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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The radiographic features of a cerebral malformation are presented. The major findings are absence of the corpus callosum, a large interhemispheric cyst, and ventricular dilatation. This cerebral dysplasia originates in the first trimester of fetal development resulting in gross psychomotor deficiencies and seizure disorders. Diagnosis which depends predominantly on encephalographic or ventriculographic findings enables formulation of a guarded prognosis.
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PMID:Agenesis of the corpus callosum with interhemispheric cyst. 107 95

Two strikingly similar patients with arrhythmogenic right ventricular dysplasia which severely impaired not only the right ventricle but also the left ventricle are described in association with familial occurrence of sudden death. A 49-year-old man experienced syncope which was due to ventricular tachycardia. Electrocardiography revealed a first degree atrioventricular block, incomplete right bundle-branch block, T wave inversions in leads II, III, a VF and V1 to V5, and multiformal ventricular extrasystoles. Echocardiography and ventricular cineangiography showed not only the right ventricular dilatation with an aneurysm in the right ventricular apex, inflow and outflow tracts, but also mild dilatation of the left ventricle with left ventricular apical and posterior aneurysms. Radionuclide angiography also disclosed dysfunction of both ventricles, especially during exercise. His family history revealed that 3 members of his family died of sudden deaths. A 56-year-old woman experienced syncope secondary to ventricular tachycardia, with left bundle-branch block. Electrocardiography showed complete right bundle-branch block, left axis deviation, and T wave inversions in leads V1 to V4. Echocardiography and ventricular cineangiography revealed not only marked right ventricular dilatation with the "triangle of dysplasia", but also a left ventricular aneurysm in the apex and posterior portion. Her elder brother died of a sudden death, and electrocardiograms of 2 members of her family showed ventricular extrasystoles and T wave inversions. These 2 cases may well be termed "familial bi-ventricular dysplasia".
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PMID:Two cases of bi-ventricular dysplasia associated with ventricular tachycardia and familial occurrence of sudden death. 172 78

The frequency of right ventricular dysplasia (RVD) in an autopsy series of young persons with sudden cardiac death in the United States has not been previously reported. We reviewed the autopsies from cases of sudden cardiac deaths in young adults in the state of Maryland and noted three cases of RVD among 547 cardiac deaths (0.55%). These three cases of RVD in young adults and three additional cases from our file are presented. Their ages ranged from 19 to 28 yr, and there were five males and one female. Five deaths occurred during strenuous exercise while the sixth was unwitnessed. Three of these cases had a documented history of arrhythmias and 1 had palpitations. In each case, autopsy revealed right ventricular dilatation with partial absence of the myocardium and extensive fatty infiltrates with and without fibrosis. In four cases, collections of chronic lymphocytic infiltrates were seen, of which two had associated myocyte necrosis. In one patient, the disease was familial, while in the remaining five it was sporadic, suggesting a nongenetic cause.
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PMID:Right ventricular dysplasia associated with sudden death in young adults. 178 62

Prenatal sonographic findings were reviewed in 30 fetuses with a single umbilical artery (SUA) to determine the reliability of ultrasound for detecting concurrent anomalies. Additional anomalies were identified in 15 fetuses, including 3 fetuses with minor anomalies and 12 fetuses with major or multiple concurrent anomalies. Minor anomalies observed in 3 fetuses included 1 case each of pelvic kidney, unilateral absent kidney, and mild cerebral ventricular dilatation. Major abnormalities detected in 12 fetuses involved a variety of organ systems and included cardiac defects, holoprosencephaly, skeletal dysplasia, hydrocephalus, omphalocele, hydrothorax, enlarged cisterna magna, and diaphragmatic hernia. Clinical and pathologic correlation showed that all fetuses were correctly categorized regarding the presence of other anomalies; none of the 15 fetuses in whom an SUA was considered an isolated finding had a significant anomaly at birth. Chromosome abnormalities were found in 6 of 12 fetuses with major abnormalities but in none of the 18 remaining fetuses. We conclude that prenatal ultrasound can reliably identify major concurrent anomalies in fetuses with SUA. In the absence of additional anomalies, prenatal detection of SUA should not alter obstetric management.
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PMID:Single umbilical artery. Prenatal detection of concurrent anomalies. 205 44

The aim of this study of 20 young subjects (28 +/- 10.6 years) with no apparent cardiac disease on clinical examination and chest X-ray was to determine the origin of complex ventricular arrhythmias: monomorphic or polymorphic ventricular extrasystoles, isolated or in valves (average 18 158 +/- 12 388 per 24 hours) and/or ventricular tachycardia (5 cases, sustained in 3). These arrhythmias were aggravated (N = 6), disappeared (N = 8) or remained unchanged (N = 5) during exercise. The inter-critical ECG showed ST changes in 5 cases. The extrasystoles had a left bundle branch block configuration in 14 cases and a right bundle branch block configuration in 9 cases. Nine patients were Grade 2 (45%) and 11 patients Grade 4B of Lown's classification. Complementary investigations (echocardiography), radionuclide investigations, right and left heart catheterisation, selective right and left ventriculography and coronary angiography) showed a high incidence of arrhythmogenic right ventricular dysplasia (N - 14) associated with left ventricular abnormalities in 13 cases: hypofixation of Thallium (N = 14) associated with left ventricular abnormalities in 13 cases: hypofixation of Thallium (N = 11), abnormal global left ventricular function (N = 13) with decreased ejection fractions in half the cases, left ventricular dilatation in a third of cases (average and diastolic volume: 109.8 ml/m2), mean velocity of circumferential fibre shortening decreased in 86% of cases (average 0.88 cir/sec), angiographic abnormalities of segmental left ventricular wall motion in 36% of cases; 2 clinically silent cases of mitral valve prolapse were associated with these left ventricular changes; these cases represent forms of arrhythmogenic cardiac disease localised to the right ventricle or involving both ventricles which should be searched for routinely in young patients with apparently normal hearts but with idiopathic and severe ventricular arrhythmias. The diagnosis can only be established by angiography. In other cases, isolated left ventricular abnormalities are detected: two cases of hypertrophic non obstructive cardiomyopathy including one apical form, a condition which may be suspected from analysis of the surface ECG and careful 2D echocardiographic study; phonomechanography may be normal; one idiopathic left ventricular aneurysm which was only diagnosed at ventriculography; one dilated cardiomyopathy affecting the left ventricle. In our series, none of the patients had coronary artery disease and two patients even had no abnormality of any of these investigations.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Complex ventricular arrhythmia in apparently healthy young subjects]. 241 70

Arrhythmogenic right ventricular dysplasia is characterized by fibrous and adipose replacement of the right ventricular myocardium and recurrent ventricular arrhythmias of left bundle branch block morphologic pattern. Sometimes the diagnosis is difficult because not all the clinical and instrumental findings are present and the separation between arrhythmogenic right ventricular dysplasia and other right ventricular cardiopathies is uncertain. In such cases the angiographic appearance of the right ventricle has been considered the "gold standard". To assess the diagnostic value of right ventricular morphology in identifying arrhythmogenic right ventricular dysplasia, we compared the angiographic findings of 8 patients with arrhythmogenic right ventricular dysplasia, 10 with biventricular dilated cardiomyopathy and 10 with Ebstein's anomaly. The following aspects were considered: deep fissuring of the anterior or inferior wall, outflow tract enlargement, contrast persistence in the right ventricle during the levophase, regional wall motion abnormalities including aneurysmal formations and tricuspid regurgitation. Aneurysmal formations of the right ventricle were found only in arrhythmogenic right ventricular dysplasia whereas the other angiographic findings were common to all the above mentioned diseases. Right ventricular angiography is an important adjunct to the clinical and instrumental diagnosis of arrhythmogenic right ventricular dysplasia, but most of its angiographic features are common to other diseases which cause right ventricular dilatation.
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PMID:[Angiographic findings in arrhythmogenic dysplasia of the right ventricle]. 280 89

Four cases of right ventricular dysplasia (RVD) and 28 cases of dilated cardiomyopathy (DCM) were studied. RVD was characterized clinically by syncope, sustained recurrent ventricular tachycardia with left bundle branch block patterns on the surface electrocardiogram, and right heart failure. Furthermore, moderate to severe dilatation of the right ventricle and depressed right ventricular function were apparent on radionuclide angiography. However, left ventricular dilatation and depressed left ventricular function were documented in DCM. Right ventricular volume was proportional to left ventricular volume in DCM, however, right ventricular volume was disproportionately greater in RVD. On the T1-201 perfusion image, left ventricular perfusion defects were delineated in 10 of 26 patients with DCM, and in one of four RVD patients. During two to eight year follow-up periods, six patients died suddenly five of whom had left ventricular perfusion defects. However, in 19 patients without left ventricular perfusion defects, only one sudden death was observed. A connecting link between sudden death and left ventricular perfusion defect is suggested.
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PMID:[Right ventricular dysplasia and dilated cardiomyopathy observed by radionuclide images]. 384 88

A forty-year-old man was admitted to our hospital because of dizziness, palpitations and an oppressive feeling in the precordium. Physical examination was normal. A chest roentgenogram revealed mild cardiomegaly and the electrocardiogram showed ventricular tachycardia of a left bundle branch block configuration which was terminated by the intravenous injection of procainamide. During sinus rhythm the electrocardiogram showed incomplete right bundle branch block, PQ prolongation and inverted T waves in leads V1 through V3. Two-dimensional echocardiography revealed only moderate right ventricular dilatation. Right ventricular angiography showed severe right ventricular dilatation and hypokinesis of the right ventricular apex and pulmonary artery infundibulum. From these characteristics signs we concluded that this adult patient had arrhythmogenic right ventricular dysplasia (ARVD), suggesting that this condition is not confined to children but may occur in adults as well.
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PMID:An adult case of arrhythmogenic right ventricular dysplasia. 400 56

Two cases of arrhythmogenic right ventricular dysplasia are described. The most important clinical features of the disease are malignant ventricular arrhythmia and sudden death. It is characterized by the loss of right ventricular musculature and by the fatty and connective tissue infiltration of the right ventricular wall. The diagnosis is based on the typical echocardiographic appearance of right ventricular dilatation, on the presence of negative T waves in leads V1-4 on the resting ECG and on ventricular tachycardia of left bundle branch block pattern. Right heart failure develops only in the late phase of the disease. Genetic defect might be an etiologic factor. In conclusion authors suggest that in case of left bundle branch block ventricular tachycardia or Adams-Stokes syndrome in young adults echocardiography and family screening are necessary.
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PMID:[Arrhythmogenic right ventricular dysplasia. Case report and review of the literature]. 826 30

A modified open patch-graft technique was used to correct congenital pulmonic stenosis in 8 dogs. Pulmonary valve dysplasia was moderate to severe in all cases, based upon clinical and echocardiographic criteria, and 3 dogs were in right-sided congestive heart failure at the time of surgery. Seven of the 8 dogs survived the surgery. One surviving dog displayed cerebral cortical dysfunction, the remaining 6 had no detectable neurological sequelae. Right ventricular failure was alleviated in all 7 surviving dogs, but right ventricular dilatation persisted post-operatively. Pulmonary valvulectomy and open patch-grafting provides an effective means of alleviating signs of congestive heart failure caused by pulmonary valve dysplasia, even in the presence of severe infundibular hypertrophy and dynamic outflow obstruction.
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PMID:Use of a modified open patch-graft technique and valvulectomy for correction of severe pulmonic stenosis in dogs: eight consecutive cases. 836 65


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