondary to the DCM rather than the causative condition. As such no antiarrhythmic therapy was considered. Fetal cardiomyopathy refers to a very rare situation where a cardiomyopathy occurs in utero. It is often a diagnosis of exclusion where, by definition, there is an absence of an underlying congenital cardiac morphological anomaly. Among fetuses with non-structural cardiac abnormalities, survival was 73%. Poor outcome was evident in fetuses with dilated cardiomyopathy in eight of

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