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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe a case of congenital heart disease no longer frequent in adults, due to an early diagnosis and surgical treatment. The patent ductus arteriosus in adults who received no therapy can present several forms of evolution, depending on its own hemodynamic profile. The present case is an example of a congenital heart disease not diagnosed during the childhood, that developed pulmonary hypertension and congestive heart failure. These clinical situations have a bad prognosis and place this patient in a group of high surgical risk. Thus, surgery is not advisable considering the bad prognosis and early high mortality rate.
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PMID:[Persistent ductus arteriosus in the adult--report of a case]. 129 Jun 45

108 women with congenital heart disease in child-bearing age (16-38 years, mean age 28) were followed up for a period of ten years. Possible complications of contraceptive methods, and incidence of full-term pregnancies, spontaneous abortions and cardiovascular complications during pregnancy and delivery, were considered. Half of these patients had a cyanotic congenital heart disease, 23 had left-to-right shunt mainly at atrial level, 18 had right and 14 left ventricular outflow tract obstruction. 60 women had previous surgical procedures for their congenital heart disease. 83% of the patients were in NYHA functional class I-II. No complications were found during oral contraceptive regimen during intrauterine device insertion with antibiotic prophylaxis, except for the development of pulmonary hypertension in one patient with an atrial septal defect. 146 pregnancies occurred, but only 89 were full-term. There was a high number of interrupted pregnancies, rarely for medical reasons. The incidence of spontaneous abortion was similar to that of normal population (18% versus 10-15%). Maternal cardiovascular complications were found in the 22% of the full-term pregnancies, but were well controlled by medical treatment. In conclusion, good family planning and pregnancies are possible in most young females with congenital heart disease. In the absence of cyanosis and pulmonary hypertension, oral contraception does not carry any particular risk. With appropriate medical care intrauterine devices may be an alternative in high risk patients. In the presence of a good cardiac function, a normal full-term pregnancy with an healty baby is the rule. A cesarean section is seldom needed.
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PMID:[Risks of contraception and pregnancy in patients with congenital cardiopathies. Retrospective study on 108 patients]. 129 7

All non-steroidal anti-inflammatory drugs (NSAIDs) are prostaglandin inhibitors, which explains their foetal toxicity. So far, no epidemiological study of their cardiopulmonary and renal effects has been carried out, but case-reports have been published. The cardiopulmonary effects of NSAIDs include closure of the ductus arteriosus, pulmonary hypertension cardiopathy and tricuspid valve insufficiency. They were responsible for 31 neonatal accidents, 8 of which were fatal (for 22 pregnant women, 7 bearing twins, 1 bearing triplets). The renal effects of NSAIDs consisted of acute renal failure with oedema, oliguria, hyponatraemia and marked hyperkalaemia. They affected 23 neonates, 8 of whom died (for 17 pregnant women, 4 bearing twins, 1 bearing triplets). A few epidemiological studies have reported foetal haemorrhages when aspirin was used by the mother as anti-inflammatory agent. In comparative trials of indomethacin as short treatment of premature labour and polyhydramnios the drug proved to be effective. In obstetrical tocolysis NSAIDs can be given in the absence of alternative therapy with beta-adrenergic agents, and their risk can be minimized by ultrasonographic examination and monitoring of foetal cardiac function and diuresis. In the field of rheumatology, corticosteroids would be a good alternative to NSAIDs for rheumatic diseases, but using NSAIDs for low back pain, sciatica, haemorrhoids, toothaches, sinusitis, etc., would not be justified in pregnant women. Self medication must be discouraged.
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PMID:[Fetal toxicity of non-steroidal anti-inflammatory agents]. 129 2

Among 388 cases of congenital heart disease with magnetic resonance imaging (MRI) from September 1990 to February 1992, we came across two cases of Ebstein's anomaly. They had been previously diagnosed as Ebstein's anomaly by echocardiography and cinecardioangiography. The first case was a three-year-old boy with complex congenital heart disease that included Ebstein's anomaly, a double-outlet right ventricle, pulmonary hypertension, tricuspid regurgitation, mitral regurgitation, a ventricular septal defect and an atrial septal defect. The second was a 13-year-old boy who also had Ebstein's anomaly, but had received a tricuspid valve replacement at the age of five. In Ebstein's anomaly, we found that MRI offers exquisite endocardial and epicardial details. We anticipate that in the future MRI will help to eliminate invasive studies.
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PMID:Magnetic resonance imaging of Ebstein's anomaly: report of two cases. 136 45

In children with congenital heart disease pulmonary vascular disease can be fatal for a variety of reasons. Even before the classical changes of advanced pulmonary vascular obstructive disease have developed, a marked increase in pulmonary vascular smooth muscle can be fatal due to pulmonary hypertensive crises. After the Fontan procedure, a modest increase in muscularity can jeopardise the outcome since there is no subpulmonary ventricle to support the pulmonary circulation. Following heart transplantation, a slight increase in muscularity can cause failure of the donor right ventricle unless that heart is already hypertrophied as in the domino procedure. In all children with pulmonary hypertension, either persistent pulmonary hypertension of the newborn or secondary to congenital heart disease the pulmonary vasculature fails to remodel normally after birth. Newborn vessels are characterized by the immaturity of the smooth muscle cells and the paucity of connective tissue. In the hypertensive lung smooth muscle differentiation and connective tissue deposition is accelerated. In children with congenital heart disease intimal changes follow. In these children the potential reversibility of disease following intracardiac repair is determined by the type of pathological change present at the time of repair. However, pulmonary hypertensive crises can occur in young children with potentially reversible disease. Operability is not synonymous with the potential reversibility of pathological lesions. Correlations between structural findings at lung biopsy and haemodynamic findings at cardiac catheterization have improved the accuracy with which the natural and unnatural history of pulmonary vascular disease can be predicted, but is still inadequate because we do not understand the functional implications of the changes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pathophysiological and metabolic manifestations of pulmonary vascular disease in children. 139 33

Heart-lung transplantation was, for many years, conceptualized as a possible treatment for patients with combined end-stage cardiac and pulmonary disease. As experience grew with heart transplantation, particularly in the 1970s, the difficulties of performing the orthotopic operation in patients with fixed pulmonary hypertension became apparent. This further impetus for combined heart-lung transplantation led to successful animal experiments in the late 1970s, and the first successful heart-lung transplant operation was performed in 1981. There has been significant evolution in the operative technique for the recipient operation, with emphasis on preservation of the phrenic, vagal, and recurrent laryngeal nerves and on meticulous hemostasis, with particular attention to the bronchial vessels of the posterior mediastinum. Donor procurement is of critical importance to the success of the operation, and criteria for donor selection have been well established. Lung preservation remained, for many years, a significant limitation, but current techniques involving the use of prostaglandin E1 have led to safe distant procurement with ischemic times up to 6 hours. The heart-lung transplant operation remains an effective modality for the treatment of patients with congenital heart disease, primary pulmonary hypertension, chronic obstructive pulmonary disease, and cystic fibrosis.
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PMID:Technique of combined heart-lung transplantation. 148 24

The association of tetralogy of Fallot and mitral stenosis is extremely rare. This is probably the first description of this association. The clinical and haemodynamic presentation is similar to that of cyanotic heart disease with pulmonary stenosis and post-capillary pulmonary hypertension. The mitral stenosis was probably congenital as it was diagnosed at 14 months of age.
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PMID:[Tetralogy of Fallot and mitral valve stenosis]. 153 Apr 4

Patients with cyanotic congenital heart disease who have obstruction proximal to the pulmonary arteries and, hence, do not develop pulmonary hypertension, can go through pregnancy successfully, if the pregnancy and delivery are adequately cared for, with possible complications in mind. We report a successful pregnancy in a woman with congenital tricuspid stenosis and a patent oval foramen.
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PMID:A successful pregnancy in a patient with congenital tricuspid stenosis and a patent oval foramen. 154 5

A postulated association between primary pulmonary hypertension (PPH) and autoimmune diseases prompted this study. To see if autoantibodies and an association with the major histocompatibility locus (MHC) accompany the familial form of pulmonary hypertension (PHT), we determined human leukocyte antigen (HLA)-class I (A,B,C) and HLA-class II (DR,DQ) typing serologically, serum immunoglobulin (Ig) isotypes, and antinuclear (ANA)-autoantibodies in three families in which more than one member had PPH (FPPH) and in a fourth family with PPH and PHT associated with congenital heart disease. The three FPPH families had 15 PPH + members, eight (in whom the alleles were determined or could be inferred) typed for HLA-DRw52 and seven for HLA-DR3,DRw52,DQw2. These three families each had one PPH+,DR3+ member, with an immunoglobulin isotype deficiency, one with IgA and two with mild IgG. The fourth family differed in that both the patient and asymptomatic relatives had varying autoantibodies and different HLA associations. This family had an anti-centromere+ child with PHT and a congenital heart lesion, a mother who died of PPH, and asymptomatic ANA+ relatives (father anti-Sm+/anti-RNP+; autoantibodies unknown in fraternal twin sister and in maternal grandmother). HLA typing showed that the proband and her fraternal twin sister received DR5,DRw52,DQw3 from their father whereas the proband received DR4,DRw53,DQw3 from the mother and her sister received DR2,DQw1. In summary, three FPPH families had multiple PPH+ members who lacked autoantibodies and typed for HLA-DR3,DRw52,DQw2, whereas a fourth family with familial PHT had PHT+ and PHT- members with autoantibodies and different HLA associations.
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PMID:Familial pulmonary hypertension: immunogenetic findings in four Caucasian kindreds. 155 3

The increase in muscular oxygen consumption that accompanies the onset of exercise is accomplished by increases in blood flow and arterial-venous O2 difference. These processes are reflected in a similar increase in pulmonary oxygen uptake (VO2), which rises in a dynamic pattern having two components and with an overall time course that may be characterized as an exponential. Because the immediate determinants of VO2 are the blood flow and respiratory gas composition in the pulmonary circulation, it was hypothesized that VO2 kinetics at exercise onset would be abnormal in patients with pulmonary vascular disease. To test this, 10 patients with pulmonary hypertension and two with pulmonary hypoperfusion caused by congenital heart disease performed constant work rate (15 +/- 16 SD watt) exercise on an upright cycle ergometer, with breath-by-breath measurement of respiratory gas exchange for determination of VO2 kinetics. The phase I increase in VO2, comprising approximately the first 30 s of exercise, was small (18 +/- 15 SD % above resting VO2). The time constant for the phase II increase in VO2 averaged 74 +/- 16 s, and the mean response time for attainment of the exercise steady state (75 +/- 17 SD s) was prolonged compared with normal values for the same work rate exercise (approximately 100 to 130% increase in phase I, and mean response time less than 25 s). In two patients who underwent surgical procedures substantially improving pulmonary hemodynamics, VO2 kinetics also improved. These findings are consistent with the concept that VO2 kinetics may be limited by pulmonary hemodynamics in the presence of disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Oxygen uptake kinetics in response to exercise in patients with pulmonary vascular disease. 158 46


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