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

57 cases of renal obstructive dysplasia (defined as the abnormal development of nephronic and ductal structures due to in utero obstruction of the urinary tract) were evaluated in terms of sonographic findings, renal and other associated anomalies, and current status of the child. More than one-third of the cases had bilateral disease and although not uniformly fatal bilateral involvement was associated with significant morbidity and mortality. In 12 of the 33 cases with unilateral dysplasia there was an association with contralateral renal problems including uretero-pelvic junction obstruction, vesicoureteral reflux and aplasia. Almost one-half of the cases had congenital anomalies, these included VACTERL association, congenital heart disease, cranial abnormalities and gastrointestinal malformations. Fifteen stillborns and 12 of the patients with bilateral involvement and four with unilateral involvement have died. Four patients are on dialysis (two with bilateral involvement and two with unilateral renal obstructive dysplasia). Only one-quarter are otherwise normal. More serious problems are reported in this mixed age population of patients with obstructive renal dysplasia than has been identified in previous studies. Management decisions of the fetus and child must be based on this new age-expanded population.
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PMID:Renal obstructive dysplasia: ultrasound diagnosis and therapeutic implications. 187 Sep 25

Early in the course of studies of the Spanish toxic oil syndrome it was recognized that vascular lesions were a major problem, most logically attributable to endothelial damage by the toxic oil. However, most clinical attention has been directed to the pulmonary complications and the evolution into a scleroderma-like illness later. In this study of 11 victims of the toxic oil syndrome careful postmortem studies of the coronary arteries and conduction system and neural structures of the heart demonstrated major injury to all those components of the heart. Obliterative fibrosis of the sinus node in four cases resembled findings in fatal scleroderma heart disease, and in eight the cardiac lesions resembled those of lupus erythematosus. The more impressive pathologic features involved the coronary arteries and neural structures, which were abnormal in every heart. The arterial disease included widespread focal fibromuscular dysplasia, but there was also an unusual myointimal proliferative degeneration of both small and large coronary arteries in five patients, four of whom were young women. In two hearts, portions of the inner wall of the sinus node artery had actually detached and embolized downstream. Coronary arteritis was rarely found. Inflammatory and noninflammatory degeneration of cardiac nerves was widespread. Fatty infiltration, fibrosis and degeneration were present in the coronary chemoreceptor. In most respects these cardiac abnormalities resemble those described in the eosinophilia-myalgia syndrome caused by an altered form of L-tryptophan. In both diseases there is good reason to anticipate more clinical cardiac difficulties than have so far been reported, and even more basis for future concern, especially relative to coronary disease and cardiac electrical instability.
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PMID:Cardiac abnormalities in the toxic oil syndrome, with comparative observations on the eosinophilia-myalgia syndrome. 191 15

In this paper, we report the cases of 4 male patients (mean age 32.7 yr) with right-ventricular dysplasia, that occurred in familial form. Diagnosis was possible according to the clinical features and to the electrocardiographic, echocardiographic and scintigraphic criteria; at Holter monitoring recorded ventricular arrhythmias with left bundle branch block configuration, associated to sinus arrhythmias (sino-auricular block). In two patients a sustained ventricular tachycardia was recorded. The patients are living and a fourteen-year-old subject is without heart disease. From our data we conclude that: the most serious clinical features occur in extensive form with right atrial and left-ventricle involvement; the clinical features were manifested at a young age and then got worse. A dominant autosomic inheritance is observed (only males were affected).
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PMID:[Arrhythmogenic right ventricular myocardiopathy. A study of a family group]. 195 45

The purpose of this study is to assess the right atrial and ventricular function by radionuclide ventriculography using continuous infusion of 81mKr in normal subjects and in patients with heart disease. 1) Reproducibility of RVEF measurement by continuous infusion of 81mKr was good (interobserver; gamma = 0.97, p less than 0.001, n = 20: intraobserver; gamma = 0.97, p less than 0.001, n = 20). This method had a excellent correlation with RVEF of 99mTc first-pass technique (gamma = 0.92, p less than 0.001, n = 20). 2) RVEF was measured in 10 normal volunteers, 76 patients with myocardial infarction (OMI), 20 patients with dilated cardiomyopathy (DCM), 5 patients with arrhythmogenic right ventricular dysplasia (ARVD) and 5 patients with primary pulmonary hypertension (PPH). In OMI and DCM, their RVEF was lower than that of normal volunteers and, in ARVD and PPH, lower than that of OMI and DCM. There was a significant inverse correlation between RVEF, mean pulmonary artery pressure and right ventricular end-diastolic volume index. 3) The effect of the location of right coronary artery (RCA) lesions on RVEF during exercise was also evaluated. Ten normal volunteers and 27 patients with OMI were studied at rest and during exercise. The patients with OMI were divided into two groups: those without proximal RCA lesions (non RCA group, n = 12) and those with proximal RCA lesions (RCA group, n = 15). Although there were no significant increases of RVEF during exercise in both group, the percent change in RVEF was less in RCA group than in non RCA group. These findings suggested that proximal RCA stenosis is a major determinant of exercise RVEF. 4) To assess the right atrial function, right atrial volume curve was measured in 10 normal volunteers, 32 patients with OMI and 4 patients with PPH. The curve was clearly divided into 4 phase; filling phase (312 +/- 40 msec), early ejection phase (276 +/- 53 msec), plateau an index of right atrial reservoir function, was 0.41 +/- 0.05 and Contractile Volume/Stroke Volume (Contr. V/SV), as an index of right atrial pump function, was 0.23 +/- 0.05 in normal volunteers. In OMI and PPH, atrial reservoir function decreased and atrial pump function increased. It was concluded that radionuclide ventriculography using continuous infusion of 81mKr was useful to assess the right heart function.
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PMID:[Assessment of right atrial and ventricular function by radionuclide ventriculography using continuous infusion of 81mKr]. 206 Sep 2

Right ventricular arrhythmogenic dysplasia (RVAD) is a typical asymptomatic arrhythmogenic cardiopathy in athletes, which is occasionally concurrent with normal ventricular function and life-threatening arrhythmias. A total of 32 athletes (28 males and 4 females, mean age, 23 years, mean follow-up, 6.7 years) were examined for severe cardiac arrhythmias with left bundle branch block. The conclusive diagnosis of RVAD was established from clinical, echocardio-, and angiographic evidence. The protocol of the examination involved Holter monitoring, loading tests, electrophysiological study, two-dimensional echocardiography, cardiac angiography of the right and left ventricles, coronary angiography. The most severe arrhythmias were observed in athletes whose mean age was 23.4 years, 20 patients had sustained ventricular tachycardia (it occurred only in 19 who were indulging in sports), 6 presented with transient ventricular tachycardia, and 1 had ventricular fibrillation. They all had been considered fit for sports. The disease proceeded severely in 16 of 32 athletes (in 13 of 16 while indulging in sports), the conditions close to syncope were seen in 9 patients (8 had sustained ventricular tachycardias and 1 had transient ventricular tachycardias), syncopes were observed in 5 patients (sustained ventricular tachycardias).
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PMID:[Stable ventricular tachycardia in arrhythmogenic dysplasia of the right ventricle in sportsmen]. 208 39

Between 1983 and 1989, 69 patients underwent endocardial fulguration for drug-resistant VT which had several relapses per month, or were incessant. The etiologies were an old myocardial infarction, (25 cases) arrhythmogenic right ventricular dysplasia, (19 cases) dilated cardiomyocathy, (9 cases) left fascicular tachycardia, (9 cases) 5 right septal idiopathic tachycardia, and 2 congenital heart disease. The ejection fraction ranged between 12 and 30% in 25 cases. VT originated in 32 patients in the right ventricle, 31 in the left, and 6 had fulguration in both ventricles. 41 patients had 1 session, 22 needed 2, 4 had three sessions and 2 had 4. Five patients died during the procedure, four of low output, and three of them before the shocks. One died of tamponade. Two others had tamponade and were successfully drained. Four patients died during the first 3 months of preexisting heart failure without VT relapse. The remaining patients have a mean follow-up of 32 months, ranging from 3 to 72 months. 32 patients are off drug and had no relapse. 23 had early relapses, and are controlled by previously ineffective drugs. However 3 of these had a late sudden death (4, 14 and 22 months). 9 are treated with a combination of drug not tested before. The success rate ranges from 60% in septal VT to 74% in ARVD, 86% in MI and 100% in idiopathic left fascicular VT. The success rate increases with the number of sessiones. Fulguration alone, or combined with pharmacological agents can be considered as an effective therapy for VT, despite that it is neither simple, nor harmless.
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PMID:[Endocardial fulguration for the treatment of ventricular tachycardia. Experience with 69 cases]. 209 54

Autopsy studies of arrhythmogenic right ventricular dysplasia (ARVD) have rarely been reported, and its etiology remains unknown. The present report describes a detailed histopathological study of two autopsied cases of ARVD. Case 1: This 21-year-old man experienced palpitation accompanied by syncope. He died after ventricular tachycardia of right ventricular origin. The heart weighed 365 g and the right ventricular cavity was markedly dilated. The distribution of fatty tissue was roughly limited to the middle layer of the free wall, replacing the myocardium with fatty degeneration. Medial hyperplasia of the small arteries within the fatty tissue was also observed. Fibroelastosis was observed in the left ventricular endocardium. In the conduction system, fatty tissue was found in the sinus node. In addition, medial hypoplasia was observed in the pulmonary arteries. Case 2: This 32-year-old man who had had an arrhythmia for 10 years died of ventricular tachycardia of right ventricular origin. His older brother also died of heart disease. His heart weighed 515 g and both the right and left ventricles were dilated. Fatty tissue, unlike that in Case 1, was shown to markedly infiltrate from the epicardium into both the right and left ventricular walls. In the right ventricular wall, muscle layers disappeared in some portions. In the conduction system, fatty tissue was observed in the sinus node. Although ARVD may be considered a syndromic entity, individual cases are different in terms of pathological morphology, with possible variations in the etiology and pathogenesis.
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PMID:[Two autopsied cases of arrhythmogenic right ventricular dysplasia]. 213 13

Moya-Moya disease has been associated to a number of disease entities including Down syndrome. Initial clinical manifestations in our patient were alternating lateralizing seizures with sudden onset hemiparesis. Ct scan demonstrated several infarcts in different stages of evolution, in the territory of left middle cerebral artery. Single digital subtraction angiography showed bilateral occlusion, predominantly on the left side of the supraclinoid portion of the interna carotid arteries with formation of collateral circulation in the diencephalic territory. The association of Moya-Moya disease and Down syndrome is not fortuitous and it is probably due to a congenital vascular dysplasia. It should be suspected in children with trisomy 21 (with or without congenital heart disease) who presents with alternating hemiplegia and convulsions or acute hemiplegia.
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PMID:[Acute hemiplegia in childhood and alternating hemiconvulsions secondary to Moya-Moya disease. Report of a case associated with Down's syndrome]. 228 71

The incidence of structural renal tract abnormalities in 109 children with documented congenital heart disease (CHD) was studied using real-time sonography. In these children, 11.9% had associated urologic anomalies, which included hydronephrosis, duplication, ectopia, agenesis, and dysplasia of kidneys. Children with associated extracardiac anomalies had a significantly higher incidence of renal tract anomalies (39.1%) compared to those with isolated CHD (4.7%). Therefore, sonographic screening of the renal tract should be performed routinely in patients with CHD, especially in those with multiple congenital defects.
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PMID:Sonographic screening for renal tract anomalies associated with congenital heart disease. 215 10

Dysplasias underlying the mitral and aortic valve diseases in the presence of valvular incompetence were identified in 110 patients with a history of valve replacement. The morphological and electron microscopy evidence together with combinations of valvular changes with Marfan's syndrome and Erdheim's disease, and in some cases with minimal changes in the aorta and peripheral vessels, which followed the type of idiopathic cystic medionecrosis without aneurysm formation support the idea of the dysplastic etiology of the detected abnormalities. The occurrence of a heart disease in the presence of dysplasia is determined by the extent of a valvular process and attending complications, such as chordal rupture or septic endocarditis.
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PMID:[Heart valve dysplasia as a cause of valvular insufficiency in cardiac surgery (clinico-morphological correlations)]. 227 75


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