Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two young Black female patients with pulmonary valve stenosis and intact ventricular septa are presented in protracted congestive cardiac failure with severe tricuspid insufficiency and, in one, atrial fibrillation. Right ventricular systolic dysfunction was manifested by peak systolic pressures below systemic level, raised end-diastolic pressures and low cardiac output, but without right-to-left shunt. These findings are in strong contrast to those found in most patients with pulmonary stenosis of long standing, where persistent impairment of right ventricular function is diastolic with a high end-diastolic pressure and reversal of an interatrial shunt which result from poor right ventricular compliance. Evidence of left ventricular dysfunction was also present in both cases. Protracted heart failure in these patients is believed to have been the result of coincidental cardiomyopathy in a racial group highly predisposed to this disorder. A diagnostic appreciation of this phenomenon is important in the evaluation of heart disease in the Black, since cardiomyopathy may modify or even mask the features of the underlying disorder.
 ...
PMID:Pulmonary stenosis and impaired myocardial function. 12 85

Two hundred fifty-one patients with Down syndrome and congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients uncerwent cardiac surgery. Motality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.
 ...
PMID:Down syndrome with congenital heart malformation. 13 59

The Hancock conduit that contains a porcine xenograft valve has been used in part of the cardiac repair of 22 patients with complex congenital heart disease. Five patients had a severe form of tetralogy of Fallot; six had pulmonary atresia; five had transposition of the great vessels, ventricular septal defect (VSD), and pulmonic stenosis; five had truncus arteriosus; and one had "corrected" transposition, VSD, and pulmonic stenosis. The hospital mortality was 2/22. This conduit has proved a satisfactory method to establish right ventricular-pulmonary artery continuity.
 ...
PMID:Discontinuity between the heart and the pulmonary circulation. Its management with the use of a valved external conduit. 43 31

Patients over 18 years of age who have undergone a surgical correction of a congenital cardiac malformation during the period 1968 through 1977 have been reviewed. Those patients with calcific aortic stenosis which was thought, but not proved, to have arisen in a congenitally malformed aortic valve, were excluded from the review. Patients with cystic medial necrosis of the aorta were similarly excluded. There were 139 patients in the series (age range from 18 years to 67 years). The most common defects were those involving the intra-atrial septum and the related great veins, 50%. Abnormalities involving the great arteries including patent ductus arteriosus and coarctation of the aorta accounted for 19%. Common defects of conal development including ventricular septal defects and Tetralogy of Fallot malformations accounted for 15%. Valvular abnormalities including pulmonic stenosis, aortic valve abnormalities and Ebstein's malformation of tricuspid valve accounted for 11.5%. Complex congenital malformations were relatively uncommon, 4%. There were two patients with a combination of acquired and congenital heart disease. There were two operative deaths in the series, both occurring in patients with complex forms of congenital heart disease (multiple ventricular septal defects, double outlet right ventricle). There were two additional postoperative hospital deaths, one occurring following repair of an atrial septal defect from massive pulmonary embolus, and another occurring six weeks following a Fontan procedure performed for tricuspid atresia. Thus, the hospital mortality for the series was 2.9%. This reviewed series reveals the incidence of operable congenital heart defects appearing in an adult cardiac surgical practice and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.
 ...
PMID:Surgical correction of congenital heart disease in the adult: experience with 139 patients. 69 29

Seven (29%) of 24 patients with de Lange's syndrome were found to have congenital heart disease. Right-sided obstructive lesions were most commonly encountered. Approximately one in five of all patients with the de Lange syndrome exhibit congenital heart disease, usually ventricular septal defect or pulmonic stenosis.
 ...
PMID:Congenital heart disease in de Lange's syndrome. 84 82

Operative mortality from surgical treatment of congenital heart disease has been steadily reduced over the past 20 years. During the same period it has become clear that a proportion of survivors have residual clinical problems. Some of these are due to imperfect repair, a number being due to factors beyond present surgical control, and some are the consequence of associated cardiovascular defects. The chief problems can be elucidated by an analysis of the results of surgical treatment of six cardiac malformations: simple pulmonary valve stenosis, coarctation of the aorta, secundum atrial septal defect, isolated ventricular septal defect, tetralogy of Fallot and transposition of the great arteries. Recognition of the sequelae of preoperative hemodynamic strain and apparently minor associated malformations is important, and it is possible to anticipate such factors. Long-term follow-up of patients after operation is particularly important.
 ...
PMID:Evaluation of late results of surgical treatment of congenital heart disease. 118 30

The past year has seen important papers on the risk of acquired immunodeficiency syndrome in the invasive radiology laboratory with emphasis on awareness and prevention. Interventional catheterization techniques in congenital heart disease are reviewed. Valvoplasty of pulmonic stenosis has the greatest success and least complication rate. The majority of complications appear to be at the vascular access site and are more common in neonates and in procedures that are ultimately unsuccessful. Intrauterine valvoplasty of the aortic valve is reviewed as is angioplasty for a recurrent coarctation of the aorta. Closure of a persistent ductus arteriosus with a percutaneously introduced prosthesis and occlusion of congenital coronary artery arteriovenous fistulae with a variety of embolic agents is reviewed. The use of balloon-expandable stents in congenital heart disease is discussed. Traumatic rupture of the aorta, aortic and great vessel involvement in Takayasu's disease, and mucopolysaccharidosis are reviewed along with imaging of congenital anomalies of the aortic arch. The diagnosis of vascular complications of renal transplants is summarized.
 ...
PMID:Advances in pediatric interventional radiology. 138 21

To determine whether the hemodynamic responses to adrenergic agonists are altered during chronic hypoxemia secondary to an intracardiac right to left shunt, we studied seven lambs with surgically created pulmonic stenosis and atrial septal defect and nine controls during infusions of isoproterenol at 0.1 and 0.5 micrograms/kg/min and dopamine at 5 and 20 micrograms/kg/min. Isoproterenol increased heart rate by 89 +/- 17% in control but only 46 +/- 6% in experimental lambs (p less than 0.05). However, because resting heart rate was higher in experimental lambs (213 +/- 7 versus 177 +/- 12 beats/min, p less than 0.05), maximal heart rates were similar (310 +/- 7 versus 326 +/- 6 beats/min; NS). Cardiac output increased during isoproterenol from 219 +/- 20 to 425 +/- 54 mL/min/kg in experimental lambs (p less than 0.05) and, similarly, from 180 +/- 20 to 425 +/- 71 in controls (p less than 0.05) (experimental versus control; NS). Dopamine also increased cardiac output similarly in both groups, at both doses, but without changing heart rate. Isoproterenol did not alter aortic oxygen saturation and increased systemic oxygen transport more than oxygen consumption. In contrast, dopamine at both doses decreased aortic oxygen saturation in experimental lambs (rest, 71 +/- 2% versus dopamine, 59 +/- 2%; p less than 0.05). With dopamine, the increase in systemic oxygen transport was equalled by an increase in oxygen consumption. Thus, circulatory responses to isoproterenol are similar in lambs with experimental cyanotic heart disease and controls, although higher resting heart rate in the experimental lambs reduces chronotropic reserve.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Comparative circulatory effects of isoproterenol and dopamine in lambs with experimental cyanotic heart disease. 185 23

The adult with congenital heart disease who undergoes cardiac catheterization at the present time is most likely to have complex heart disease and is left with clinically important sequelae or residual defects, ventricular dysfunction or arrhythmias. Residual defects such as paravalvular leaks, coronary fistulas and pulmonary artery narrowings may be corrected with transcatheter techniques. Patients with simple forms of congenital heart disease (for example, atrial septal defect, patent ductus arteriosus, aortic valve stenosis, pulmonary valve stenosis) will go to the catheterization laboratory for treatment, not diagnosis. Certain lesions previously considered benign (for example, patent foramen ovale) may require definitive interventional therapy to reduce the risk of stroke from paradoxic embolism.
 ...
PMID:The adult with congenital heart disease: cardiac catheterization as a therapeutic intervention. 185 95

A prohibitive perioperative mortality has been previously ascribed to pediatric heart transplantation after palliative operations for congenital heart disease involving the pulmonary arteries. Of 46 children who have undergone heart transplantation at our institution between June 1984 and February 1990, 7 (15%; mean age 8 +/- 3 years; range 1 to 18 years) have previously undergone such operations: right ventricle to pulmonary artery conduit/homograft for levo-transposition of the great arteries (2), Waterston shunt for tricuspid and pulmonary atresia (1), pulmonary artery banding for single ventricle (1), Fontan procedure for single ventricle (1), first-stage Norwood procedure for hypoplastic left heart syndrome (1), and classic right Blalock-Taussig shunt for atrioventricular canal with pulmonic stenosis (1). Three categories of pulmonary artery anatomy that require different approaches to reconstruction at the time of transplantation are recognized: abnormalities of position, pulmonary outflow obstruction, and previous systemic- or atrial-pulmonary connections. At operation, individualized pulmonary arterial reconstruction was employed, including use of previously created right ventricular-pulmonary artery conduits/homografts and angioplasty (with and without pericardial patches). Transplantation was successful in all patients. Posttransplant right ventricular-pulmonary artery pressure gradients and pulmonary vascular resistance indices were acceptable, with a tendency to decrease with time. Two patients had critical right ventricular failure postoperatively; one of them required support with extracorporeal membrane oxygenation. There was no perioperative mortality, with three deaths occurring from 5 to 39 months after transplantation. All surviving patients are in New York Heart Association functional class I. Techniques borrowed from the repair of congenital cardiac lesions can be applied to subgroups of children undergoing heart transplantation. Additional length of donor aorta and pulmonary artery should be harvested for possible use in designing pulmonary artery connections. Previous palliative operations involving the pulmonary arteries with associated complex pulmonary artery anatomy are not of themselves an insurmountable obstacle to successful heart transplantation.
 ...
PMID:Pediatric heart transplantation after operations involving the pulmonary arteries. 188 Nov 78


1 2 3 4 5 6 7 8 9 10 Next >>