Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chest roentgenograms in 13 infants and angiocardiograms in 11 infants with persistent transitional circulation (PCT) syndrome were reviewed. Chest radiographs typically revealed congestive heart failure with pulmonary venous congestion, cardiomegaly, hepatomegaly, and pleural effusions. Angiocardiography demonstrated a right-to-left shunt through the ductus arteriosus, ventricular dilatation, poor myocardial function, and ventricular emptying. Cardiopulmonary abnormalities resolved over a period of 2-6 days in survivors. PTC syndrome as seen at the authors' institution has been an important and relatively common cause of congestive heart failure and cyanosis in the newborn. The incidence of congestive failure was higher than in other reported series.
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PMID:Persistent transitional circulation. Roentgenographic findings in thirteen infants. 13 73

Persistence of the fetal circulation is a recently recognized cause of severe cyanosis in newborn full term infants. Abnormally elevated pulmonary vascular resistance apparently stimulated by hypoxia, acidosis, and/or hyperviscosity results in cyanosis due to large right-to-left shunts through persistent fetal channels (patent foramen ovale and patent ductus arteriosus). Initial chest radiographs demonstrate clear lungs, decreased, normal, or mildly prominent pulmonary vascularity, and normal to moderately enlarged cardiac silhouettes. Angiocardiography, when required to rule out cyanotic congenital heart disease, demonstrates normal intracardiac anatomy, normal great vessel relationships, and right-to-left shunting across the patent foramen ovale and patent ductus arteriosus. Significant tricuspid regurgitation occurs in some of these infants, associated with variable right ventricular dilatation; the left ventricle is normal. The majority of babies with this condition ultimately survive. Treatment consists of intensive care including oxygen therapy and correction of acidosis. Vasodilators such as tolazoline may be helpful.
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PMID:Persistence of the fetal circulation: radiologic considerations. 40 2

A 76-year-old man was referred to our hospital with complaints of productive cough, dyspnea and peripheral cyanosis. The chest X-ray film indicated the pulmonary emphysema and acute bronchitis, but no abnormal intracardiac calcification. The electrocardiogram revealed a peaked P-wave, complete left bundle branch block, and ventricular premature contraction. Chest tomography demonstrated abnormal intracardiac calcium deposition in the right heart region. Two-dimensional echocardiography revealed the tricuspid annular calcification in the postero-lateral portion, showing a synchronous movement with tricuspid annular motion throughout the cardiac cycle. The size of calcification was 10 x 14 mm. The tricuspid valve showed no significant regurgitation. Left ventricular dilatation, associated with mild mitral regurgitation and impairment of systolic function (EF = 49%) was revealed by echocardiography. Serum examination revealed positive in Wassermann reaction. This case of tricuspid annular calcification might be caused by atherosclerotic degenerative change related to the aging process, or by an unknown mechanism related to pulmonary emphysema.
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PMID:[A case of tricuspid annular calcification]. 179 47

To investigate the pathophysiology of chronic cyanosis, we subjected 14 adult mongrel dogs to diversion of the inferior vena cava to the right inferior pulmonary vein. This produced a mean oxygen tension of 42 +/- 2 mm Hg and a calculated right-to-left shunt of 52.0% +/- 3.9%. These animals (Group C) and 15 normal dogs (Group N) were subjected to cardiopulmonary bypass with 20 minutes of normothermic global ischemia. Functional indices studied were rate of rise of left ventricular pressure and the end-systolic pressure/volume ratio. Metabolic status was assessed by obtaining transmural myocardial biopsy specimens for measurement of adenosine triphosphate content. Myocardial blood flow was measured with radiolabeled microspheres. There were no significant differences between Group C and Group N in either functional index or blood flow measurement prior to global ischemia. At 45 minutes after ischemia, Group N animals had a significantly greater rate of rise of left ventricular pressure (at a left ventricular end-diastolic pressure of 0, 5, 10, and 15 mm Hg, p less than 0.025 to 0.05) and subendocarial perfusion (endocardial/epicardial flow ratio 0.961 +/- 0.037 versus 0.815 +/- 0.021, p less than 0.01). At 90 minutes after ischemia, Group N animals exhibited a significantly higher end-systolic pressure/volume ratio (4.9 +/- 0.7 versus 3.0 +/- 0.4 mm Hg/ml, p less than 0.05), rate of rise of left ventricular pressure (at an end-diastolic pressure of 0 to 20 mm Hg, p less than 0.005 to 0.05), and endocardial/epicardial flow ratio (1.065 +/- 0.046 versus 0.829 +/- 0.059, p less than 0.01). No differences in adenosine triphosphate content were found at any sampling period. The Group C left ventricles exhibited no hypertrophy but were significantly dilated compared to Group N (38.8 +/- 0.3 versus 30.1 +/- 0.2 mm, p less than 0.05). Inferior vena cava to pulmonary vein diversion produces cyanosis with left ventricular dilatation but without hypertrophy. It is proposed that abnormal loading characteristics of the left ventricle are responsible for the functional derangements that result from global ischemia.
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PMID:Pathophysiology of chronic cyanosis in a canine model. Functional and metabolic response to global ischemia. 402 30

Three newborn infants who had severe congestive heart failure caused by an arteriovenous malformation of the vein of Galen were evaluated by two-dimensional ultrasonography. Examination of the heart from standard echocardiographic planes showed right atrial and right ventricular dilatation. The superior vena cava, ascending aorta and the vessels to the head and neck were enlarged. Contrast two-dimensional echocardiography from a peripheral vein showed right-to-left atrial shunting and early superior vena cava recirculation. With the transducer positioned in the anterior fontanel or against the temporal bone, two-dimensional sector scans of the brain were obtained in the coronal, sagittal and transverse planes. A large echo-free space that represented an aneurysm of the vein of Galen was seen within the brain. During contrast injection into a peripheral vein, microcavitations that passed from right to left at the atrial level were seen filling the vein of Galen aneurysm. In two infants in whom beryllium wire was placed in the fistula to induce thrombosis, postoperative ultrasonography showed a dense mass of echoes arising from the wire and persistent left-to-right shunting through the fistula. In infants presenting in the first week of life with congestive heart failure and cyanosis of unknown etiology, two-dimensional ultrasonography provides a rapid, safe method for detecting intracranial arteriovenous malformation.
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PMID:Detection of intracranial arteriovenous fistula by two-dimensional ultrasonography. 645 32

Left ventricular function was studied with quantitative biplane cineangiocardiography in 39 preoperative and 23 postoperative patients with cyanotic congenital heart disease. Diagnoses included pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (group 1), tricuspid atresia (group 2) and pulmonary atresia with ventricular septal defect (group 3). Preoperative patients ranged in age from 1 day to 7 years and postoperative patients from 7 weeks to 23 years. Left ventricular end-diastolic volume was increased in preoperative patients in groups 1 and 2 (132 and 136 percent of normal, respectively) but was normal in patients in group 3. Left ventricular ejection fraction was decreased to a similar extent in preoperative groups 1 to 3: 0.54, 0.55 and 0.56, respectively. After a shunt procedure left ventricular end-diastolic volume increased to 228 and 266 percent of normal in groups 1 and 2, respectively, but remained within normal limits in group 3. Left ventricular ejection fraction was normal in postoperative group 1 patients, whose ages averaged 1.8 years, but remained decreased in group 2 and 3 patients, whose ages averaged 8.1 and 5.6 years, respectively. Duration of cyanosis and degree of left ventricular dilatation appear to be important variables in regard to pump function in patients with cyanotic congenital heart disease.
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PMID:Left ventricular function in cyanotic congenital heart disease. 737 22

Cardiac complications contribute significantly to morbidity and mortality in HIV-infected children. There have been few reports of cardiac manifestations in HIV-infected children in developing countries. The aims of this study were to evaluate the clinical manifestations and echocardiographic findings in Thai children with HIV infection and determine the clinical predictors of left ventricular dysfunction and pulmonary hypertension. We retrospectively reviewed the medical records of 27 infants infected with HIV perinatally who presented with cardiovascular problems at a tertiary care hospital between 1995 and 2000. The mean age at initial cardiac evaluation was 36 months (range 8-65). Signs and symptoms included dyspnoea in all cases, oedema in 12 (44%), finger clubbing in 11 (41%), cyanosis in 6 (22%) and S(3) gallop in 8 (30%). Echocardiographic abnormalities included pericardial effusion in 12 (44 %), right ventricular dilatation in 12 (44%), pulmonary hypertension in 11 (41%), diminished left ventricular fractional shortening in 10 (37%), left ventricular dilatation in 9 (33%) and combined ventricular dilatation in 2 (7%). Left ventricular dysfunction did not correlate with HIV CDC classification, age, nutritional status or clinical signs and symptoms.
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PMID:Cardiac manifestations in HIV-infected Thai children. 1518 44

A patient with a severe neonatal variant of 3-methylcrotonyl-CoA carboxylase (MCC) deficiency is reported. The first child of healthy consanguineous Turkish parents presented on the second day of life with dehydration, cyanosis, no sucking, generalized muscular hypotonia, encephalopathy, respiratory depression requiring mechanic ventilation, macrocephaly, severe acidosis and hypoglycaemia. Elevated C5-OH-carnitine in dried blood spot by tandem MS and elevated urinary excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine suggested MCC deficiency, confirmed by enzyme analysis in cultured fibroblasts. Cerebral ultrasonography and cranial CT findings revealed progressive changes such as disseminated encephalomalacia, cystic changes, ventricular dilatation and cerebral atrophy. Treatment with high-dose biotin and protein-restricted diet was ineffective and the patient died at the age of 33 days with progressive neurological deterioration. Mutation analysis revealed a homozygous mutation in the splice acceptor site of intron 15 in the MCC beta-subunit. Early-onset severe necrotizing encephalopathy should be included in the differential diagnosis of isolated MCC deficiency.
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PMID:Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome. 1587 10

A nine-year-old male Maltese dog was presented with an eight-month history of inspiratory stridor leading to exertional dyspnoea and cyanosis. Myokymic contractions in the palatolingual muscles were noticed and confirmed by electromyography. Brain computer tomography-scan showed ventricular dilatation. Cerebrospinal fluid analysis revealed a slightly elevated protein level. Treatment with slow-release phenytoin was unsuccessful and symptoms gradually worsened over the next nine months. At post-mortem examination a small pituitary adenoma was found. Apart from a single canine report of facial myokymia, this is the only other description of spontaneous focal myokymia in animals. Palatolingual myokymia has only been reported in one human being. Although the co-occurrence with a pituitary adenoma might be incidental, a paraneoplastic pathogenetic mechanism is proposed. Its unique clinical presentation adds a new, albeit uncommon, syndrome to the differential diagnosis of upper airway complaints in dogs.
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PMID:Inspiratory stridor secondary to palatolingual myokymia in a Maltese dog. 2007 Apr 91

Pediatric cardiomyopathies are a heterogenous group of conditions of which dilated cardiomyopathies are the most common clinicomorphologic subtype. However, the etiology and pathogenesis of many cases of dilated cardiomyopathies remain unknown. We describe a series of 5 cases of a rare but clinically and histologically distinctive dilated cardiomyopathy that was uniformly lethal in early infancy. The 5 cases include 2 pairs of siblings. There was parental consanguinity in 1 of the 2 pairs of siblings. Death occurred in early infancy (range, 22-67 days; mean, 42 days) after a short history of general lethargy, decreased feeding, respiratory distress, or cyanosis. There was no specific birth or early neonatal problems. Autopsy revealed congestive cardiac failure and enlarged, dilated hearts with ventricular dilatation more pronounced than atrial dilatation, and endocardial fibroelastosis. Histology showed prominent hypertrophic nuclear changes of cardiac myofibers and markedly increased myocyte mitotic activity including occasional atypical mitoses. Immunohistochemical staining for Mib1 showed a markedly increased proliferative index of 10% to 20%. Ancillary investigations, including molecular studies, did not reveal a primary cause for the cardiomyopathies. This distinctive dilated cardiomyopathy characterized by unusual histologic features of myocyte nuclear hypertrophy and marked mitotic activity is lethal in early infancy. Its occurrence in 2 pairs of siblings suggests familial inheritance. Although the underlying molecular pathogenesis remains to be elucidated, it is important to recognize this distinctive entity for purposes of genetic counseling.
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PMID:Mitogenic cardiomyopathy: a lethal neonatal familial dilated cardiomyopathy characterized by myocyte hyperplasia and proliferation. 2030 41


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