Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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

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

Percutaneous endoscopic gastrostomy (PEG) is used to provide nutrition for patients who are unable to eat but have a functionally intact gut. Clinical guidelines for PEG are uncertain and have been derived mainly from referral practices. We performed a population-based cohort study in 97 residents of Olmsted County, Minnesota, referred for PEG between January 1982 and December 1988 to determine complications, duration of tube feeding, and survival. Follow-up continued until death or February 1990. Inpatient and outpatient records were reviewed to determine indications, comorbid conditions, level of consciousness, and limitations in activities of daily living. Outcomes determined after referral for PEG included type and number of complications, tube removal, and survival. Statistical methods used included Kaplan-Meier and proportional hazards regression analyses. PEG placement was successful in 94% of patients. Although complications occurred in 70% of patients, they usually were minor (88%) and most occurred within 3 months. In 24 patients, tubes were removed because eating was resumed. The probability of surviving 30 days, 1.5 years, and 4 years after referral for PEG was 78%, 35%, and 27%, respectively. The major causes of death within and after 30 days were pneumonia, heart disease, and vascular disease of the central nervous system. An increased risk of death after referral for PEG placement was associated with older age, male gender, diabetes, and specific indications for PEG. If validated in other population-based studies, these predictors of survival after referral for PEG placement could be used to identify patients with a low probability of survival who may not benefit from PEG.
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PMID:Predictors of outcome after percutaneous endoscopic gastrostomy: a community-based study. 143 74

Pulmonary arterial structure was examined in lung biopsies from 26 children with severe pulmonary hypertensive congenital heart disease, aged 2 months-18 years, in whom the mean pulmonary arterial pressure was 55 (range 35-105) mmHg, using light, transmission, and scanning electron microscopy. Qualitative and quantitative techniques were applied and findings compared with those in age-matched controls. At 2 months, the smooth muscle cells showed hyperplasia, hypertrophy (mean cell diameter increased, P less than 0.001), and accelerated differentiation. In all pulmonary hypertensive cases aged less than 6 months, the contractile myofilament concentration was similar to the normal concentration at 6 months. Medial connective tissue was excessive for age. Smooth muscle cells within the intima (intimal proliferation) were first seen at 7 months, lying beneath a new internal elastic lamina. They showed a reduction in myofilament concentration in the more fibrotic lesions. In all cases, endothelial cells were abnormally thick (P less than 0.001) and elongated. Cytoskeletal remodelling was indicated by an increase in micro- and intermediate filament volume density (P less than 0.05 for both). The severity of endothelial damage was related to vessel size and position in the arterial pathway. These findings indicate that pulmonary vascular disease begins at or soon after birth with abnormal pulmonary vascular remodelling which leads to obliterative pulmonary vascular disease.
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PMID:Onset and evolution of pulmonary vascular disease in young children: abnormal postnatal remodelling studied in lung biopsies. 156 Mar 20

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

We report on the incidence of new macrovascular disease among the 497 members of the London Cohort of the WHO Multinational Study of Vascular Disease in Diabetics (aged 35-54 years at recruitment) over a mean 8.33 year follow-up period. Overall at the end of the follow-up period the prevalence of macrovascular disease in the cohort was 45%; 43% of the subjects showed evidence of ischaemic heart disease, 4.5% of cerebrovascular disease and 4.2% of peripheral vascular disease. The incidence rates for new disease in those subjects who were free at baseline expressed per 1000 patient years of follow-up were: ischaemic ECG abnormality 23.6 (patients with insulin-dependent diabetes 19.8, patients with non-insulin-dependent diabetes 28.1), myocardial infarction 17.6 (patients with insulin-dependent diabetes 16.5, patients with non-insulin-dependent diabetes 18.8), all ichaemic heart disease 31.7 (patients with insulin-dependent diabetes 30.3, patients with non-insulin-dependent diabetes 33.4), cerebrovascular disease 5.9 and peripheral vascular disease 5.2. Incidence rates were generally similar among men and women except for myocardial infarction in patients with non-insulin-dependent diabetes where men had a significantly higher incidence rate. Macrovascular disease is a major problem in patients with diabetes and in this age group is mainly manifested as ischaemic heart disease.
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PMID:Incidence of macrovascular disease in diabetes mellitus: the London cohort of the WHO Multinational Study of Vascular Disease in Diabetics. 193 62

Sixty patients with congenital heart disease (CHD), age range 3 months-45 years (median 2 years), underwent lung biopsy to exclude pulmonary vascular disease (PVD): 25 had an atrioventricular (AV) septal defect, 14 a ventricular septal defect (VSD), 7 a complete transposition of the great arteries (TGA) + VSD, 3 an atrial septal defect, and 2 a patent ductus arteriosus; 9 had other malformations. Scoring of histological section (0-4) according to a modified Heath-Edwards classification disclosed that 30 patients had severe "irreversible" PVD (greater than or equal to grade 3) (11 AV septal defect, 8 VSD, 6 TGA + VSD and 5 others); 8 patients were younger than 1 year. Pulmonary vascular resistance (PVR) was calculated in 51 patients and exceeded 7 U/m2 in 1 of 2 patients with grade 0, in 9 of 18 with grade 1, in 1 of 4 with grade 2, in 11 of 18 with grade 3, and in 8 of 9 with grade 4. PVR was also calculated after 100% oxygen administration in 19 patients, 14 of whom had a resting PVR greater than or equal to 7 U/m2. PVR persisted greater than or equal to 7 U/m2 in 5 patients: 4 had PVD greater than or equal to grade 3 and died after surgery. PVR fell to under 7 U/m2 in 14 patients, 3 of whom had PVD greater than or equal to grade 3:1 patient with grade 4 was not operated upon and 2 with grade 3 were operated upon: all are alive. Follow-up in discharged living patients was 100%, with a mean of 50 months. Fifty-five patients underwent surgery with 10 early and 2 late deaths. Among the 12 subjects with a fatal outcome, 10 had irreversible lesions. The 5 non-operated patients all had irreversible lesions at biopsy, and are still alive.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Is lung biopsy useful for surgical decision making in congenital heart disease? 202 37

Heart transplantation has evolved from an experimental procedure with a low survival rate to a therapeutic option for patients with end-stage heart disease. The most common diagnosis among recipients is cardiomyopathy, followed by coronary artery disease and vascular disease. As immunosuppression techniques have improved and protocols standardised heart transplantation has been successfully employed by many centres. A growing insight into the risks and benefits of cardiac transplantation means that we can stratify an individual patient's risk, thereby enhancing both selection for and timing of transplantation. The number of suitable donors available is limited so we should ensure that donor's hearts go those patients who stand to gain the greatest benefit after operation. The cost-benefit ratio has been shown to be good and heart transplantation is nowadays a routine procedure in cardiac surgery.
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PMID:Present status of heart transplantation. 206 97

We performed a prospective study in 106 patients with acute stroke. The main purpose was to evaluate the associated diseases and to determine their prevalence and incidence in two different types of cerebrovascular disease: the intracerebral hemorrhage (HI) and ischaemic events (AI). The studied population included 54 men and 52 women with a mean age of 66.8 +/- 10.3 years. A clinical examination was performed in all patients by different specialists and all were submitted to diverse complementary tests, including a computed tomography scan of the brain (TAC) and an echocardiogram (ECO). We found 24 (23%) HI and 82 (77%) AI. In the past history, previous stroke were more prevalent in AI (p less than 0.01). Heart disease was present in 87 (82%) patients but, among them, only atrial fibrillation which was found in 19 (18%) patients, was significantly more frequent in AI (p less than 0.02). Hypertension (HTA) existed in 79 (75%) patients, respiratory complications and periferic vascular disease in 9 (8%), diabetes in 44 (42%) and dyslipidemia in 31 (29%) patients. No significant difference was found between the two groups of stroke regarding these diseases; however, there was a tendency for HTA and diabetes to be more prevalent in HI and for periferic vascular disease in AI. In the blood tests, high haematocrit was found in 35 (33%) patients, anemia in 21 (20%), hypercholesterolemia in 17 (16%), hypertrigliceridemia in 18 (17%) and uremia or creatinemia or ionic alteration in 32 (30%) patients, without any difference in their prevalence and incidence in the two groups of stroke. In conclusion, in this prospective study of patients with an acute stroke, there was 23% of HI and 77% of AI, a high prevalence of previous stroke, heart disease and HTA, but only the previous stroke and, within heart disease, the atrial fibrillation were significantly more frequent in the AI group. Also, periferic vascular disease had a tendency to be more frequent in AI, as well as diabetes and HTA had in HI.
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PMID:[The patient with acute cerebrovascular disorders: assessment of associated diseases]. 208 57

A retrospective review was made of 59 open lung biopsy specimens taken between 1984 and 1988 from children with congenital heart disease who were at risk for pulmonary vascular disease. Thirty-seven patients (ranging in age from 3.5 months to 23 years; median age, 14 months) had a primary left-to-right shunt (group A) and 22 patients (ages 1 to 15 years) had palliated cyanotic heart disease (group B). Forty-five of the lung biopsy specimens were requested as frozen sections. In both groups lung biopsy specimens were graded by the Heath-Edwards classification and correlated against preoperative hemodynamic data and outcome. In group A patients, carefully measured pulmonary vascular resistance and pulmonary/systemic vascular resistance ratio were reliable indicators of the structural state of the pulmonary vascular bed, obviating the need for routine lung biopsy. Pulmonary/systemic vascular resistance ratios greater than 0.45 accurately predicted all patients with irreversible pulmonary vascular disease, and pulmonary vascular resistance greater than 7 units.m2 accurately predicted all but one case of disease. Reversibility of pulmonary vascular changes is not synonymous with immediate postoperative survival: Fatal postoperative pulmonary hypertensive crises occurred in the presence of reversible pulmonary disease. Of those considered for the Fontan procedure, a mean pulmonary artery pressure less than 30 mm Hg and pulmonary vascular resistance less than 3 units.m2 correlated with Heath-Edwards grade I or normal lung biopsy results. In 36% of group B patients, reliable assessment of pulmonary vascular resistance could not be made, indicating a possible need for open lung biopsy procedures. When lung biopsy procedures were used as an isolated procedure, they were more dangerous (20% mortality, 13% morbidity) than previously reported. Intraoperative frozen sections are not adequate to accurately assess pulmonary vascular changes (9% error); serial paraffin sections are required.
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PMID:The clinical value and risks of lung biopsy in children with congenital heart disease. 210 1

Hypoxic vasoconstriction has been the subject of many studies, but little is known about the interaction of hypercapnia and the pulmonary circulation. We performed two haemodynamic studies on each of three patients with pulmonary vascular disease secondary to congenital heart disease. On the first occasion ventilation was inadequate due to technical problems, and the patients were therefore hypercapnic (arterial pCO2 greater than 5.3 kPa). On the second occasion, they were normocapnic. Pulmonary vascular resistance was measured on each occasion while the patients were breathing 100% oxygen (alveolar hyperoxia) and while epoprostenol (prostacyclin) was infused at doses of 5-20 ng/kg/min. Pulmonary vascular resistance was elevated in the presence of hypercapnia and, despite oxygen and epoprostenol, could not be reduced to the levels observed in the normocapnic study. We conclude that hypercapnia causes significant vasoconstriction in infants; and that epoprostenol is a relatively ineffective pulmonary vasodilator in infants who are hypercapnic due to inadequate ventilation. Where possible, respiratory acidosis should be corrected before using oxygen or epoprostenol as a pulmonary vasodilator.
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PMID:Interactions between alveolar hypercapnia and epoprostenol on the pulmonary circulation: clinical and pharmacological implications. 213 21


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