Images in cardiology. Arterial tortuosity syndrome in a newborn.
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Angiography↗
Biomedical subjects
P Ou
Publications and source records attributed to P Ou.
[Multislice CT scan in congenital heart disease].
The management of congenital cardiovascular malformations is a diagnostic challenge. It requires accurate assessment of the intra- and extra-cardiac malformations. Three-dimensional imaging by the multislice CT scanner is now a routine investigation for congenital heart disease, complementary to echocardiography, and, increasingly, an alternative to conventional angiography. Three dimensional imaging is particularly useful in the diagnosis of complex congenital heart disease, in the preparation of complex investigations of interventional cardiac catheterisation and the postoperative evaluation of surgical repair. This report describes the different acquisition protocols adapted for children and illustrates the present role of volumic CT scanner in congenital heart disease by clinical examples of everyday clinical practice.
Child↗
[Transcatheter exclusion of a giant aneurysm of the left pulmonary artery using covered stents].
An 11 year-old boy with an Alagille syndrome was referred for an urgent echocardiography because of new onset of febrile hemoptysis. Follow-up in our institution to that point had shown severe hypoplastic pulmonary arteries. The diagnosis of tricuspid endocarditis complicated by pulmonary embolisms and aneurysm of the left pulmonary artery was done. Despite effective antiobiotics, the evolution was marked by rapid progression in size of the left pulmonary aneurysm. Since the surgery was not an option because of high risk of death, this aneurysm was treated by a trans-catheter technique using covered stents (CP stents, Numed Inc). Its exclusion allowed to stop its progression in size and to avoid its rupture.
Aneurysm, Infected↗
[Mycoplasma pneumoniae myopericarditis in children].
Mycoplasma pneumoniae myocarditis is a rare condition, potentially very serious and seldom described in children. It is classically attributed to direct invasion or to an indirect immunological mechanism. The authors report the case of a 10 year old boy with myopericarditis, proved by authentic seroconversion, complicating congenital mitro-aortic valvular disease. In this case, the spectacular response to steroid therapy was in favour of an indirect immunological causal mechanism of the left ventricular dysfunction and pericardial involvement.
Child↗
[Spontaneous idiopathic chylopericardium in childhood].
Here we report a case of a primary idiopathic chylopericardium in a 13 years old child. Pericardial effusion was diagnosed because the child suffered chest pain and fatigue. Pericardial drainage was performed and 800mL of chylous fluid was evacuated. Extensive investigations were performed but no cause could be found. Thoracic CT scan, lymphoscintigraphy and MRI did not evidence any communication between the thoracic duct and pericardium. After 2 recurrences of pericardial effusion while the child was on a medium chain triglycerides regimen, it was decided to ligate the thoracic duct and to do a partial pericardectomy. The result was excellent with complete resolution of the pericardial effusion and no recurrence since 3 years.
Adolescent↗
[Does hypertension need treatment following correction of coarctation in childhood?].
Does hypertension need treatment following correction of coarctation in childhood? The results of the surgical repair of aortic coarctation (CoA) are excellent. Prenatal diagnosis of this defect is made in 40% of the cases and this allowed a reduction of preoperative mortality. Beside these successes, patients who underwent a CoA repair in infancy remain at high risk for resting hypertension (HT) later in life. Indeed, half of the adolescents are hypertensive and 2/3 of the patients around 30 years. This HT is responsible for an increased mortality mostly related to cardiovascular events. Screening for HT and its risk factors is the main objective of the follow-up. Some residual anomalies such as recoarctation or hypoplasia of the horizontal aorta may be treated either by surgery or by interventional catheterisation. Recently, new contributors to hypertension have been identified such as abnormal geometry of the aortic arch or alteration of the mechanical properties of the arterial wall. In a given patient, the co-existence of vascular dysfunction and abnormal geometry of the aortic arch confers a high risk for HT. The indications to treat exercise HT or the type of antihypertensive drug to be given remain unsolved questions. Prevention may rely on substantial modifications of the surgical techniques to optimise the aortic arch geometry. The indications to correct abnormal geometries at high risk without recoarctation are not yet defined. The long-term benefit of either preventive or curative strategies might be difficult to evidence and will probably need the analysis of intermediate markers such as vascular function and left ventricular hypertrophy.
Aortic Coarctation↗
Right to left shunt through interatrial septal defects in patients with congenital heart disease: results of interventional closure.
OBJECTIVE: To study the effects of closure of interatrial communications associated with a right to left shunt in patients with congenital heart disease (CHD) who had a biventricular repair. DESIGN: Retrospective study. SETTING: Tertiary referral centre. PATIENTS: 15 patients with CHD with right to left shunt through an interatrial communication: three had repaired tetralogy of Fallot, five had repaired pulmonary atresia with intact ventricular septum, four had Ebstein's disease, and three had other CHDs. Two patients had had a stroke before closure of the interatrial communication. INTERVENTIONS: Percutaneous atrial septal defect (n = 6) or persistent foramen ovale (n = 9) closure. All patients underwent an exercise test before and after interatrial communication closure. RESULTS: Five patients were cyanotic at rest. During exercise, mean (SD) oxygen saturation diminished from 93.9 (3.8)% to 84.3 (4.8)% (p < 0.05). Interatrial communication closure led to an immediate increase of oxygen saturation from 93.9 (3.8)% to 98.6 (1.6)% (p < 0.05). At a median follow up of three years (range 0.5-5) all but one patient with a residual atrial septal defect had normal oxygen saturation at rest and during exercise. Maximum workload increased from 7.2 (1.9) to 9.0 (2.2) metabolic equivalents (p < 0.001). CONCLUSIONS: Percutaneous closure of interatrial communications associated with a right to left shunt allows restoration of normal oxygen saturation at rest, avoidance of desaturation during exercise, and improvement of exercise performance in patients with CHD.
Adolescent↗
[A rare cause of cardiomyopathy in childhood: propionic acidosis. Three case reports].
Dilated cardiomyopathy is a rare and unrecognised complication of propionic acidosis. It usually progresses to chronic cardiac failure with a poor prognosis. The authors report three cases of cardiomyopathy due to this condition. One of the children underwent liver transplantation which cured the cardiac complication.
Acidosis↗
[Study on the prevalence rate of primary osteopenia in the middle-aged and the elderly population in some parts of China].
OBJECTIVE: To understand the distribution of prevalence rate of primary osteopenia (POPA) in the middle-aged and the elderly in some parts of China. METHODS: Bone mineral density (BMD) was measured and questionnaire was administered to 5 600 people aged above 40 in five administrative areas in China, through a stritified-multi-steps-cluster sampling method. RESULTS: The total prevalence rate of primary osteopenia in some parts of China was 15.8%, according to the most significant parts of the rate of loss. Of them, the prevalence rate of males was 20.0% compared to 12.4% in females (P < 0.01); There were also differences on POPA prevalence among provinces, age groups and areas between urban and rural (P < 0.01). CONCLUSION: Although prevention and treatment of primary osteopenia were important strategy in males however it should not be ignored in females as well.
Adult↗
A discontinuous method for catalase determination at 'near physiological' concentrations of H2O2 and its application to the study of H2O2 fluxes within cells.
We describe a discontinuous method for the measurement of catalase using the Ferrous Oxidation in Xylenol orange (FOX) assay. Samples containing catalase are incubated with H2O2 for varying time intervals prior to rapid mixing of aliquots of the incubation mixtures with FOX reagent, which measures residual H2O2. Absorbance is then read at 560 nm after 30-min incubation at room temperature. Decay of H2O2 is proportional to catalase activity in the original sample. An adaptation of the method involves exposure of intact cells to H2O2-generating systems in the presence of aminotriazole. The extent of catalase inactivation allows estimates of total H2O2 accumulation within the cell.
Amitrole↗
Activation of aldose reductase in rat lens and metal-ion chelation by aldose reductase inhibitors and lipoic acid.
Sorbitol formation in rat lenses incubated with high levels of glucose was related to activation of aldose reductase (AR). The hyperglycaemia-activated aldose reductase was inhibited by alpha-lipoic (thioctic) acid, O-phenanthroline and aldose reductase inhibitors (ARIs) including Zeopolastat (ZPLS), Sorbinil (SBN) and AL-1576. This study also examined ARIs for the ability to chelate metal ions. We found that ARIs suppress copper-dependent ascorbate oxidation, lipid peroxidation and hydrogen peroxide production in erythrocytes. ARIs also increased partition of copper ions into noctanol, which indicates formation of lipophilic complexes. Our data support the hypothesis that transition metals may be involved in activation of the polyol (aldose reductase) pathway. Also, ARIs function as metal-chelating antioxidants that may contribute to their therapeutic role for diabetic complications.
1-Octanol↗
Thioctic (lipoic) acid: a therapeutic metal-chelating antioxidant?
Thioctic (alpha-lipoic) acid (TA) is a drug used for the treatment of diabetic polyneuropathy in Germany. It has been proposed that TA acts as an antioxidant and interferes with the pathogenesis of diabetic polyneuropathy. We suggest that one component of its antioxidant activity requiring study is the direct transition metal-chelating activity of the drug. We found that TA had a profound dose-dependent inhibitory effect upon Cu(2+)-catalysed ascorbic acid oxidation (monitored by O2 uptake and spectrophotometrically at 265 nm) and also increased the partition of Cu2+ into n-octanol from an aqueous solution suggesting that TA forms a lipophilic complex with Cu2+. TA also inhibited Cu(2+)-catalysed liposomal peroxidation. Furthermore, TA inhibited intracellular H2O2 production in erythrocytes challenged with ascorbate, a process thought to be mediated by loosely chelated Cu2+ within the erythrocyte. These data, taken together, suggest that prior intracellular reduction of TA to dihydrolipoic acid is not an obligatory mechanism for an antioxidant effect of the drug, which may also operate via Cu(2+)-chelation. The R-enantiomer and racemic mixture of the drug (alpha-TA) generally seemed more effective than the S-enantiomer in these assays of metal chelation.
Antioxidants↗
Erythrocyte catalase inactivation (H2O2 production) by ascorbic acid and glucose in the presence of aminotriazole: role of transition metals and relevance to diabetes.
Erythrocytes exposed to ascorbic acid in the presence of aminotriazole undergo a dose- and time-dependent inactivation of endogenous catalase which is proportional to environmental hydrogen peroxide (H2O2) concentrations. The production of H2O2 seems to be dependent upon the availability of transition metal chelatable by o-phenanthroline (OPT), although the kinetics of catalase inactivation and H2O2 production by externally added copper ions in the presence of OPT is complex. Furthermore, although glucose is also able to undergo a transition-metal-catalysed oxidation yielding H2O2, the production of H2O2 by glucose seems to be a minor process by comparison with ascorbic acid oxidation. Indeed, on the basis of these data, transition-metal-catalysed ascorbic acid oxidation is likely to be a more important source of oxidative stress in the diabetic state than hyperglycaemia.
Amitrole↗
Aminoguanidine: a drug proposed for prophylaxis in diabetes inhibits catalase and generates hydrogen peroxide in vitro.
Aminoguanidine (AG) has been proposed as a drug of potential benefit in prophylaxis of the complications of diabetes. We show here that AG irreversibly inhibits catalase with an efficacy similar to aminotriazole. AG also produces hydrogen peroxide, in a transition metal-catalysed process which may be partially dependent upon prior hydrolysis of AG to semicarbazide and hydrazine. These observations may be of importance in proposals for the long term administration of AG in diabetes.
Amitrole↗
[Aortic arch shape and flow dynamics].
Post-operative deformation of the aortic arch architecture is associated with an increased risk of hypertension following correction of coarctation. In addition to morphological analysis, MRI allows a functional analysis of the thoracic aorta. We report three examples which illustrate the direct relationship between aortic arch morphology and blood flow in the thoracic aorta.
Adolescent↗
[Relationship between aortic arch shape and blood pressure response after coarctation repair].
The mechanisms of secondary hypertension after repair of coarctation of the aorta are not well understood. Abnormalities of the architecture of the aortic arch and their consequences on blood pressure have not been studied. In order to study the relationship between abnormalities or aortic arch architecture and resting blood pressure ninety-four patients without re-coarctation were followed up prospectively from 1997 to 2004 (mean age 16.9 +/- 8.1 years; mean weight 57.5 +/- 18.3 Kg; interval since surgery 16.3 +/- 5.4 years). All underwent MRI angiography of the thoracic aorta which enabled the abnormalities to be classified in 3 groups: gothic arch, crenellated arch and roman arch. Twenty-four patients (25.5%) were hypertensive and 70 (74.4%) normotensive. There were 40 gothic arches (42.5%). 14 crenellated arches (15%) and 40 roman arches (42.5%). Gothic arches were more commonly observed in the hypertensive patients (18/40, [45%, 95% CI 31-62]) than the crenellated arches (4/14, [28.5%, 95% CI 7-48]) or the roman arches (2/40, [5%, 95% CI 2-12]). Only the gothic arch was independently correlated with hypertension on multivariate analysis. The authors conclude that gothic deformation of the aortic arch is an independent predictive factor of hypertension in patients operated for coarctation with an excellent result on the isthmic region. Patients with a gothic appearance of their aortic arch should be followed up closely.
Adolescent↗