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

Standardised exercise electrocardiography was performed in 50 patients undergoing endocavitary electrophysiological investigation of sinus node function. There was no previous history of cardiac failure or coronary insufficiency, and the patients, who were not trained athletes, were investigated after interruption of any medication which could affect sinus node function. The endocavitary investigations comprised overdrive suppression with rapid atrial pacing (Mandel) premature extrastimulus method (Strauss) and stimulation at a relative constant prematurity of 50% (Normal values: A2 A3 - 2d - return cycle - sinoatrial conduction time - = 0,77 A1 A1 - spontaneous cycle - +345). A significant difference in the maximal heart rate on stress testing between normal subjects and patients with sinus node dysfunction was observed only when values obtained with the relative constant prematurity method was taken into consideration (24 cases) the relative constant prematurity method became more significant (p less than 0,001). This held true to a lesser degree for Mandel's method (p less than 0,02), but no significant difference was obtained with Strauss' method. The ratio of maximal heart rate to theoretical maximal heart rate for age gave similar results. The correlation between stress testing and the relative constant prematurity method is probably related to intra sinusal shift of the dominant pacemaker. Both investigations would seem to test the functional properties and the reserve of the sinus node, taking extrinsic factors such as vago sympathetic stimulation into account. Patients with functional sinus node dysfunction increase their heart rates normally on exercise (average maximum of 170 bpm); a maximal heart rate of less than 120 bpm is very suggestive of organic sinus node dysfunction.
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PMID:[Contribution of the exercise test to the diagnosis of sinus dysfunction. Correlation with endocavitary explorations]. 678 90

The presentation, pattern of acute illness, and incidence of learning difficulties are described in 63 (33 boys, 30 girls) children with salt wasting 21-hydroxylase deficiency, drawn from a cohort study of congenital adrenal hyperplasia in the South West Region of England between 1968 and 1988. Thirty boys presented with a salt losing crisis from birth whereas the other three boys presented between 2 and 14 months of age with failure to thrive and hyponatraemia. Diagnostic uncertainty led to 13 (43%) of 30 girls developing a salt losing crisis. Five girls were misassigned as boys at birth. There were four deaths in the group, two due to salt losing crisis, one to complications of prematurity possibly compounded by 21-hydroxylase deficiency, and one from heart failure probably related to an excess of steroids. Acute admissions were common, especially during the first year of life, with convulsions in 7% of admissions. The 9% incidence of hypoglycaemia was considered to be an underestimate as blood glucose was measured in only 56 (22%) of 254 admissions. No convulsions occurred in the 38 (15%) admissions where the parents had given intramuscular hydrocortisone before bringing the child to hospital. A high incidence of learning difficulties was found among the 59 surviving children (9/30 (30%) boys and 6/29 (21%) girls), and in only two children could any factor other than 21-hydroxylase deficiency be invoked. Analysis of the subgroup with learning difficulties indicated that they were more ill at presentation with a significantly higher incidence of hypoglycaemia, and that growth in the first year was significantly worse. It is concluded that congenital adrenal hyperplasia remains a formidable disorder with an appreciable mortality and morbidity. The high incidence of learning difficulties seen in salt wasting 21-hydroxylase deficiency needs further attention. A prospective study is indicated to examine the effect of neonatal screening on morbidity from congenital adrenal hyperplasia, particularly the intellectual impairment seen in this study.
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PMID:Presentation, acute illness, and learning difficulties in salt wasting 21-hydroxylase deficiency. 813 66

Acardiac fetus is a rare lethal fetopathy usually encountered in monozygous pregnancies. Ultrasound prenatal diagnosis has enabled an increasing number of observations and raised the need for an adequate therapeutic approach since the spontaneous prognosis for the healthy twin is unfavorable in half of the cases. An acardiac fetus was identified at 12 weeks gestation in a 36-year-old woman. Growth of the healthy fetus was carefully monitored and progressed normally to delivery by cesarean section of a 2.900 kg boy at 36 weeks. At delivery, the acardiac fetus was found to be totally free of any attachment, floating in the remaining fluids. Pathology examination showed a 16 g macerated fetus with a cephalic extremity, a ventral pedicle and a syrenomelic caudal extremity. The caryotype was not significative. Acardiac fetus occurs in less than 1% of multiple pregnancies and can develop in single pregnancies. Twin reversed arterial perfusion has been recognized as necessary for development of the perfused fetus. Genetic and immunologic theories have been proposed to explain the pathogenesis which remains unknown. Clinical management depends on the spontaneous development of the acardiac fetus and the deleterious consequences for the healthy twin resulting from heart failure, anasarca or prematurity. Medical management with digoxin, or selective extraction by hysterotomy may improve prognosis but results have been variable. Echoguided umbilical cord ligation has also been proposed to improve maternal mortality. Therapeutic abstention is no longer indicated at prenatal diagnosis of an acardiac fetus and a healthy twin despite the risk of invasive treatment.
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PMID:[Acardiac fetus]. 894 22

Twenty-five neonates (16 boys and 9 girls) who had atrial flutter were identified. Diagnosis was made on or before the first day of life in 18 (72%). Heart failure were present in 9 patients, and hydrops fetalis was present in another 5. Atrial and ventricular rates did not differ between symptomatic and asymptomatic patients. Atrioventricular conduction was variable in 16 patients, and documented 1:1 conduction occurred in 5. Digoxin was the initial drug therapy given to 21 patients, with 7 reverting to sinus rhythm. Electrical cardioversion (pacing or synchronized shock) was attempted in 13 of the 14 cases in which digoxin was not successful and was attempted as the first treatment in 3 cases. Sustained sinus rhythm was achieved in 9. Two infants died of complications from prematurity but without having been successfully converted to sinus rhythm. No patient had atrial flutter during long-term follow-up (median 23 months). Neonatal atrial flutter has significant morbidity but an excellent long-term prognosis.
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PMID:Neonatal atrial flutter: significant early morbidity and excellent long-term prognosis. 906 Jul 98

Congenital hyperthyroidism is a very rare disease. But, for each affected child it has to be considered as a serious condition because of the negative impact of hyperthyroidism on fetal and postnatal development. If the manifestation occurs during fetal life tachycardia, cardiac arrhythmia, growth retardation and, most significant, prematurity are the consequences. Postnatal signs of hyperthyroidism are irritability, tachycardia, hypertension, poor weight gain and thyroid enlargement. Even cardiac failure may occur if hyperthyroidism is severe and treatment not adequate which explains the high early mortality rate of 16%. The main complication of persistent hyperthyroidism in the neonatal period and during infancy is craniosynostosis. Severe developmental delay or even mental retardation can be the consequence of inadequate high T4-levels during fetal and neonatal life. Congenital hyperthyroidism was first recognized in infants born to mothers with Graves' disease. The description of transplacental passage of the maternal thyroid stimulating antibodies elucidated the molecular mechanism in this major group of patients with "autoimmune congenital hyperthyroidism". In contrast to this transient, self-limited character of "autoimmune congenital hyperthyroidism", due to the clearance of maternal antibodies from the infant's circulation, some cases of persistent congenital hyperthyroidism without signs of thyroid autoimmunity have been recognized. Activating mutations in the thyroid-stimulating hormone receptor were described recently as the underlying molecular pathogenesis in this group of "non-immune congenital hyperthyroidism". Therefore the possibility of a molecular differential diagnosis of both groups of congenital hyperthyroidism now exists and opens the opportunity of optimal treatment for each patient.
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PMID:Congenital hyperthyroidism. 943 7

We retrospectively identified 92 children aged </=9 years with isolated secundum atrial septal defect (ASD) with preoperative height or weight z score < or =1.0 (16th percentile), respectively. Children with additional cardiac malformations, chromosomal abnormalities, recognized syndromes, pulmonary hypertension, heart failure, prematurity (<34 weeks' gestation), or use of cardiac medications were excluded from further analysis. After exclusion, 24 low-weight and 25 low-height children were compared with 48 and 50 age-, gender-, and size-matched normal controls, respectively. Univariate and multivariate analyses were performed separately for height and weight groups for achieving an end point of improvement in height or weight z score by +0.5 after repair. There were no significant preoperative demographic differences. After repair, 50% of low-weight patients improved +0.5 SD in weight within 2.6 years versus 5.6 years for controls (p = 0.02). Similarly, 50% of low-height patients improved +0.5 SD in height within 1.7 years versus 11.6 years for controls (p <0.001). Multivariate analysis also indicated a benefit of repair with relative risks of 2.35 (95% confidence interval 1.20 to 4.62; p = 0.01) and 5.11 (95% confidence interval 2.45 to 10.65; p <0.0001), respectively, for improving height or weight by +0.5 SD in patients with ASD relative to controls. Furthermore, children heavier at repair were less likely to improve in weight (relative risk 0.56 [0.34, 0.92] per 1.0 z [p = 0.02]). Likewise, children older at repair were less likely to improve in height (relative risk 0.77 [0.64, 0.92] per year of age [p = 0.003]). We conclude that undersized children with ASD exhibit improved growth after repair. Poor growth should be considered a relative indication for early ASD repair.
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PMID:Impact of anatomic closure on somatic growth among small, asymptomatic children with secundum atrial septal defect. 1085 95

Hypertrophic cardiomyopathy (HCM) is a rare, often familial condition, which may be complicated by syncope, atrial or ventricular arrhythmias and episodes of cardiac failure. This genetic disease affects young people and may be observed in women wishing for a pregnancy. The duration and outcome of such pregnancies has not been extensively studied. The authors undertook a retrospective study by questionnaire to compare the pregnancies of 41 women with HCM, a total of 150 pregnancies, with those of 39 unaffected women from the same families: a total of 132 pregnancies. None of the women died, there were no hospital admission for cardiac causes and there was no aggravation of functional status (31% of women with HCM had symptoms before pregnancy compared with 27% during pregnancy). The foetal prognosis was good with no increase in prematurity or neonatal crises. Only the women with symptoms before pregnancy had an increased risk of foetal prematurity compared with healthy women (18% versus 5%). These results indicate the good tolerance of pregnancy of women with HCM and should lead to a revision of systematic medical contra-indication of pregnancy in these patients.
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PMID:[Familial hypertrophic cardiomyopathy. French study of the duration and outcome of pregnancy]. 1193 43

First cause of hyperthyroidism among women of childbearing age, Graves' disease raises the risk of maternal and fetal complications, including eclampsia, cardiac failure, abortion, prematurity, fetal death, all of which can be avoided if maternal hyperthyroidism is closely controlled. The risk of transplacental hyperthyroidism has been shown to correlate to the titre of anti-TSH receptor antibodies and has to be evaluated not only in women treated for Graves' disease during pregnancy, but also in women who have previously received radio iodine treatment or undergone surgery for Graves' disease: TSH-receptor antibodies may indeed remain at a high level several years after initial treatment. Both methimazole and propylthiouracil are equally effective to restore maternal euthyroidism. Accumulation of case-reports relating congenital malformations (mostly aplasia cutis, but in some cases, severe malformations) among the offspring of methimazole-treated women suggests the possibility of a teratogenic effect of methimazole. Despite the fact that the link between severe congenital defects and methimazole exposure during pregnancy is not formally established, propylthiouracil should be preferred to methimazole for the treatment of young hyperthyroid women.
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PMID:[Antithyroid agents and embryopathies]. 1506 47

Twin reversed arterial perfusion (TRAP sequence), or acardiaus, is a rare and serious complication of monozygotic multiple gestation. The acardiac fetus suffers from lethal anomalies. The normal co-twin also runs a high risk of perinatal morbidity and mortality due to heart failure and prematurity. Four cases of acardius are presented. The role of sonography in the condition's diagnosis and management is discussed.
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PMID:Twin reversed arterial perfusion: report of four cases. 1537 49

Ebstein's anomaly, an uncommon malformation of the tricuspid valve, has an extremely variable natural history, depending on a wide spectrum of pathological features. We here described a case of a patient with Ebstein's anomaly who gave birth to 2 healthy unaffected full-term infants after two successful pregnancies; a third pregnancy miscarried at the 11th week. The anomaly was diagnosed during childhood, was not associated with other cardiac anomalies, cyanosis or preexcitation and the echocardiographic degree of severity was low (grade 1). All of these factors are considered predictors of a good survival and prognosis. During the pregnancies, no arrhythmias, cyanosis or signs of cardiac failure were observed and the patient's NYHA functional class (1) remained unchanged. Our case is the only published case of two successful term pregnancies in Ebstein's anomaly, it confirms the importance of echocardiographic evaluation and that the probability of maternal and neonatal events may be predicted from the baseline characteristics of the mother. Pregnancy is well tolerated in the absence of important maternal cardiomegaly, cyanosis and arrhythmias and in those patients with mild cardiac dysfunction as evaluated at echocardiography and a low NYHA class, but it is associated with an increased risk of abortions and prematurity.
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PMID:Ebstein's anomaly and pregnancy: a case report. 1556 1


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