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

Reactive airway disease has only rarely been associated with pulmonary hypertension. We treated two patients with congenital heart disease and asthma who had increased pulmonary arterial pressure at cardiac catheterization. Pulmonary hypertension could not be explained solely by the cardiac lesion, nor by respiratory mechanical factors, as the patients did not have wheezing during the catheterization study. After long-term treatment with bronchodilators, corticosteroids, and oxygen, and coincident with improvement in the airway disease, there was catheterization-proved diminution of pulmonary hypertension. Whether asthma and pulmonary hypertension were causally linked is unknown, but further work seems indicated to elucidate the relationship between bronchoconstriction and pulmonary vasoconstriction. Furthermore, aggressive management of even mild reactive airway disease may be warranted in patients with pulmonary hypertension, regardless of apparent cause.
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PMID:Pulmonary hypertension and asthma in two patients with congenital heart disease. 275 75

Using an immunohistochemical technique, the development of the cytoskeletal proteins desmin, vimentin, and actin (using alpha isotype and non-isotype specific antibodies) was assessed using a semi-quantitative grading system in the pulmonary vascular smooth muscle of nine normal pigs and 19 normal humans at different ages, and in 13 children with pulmonary hypertensive congenital heart disease. In the normal of both species, immunostaining for vimentin decreased after birth and then increased gradually while immunostaining for desmin and alpha actin increased steadily with age. In pulmonary hypertension, immunostaining for alpha actin and vimentin showed an accelerated increase at between 2 and 8 months. Also, the media showed regional differences in immunostaining which preceded the development of intimal proliferation. The inner media showed less immunoreactivity for all cytoskeletal proteins studied than did the outer media. Within areas of intimal proliferation many cells were immunonegative. These results suggest that the cytoskeletal features of medial smooth muscle cells are remodelled in the normal infant; that this process is altered from at least 2 months in the pulmonary hypertensive infant; and that the smooth muscle cells immediately beneath the internal elastic lamina are remodelled before migrating to form intimal proliferation. Changes in cytoskeletal composition can be related to the previously described postnatal maturation of pulmonary vascular smooth muscle cells.
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PMID:Cytoskeletal features of immature pulmonary vascular smooth muscle cells: the influence of pulmonary hypertension on normal development. 276 90

Total anomalous pulmonary venous connection (TAPVC) is a cardiac disorder that seldom permits survival into adulthood without surgical correction in infancy. We reported a successful total correction in a 47-year-old man with TAPVC. Cardiovascular angiography demonstrated the features of Darling I a type TAPVC. Cardiac catheterization showed Qp/Qs: 5.8, Pp/Ps: 0.31, Rp/Rs: 0.06 and mild pulmonary hypertension. On operation, the posterior wall of left atrium anastomosed to the common pulmonary vein over 4 cm length, ASD (5.0x3.8 cm) was closed with patch, and the communicating vein was ligated. The patient made uneventful postoperative recovery and is well 3 months following surgery.
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PMID:[Total anomalous pulmonary venous connection in adult: report of a case]. 277 53

Despite success in adults, heart transplantation (HT) is still considered by many as only desperation therapy for children with end-stage heart disease. Thus, of 30 pediatric patients undergoing HT at our center, only seven (23%) patients had not developed pulmonary hypertension with increased pulmonary vascular resistance (PH) or hemodynamic decompensation (HD) requiring inotropic support at the time of transplantation. We have retrospectively reviewed the effect of preoperative PH, HD, and seven other potential risk factors on survival of our pediatric heart transplant recipients. All 30 patients, aged 5 days to 18 years, had New York Heart Association class III or IV symptoms. Twenty had idiopathic cardiomyopathy, nine had congenital lesions, and one infant had a large left ventricular tumor. A univariate and multivariate Cox proportional-hazards analysis was performed examining the effects of nine variables on survival after transplantation: PH, HD, age, need for hospitalization, congenital heart disease, need for surgical pulmonary artery reconstruction, prior stroke, history of cardiac arrest(s), and mechanical ventilator dependence. One-year actuarial survival for the entire series was 66% and was 100% for the seven patients with neither PH nor HD. None of the nine potential risk factors was a statistically significant predictor of risk, yet the combined presence of PH and HD represented a highly significant predictor of mortality (relative risk, 4.08: 1; p less than 0.002). One-year actuarial survival of the 10 patients with this combination was 30% versus 84% of those without the combination.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Optimal timing of pediatric heart transplantation. 280 8

We studied 15 cases of the scimitar syndrome. The diagnosis was suspected in 87% of the patients by x-rays studies. Bronchography is useful in order to detect bronchial anomalies, such as hypoplasia or agenesis of the medial lobe which is responsible for the left isomerism. The hemodynamic study is important in symptomatic patients in order to demonstrate associated congenital heart disease as well as the degree of pulmonary hypertension. Angiography is fundamental in determining the anomalous venous drainage of the right lung, whether complete or partial, and in demonstrating the exact site of the connection which may be to the right atrium or more frequently to the inferior vena cava above or below the diaphragm. There are variant forms in so far as the pathway, extension and connection of the anomalous venous trunk which gives rise to the scimitar sign. This syndrome is due to a persistent connection of the right pulmonary veins to the proximal portion of the right vitelin vein which normally forms the suprahepatic segment of the inferior vena cava. Asymptomatic patients should have medical treatment symptomatic ones with an associated congenital heart disease and moderate pulmonary hypertension must be subject to surgery.
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PMID:[Scimitar syndrome]. 294 26

Recent ultrastructural studies have shown that in the normal lung the pulmonary arteries adapt to extra-uterine life not by reducing the amount of vascular smooth muscle, but by reorganizing the components of the arterial wall, thereby reducing wall thickness and increasing lumen diameter. In persistent pulmonary hypertension of the newborn, either primary or secondary to hypoxia at birth, arterial wall reorganization fails to occur. Smooth muscle cells increase their contractile myofilaments and deposit connective tissue around themselves while the artery remains in its undilated state. The newborn lung is more vulnerable to hypoxia than the lung which has adapted normally in room air for three days or more. In pulmonary hypertension secondary to congenital heart disease, the type of pathological abnormalities and their natural history is determined by the type of intracardiac abnormality. Recent studies on normal lung development and on primary and secondary pulmonary hypertension have enhanced our understanding of the relation between structure and function in the immature pulmonary circulation.
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PMID:Insights into primary and secondary pulmonary hypertension in childhood. 294 37

This study was undertaken to characterize the clinical pictures of long-standing overload of the right ventricle and to investigate its hemodynamics. 1. Myocardial cell injury of the right ventricle was studied. Histopathological examinations were performed by biopsy in patients with atrial septal defect (ASD). Cell diameter was more widely distributed in the disease compared to the control. The range of distribution was more markedly scattered in ASD with congestive heart failure. Change in the ultra-fine structure was also more remarkable in ASD with than without pump failure. 2. ASD with impending myocardial infarction was discussed as an example of associated acquired disease. 3. The occurrence of pulmonary hypertension could be monitored with recently developed color Doppler method. A representative case of ASD and pulmonary hypertension was presented and discussed. 4. Right ventricular overloading associated with arrhythmias was presented. The effects of arrhythmias on Ebstein's anomaly and Uhl disease were examined. In these anomalies, development of pump failure and life-threatening arrhythmias were serious problems in managing old patients. In summary, histo-pathological examinations, monitoring of pulmonary artery pressure by Doppler echocardiography and management of arrhythmias were necessary to evaluate prognosis of congenital heart disease in the elderly.
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PMID:[Congenital heart disease with right ventricular overloading in the elderly]. 307 95

The paper gives a survey of indication, technique, immunosuppression as well as results of the isolated heart transplantation. The isolated heart transplantation represents a genuine therapeutic alternative for many hopeless patients with heart disease who cannot be helped neither with medicamentous nor with the usual cardiosurgical methods. In future the combined heart-lung transplantation might be an essential enlargement of the treatment of patients with terminal heart diseases and with a pulmonary hypertension or of patients with a terminal pulmonary disease.
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PMID:[Allogeneic heart-lung transplantation--present state and perspectives]. 309 82

Right ventricular systolic pressure is an important parameter in paediatric cardiology. A critical haemodynamic situation is attained if it exceeds the systemic pressure: strain proximal to severe pulmonary stenosis or the result of pulmonary hypertension. In these circumstances, the patient must be closely followed up at the least, and appropriate treatment, often surgical, has to be instituted. Ambulatory methods of assessing this parameter are either unreliable, like the surface electrocardiogram, or relatively complex, like certain ultrasonic methods. We therefore looked for a method relating the systolic pressures of the two ventricles and the end systolic geometry of the left ventricle as assessed simply from a short axis view of the left ventricle at the level of the junction between the chordae and papillary muscles. Two orthogonal diameters are measured and the ratio of the two dimensions calculated: this ratio which we call the "septal curvature" reflects left ventricular compression by the pressure that the right ventricle exerts through the interventricular septum. Seventy-two children aged 6 hours to 18 years (average 5.2 years) hospitalised for pre or post-operative investigation of congenital heart disease were evaluated by this method. The results were compared by statistical analysis with the ratio of ventricular pressures measured directly almost simultaneously during cardiac catheterisation. In general, a linear relation was observed between septal curvature (x) and ratio of pressures (y); y = 0.88, x - 0.63 and R = 0.92.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Evaluation of the systolic pressure of the right ventricle by echocardiographic study of left ventricular geometry]. 309 70

Under long-term oral treatment with nifedipine (N) 2 of 3 patients (2 children with VSD and Eisenmenger, 1 patient with Truncus I after corrective surgery) showed a remarkable improvement in exercise tolerance and reduction in the total pulmonary resistance. Therefore a single-dose testing with N was performed in 9 patients aged 2 to 20 years with primary pulmonary hypertension (n = 2), secondary pulmonary hypertension because of congenital heart disease with intracardiac shunts (n = 6) and after correction of Truncus Type I (n = 1). Before and after the injection of 0.5 mcg/kg of N into the main pulmonary artery, pulmonary artery pressure, cardiac output and systolic blood pressure were measured at 1 minute intervals during right heart catheterization. The maximal reaction occurred 3 to 4 minutes after the injection. The total pulmonary vascular resistance decreased significantly from 2684.2 +/- 1829.2 to 1300.2 +/- 1117.0 dyn.sec.cm-5.m2 (p less than 0.025), the cardiac index increased significantly from 3.1 +/- 1.65 to 5.73 +/- 1.95 l/min.m2 (p less than 0.01) concomitantly with a mild reduction in the mean pulmonary artery pressure. No significant change in heart rate and systolic blood pressure was seen; no side effects were observed. 4 of the 5 patients with the highest pulmonary vascular resistance showed the best positive reactions. Therefore these 9 patients may benefit from a long-term oral treatment with nifedipine.
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PMID:[Hemodynamic studies of nifedipine (Adalat) in pulmonary hypertension in childhood]. 310 Dec 92


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