Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary arterial hypertension in children can occur secondary to shunt lesion like ventricular septal defect, patent ductus arteriosus or it may be idiopathic, the so called primary pulmonary hypertension (PPH). The progression of PPH is usually rapid in children as compared to adults and the mean survival is 2-3 years after the diagnosis is made. Histological changes in the form of medical muscular hypertrophy, intinal hyperplasia and later angiomatous, plexiform lesions occur in pulmonary vasculature. The pulmonary vasculature normally is a high flow, low resistance circuit and allows large blood flow without marked increase in pulmonary arterial pressure. However, with prolonged increased flow or any other vasoconstrictor stimulus, histological changes start occurring in the pulmonary bed resulting in increasing pressure in pulmonary artery. Right ventricular hypertension follows resulting in right ventricular hyypertrophy and later dysfunction. Life threatening arrhythmias may result in sudden death in some of these patients. Clinical presentation is in the form of exertional dyspnoea with syncope at times. Over 50% of children with PPH are helped by vasodilators. They may be treated with calcium channel blockers (e.g. nifedipine, dose titrated to blood pressure) orally. Those not responding to oral vasodilators can be put on chronic inhaled nitric oxide or continuous intravenous prostacyclin infusion. Chronic anticoagulation therapy may also increase survival. In symptomatic cases, blade/balloon atrial septostomy may increase survival in patients of PPH with intact atrial sptum. For children not responding to medical therapy, lung transplantation may be the answer in near future.
 ...
PMID:Diagnosis and management of primary pulmonary hypertension. 1112 20

There are patients with congenital heart disease and fatal pulmonary hypertension in whom the medial hypertrophy of the small pulmonary arteries is quite beyond the extent of ordinary cases of hypertension, a condition described as pulmonary hypertension with extremely thickened media of small pulmonary arteries (PH/ETM). Lungs from 6 infants, all younger than 2 years of age, who had congenital heart disease and fatal pulmonary hypertension, were analyzed by accurately measuring the media using Suwa's method. In PH/ETM, the media of the small pulmonary arteries was shown to be not only unusually thick, but extending toward the periphery, whereas the intimal changes were unexpectedly mild. In the PH/ETM group, the % wall thickness at a diameter of 50 microm (%Tw(50)), determined from regression analysis, was 23.2+/-1.3%, which was significantly higher than in either the control (10.3+/-1.2%) or ventricular septal defect group (18.9+/-1.6%). In persistent pulmonary hypertension of the newborn (PPHN), it was 22.3+/-1.8%, not significantly different from PH/ETM. The striking medial hypertrophy in PH/ETM and PPHN was apparently confined to small pulmonary arteries and in both conditions is likely to be the result of maldevelopment of these arteries. Surgical intervention may trigger a critical elevation of the pulmonary arterial resistance.
 ...
PMID:Extremely thickened media of small pulmonary arteries in fatal pulmonary hypertension with congenital heart disease--a morphometric and clinicopathological study. 1119 81

The year 2000 was rich in events, either spectacular news or confirmed improvement of on-going advances, as far as paediatric cardiology is concerned. The selection presented by the authors includes the first percutaneous implantation in a human being of a biological (bovine) valve which was sewn on a stent, compressed into a catheter and inserted against a stenotic and leaking procine bioprosthesis in a right-ventricle to pulmonary-artery conduit. This may be a new way to further valve replacements as alternatives to surgery. Balloon dilation of late postoperative recoarctations is now also improved with the use of stents able to maintain the result and to avoid traumatic injuries, with new coaxial double balloons making the procedure easier and safer. This is probably one of the main elements in reducing this very particular form of hypertension, the anatomic cause of which is often difficult to understand. As for yesterday's daring innovations now becoming near-routine protocols, two examples are developed. First, the rehabilitation of pulmonary arteries in pulmonary atresia with ventricular septal defect and complex pulmonary blood supply, both by true pulmonary vessels and by collaterals, both being stenotic and/or hypoplastic, anastomosed or not. The anatomic and functional details of such a vascular setting should be accurately understood and treated by early and aggressive surgery and interventional procedures in order to promote antegrade flow, distal angiogenesis, and, finally, active and harmonious vascular growth compatible with complete repair. The second example is Friedreich's ataxia in which, within 3 years of the discovery of the pathogenic mechanism, the deficiency in frataxin and its intra-cellular toxic consequences have been demonstrated, leading to a logical medical therapy which proves to be effective in treating (and maybe in preventing) the severe hypertrophic cardiomyopathy associated to this disease.
 ...
PMID:[The best in 2000 on pediatric cardiology]. 1126 Aug 39

Hypoplastic left heart syndrome (HLHS) is a complex combination of cardiac malformations that probably results from multiple developmental errors in the early stages of cardiogenesis and that, if left untreated, invariably proves fatal. A variety of chest radiographic findings are seen in patients with HLHS, including an enlarged cardiac silhouette (notably a prominent right atrium), pulmonary venous hypertension, an atrial septal defect, and valvular stenosis or atresia. The recent evolution of palliative surgical procedures (modified Norwood procedure, bidirectional cavopulmonary shunt, modified Fontan procedure, aortic valvuloplasty, heart transplantation) has increased the survival rate in children with HLHS. Echocardiography allows accurate assessment of the size and location of the ductus arteriosus, the hemodynamics of the aortic root, the patency and size of the foramen ovale or atrial septal defect, and the presence of a ventricular septal defect to help determine whether surgical intervention is appropriate and, if so, to facilitate planning. Pediatric radiologists now view radiologic images obtained in patients with HLHS before surgical intervention and at important intervals during treatment. Familiarity with the malformations that characterize HLHS and the surgical procedures used to enhance postnatal survival will help pediatric radiologists provide better care for patients with this relatively common pathologic condition.
 ...
PMID:Hypoplastic left heart syndrome. 1135 17

The authors present the case of a 26 years old female, 56 kg weight and 154 cm height, with a giant patent ductus arteriosus (2.4 cm of internal diameter), ventricular septal defect, discrete preductal narrowing of the aortic arch and pulmonary artery hypertension that did not diminished after 100% oxygen breathing. The authors speculate about the origin of the giant ductus here presented, based on hemodynamic and embryological data.
 ...
PMID:Unusual giant patent ductus arteriosus associated with ventricular septal defect and discrete aortic coarctation. A case report. 1156 7

In 1979, we first reported occurrence of biventricular hypertrophy in the original normotensive Wistar-Kyoto (WKY) strain obtained from the National Heart, Lung, and Blood Institute, which was derived directly from the Kyoto laboratory of Okamoto. At that time, we recommended that both ventricles be weighted when WKY are studied so that invalid conclusions are not made. Because no paper confirmed these findings for almost 20 years, heart weights were reported in only a few WKY studies, and the cause of this biventricular hypertrophy remained unknown, we re-evaluated this problem in commercially available rats. We, therefore, investigated WKY rats using transthoracic echocardiography to define the congenital heart defect. Up to 28% of commercially available WKY rats demonstrated severe congenital cardiac abnormalities associated with biventricular hypertrophy. Ventricular septal defect with pulmonary regurgitation was the most commonly encountered cardiac defect; other abnormalities included patent ductus arteriosus, and valvular defects. Pathologic and invasive hemodynamic studies confirmed these echocardiographic findings. Because this defect occurs in a large number of WKY rats obtained commercially from 2 different sources, investigators using this strain must carefully measure both ventricular weights to be certain that inappropriate and invalid conclusions are not derived therefrom.
Hypertension 2002 Aug
PMID:High rate of ventricular septal defects in WKY rats. 1215 9

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.
 ...
PMID:[Tetralogy of Fallot with right pulmonary artery origin from the ascending aorta: noninvasive diagnosis and surgical correction in a 7-week-old infant]. 1244 70

Atrial septal defects, atrio-ventricular septal defects, ventricular septal defects and the persistent arterial duct are the most common congenital heart defects which may cause, in the presence of a significant left-to-right shunt, chronic volume overload of the heart and lead to the development of pulmonary arterial hypertension. Repair is indicated to avoid these complications and evolution to right-to-left shunting (Eisenmenger syndrome). Although the long-term results of surgical interventions in uncomplicated congenital heart defects were excellent, percutaneous techniques to repair the defects became a focus of attention. The persistent arterial duct, the secundum type atrial septal defect, and, more recently, the muscular and perimembranous ventricular septal defect are currently eligible for percutaneous closure. By avoiding a sternotomy or a thoracotomy, complaints of pain become exceptional and the duration of hospitalisation is shortened. Percutaneous closure is not only better tolerated than surgery, it may also imply favourable economical aspects.
 ...
PMID:Left-to-right shunting in common congenital heart defects: which patients are eligible for percutaneous interventions? 1284 9

A 4-year-old boy in whom a muscular ventricular septal defect (VSD) had been diagnosed at birth presented with limited exercise tolerance and mild cardiomegaly. The moderately large defect was closed with an umbrella device via percutaneous heart catheterisation. Thereafter, his symptoms disappeared and the dimensions of the left heart returned to normal. A VSD occurs in 1.5-3.5 per 1,000 live births. In symptomatic patients with a medium or large-sized VSD, surgical closure is indicated to prevent the development of a fixed pulmonary resistance hypertension, ventricular dysfunction and the risk of endocarditis. Depending on the size and localisation of the defect, closure with an umbrella device may be chosen. The initial results have been promising in children. The safety and efficacy in the long term are still unknown.
 ...
PMID:[Closure of a ventricular septum defect in a 4-year-old boy during percutaneous heart catheterisation]. 1508 28

We report the combined use of inhaled nitric oxide (iNO) and sildenafil to selectively lower pulmonary vascular resistance (PVR) and reverse right-to-left shunt flow across a congenital ventricular septal defect (VSD) in a 50-year-old man. The patient developed right-to-left shunt flow with profound hypoxia because of an acute pulmonary embolism superimposed on underlying pulmonary hypertension. The dramatic improvement in oxygenation and PVR with sildenafil plus iNO has been sustained with sildenafil monotherapy. To our knowledge, use of this combination therapy in patients with decompensated pulmonary arterial hypertension (PAH) and a predisposition to right-to-left shunting has not been previously reported.
 ...
PMID:Pulmonary thromboembolism superimposed on a congenital ventricular septal defect in a 50-year-old man inhaled nitric oxide and sildenafil to the rescue. 1519 35


<< Previous 1 2 3 4 5 6 7 8 9 10