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

From 1987 to 1991, heart transplantation was undertaken in 49 infants and children with either end-stage cardiomyopathies (28 patients) or severe congenital heart disease (21 patients including 16 having already been surgically but unsuccessfully treated). Their age ranged from 13 days to 15 years (mean = 4.5 +/- 4.2 years; median = 2.5 years). There were 12 early and 7 late deaths (overall mortality = 38%), mainly due to graft dysfunction, acute or chronic rejection, and infectious complications, mostly viral. Optimal criteria in selecting both donors and recipients are crucial to reduce early mortality and should never be transgressed despite the critical shortage of organs. The actuarial probability of survival was 64% at 1 year and 57% at 5 years. Our 30 mid-term survivors (62%) were submitted to a close follow up programme which includes endomyocardial biopsies, even in the very young, since non invasive criteria failed to mark every rejection episode. Maintenance therapy was always steroid-free to start with (cyclosporin+azathioprine) but in almost one half of our oldest survivors, it failed to avoid rejection and we had to add low-dose oral steroids for at least several months. Epstein-Barr virus related lymphoproliferations occurred in four patients, two of whom died and two recovered with specific therapy. Renal function was closely monitored: tubular and interstitial lesions were found on renal biopsies and were associated with moderate functional changes. The quality of life of the children who survived heart transplantation was considered as near normal in a little more than one half of the cases but many issues (late coronary disease, drug toxicity, long-term compliance to follow up and therapy) remain significant concerns for the future.
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PMID:Heart transplantation in children: mid-term results and quality of life. 134 6

Reliable, prenatal detection of congenital heart disease has become possible over the past decade with the evolution of fetal echocardiography. We have documented the outcome of 170 cardiac defects diagnosed prenatally since 1984. Of 170 cases, 55 (32%) had major extracardiac malformations and 45 (28%) chromosomal abnormalities (16 had both). Elective termination was chosen in 77 (45%) pregnancies. Of 93 continuing pregnancies 15 were stillborn and 43 died postnatally (48% of these fetuses and infants had extracardiac or chromosomal anomalies, or both). Thirty-five patients survive at 1 to 80 months (mean 36). Aneuploidy or extracardiac defects are present in 20% of survivors. Nonimmune hydrops secondary to cardiac failure was present in 7 continuing pregnancies and none of these patients survived. The prognosis of prenatally diagnosed cardiac lesions is negatively influenced by the presence of cardiac failure, aneuploidy or extracardiac malformations, or a combination of these. Optimal counseling and management requires the identification of these conditions when present.
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PMID:Outcome of prenatally detected cardiac malformations. 159 Feb 38

Optimal strategies for identifying children with hypercholesterolemia have not been established. Several groups have advocated that testing of serum cholesterol levels be limited to those children who have family histories of hyperlipidemia or premature coronary heart disease. We studied the ability of comprehensive family histories to identify children with hyperlipidemia in a group of 114 children (mean age, 8 +/- 4 years) who were referred for treatment of hypercholesterolemia. A positive family history was defined according to guidelines of the American Academy of Pediatrics. The mean fasting total cholesterol in the children was 5.74 +/- 1.42 mmol/L (222 mg/dL). Family history was negative for hypercholesterolemia or premature coronary heart disease in 22 (22%) of 100 children with total cholesterol levels greater than the 75th percentile for their ages, in 13 (18.3%) of 71 children with total cholesterol levels greater than the 95th percentile for their ages, and in four (11.8%) of 34 children with presumed heterozygous familial hypercholesterolemia. Of the 78 children who had both hypercholesterolemia and positive family histories, hyperlipidemia was reported in 72 families, whereas premature heart disease was reported in only 27. We conclude that in a population of children referred because of known hypercholesterolemia, a detailed family history not only fails to identify many children with mild hypercholesterolemia, but also fails to identify a significant proportion of children with markedly elevated cholesterol levels. Additionally, in families of children with hypercholesterolemia, a history of hyperlipidemia is more common than a history of premature heart disease.
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PMID:Family history fails to identify many children with severe hypercholesterolemia. 198 31

Congenital heart disease as a complicating factor in pregnancy has assumed increasing clinical importance because improved techniques of surgical repair have resulted in a larger proportion of affected women living to the reproductive age. The most serious forms are those associated with pulmonary hypertension (such as the Eisenmenger syndrome), which carry a prohibitively high risk of maternal death. Complex forms of cyanotic heart disease, of which the commonest is the tetralogy of Fallot, are only slightly less dangerous. It has recently been recognized that children born to women with congenital heart disease are at increased risk of having cardiac defects; fetal echocardiography is therefore an important diagnostic test. Optimal care of the pregnant woman with congenital heart disease is best provided by a team consisting of internist-cardiologist, obstetrician-perinatologist, obstetric anesthesiologist, and ultrasonographer-echocardiographer.
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PMID:Pregnancy and congenital heart disease. 217 37

Patients with advanced heart failure may be candidates for mechanical circulatory support, heart transplantation, or both. Optimal medical therapy in such patients, who are frequently unstable clinically, is focused on traditional and newer methods of inotropic support. Accordingly, the response to intravenous and oral enoximone, an experimental compound with phosphodiesterase inhibitory properties, was examined in 25 patients with unstable, severe chronic heart failure as a result of ischemic or myopathic heart disease. Eight hospitalized patients had far-advanced failure and were dependent on dobutamine, dopamine, or both to support their cardiocirculatory status (group 1). Seventeen patients had severe failure and were hospitalized electively because of their clinical instability despite digoxin, diuretics, and vasodilators (group 2). Intravenous (1 to 2 mg/kg) and oral (1 to 2 mg/kg) enoximone significantly (p less than 0.05) improved right and left heart function, and the salutory hemodynamic response was sustained for 6 to 8 hours. In group 1, catecholamines were discontinued and clinical stability was reestablished on oral enoximone; stability was also restored in group 2. Nevertheless, 19 patients died on long-term enoximone therapy with approximately half succumbing to their heart failure. Thus enoximone, a compound with inotropic and vasodilator properties, was useful in the acute and short-term management of unstable, chronic heart failure and had an additive hemodynamic benefit to dobutamine, dopamine, or both. Enoximone may therefore be a useful adjunct to stabilizing these patients before mechanical circulatory support or transplantation. The advanced degree of myocardial failure in these patients, however, precludes enoximone together with standard medical therapy from having a more favorable impact on clinical outcome in these patients.
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PMID:Enoximone (MDL 17,043), a phosphodiesterase inhibitor, in the treatment of advanced, unstable chronic heart failure. 295 97

As the number of individuals over age 65 years continues to increase, the elderly will comprise an ever-growing portion of each physician's practice. While heart disease in the elderly may be similar in many respects to that in younger patients, major age-related differences do exist with regard to etiology, pathogenesis, natural history, clinical presentation, and management. Optimal management of the older cardiac patient requires that these differences be taken into account. In prescribing a medical regimen, it is important to keep in mind that age-related decreases in renal function, hepatic perfusion, and drug metabolism may result in higher drug levels and that the elderly are also often more prone to adverse drug effects, especially central nervous system changes. One should remember that chronology is not necessarily synonymous with physiology, and much depends on the individual patient in terms of the lifestyle he/she led and that which he/she wishes to have. Advanced age may be associated with greater interventional morbidity and mortality, but age alone should not be a contraindication to aggressive medical or surgical therapy.
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PMID:Heart disease in the elderly. 354 64

Optimal care of the patient with heart disease undergoing noncardiac surgery requires that the members of the surgical team, including anesthesiologist, internist-cardiologist, and surgeon, be familiar with the cardiovascular response to surgery, preoperative cardiac risk stratification, and the unique pathogenesis of cardiac complications that may occur in the perioperative period. Preoperative evaluation and computation of cardiac risk, anesthetic considerations, along with perioperative care of the patient with ischemic heart disease, valvular heart disease, congestive heart failure, arrhythmias and conduction disorders, and hypertension is discussed.
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PMID:Noncardiac surgery in the patient with heart disease. 355 69

The development of chylothorax is a serious and often life-threatening clinical entity. Optimal management of this problem has not been well defined to date. We reviewed our experience with chylothorax in patients of all ages during the past 10 years. Ages ranged from 2 days to 69 years. The etiologies were traumatic in 17 and congenital or idiopathic in three. Six patients (five infants) were treated nonoperatively with either repeated thoracenteses or chest tube drainage. Fourteen patients (11 infants) underwent operative treatment: transthoracic thoracic duct ligation (five patients), pleuroperitoneal shunting (seven), pleuroperitoneal shunting combined with reoperation on a patient with congenital heart disease (one), and reoperation alone on a patient with congenital heart disease (one). Duration of preoperative therapy ranged from 9 days to 2 months (average 3.3 weeks). Five of six (83.3%) patients treated nonoperatively died. Of the surgically treated group, only two of 14 (14.3%) died, and 11 of the 12 survivors had resolution of the chylothorax and immediate clinical improvement. Our experience suggests that both pediatric and adult patients respond poorly to nonoperative treatment of chylothorax and that this treatment has a high mortality rate. Post-traumatic and congenital chylothorax should be treated operatively after a limited trial (1 to 2 weeks) of nonoperative therapy. Pleuroperitoneal shunting may offer a reasonable and effective alternative to thoracotomy and thoracic duct ligation.
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PMID:Chylothorax: an assessment of current surgical management. 396 5

As a result of hypoxia following right-to-left shunts, cerebral bacterial spreading and an altered blood-brain-barrier permeability, brain abscesses (BA) are typical complications in patients with cyanotic congenital heart disease. In 483 prospectively followed patients the incidence of BA was 0.45%/year. It was higher (0.57%/year) for patients with tetralogy of Fallot where the cumulative risk within the first two decades of life was 12.1 +/- 1.7%. The risk of BA complicating cyanotic heart disease is inconstant and continuously increasing up to approximately age 12 (instantaneous risk at that time: 1.75 +/- 0.12%), decreasing thereafter. With respect to etiology, infectious endocarditis, infections per continuitatem, bacterial meningitis, bacterial lung diseases with intrapulmonary shunts, and thromboembolic complications of systemic infections have to be differentiated. The stepwise diagnosis includes CCT to demonstrate the typical contrast enhancement and a lumbar puncture which shows granulocytic pleocytosis. If the cerebral spinal fluid fails to demonstrate the typical findings, cerebral angiography may be necessary to exclude a malignant vascularized neoplasma. In cases of doubt, stereotactic cerebral biopsy should be performed. Optimal antibiotic therapy after determining the minimal bactericidal concentration and combination of antibiotics is of utmost prognostic significance. Cranial computed tomography should be repeated after 6, 14, and 24 days. Infections resistant to antibiotics may necessitate local instillation of antibiotics.
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PMID:[Brain abscess in patients with cyanotic heart defects]. 751 30

Because many infants who require cardiac operation have cyanotic heart disease, we determined whether the existing calcium content of St. Thomas' II solution (1.2 mmol/L) is optimal to protect the immature rabbit heart hypoxemic from birth during subsequent ischemia. Modified hypothermic St. Thomas' II solutions (calcium content, 0 to 2.4 mmol/L) were compared with hypothermic Krebs bicarbonate buffer in protecting chronically hypoxemic (PaO2 = 34 +/- 11 mmHg, SaO2 = 63% +/- 3%) versus normoxemic (PaO2 = 76 +/- 11 mmHg, SaO2 = 92% +/- 3%) immature hearts (7 to 12 days old) during ischemia. Hearts (n = 6 per group) underwent aerobic 'working' perfusion with Krebs bicarbonate buffer and cardiac function was measured. The hearts were then arrested with a 3 minute infusion of either cold (14 degrees C) Krebs buffer (1.8 mmol calcium/L) as hypothermia alone or modified St. Thomas' II solution before 6 hours of hypothermic (14 degrees C) global ischemia. Hearts were reperfused and postischemic enzyme leakage and recovery of function were measured. A bell-shaped dose-response profile was observed for recovery of postischemic aortic flow but not for postischemic creatine kinase leakage, with improved protection occurring at lower calcium concentrations. Optimal myocardial protection occurred at a calcium content of 0.4 mmol/L, which was significantly better than with hypothermia alone or standard St. Thomas' II solution. We conclude that the existing calcium concentration of St. Thomas' II solution is responsible, in part, for its inadequate protection of immature myocardium hypoxemic from birth during ischemia.
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PMID:Calcium and cardioplegic protection of the ischemic immature heart: impact of hypoxemia from birth. 794 63


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