Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UNIPROT:P50583 (asymmetrical)
12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During 1970-87 43 patients with unexplained pulmonary hypertension (mean pulmonary arterial pressure greater than 25 mm Hg) were admitted to the Brompton Hospital and classified by angiographic criteria as having either symmetrical peripheral pulmonary artery pruning (thought to represent primary plexogenic pulmonary arteriopathy), n = 21, or asymmetrical pulmonary arterial occlusions (thought to represent chronic thromboembolic disease), n = 22. Patients with symmetrical pulmonary arteriopathy had significantly higher mean pulmonary arterial pressures (67 mm Hg) at the time of presentation than those with asymmetrical pulmonary arteriopathy (49 mm Hg). Clinical distinction between these two groups was impossible. Survival from the time of diagnosis was similarly poor in the two groups (26 weeks and 38 weeks) and did not correlate with any of the haemodynamic measurements. The difficulties in making distinctions between these conditions are discussed.
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PMID:Clinical correlates of angiographically diagnosed idiopathic pulmonary hypertension. 228 38

Idiopathic restrictive cardiomyopathy is a rare myocardial disease characterized by restrictive physiology without a specific histologic basis. To assess its clinical, hemodynamic, morphologic and prognostic details we retrospectively evaluated all the patients hospitalized in our Institute from 1974 to 1988. Nine patients, aged 42 +/- 16 years, M/F ratio = 0.29, who represent 64% of all the restrictive myocardial diseases biopsied were identified. Severe cardiac heart failure (3-4 NYHA) and arrhythmias (ventricular and supraventricular) were extremely common. The electrocardiogram showed several non specific signs: low voltage of QRS in peripheral leads (4/7), pseudo-infarctional aspects (3/7), mono or biventricular hypertrophy (3/7) disturbance of ventricular conduction (3/7), aspecific abnormalities of ventricular repolarization (3/7). All patients showed a prolonged QTc. M-mode and 2-dimensional echocardiography demonstrated in 6 cases biatrial enlargement, normal or slightly enlarged ventricles, normal or moderately depressed fractional shortening; biventricular concentric hypertrophy was detected in 3 cases, asymmetrical septal hypertrophy in 1. Five patients showed pericardial effusion. Cardiac catheterization disclosed an increase of left and right ventricular end-diastolic pressures (8/8) with a dip-plateau pattern and/or characteristic W waveform in the atrial pressure tracing (9/9). Passive pulmonary hypertension was detected in 6/9 cases. The cardiac index was decreased in 4/8 cases. Left ventricular angiography showed mitral regurgitation in 5/8 patients, tricuspidal in 5/8. Ejection fraction was decreased in 3/8 cases. Endomyocardial biopsy showed interstitial fibrosis (8/9), cellular hypertrophy and/or nuclear alterations (7/9), slight endocardial thickening (2/9). At a mean follow-up of 22 +/- 15 months 3 patients died and 2 underwent heart transplantation. In conclusion idiopathic restrictive cardiomyopathy is one of the most frequent forms of restrictive myocardial diseases in our geographic area. Severe congestive heart failure and arrhythmias are extremely common. The disease can be suspected by clinical, electrocardiographic and echocardiographic features, but the final diagnosis requires cardiac catheterization and endomyocardial biopsy. Prognosis is severe and heart transplantation must be considered in the cases with severe heart failure.
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PMID:[Idiopathic restrictive cardiomyopathy: clinical, hemodynamic, histologic and prognostic profile]. 260 84

The authors report the clinical, ECG, PCG and echocardiography data obtained in mountaineers suffering from associated essential hypertension and high-altitude pulmonary hypertension. Demonstrate the advisability of distinguishing the high-altitude hypertrophic cardiomyopathy syndrome (HHCS) in part of mountaineers with essential hypertension living permanently at an altitude of 3600-4200 m over the sea level. The HHCS is marked by a lot of the clinical and echocardiographic signs which are regarded as characteristic of hypertrophic cardiomyopathy, particularly by appreciable asymmetrical hypertrophy of the interventricular septum. Criteria for the differential diagnosis between the HHCS and idiopathic hypertrophic subaortic stenosis are suggested. The possible mechanisms by which the HHCS develops in part of mountaineers are discussed.
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PMID:[Syndrome of high-altitude hypertrophic cardiomyopathy]. 404 Feb 72

Intrauterine growth restriction (IUGR) is characterized by fetal growth less than normal for the population and growth potential of a given infant. IUGR can be symmetrical with low weight, length and head circumference indicative usually of a process with its origin early in pregnancy or asymmetrical with sparing of head circumference and length due to processes occurring later in gestation. The acute neonatal consequences of IUGR are perinatal asphyxia and neonatal adaptive problems. These adaptive problems that include respiratory distress due to meconium aspiration, persistent pulmonary hypertension or pulmonary hemorrhage, abnormalities of glucose regulation, temperature instability, and polycythemia are reviewed in this article. Issues specific to the IUGR preterm infant are reviewed as well including an increased incidence of chronic lung disease, necrotizing enterocolitis, retinopathy of prematurity and postnatal growth failure.
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PMID:The IUGR newborn. 1848 25

Unilateral pulmonary anomalies are rare events of unknown etiology and large clinical variability. Neonatal history does not allow for a reliable prognosis. Interdisciplinary mangament includes prenatal diagnostics and obstetrics, genetics, neonatology, pediatric cardiology and surgery as well as pediatric orthopedics. Neonatal history and long-term follow-up in three patients are presented here including a discussion of prenatal diagnostics and the embryo-genetic basics of lung development. In three term neonates the diagnoses of unilateral pulmonary agenesis, aplasia and dysplasia, respectively, were based on angiography, MRI and bronchoscopy. Neonatal presentation and long-term consequences were studied in the context of the current literature. Neonatal complications ranged from mild repiratory distress to pulmonary failure requiring mechanical ventilation. One patient developed scoliosis on long-term follow-up. Cardiac failure or pulmonary hypertension did not occur during follow-up, in one case lung malformation was accompanied by VACTER-association. Unilateral lung malformation is frequently associated with other, singular or complex anomalies (e.g., renal and vascular). A possible relationship to disrupted regulation of embryo-genetic factors such as T-BOX genes, PITX2 and growth factors ( FGF10), which regulate ASYMMETRICAL pulmonary morphogenesis is discussed. Disruptive unilateral pulmonary malformations may serve as a model for embryological lung development and other anomalies (e.g., congenital diaphragmatic hernia, unilateral hypoplasia and CCAM). Prenatal diagnosis is characterized by unilateral hyperechogenicity of the affected lung. Neonatal presentation is determined by mediastinal shift which may be corrected by tissue-expander implantation. Associated anomalies require cytogenetic analysis and sequencing of currently known mutations. Long-term follow-up by echocardiography and pulmonary function testing is mandatory in these patients.
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PMID:[Unilateral pulmonary agenesis, aplasia and dysplasia]. 1931 94

Objective laboratory tools are needed to monitor developing organ damage in sickle cell disease (SCD). Circulating endothelial cells (CECs) are indicative of vascular injury. We determined whether elevated CEC can be detected in asymptomatic SCD with the CellSearch system and whether the CEC count is related to clinical and blood-based biomarkers of disease severity. Fifteen consecutive clinically asymptomatic HbSS patients and 15 matched HbAA controls were analyzed for CEC counts, laboratory parameters of disease severity (Hb, leukocyte counts, HbF%), plasma levels of markers for endothelial activation (sVCAM-1, VWF:Ag) and of endogenous inhibitors of nitric oxide synthase (asymmetrical dimethylarginine [ADMA]). CEC counts were significantly higher in patients (12 cells/mL, IQR 8-29) as compared to controls (4 cells/mL, 3-10) (P=0.007). CEC counts were significantly higher in patients with pulmonary hypertension (PHT) (P=0.015), and increased with increasing number of affected organs (0-4 involved organs, P=0.002). No significant correlations between CEC and any other laboratory parameter were detected. In conclusion, CECs could prove to be an important new tool for assessing developing vasculopathy and organ damage in SCD.
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PMID:Circulating endothelial cells: a potential parameter of organ damage in sickle cell anemia? 1935 55

Arginine is the substrate for nitric oxide synthases (NOS), and arginine availability regulates the production of nitric oxide. Through the activity of methyltransferases, arginine can be methylated to form monomethylarginine (NMMA), asymmetrical dimethylarginine (ADMA), and symmetrical dimethylarginine (SDMA). NMMA and ADMA directly inhibit NOS, whereas SDMA inhibits the cellular import of arginine through the cationic amino acid transporter. Increased levels of methylarginine compounds have been associated with many diseases including atherosclerosis, renal failure, pulmonary hypertension, and preeclampsia. Previous HPLC methods to measure these molecules rely on derivatization with ortho-phthalaldehyde, which is unstable and requires immediate pre- or post-column reactions. We have identified a new fluorometric agent that is stable for at least 1 week and provides chromatographic properties that facilitate separation of these chemically similar compounds by reverse phase chromatography.
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PMID:Measurement of arginine metabolites: regulators of nitric oxide metabolism. 2451 May 41

Pulmonary arterial hypertension (PAH) is a debilitating and deadly disease with no known cure. Heart failure is a major comorbidity and a common cause of the premature death of patients with PAH. Increased asymmetrical right ventricular hypertrophy and septal wall thickening compress the left ventricular cavity and elicit diastolic heart failure. In this study, we used the Sugen5416/hypoxia/normoxia-induced PAH rat to determine whether altered pyridine nucleotide signaling in the failing heart contributes to 1) increased oxidative stress, 2) changes in metabolic phenotype, 3) autophagy, and 4) the PAH-induced failure. We found that increased reactive oxygen species, metabolic maladaptation, and autophagy contributed to the pathogenesis of right ventricular remodeling and hypertrophy that lead to left ventricular diastolic dysfunction. In addition, arterial elastance increased in PAH rats. Glucose-6-phosphate dehydrogenase is a major source of pyridine molecule (nicotinamide adenine dinucleotide phosphate), which is a substrate for nicotinamide adenine dinucleotide phosphate oxidases in the heart. Dehydroepiandrosterone, a 17-ketosteroid that reduces pulmonary hypertension and right ventricular hypertrophy, inhibited glucose-6-phosphate dehydrogenase, decreased oxidative stress, increased glucose oxidation and acetyl-coA, and reduced autophagy in the hearts of PAH rats. It also decreased arterial stiffness and improved left ventricular diastolic function. These findings demonstrate that pyridine nucleotide signaling, at least partly, mediates PAH-induced diastolic heart failure, and that reduction of glucose-6-phosphate dehydrogenase-derived nicotinamide adenine dinucleotide phosphate is beneficial to improve left ventricle diastolic function.
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PMID:Increased reactive oxygen species, metabolic maladaptation, and autophagy contribute to pulmonary arterial hypertension-induced ventricular hypertrophy and diastolic heart failure. 2526 98

Introduction: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) results from progressive thrombotic occlusion of the pulmonary arteries. It is treated by surgical removal of the occlusion, with success rates depending on the degree of microvascular remodeling. Surgical eligibility is influenced by the contributions of both the thrombus occlusion and microvasculature remodeling to the overall vascular resistance. Assessing this is challenging due to the high inter-individual variability in arterial morphology and physiology. We investigated the potential of patient-specific computational flow modeling to quantify pressure gradients in the pulmonary arteries of CTEPH patients to assist the decision-making process for surgical eligibility. Methods: Detailed segmentations of the pulmonary arteries were created from postoperative chest Computed Tomography scans of three CTEPH patients. A focal stenosis was included in the original geometry to compare the pre- and post-surgical hemodynamics. Three-dimensional flow simulations were performed on each morphology to quantify velocity-dependent pressure changes using a finite element solver coupled to terminal 2-element Windkessel models. In addition to transient flow simulations, a parametric modeling approach based on constant flow simulations is also proposed as faster technique to estimate relative pressure drops through the proximal pulmonary vasculature. Results: An asymmetrical flow split between left and right pulmonary arteries was observed in the stenosed models. Removing the proximal obstruction resulted in a reduction of the right-left pressure imbalance of up to 18%. Changes were also observed in the wall shear stresses and flow topology, where vortices developed in the stenosed model while the non-stenosed retained a helical flow. The predicted pressure gradients from constant flow simulations were consistent with the ones measured in the transient flow simulations. Conclusion: This study provides a proof of concept that patient-specific computational modeling can be used as a noninvasive tool for assisting surgical decisions in CTEPH based on hemodynamics metrics. Our technique enables determination of the proximal relative pressure, which could subsequently be compared to the total pressure drop to determine the degree of distal and proximal vascular resistance. In the longer term this approach has the potential to form the basis for a more quantitative classification system of CTEPH types.
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PMID:The Use of Biophysical Flow Models in the Surgical Management of Patients Affected by Chronic Thromboembolic Pulmonary Hypertension. 2959 74

Tricuspid valve regurgitation is generally functional in nature due to right-sided dysfunction in the setting of left-sided concomitant cardiac disease or pulmonary hypertension. Patients living with tricuspid regurgitation often experience numerous limitations as a result of right-sided heart failure symptoms. Patients with significant tricuspid disease, whether native, repaired, or replaced valve, often present with significant symptoms but may not be ideal candidates for operation or, eventually, reoperation. Transcatheter techniques to either repair or replace the tricuspid valve are a burgeoning frontier in structural cardiac interventions. Anatomical challenges include the large and asymmetrical annulus, paucity of calcification, adjacency of the right coronary artery system, and fragility of the valve tissue. Current approaches under investigation in feasibility and early phase clinical trials include edge-to-edge repair, coaptation enhancement, annuloplasty, heterotopic caval valve implantation, and percutaneous tricuspid valve replacement. Although there are limitations to the currently available transcatheter options for the patients, the initial data demonstrate the relative safety of using existing devices with good results and functional improvement. Hopefully, the emerging interest into interventional therapy of tricuspid valve disease will bring back the "forgotten valve" into the conscience of the cardiological and surgical community. This review intends to summarize the current strategies and evidences in transcatheter tricuspid valve intervention and enlightening new avenues for future clinical studies.
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PMID:Transcatheter Tricuspid Valve Interventions: Current Approaches and Future Perspectives. 3103 17


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