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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital atrioventricular block is a congenital disorder of the cardiac conducting system. Pacemaker implantation is the treatment of choice for patients with congenital A-V block if prior medical treatment fails. The criteria for pacemaker implantation in infants and children are (1) frequent syncope (2) heart failure (3) ventricular rate below 55 beats/min (4) ventricular arrhythmia (5) QRS widening in the ECG (6) moderate to severe exercise intolerance. Our experience with a 15-month-old male child is herein presented. The patient suffered from frequent attacks of upper respiratory tract infection, bradycardia and complete A-V block as found on electrocardiograms. At 15 months old, he was admitted again due to respiratory tract infection and several attacks of syncope. Stokes-Adams syndrome was noted several times despite medical treatment. Following implantation of a VVI mode epicardiac pacemaker, the patient's condition, including resistance to heart failure and respiration, were improved dramatically. He was followed up regularly for 4 years, his heart beat remaining stable.
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PMID:[Permanent pacemaker with implantation in a young child with congenital complete atrioventricular block: a case report]. 203 May 27

An anomalous left coronary artery originating from the pulmonary artery is a rare, but frequently lethal congenital disorder. A wide range of symptoms due to heart failure and myocardial ischaemia may appear soon after birth. We describe a young woman who was admitted to the hospital after resuscitation for ventricular fibrillation. An anomalous origin of the left coronary artery from the pulmonary trunk was diagnosed. An echocardiographic evaluation in this adult patient illustrated the haemodynamic disturbances in the coronary circulation. The thallium scintigram showed reversible perfusion defects on exercise. Ischaemia may be implicated in the pathogenesis of the arrhythmia. Our patient was treated with an arterial graft and ligation of the left coronary artery at its origin.
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PMID:[Aberrant origin of the left coronary artery from the pulmonary artery as cause of ventricular fibrillation in a young woman]. 847 75

A most significant life event in the first years of life is a disease, especially if it is of early onset, severe, life threatening, with an uncertain prognosis, and with the necessity of frequent diagnostic and therapeutic interventions. Psychological implications are a significant parts of the illness, not a marginal component; they can affect prognosis and outcome. The authors describe the different psychological implications of an experience of chronic disease in children and adolescents and their families (parents and siblings). Congenital disease (for example congenital heart failure) has a peculiar significance: since it is diagnosed early, it influences mother-infant interactions from the beginning, in a crucial moment of the infant's psychological development; diagnostic and therapeutical interventions are early and frequent; congenital defects determine the strongest guilt feelings in the parents. Some specific psychological aspects can be described: the weakening of the Bodily self, the inhibition of thinking, the theories the child and the family formulates on the disease, the death feelings. Emotional features in children and adolescents with congenital cardiopathy are described: inhibition of emotions, marked anxiety, depressive reaction, with loneliness, low self-esteem and inadequacy, emotional lability, with oscillation between omnipotence and inadequacy; impulsiveness; weakness of self identity; especially in bodily Self. Some psychopathological aspects in children and adolescents with heart transplant and their families are also described. Intellectual level of patients with congenital heart disease is in the normal range, although significantly lower than normal controls. There is a positive correlation between worsening of intellectual functioning and clinical severity of the heart disease; this clinical severity is related both to restrictions in normal daily life activities, and blood oxygen saturation. It is hard to tease apart the role of early physical limitations versus the role of chronic hypoxia, in affecting intellectual development. Some methodological considerations are described, relating to the role of the physician, the psychological support to the children and adolescents and their families, the problem of the shared-cares between main centres and local hospitals, where primary health-care team operates.
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PMID:[Psychopathology of chronic diseases in children and adolescents. Congenital cardiopathies]. 909 31

Noncompaction of the ventricular myocardium is a rare congenital disorder characterized by the presence of numerous prominent trabeculations and deep intertrabecular recessess which communicate with the left ventricular cavity. The disease uniformly affects the left ventricle, and sometimes also affects the right ventricle. Echocardiographic findings are important clues for the diagnosis. Clinical symptoms include signs of left ventricular systolic dysfunction even to the point of heart failure, ventricular arrhythmias, and embolic events. We describe an illustrative case of isolated noncompaction of the left ventricular myocardium in a two-year-old child with the typical clinical and echocardiographic features of the disease. The literature on the topic is reviewed.
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PMID:Isolated noncompaction of the ventricular myocardium. 1185 89

A 83-year-old man with an implanted pacemaker was admitted to our hospital for dyspnea. Echocardiography revealed left ventricular dilation and diffuse hypokinesis mimicking dilated cardiomyopathy. Multiple trabeculations and deep intertrabecular recesses were prominent in the apical and posterior walls. Computed tomography with contrast medium also showed prominent trabeculations. Thallium-201 and iodine-123-beta-methyl-p-iodophenyl-pentadecanoic acid scintigraphy demonstrated fixed defects in the inferior wall. These findings were consistent with isolated noncompaction of the ventricular myocardium. The patient's symptoms of heart failure improved in response to treatment, including carvedilol. Isolated noncompaction of the ventricular myocardium is recognized as a rare congenital disorder of infancy with fatal outcomes, with some reports of cases in adults. The present patient is the most elderly described to date.
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PMID:[Isolated left ventricular noncompaction in the elderly: a case report]. 1533 81

Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart failure and arrhythmias are the most prominent. Accordingly, these patients need frequent follow-up by physicians with specific knowledge in the field of congenital heart disease. However, planning of care for this population is difficult, because the number of patients currently living with congenital heart disease is difficult to measure. Birth prevalence estimates vary widely according to different studies, and survival rates have not been well recorded. Consequently, the prevalence of congenital heart disease is unclear, with estimates exceeding the number of patients currently seen in cardiology clinics. New developments continue to influence the size of the population of patients with congenital heart disease. Prenatal screening has led to increased rates of termination of pregnancy. Improved management of complications has changed the time and mode of death caused by congenital heart disease. Several genetic and environmental factors have been shown to be involved in the etiology of congenital heart disease, although this knowledge has not yet led to the implementation of preventative measures. In this Review, we give an overview of the etiology, birth prevalence, current prevalence, mortality, and complications of congenital heart disease.
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PMID:The changing epidemiology of congenital heart disease. 2104 84

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of unknown etiology characterized by venous malformations or varicose veins, cutaneous capillary malformation and hypertrophy of soft tissues with limb (usually asymmetric lower extremity) involvement. Peripartum cardiomyopathy (PPCM) is characterized by rapid onset heart failure during the final month of pregnancy or within five months of delivery, in the absence of identifiable risk factors or previous heart disease. The aim of this study was to illustrate the correlation between the KTS and the onset of PPCM in women with twin pregnancies. Our case is a 35-year-old woman, gravida II para I, with KTS, twin pregnancy and PPCM. We can assume that, as the heart of a women with KTS usually works with a low preload reserve due to the widespread venous varicosities, if a significant increase in preload occurs, it may lead to the onset of cardiac dilatation and thus PPCM.
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PMID:Peripartum cardiomyopathy and Klippel-Trenaunay syndrome. 2107 13

The double-chambered right ventricle (DCRV) is a rare congenital cardiac disease in dogs, and its detailed epidemiological and morphological features are not clearly understood. By investigating the profile, clinical signs, and characteristics of examination findings of eleven dogs with DCRV by means of a retrospective study, we attempted to clarify the epidemiology and morphology of the condition. The study group consisted of nine males and two females. Breeds included Pug (n=3), Miniature Dachshund (n=1), French Bull-dog (n=1), Shiba (n=1), and Retrievers (n=5). The attachment site of the anomalous muscular bundle was continuous with the cardiac apex in nine dogs, and it was attached to the right ventricle free wall in the other two dogs. In dogs with DCRV, at least one of the following conditions was present concurrently: congenital or acquired tricuspid valve regurgitation (TR), ventricular septal defect, and atrial septal defect. Also, the pressure difference between the two chambers increased over time, and progressive right-sided heart failure was observed. In summary, DCRV occurs in small breeds of dog as well as in large breeds of dog and it may be more prevalent in males. The existence of two types of DCRV in dogs was established. Dog with DCRVs will have a high incidence of concurrent cardiac abnormalities. Concurrent TR may be either congenital or acquired. DCRV is a congenital disorder, but the clinical condition progresses as the dog develops.
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PMID:Epidemiological and morphological studies of double-chambered right ventricle in dogs. 2164 54

Genetic causes for autosomal recessive forms of dilated cardiomyopathy (DCM) are only rarely identified, although they are thought to contribute considerably to sudden cardiac death and heart failure, especially in young children. Here, we describe 11 young patients (5-13 years) with a predominant presentation of dilated cardiomyopathy (DCM). Metabolic investigations showed deficient protein N-glycosylation, leading to a diagnosis of Congenital Disorders of Glycosylation (CDG). Homozygosity mapping in the consanguineous families showed a locus with two known genes in the N-glycosylation pathway. In all individuals, pathogenic mutations were identified in DOLK, encoding the dolichol kinase responsible for formation of dolichol-phosphate. Enzyme analysis in patients' fibroblasts confirmed a dolichol kinase deficiency in all families. In comparison with the generally multisystem presentation in CDG, the nonsyndromic DCM in several individuals was remarkable. Investigation of other dolichol-phosphate dependent glycosylation pathways in biopsied heart tissue indicated reduced O-mannosylation of alpha-dystroglycan with concomitant functional loss of its laminin-binding capacity, which has been linked to DCM. We thus identified a combined deficiency of protein N-glycosylation and alpha-dystroglycan O-mannosylation in patients with nonsyndromic DCM due to autosomal recessive DOLK mutations.
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PMID:Autosomal recessive dilated cardiomyopathy due to DOLK mutations results from abnormal dystroglycan O-mannosylation. 2224 4

Left ventricular noncompaction cardiomyopathy (LVNC) is a relatively rare congenital disorder prominently characterized by prominent trabeculations and intertrabecular recesses that communicate with the ventricular cavity rather than the coronary circulation. LVNC can occur in isolation or coexist with other cardiac and/or systemic anomalies, in especial neuromuscular disorders. The clinical presentation varies ranging from asymptomatic patients to patients who develop ventricular arrhythmias, thromboembolism, heart failure and sudden cardiac death. Although LVNC is commonly diagnosed by echocardiography, there are also other useful diagnostic techniques, including contrast ventriculography, CT and MRI. Now, it is being diagnosed more frequently in patients of all ages because of increased awareness and improvements in imaging methods. We described the case of a woman who presented with heart failure for the first time at 62 years of age. The diagnosis was LVNC. Transthoracic echocardiography showed a trabeculated, sponge-like appearance of the ventricular apical and inferolateral segments. After medical management, the patient was asymptomatic at the 1-month follow-up examination. Now we discussed the diagnosis of this case and reviewed the medical literature that pertained to LVNC.
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PMID:Left ventricular noncompaction cardiomyopathy: a case report and literature review. 2566 15


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