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

Sixty-nine children with Down syndrome (mongolism, trisomy 21), with atrial septal defect, patent ductus arteriosus, ventricular septal defect, or endocardial cushion defects, and 315 children with similar cardiac anomalies without this syndrome underwent cardiac catheterization during an 8-year period from 1964 to 1973. Only patients under 17 years of age were included in the study. Nine tenths of the children with Down syndrome but only one fourth of the control group had abnormally high pulmonary arterial pressures. For example, 9 of 11 children with defects of the atrial septum and Down syndrome had pulmonary hypertension; in contrast, only 5 of 55 control subjects with similar defects had pulmonary hypertension. The data suggest that children with congenital heart disease and Down syndrome have an unusually high pulmonary vascular resistance and a propensity for early development of severe damage to the pulmonary vascular bed.
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PMID:The pulmonary vascular bed in children with Down syndrome. 12 55

The authors have followed up 26 children suffering from severe scoliosis associated with congenital heart disease up to the end of puberty. The curve was usually a very severe idiopathic scoliosis developing early and requring surgical treatment. Two types may be distinguished: I. Scoliosis without excessive surgical risk, in patients in whom the heart disease is not associated with cyanosis or where the heart condition has already been treated surgically and in which there are no clinical, radiological or electrical signs of cardiac failure. 2. Scoliosis with considerable surgical risk because of heart disease with cyanosis not treated surgically, or with signs of heart failure or pulmonary hypertension. In such cases, the surgical treatment of the scoliosis is likely to endanger life.
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PMID:[Scoliosis and congenital cardiopathies]. 13 60

The natural history of congenital heart disease in older patients will become increasingly difficult to delineate, since many of uncomplicated defects were repaired early in life. Therefore the frequency of clinical and hemodynamic progress in non-operated patients with atrial septal defects seems to be of great interest. We are able to compare 27 patients aged more than 60 years with a group between 40 and 59 years. In 80 out of 103 patients totally, the clinical findings could be completed with catheterization data. The progress of deterioration with age in spite of intensive medical care could be demonstrated by mostly statistical significant signs of greater right-ventricular overload in the Ecg, cardiac arrhythmias--usually atrial fibrillation--changes in the X-ray findings and increasing pulmonary hypertension.
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PMID:[Atrial septal defect in the aged (author's transl)]. 15 Jul 10

Clinical studies have suggested that patients with Down syndrome have precocious development of pulmonary hypertension, even in the absence of congenital heart disease. To examine the pathologic basis of this impression, we studied 82 patients with Down syndrome autopsied at The Johns Hopkins Hospital from 1913 to present. The patients ranged in age from 26 hours to 25 years; 41 (50%) were female. Atrioventricular canal defect was present in 40 patients, nine of whom also had pulmonary stenosis. Eight had isolated ventricular septal defect and seven had other malformations. Histologic sections of the lung from each patient were studied and the degree of hypertensive pulmonary vascular disease graded. Age and sex-matched controls without cardiovascular manifestations, all other autopsied patients with atrioventricular canal defect not associated with Down syndrome, and age-matched patients with ventricular septal defect or other malformations were similarly studied for pulmonary vascular changes. Comparison of the 27 patients with Down syndrome and no cardiovascular malformations with normal controls showed no differences, with one notable exception: an 8-month-old child with Down syndrome had severe HPVD secondary to idiopathic pulmonary hypertension. HPVD in patients with the various cardiovascular malformations was similar for those groups with Down syndrome and those without, when HPVD was considered as a function of the patients' age and the type of malformation. We conclude that, in general, patients with Down syndrome have no predisposition to develop severe or precocious hypertensive pulmonary vascular disease.
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PMID:Hypertensive pulmonary vascular disease in Down syndrome. 15 39

The results of functional pharmacological tests with nitroglycerine and carbon dioxide gas are presented for patients with congenital heart diseases and pulmonary hypertension, as shown during right heart catheterization. It was established that nitroglycerine, taken sublingually, does not produce any direct effect on the pulmonary artery pressure, and the delayed changes in haemodynamics are a consequence of the altered regimen of pressure in the general circulation. When carbon dioxide gas was administered into the pulmonary artery, it proved to produce a direct effect upon the receptors of the pulmonary arterial bed. However, along with the hypotensive effect, in some cases the carbon dioxide gas increases the pressure in the pulmonary artery due to the embologenic effect of the gas bubbles. Both agents affect the arterial pressure irrespective of the patient's age, the nature of the heart disease, and the degree of pulmonary hypertension.
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PMID:[Study of the pulmonary vessel reaction to administration of carbon dioxide and nitroglycerin in congenital heart defects complicated by high pulmonary hypertension]. 40 59

The informative value of some indices of myocardial contractility, calculated from curves of pressure in the right ventricle and pulmonary artery, was determined in 119 patients suffering from mitral heart disease with various grades of pulmonary hypertension in 25 patients who did not have a heart disease. The dependence of these indices on the grade of pulmonary hypertension and severity of the clinical symptoms of right-ventricular insufficiency was also studied. The Siegel-Sonnenblick contractility index proved to be the most informative index of myocardial contractile function. It was reduced in patients with right-ventricular insufficiency. With an increase in pulmonary hypertension, the average rate of increase in intraventricular pressure in the phase of isovolumic contraction grows sharply, which indicates to considerable hyperfunction of the right ventricle.
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PMID:[Myocardial contractile capacity of the right ventricle in lesser circulation hypertension and in right ventricular insufficiency]. 42 35

Lung biopsy specimens were taken from 39 infants younger than 12 months of age with congenital heart disease and severe pulmonary hypertension (Pp/Ps greater than or equal to 0.75) accompanied by respiratory distress. The pathological change in lung specimens and clinical courses were compared. These 39 infants underwent surgical treatment of patent ductus arteriosus (PDA), seven patients; ventricular septal defect (VSD), 13 patients; and complex heart anomaly, 19 patients. The common pathological findings of the lung specimens taken from these infants were lymphoid cellular infiltration and thickening of the alveolar septum, which we have called "septitis" in the present study. In most cases pulmonary vascular obstructive change was within Grade 2 of the Health-Edwards criteria. Septitis was classified into three categories: mild, moderate, and severe. Only three of the 19 infants with severe septitis survived postoperatively, whereas 10 of the 12 infants with moderate septitis and all eight with mild septitis could be successfully weaned. The cause of septitis remains unidentified. We have found the patient's age and pulmonary hypertension to be closely related to the grade of septitis in this study. Septitis plays a much more important role than pulmonary vascular obstructive change in the prognosis of pulmonary hypertensive heart disease in early infancy.
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PMID:Lung pathology in infants with severe pulmonary hypertension and cardiac disease. 43 Nov 7

The authors placed Swan-Ganz catheters in 11 preterm and 2 term infants with severe cardiopulmonary distress. The infants ranged in weight from 1100-4000 g. The procedure was performed in the neonatal ICU by jugular venous cutdown. Intracardiac pressures and oxygen saturations were measured in each chamber entered. The authors also evaluated the presence of right to left shunting through the patent ductus arteriosus and assessed the degree of pulmonary arterial hypertension. In 5 hypoxemic infants, the authors found a patent ductus arteriosus with pulmonary hypertension. Intermittent measurement of PAP served as a guide to further therapy. In 2 infants, the unexpected diagnosis of cyanotic congenital heart disease was made. The placement of flow directed pulmonary arterial catheters in critically ill infants can be performed safely at the bedside and can provide useful diagnostic and therapeutic information.
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PMID:Swan-Ganz catheterization in the critically ill newborn. 45 10

The authors had under their observation 389 patients over 16 years of age with congenital heart diseases, 157 of whom were operated on. The specific features of the diagnosis, course, and operative treatment of the most commonly encountered congenital heart disease among adults were studied. Peculiarities were revealed in the clinical symptomatology in adults with congenital heart diseases complicated by pulmonary hypertension. The authors discuss the problems of rehabilitation of adults who had been operated on, in the light of the study of the late postoperative results.
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PMID:[Congenital heart defects in persons over 16]. 51 57

Disturbances of heart rhythm, observed during 700 heart catheterizations in infants and children, are discussed. Paroxysmal supraventricular tachycardia has been observed in 25 investigations (3,6%), sinus bradycardia in 18 (2,6%), junctional rhythm in 10 (1,4%), second degree AV-block in 9 (1,3%), ventricular fibrillation in 8 (1,1%), sinus tachycardia in 7 (1%), complete block in 7 (1%), asystole and atrial flutter in 2 (0,3%) each, and ventricular tachycardia in 1 (0,15%). Supraventricular tachycardia occurred equally in all ages without preference of a special malformation. The two patients with WPW-syndrome, however, showed this disorder in each of three catheterizations. Propranolol and verapamil succeeded in terminating the attacks. Junctional rhythm and sinus tachycardia presented equal behavior and benignity. Sinus bradycardia, second and third degree AV-block, and especially ventricular fibrillation occurred mostly in neonates and infants, many of them cyanotic and suffering from complex malformations and therefore needing multiple catheter manipulations. Bradycardia was in two, asystole in one of the very sick neonates associated with subsequent death within 24 hours. Once asystole resulted in immediate death after pulmonary angiography in a child with severe pulmonary hypertension. Ventricular fibrillation could be terminated promptly by DC countershock in all patients, but three of the children died subsequently. Complete block occurred only in children with systemic right ventricular pressure, 4 of the 7 patients having pulmonary hypertension, too. In two instances the block subsided spontaneously, the rest could successfully be treated with orciprenaline (Alupent R). Life threatening arrhythmias became less frequent as a consequence of earlier investigation, if severe heart disease was suspected, and by closer control of cyanosis, acidosis and temperature before, during, and after catheterization.
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PMID:[The risks involved in the heart catheter examination. A retrospective evaluation of the complications after 700 examination. III. Irregularities of heart (author's transl)]. 53 Jul 27


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