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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this clinical study, 20 high risk infants and neonates were monitored to identify and characterize pulmonary hypertensive crises following surgery for congenital cardiac defects. Monitoring included right ventricular or pulmonary artery pressure catheters and transcutaneous oximetry. Eleven patients also had continuous analog recording of hemodynamic data so that antecedents of crises and the sequence of events following treatment could be reconstructed. Eleven of the 20 patients had one or more crises. Six of these ultimately died whereas 5 patients survived with aggressive vasodilator therapy. Four patients without crises but with episodic pulmonary hypertension benefitted from pulmonary vasodilator therapy to ease weaning from ventilatory support. Typically, each crisis was associated with a stress event. Crises were difficult to ablate if not rapidly treated and multiple crises would often cluster following an initial event. High dose narcotic (fentanyl) analgesia was found to be important in the postoperative management. Tolazoline and oxygen were the most consistently useful vasodilators, but isoproterenol and nitrates also played a role. Five of the children who died were examined post mortem: histologically, there was increased pulmonary arterial muscularization in 2, in none were there changes of fixed pulmonary vascular disease. The postoperative management must be individualized on the basis of monitored responses of pulmonary circulation.
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PMID:Pulmonary hypertensive crises following surgery for congenital heart defects in young children. 177 78

This report describes a combination of a ventricular septal defect and partial anomalous pulmonary venous drainage in an infant with right lung agenesis. Unilateral agenesis of the lung has been rarely described. Although this condition is compatible with long survival, morbidity and mortality are increased by the associations with other malformations both intra- and extracardiac. In unilateral lung agenesis although there is a compensatory increase in volume of the single lung, there is a reduction in the number of branches of the pulmonary artery and the whole right ventricular output passes through a reduced vascular bed. In isolated unilateral agenesis of the lung, pulmonary hypertension is present in about 19% of cases, while in patients with additional left to right shunt the incidence is 88%. The basic condition of increased pulmonary blood flow (single lung) and the undiagnosed partial anomalous pulmonary venous drainage may explain why the early closure of the ventricular septal defect did not prevent the progressive pulmonary vascular disease. We discuss shortly Tolazoline as pulmonary vasodilator.
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PMID:Right lung agenesis. A case report. 715 88