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

An isochromosome for the long arm of chromosome number 18 - 46,XY,i(18q) - was found in an infant who had features of both trisomy 18 and 18p- syndromes. Findings compatible with trisomy 18 included postmature delivery, prominent occiput, severe congenital heart disease, overlapping fingers, and rocker-bottom feet. Those of 18p- syndrome, which frequently resembles Turner syndrome, were downward obliquity to the palpebral fissures, short, webbed neck, low posterior hairline, and widely-spaced nipples. The infant died of heart failure at 3.5 months of age. Parental karyotypes were normal.
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PMID:Features of trisomy 18 and 18p- syndromes in an infant with 45,XY,i(18q). 48 37

A case of a week old female baby, admitted because of apathy, hypothermia, dyspnea, jaundice and cyanosis is described. She had the characteristic phenotype of Turner's syndrome with normal karyotype. Signs of severe heart failure were present. Therapy with diuretics, digoxin, dopamine and mechanical ventilation were unsuccessful, and the patient died several hours after her admission. The anatomopathological examination revealed the presence of hypoplastic left heart syndrome with mitral atresia and aortic atresia, atrial septal defect, double outlet right ventricle, and a patent ductus arteriosus.
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PMID:Noonan's syndrome associated with hypoplastic left heart. 235 79

Growth hormone (GH), probably acting indirectly through locally produced insulin-like growth factor I, stimulates myocardial hypertrophy and increases myocyte contractility. In experimental models insulin-like growth factor I appears to be a key regulator of ventricular hypertrophy. Many adults with growth hormone deficiency (GHD) have reduced left ventricular mass, a lower ejection fraction, and reduced exercise tolerance. Elevated serum lipid levels, increased visceral fat, and early atheroma formation may contribute to an increased mortality rate from cardiovascular disease in these persons, but GH replacement therapy appears to correct many of these abnormalities. GH excess (acromegaly) results in cardiac hypertrophy that can progress to cardiac failure. Treatment with octreotide at least partially reverses cardiac hypertrophy and dysfunction. GH treatment may induce beneficial cardiac hypertrophy in adults without GHD who have dilated cardiomyopathy. Significant cardiac dysfunction has not been reported in children with GHD who are treated with GH, nor have adverse cardiac effects been reported with GH in short children without GHD, including those with Turner syndrome. We now have extensive experience with the therapeutic use of GH in children with cardiac structural abnormalities (e.g., Turner and Noonan syndromes, congenital heart disease), and such use appears to be safe. Furthermore, cardiac complications of GH in children without cardiac disease are rare. Continued observation to ensure that GH therapy has no long-term effects on cardiac anatomy or function in children is necessary.
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PMID:Is growth hormone good for the heart? 925 33

Nonimmune hydrops fetalis (NIHF) is used to describe fetuses and newborns with generalized edema and cavity effusions. It is helpful to alert physicians about the presence of anemia, heart failure, and/or hypoproteinemia, but this diagnosis is frequently overlooked. We reviewed the autopsy files from 1990 to 2000, selected all cases with NIHF including clinical information (with maternal laboratory tests and ultrasound), and classified patients by etiology. Among 840 stillborn autopsies during the 11-year period, we found 51 with NIHF (6.07%). The clinical summary had mentioned hydrops in 14 patients and the etiology in another 7 by fetal ultrasonography, but without addressing the possibility of hydrops. In the remaining 30 cases neither hydrops nor an etiology was mentioned. Other pertinent diagnoses were maternal diabetes mellitus (4), congenital heart disease (3), and cystic hygroma (2). The following diagnoses were made in one instance each: cardiac tumor, twin transfusion syndrome, congenital adenomatoid malformation, syphilis, Turner syndrome, and cerebral arteriovenous malformation. Postmortem and placental examination confirmed the following etiologies: congenital infections (17); placental pathology significant enough to explain NIHF (10); cardiovascular diseases (8) (further classified as congenital heart disease [3], rhabdomyoma [1], and vascular malformations [4]); chromosomal abnormalities (6); uncontrolled maternal diabetes (4); intrathoracic lesions (2); prune-belly syndrome (2); and idiopathic NIHF (2). Only 3.9% of the cases studied had no identifiable etiology. The cause of hydrops was confirmed by autopsy in 47 fetuses (92%), which further supports the importance of performing an autopsy. Thirty-two cases (62.74%) had placental abnormalities helpful to the etiology (parvovirus, syphilis, Turner's syndrome, etc.). In 20 instances, the clinical summary had no mention of either hydrops or any of the diseases leading to it. The autopsy in conjunction with placental examination and fetal ultrasound represent the best combination to determine the etiology of NIHF among stillborn fetuses.
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PMID:Value of autopsy in nonimmune hydrops fetalis: series of 51 stillborn fetuses. 1201 30

A 21-year-old woman with Turner syndrome was admitted to hospital because of progressive heart failure. Echocardiography revealed a bicuspid aortic valve with one cusp ruptured due to infective endocarditis and anomalous pulmonary venous drainage. The patient underwent successful aortic valve replacement.
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PMID:[Congenital disorders of the cardiovascular system and their complications in a 21-year-old woman with Turner syndrome--a case report]. 1826 87

Turner's syndrome is characterized by an ovarian failure, which occurs in most cases before puberty and leads to infertility. In vitro fertilization with oocyte donation has dramatically transformed the prognosis of infertility of these women. However, in the same time, it has become obvious that pregnancies in Turner's syndrome are at very high risk of possible sudden death because of a specific risk for cardiovascular complications involving aortic root dissection. We report the case of a serious cardiac failure occurred during a twin pregnancy obtained by oocyte donation in a 39-year-old patient with Turner's syndrome. Pregnancy outcome was hopefully favourable thanks to a foetal extraction at 27 weeks of amenorrhoea. If the most reported cases of maternal deaths in patients with Turner's syndrome are associated with an aortic root dissection, our observation is characterized by a full normal cardiologic assessment before the pregnancy and by the absence of aortic root dilatation during pregnancy. This case also illustrates the very high risk of pregnancy in women with Turner's syndrome and the importance of a multidisciplinary care by professionals informed and been used to this obstetric practice.
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PMID:[Oocyte donation in patients with Turner syndrome: A high-risk pregnancy]. 1920 Sep 42

Partial anomalous venous connection (PAPVC) is a congenital cardiovascular defect where one or more (but not all) of the pulmonary veins return anomalously back to the right atrium, either via a direct or indirect connection. It often occurs with other cardiac defects, most commonly a secundum atrial septal defect. Individuals with a large degree of shunting will present with dyspnea, fatigue, and, in some cases, heart failure. Clinical associations and variants of PAPVC include scimitar syndrome, pseudo-scimitar or meandering right pulmonary vein, sinous venosus defects, malposition of the septum primum, and Turner syndrome. The patient in this case, a previously healthy, 6-month-old, full-term male, presented to the emergency department for evaluation of respiratory distress and wheezing. The infant was first seen in his pediatrician's office, where he was noted to be tachypneic and wheezing. He was feeding without difficulty, voiding well, and was active and playful. The patient had passed critical congenital heart disease screening after his birth and prior to discharge, and the family history was negative for any respiratory or cardiac conditions. Cardiac magnetic resonance imaging is becoming the mode of choice for diagnosis of PAPVC. The definitive treatment is surgical correction, but surgery is not indicated in all cases, especially if the patient is asymptomatic and the degree of shunting is small. Patients with isolated PAPVC who undergo surgical correction have good long-term outcomes. In this case, the patient underwent the Warden procedure, which causes an increased risk of superior vena cava stenosis or obstruction to the right atrium. This patient will require lifelong follow-up to assess for new onset pulmonary venous obstruction.
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PMID:An unusual cause of respiratory distress in an infant. 2487 94