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)

Open lung biopsy has proven beneficial in the treatment of life-threatening pulmonary diseases. Its safety and efficacy in infants and children placed on extracorporeal membrane oxygenation (ECMO) for severe respiratory failure is not known. The authors reviewed eight cases (4 neonates, 3 infants, 1 child) who underwent open lung biopsy while on ECMO. The primary diagnoses were pneumonia (4), lymphoma (1), primary pulmonary hypertension (1), and complex congenital heart disease (2). The patients underwent biopsy after they had been on ECMO an average of 9.6 days (range, 1 to 14 days). Biopsy results confirmed the clinical diagnosis in five patients, two of whom had irreversible alveolar destruction resulting in ECMO withdrawal. Three patients had pathological diagnoses, which resulted in major therapy revisions (1 fungal infection and 2 noninfectious lesions that required steroid treatment). The overall average duration of ECMO treatment was 16.3 days (range, 10 to 24 days). Three patients were weaned successfully from ECMO, but only one infant survived to discharge. One nonlethal bleeding complication occurred after biopsy. Open lung biopsy is well tolerated during ECMO. It accurately determines pulmonary pathology and provides valuable prognostic information. Earlier biopsy for patients whose diagnoses are uncertain or who are not responding to ECMO may improve the mortality rate for this high-risk group.
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PMID:Open lung biopsy in pediatric patients on extracorporeal membrane oxygenation. 972 18

Echocardiographic assessment of right ventricular function remains difficult and challenging. However, there is considerable clinical need for a simple, reproducible, and reliable parameter of right ventricular function in patients with right-sided heart disease. The purpose of this study was to assess the clinical value of a Doppler-derived index, combining systolic and diastolic intervals of the right cycle, in assessing global right ventricular function in patients with primary pulmonary hypertension. The study population comprised 26 consecutive patients with primary pulmonary hypertension and 37 age-matched normal subjects. The sum of right ventricular isovolumetric contraction time and isovolumetric relaxation time was obtained by subtracting right ventricular ejection time from the interval between cessation and onset of the tricuspid inflow velocities with pulsed-wave Doppler echocardiography. An index of combined right ventricular systolic and diastolic function was obtained by dividing the sum of both isovolumetric intervals by ejection time. The index was compared with available parameters of systolic or diastolic function, clinical symptoms, and survival. Right ventricular isovolumetric contraction time and isovolumetric relaxation time were prolonged significantly in patients with primary pulmonary hypertension (85 +/- 41 msec and 135 +/- 43 msec) compared with normal subjects (38 +/- 7 msec and 49 +/- 9 msec, respectively; p < 0.001). Ejection time was shortened significantly in patients with pulmonary hypertension (241 +/- 43 msec versus normal [322 +/- 21 msec]; p < 0.001). However, the index was the single most powerful variable to discriminate patients with primary pulmonary hypertension from normal subjects (0.93 +/- 0.34 versus 0.28 +/- 0.04; p < 0.001) and was the strongest predictor of clinical status and survival. The index was not significantly affected by heart rate, right ventricular pressure, right ventricular dilation, or tricuspid regurgitation. It is well known that right ventricular systolic and diastolic dysfunction coexist in patients with primary pulmonary hypertension. This article reports the use of an easily obtainable Doppler-derived index that combines elements of systolic and diastolic function. This index appears to be a useful noninvasive means that correlates with symptoms and survival in patients with primary pulmonary hypertension.
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PMID:Doppler echocardiographic index for assessment of global right ventricular function. 1098 96

The hemodynamic effects of acute oral administration of a newly-developed prostacyclin analogue (beraprost sodium; 1-2 micrograms/kg), inhaled nitric oxide (NO; 20 ppm) and tolazoline hydrochloride (1 mg/kg) were measured in 17 children (mean age 1 year and 9 months) with pulmonary hypertension complicating congenital heart disease or primary pulmonary hypertension. Beraprost, NO and tolazoline achieved approximately equivalent reductions in pulmonary vascular resistance (20%, 26% and 18%, p < 0.05), but the greatest percentage decrease of pulmonary to systemic resistance ratio was obtained after administration of NO (33%, p < 0.05). Furthermore, combined administration of beraprost and NO produced the maximum effect of pulmonary vasodilation without adverse effects (49%). Beraprost appears to be an effective and available substitute for NO and tolazoline in screening for pulmonary vasodilator responsiveness. The combined use of beraprost and NO may provide an alternative treatment for pulmonary hypertension in children without serious complications.
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PMID:Acute effect of oral prostacyclin and inhaled nitric oxide on pulmonary hypertension in children. 912 38

Microangiopathic hemolytic anemia and thrombocytopenia have been reported in patients with primary pulmonary hypertension, but not in patients with congenital heart disease even if accompanied with pulmonary hypertension. We present a 7-year-old boy with atrial septal defect and pulmonary hypertension who developed microangiopathic hemolysis and thrombocytopenia. Microangiopathic hemolytic anemia and thrombocytopenia should be remarked as a complication in patients with congenital heart disease.
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PMID:Microangiopathic hemolytic anemia and thrombocytopenia in a child with atrial septal defect and pulmonary hypertension. 916 53

Deceleration time (DT) of the early transmitral flow velocity has recently been highlighted as a simple, noninvasive indicator of pulmonary arterial wedge pressure. In patients with pulmonary hypertension without left-sided heart disease, however, increased right ventricular pressure may result in an abnormal ventricular septal motion, which may impact on left ventricular (LV) early diastolic filling. We sought to determine if DT may be influenced by the severity of pulmonary hypertension in patients without left-sided heart disease. Doppler-derived transmitral flow and hemodynamic parameters were simultaneously assessed in 26 patients with pulmonary hypertension (primary pulmonary hypertension = 11; chronic thromboembolism = 15). Transmitral Doppler variables including DT were correlated with hemodynamics and LV deformity index measured in early diastole with 2-dimensional echocardiography. DT significantly correlated with the total pulmonary resistance (r = -0.70, p <0.001). Multivariate analysis revealed that DT was independently determined by total pulmonary resistance, but not by pulmonary arterial wedge pressure, heart rate, or patient's age in our study population. DT showed a correlation with LV deformity index (r = -0.74, p <0.001). These results indicate that DT may shorten in association with the severity of pulmonary hypertension and that the shortened DT in pulmonary hypertension may be attributable to right ventricular pressure overload which causes geometric changes.
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PMID:Shortening of Doppler-derived deceleration time of early diastolic transmitral flow in the presence of pulmonary hypertension through ventricular interaction. 918 41

Glomerulomegaly is a histologic finding present in idiopathic pulmonary hypertension, congenital cyanotic heart disease, morbid obesity associated with sleep apnea syndrome, sickle cell disease, and polycythemic states. This study examines the case of a 34-yr-old woman with idiopathic pulmonary artery hypertension who presented with nephrotic-range proteinuria. Kidney biopsy revealed enlarged glomeruli with mesangial-proliferative glomerulonephritis. A review of the pertinent literature and a discussion of the proposed pathophysiologic mechanisms leading to glomerulomegaly are presented.
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PMID:Glomerulomegaly and proteinuria in a patient with idiopathic pulmonary hypertension. 940 1

Morphogenesis in the human body is mediated to an important, but not exclusive, extent by apoptosis. That this is true in the heart is illustrated by primary pulmonary hypertension, which includes medial degeneration in both coronary and pulmonary arteries, normal and abnormal postnatal involution of the right ventricle, familial progressive heart block ending in fatal arrhythmias, and the long QT syndrome. Apoptosis occurs in ventricular myocardium, specialized myocytes of the conduction system, endothelium and medial smooth muscle of small arteries, cardiac nerves and ganglia and fibroblasts. The normal onset, duration and termination of apoptosis act beneficially. If apoptosis fails to occur or is too long continued, the result is harmful. Little is known of the signals controlling apoptosis in the human heart. Future research into these questions may radically change many of our present concepts of congenital heart disease.
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PMID:Apoptosis in congenital heart disease. 945 42

Abnormal biosynthesis of thromboxane and prostacyclin has been implicated in patients with primary pulmonary hypertension and secondary pulmonary hypertension associated with congenital heart disease, and could be involved in the pathogenesis of pulmonary vascular disease. The chronic effects of an oral prostacyclin analogue, beraprost sodium, on thromboxane and prostacyclin biosynthesis and on pulmonary circulation were investigated in 15 children with pulmonary hypertension. The plasma concentrations of thromboxane B2 and 6-keto-prostaglandin F1 alpha were measured, as was the urinary excretion of 11-dehydro-thromboxane B2 and 2,3-dinor-6-keto-prostaglandin F1 alpha, which are stable metabolites of thromboxane A2 and prostacyclin, respectively. In patients with pulmonary hypertension, the plasma concentration of thromboxane B2 and the ratio of thromboxane B2 to 6-keto-prostaglandin F1 alpha were greater than in healthy controls: 210 +/- 49 versus 28 +/- 4 pg/mL (P < 0.05) and 32.6 +/- 8.9 versus 5.7 +/- 1.8 (P < 0.01), respectively. After 3 months of administration of beraprost, the plasma concentration of thromboxane B2 and the ratio of thromboxane B2 to 6-keto-prostaglandin F1 alpha were reduced significantly: 210 +/- 49 to 98 +/- 26 pg/mL (P < 0.01) and 32.6 +/- 8.9 to 18.0 +/- 6.7 (P < 0.05), respectively. In contrast, the plasma concentrations of 6-keto-prostaglandin F1 alpha in patients were slightly but not significantly higher than in controls, and did not change significantly after administration of beraprost. The concentrations of 11-dehydro-thromboxane B2 and 2,3-dinor-6-keto-prostaglandin F1 alpha in urine correlated significantly with thromboxane B2 and 6-keto-prostaglandin F1 alpha, respectively, in plasma. Beraprost improved the imbalance of thromboxane and prostacyclin biosynthesis and has a potential efficacy for preventing the progressive development of pathological changes in pulmonary vasculature.
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PMID:Chronic effects of oral prostacyclin analogue on thromboxane A2 and prostacyclin metabolites in pulmonary hypertension. 958 94

1. The number of heart transplant operations performed in the United States has decreased by 16 procedures between 1995 (2,360) and 1996 (2,344). Following a period of rapid growth from 1990 (203) through 1995 (871), there was a decrease of 71 lung transplant procedures between 1995 (871) and 1996 (800). 2. The most frequently reported indication for heart transplantation in the US was coronary artery disease (44.88%). For other thoracic transplants, the most frequently reported indications included cystic fibrosis (31.85%) for double lung, emphysema/COPD (55.88%) for single lung and congenital heart disease (48.72%) for heart-lung transplants. The most frequently reported diagnoses for thoracic transplantation outside the US included cardiomyopathy (47.4%) for heart, cystic fibrosis (33.0%) for double lung, idiopathic pulmonary fibrosis (29.1%) for single lung and primary pulmonary hypertension (23.4%) for heart-lung transplants. 3. US heart transplant recipients were predominantly male (77.6%), older than age 50 (55.4%) and white (82.3%). In contrast, US lung transplant recipients were predominantly female (52.1%), aged 35-64 (73.5%) and white (89.5%). No significant variance from the US recipient demographic profile was noted for non-US recipients in this analysis. 4. The mean donor age for heart and lung transplants has risen slightly with an increase in mean age of 3.12 years for heart donors and 4.72 years for lung donors from 1987-1997. 5. The one-year survival rate for thoracic transplants performed in the US was 84.8% for heart, 70.1% for lung and 73.4% for heart-lung in 1996. Five-year survival for US thoracic transplants was 66.5% for heart and 43.2% for lung transplants performed in 1992. 6. There was little change in heart transplant survival based on transplant era (1987-89, 1990-92 and 1993-95). Lung recipients transplanted in the 1993-95 era showed a 16% increase in survival compared with those transplanted in the 1987-89 era at the 48-month time point. 7. The most important risk factor for US heart recipients at one month, one year, and conditionally at 5 years after transplantation was receipt of a previous heart transplant. Other substantial long-term risk factors included donor age and non-white, non-black recipient. 8. The most important risk factors for mortality in US lung recipients were the order of the transplant (primary or repeat) and the patient's medical condition at time of transplant. Diagnosis, recipient age and recipient race were highly influential risk factors for conditional 5-year mortality. 9. For heart and lung recipients, the major cause of hospitalization during the first 2 years after transplantation was infection.
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PMID:Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT International Registry for thoracic organ transplantation. 991 89

Based on the clinical observation that patients with right or left heart failure often present with hyperuricaemia, the relation between serum urate values and haemodynamic variables was studied in patients with primary pulmonary hypertension (PPH) as well as in patients with advanced ischaemic heart disease or dilated cardiomyopathy. The study was a retrospective analysis of 39 patients with PPH and 36 patients with left heart disease, examining serum urate levels in association with haemodynamic variables. Elevated urate concentrations were found in 79% of the PPH patients. There was no association between serum urate levels and mean pulmonary artery pressures, but a significant correlation was found between urate levels and the cardiac index (r=0.48; p=0.0021) and an even stronger correlation between serum urate levels and mean right atrial pressures (r=0.83; p<0.0001). A similar association was found in a subgroup of 21 PPH patients not receiving diuretics. In 36 patients with ischaemic heart disease or dilated cardiomyopathy, hyperuricaemia was present in 78% and was significantly associated with elevated right atrial pressures (r=0.40; p=0.031) and even more so with elevated left atrial pressures (r=0.55; p=0.0005) but not with the cardiac index (r=0.034; p=0.86). The data show that hyperuricaemia in patients with cardiac dysfunction is closely related to elevated right or left atrial filling pressures.
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PMID:Hyperuricaemia in patients with right or left heart failure. 1023 47


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