Arrythmogenic Right Ventricular Dysplasia

A 10-year-old well and asymptomatic female was referred for screening of acute right ventricular dilatation (ARVD) as she had an elder brother diagnosed with ARVD whom died of sudden cardiac death. Electrocardiography (ECG), transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) were performed. Results of these investigations were suggestive of ARVD. Despite being a rare cardiac disease and largely unrecognised in children and young adult population, ARVD is an important cause of ventricular arrhythmias in this group of patients and is one of the causes of sudden cardiac death (SCD) in this population.

Papel da lipoperoxidação na intensificação da remodelação causada pelo betacaroteno após o infarto / Rol de la lipoperoxidación en la intensificación de la remodelación ocasionada por el betacaroteno tras infarto / Role of lipoperoxidation in the remodeling intensification induced by beta-carotene after infarction

FUNDAMENTO: Os mecanismos envolvidos na maior remodelação causada pelo betacaroteno após o infarto são desconhecidos. OBJETIVO: Analisar o papel da lipoperoxidação na remodelação ventricular após o infarto do miocárdio, em ratos suplementados com betacaroteno. MÉTODOS: Ratos foram infartados e distribuídos em dois grupos: C (controle) e BC (500mg/kg/dieta). Após seis meses, foram realizados ecocardiograma e avaliação bioquímica. Utilizamos o teste t, com significância de 5 por cento. RESULTADOS: Os animais do grupo BC apresentaram maiores médias das áreas diastólicas (C = 1,57 ± 0,4 mm²/g, BC = 2,09 ± 0,3 mm²/g; p < 0,001) e sistólicas (C = 1,05 ± 0,3 mm²/g, BC = 1,61 ± 0,3 mm²/g; p < 0,001) do VE, ajustadas ao peso corporal do rato. A função sistólica do VE, avaliada pela fração de variação de área, foi menor nos animais suplementados com betacaroteno (C = 31,9 ± 9,3 por cento, BC = 23,6 ± 5,1 por cento; p = 0,006). Os animais suplementados com betacaroteno apresentaram valores maiores da relação E/A (C = 2,7 ± 2,5, BC = 5,1 ± 2,8; p = 0,036). Não foram encontradas diferenças entre os grupos em relação aos níveis cardíacos de GSH (C = 21 ± 8 nmol/mg de proteína, BC = 37 ±15 nmol/mg de proteína; p = 0,086), GSSG (C = 0,4 (0,3-0,5) nmol/g de proteína, BC = 0,8 (0,4-1,0; p = 0,19) de proteína; p = 0,246) e lipoperóxidos (C = 0,4 ± 0,2 nmol/mg de tecido, BC = 0,2 ± 0,1 nmol/mg de tecido; p = 0,086). CONCLUSÃO: A maior remodelação em animais infartados e suplementados com betacaroteno não depende da lipoperoxidação.

BACKGROUND: The mechanisms involved in the biggest remodeling caused by the post-infarct beta-carotene are unknown. OBJECTIVE: To analyze the role of lipoperoxidation in the ventricular remodeling after infarct of the myocardium in rats supplemented with beta-carotene. METHODS: Rats were infarcted and divided into two groups: C (control) and BC (500mg/kg/regimen). After six months, echocardiogram and biochemical evaluation were performed. The t test was used, with 5 percent significance. RESULTS: The animals from BC group presented highest means of the diastolic (C = 1.57 ± 0.4 mm²/g, BC = 2.09 ± 0.3 mm²/g; p < 0.001) and systolic (C = 1.05 ± 0.3 mm²/g, BC = 1.61 ± 0.3 mm²/g; p < 0.001) areas of LV, which were adapted according to the rat's body weight. The systolic function of LV, evaluated by the area variation fraction, was lower in the animals supplemented with beta-carotene (C = 31.9 ± 9.3 percent, BC = 23.6 ± 5.1 percent; p = 0.006). The animals supplemented with beta-carotene presented higher values of the E/A relation (C = 2.7 ± 2.5, BC = 5.1 ± 2.8; p = 0.036). No differences were found between the groups concerning the cardiac levels of the GSH (C = 21 ± 8 nmol/mg of protein, BC = 37 ± 15 nmol/mg of protein; p = 0.086), GSSG (C = 0.4 (0.3-0.5) nmol/g of protein, BC = 0.8 (0.4-1.0; p = 0.19) of protein; p = 0.246) and lipoperoxides (C = 0.4 ± 0.2 nmol/mg of tissue, BC = 0.2 ± 0.1 nmol/mg of tissue; p = 0.086). CONCLUSION: The highest remodeling in infarcted rats supplemented with beta-carotene does not depend on the lipoperoxidation.

FUNDAMENTO: Los mecanismos implicados en la mayor remodelación ocasionada por betacaroteno tras el infarto son desconocidos. OBJETIVO: Analizar el rol que juega la lipoperoxidación en la remodelación ventricular tras el infarto de miocardio, en ratas suplementadas con betacaroteno. MÉTODOS: Se había inducido a un infarto a las ratas y se las distribuyó en grupos: C (control) y BC (500mg/kg/dieta). Tras seis meses, se realizaron ecocardiograma y evaluación bioquímica. Utilizamos la prueba t, con significancia del 5 por ciento. RESULTADOS: Los animales del grupo BC presentaron mayores promedios de las áreas diastólicas (C = 1,57 ± 0,4 mm²/g, BC = 2,09 ± 0,3 mm²/g; p < 0,001) y sistólicas (C = 1,05 ± 0,3 mm²/g, BC = 1,61 ± 0,3 mm²/g; p < 0,001) del VI, ajustadas al peso corporal de la rata. La función sistólica del VI, evaluada por la fracción de variación de área, fue menor en los animales suplementados con betacaroteno (C = 31,9 ± 9,3 por ciento, BC = 23,6 ± 5,1 por ciento; p = 0,006). Los animales suplementados con betacaroteno presentaron valores mayores de la relación E/A (C = 2,7 ± 2,5, BC = 5,1 ± 2,8; p = 0,036). No se encontraron diferencias entre los grupos con relación a los niveles cardiacos de GSH (C = 21 ± 8 nmol/mg de proteína, BC = 37 ±15 nmol/mg de proteína; p = 0,086), GSSG (C = 0,4 (0,3-0,5) nmol/g de proteína, BC = 0,8 (0,4-1,0; p = 0,19) de proteína; p = 0,246) y lipoperóxidos (C = 0,4 ± 0,2 nmol/mg de tejido, BC = 0,2 ± 0,1 nmol/mg de tejido; p = 0,086). CONCLUSIÓN: La mayor remodelación en animales infartados y suplementados con betacaroteno no depende de la lipoperoxidación.

The role of oxidative stress and lipid peroxidation in ventricular remodeling induced by tobacco smoke exposure after myocardial infarction

OBJECTIVE: To evaluate the roles of oxidative stress and lipid peroxidation in the ventricular remodeling that is induced by tobacco smoke exposure after myocardial infarction. METHODS: After induced myocardial infarction, rats were allocated into two groups: C (control, n=25) and ETS (exposed to tobacco smoke, n=24). After 6 months, survivors were submitted to echocardiogram and biochemical analyses. RESULTS: Rats in the ETS group showed higher diastolic (C = 1.52 ± 0.4 mm2, ETS = 1.95 ± 0.4 mm2; p=0.032) and systolic (C = 1.03 ± 0.3, ETS = 1.36 ± 0.4 mm2/g; p=0.049) ventricular areas, adjusted for body weight. The fractional area change was smaller in the ETS group (C = 30.3 ± 10.1 %, ETS = 19.2 ± 11.1 %; p=0.024) and E/A ratios were higher in ETS animals (C = 2.3 ± 2.2, ETS = 5.1 ± 2.5; p=0.037). ETS was also associated with a higher water percentage in the lung (C = 4.8 (4.3-4.8), ETS = 5.5 (5.3-5.6); p=0.013) as well as higher cardiac levels of reduced glutathione (C = 20.7 ± 7.6 nmol/mg of protein, ETS = 40.7 ± 12.7 nmol/mg of protein; p=0.037) and oxidized glutathione (C = 0.3 ± 0.1 nmol/g of protein, ETS = 0.9 ± 0.3 nmol/g of protein; p=0.008). No differences were observed in lipid hydroperoxide levels (C = 0.4 ± 0.2 nmol/mg of tissue, ETS = 0.1 ± 0.1 nmol/mg of tissue; p=0.08). CONCLUSION: In animals exposed to tobacco smoke, oxidative stress is associated with the intensification of ventricular remodeling after myocardial infarction.

Prenatal Diagnosis of Pallister-Killian Syndrome Associated with Pulmonary Stenosis and Right Ventricular Dilatation / 대한진단검사의학회지

Pallister-Killian syndrome (PKS) is a rare disorder characterized cytogenetically by tetrasomy 12p for isochromosome of the short arm of chromosome 12. PKS is diagnosed by prenatal genetic analysis through chorionic villous sampling, genetic amniocentesis, and cordocentesis, or by chromosomal analysis of skin fibroblasts, but is not usually detected by chromosomal analysis of peripheral blood cells. Herein, we report a case of a gravida at 23 weeks gestation with pulmonary stenosis and right ventricular dilation of the heart which were detected by sonography. Fluorescence in situ hybridization and a multicolor banding technique were performed to verify the diagnosis as 47,XX, +mar.ish i(12)(p10)(TEL++)[16]/46,XX[4], and an autopsy confirmed the cardiac anomalies detected on antenatal sonography.

The changes of cerebrospinal fluid non-protein-bound iron and malondialdehyde(MDA) in preterm infants with severe intraventricular hemorrhage (IVH) after serial lumbar punctures / 中国小儿急救医学

Objective To investigate the changes and clinical significance of cerebrospinal fluid (CSF) non-protein-bound iron and malondialdehyde(MDA) in preterm infants with severe intraventricular hemorrhage (IVH) after serial lumbar punctures.Methods Non-protein-bound iron and MDA of CSF in twenty severe IVH preterm infants with first and end lumbar puncture were examined respectively.The control samples were determined for once from first to second week after birth.Results Both Non-protein-bound iron and MDA levels of the first CSF sample in twenty severe IVH preterm infants were higher than that in control[(0.66±0.38)μmol/L vs (0.24±0.12)μmol/L(P<0.01);(1.21±0.41)μmol/L vs (0.89±0.35)μmol/L(P<0.05)].Non-protein-bound iron levels of first and end CSF sample in severe IVH with posthemorrhagic ventricular dilatation (PHVD) were both higher than those without PHVD [(0.75±0.34)μmol/L vs (0.54±0.31)μmol/L;(0.57±0.21)μmol/L vs (0.35±0.18)μmol/L](P<0.05).However,there was no significant difference in MDA levels between both groups.The levels of non-protein-bound iron of end CSF in severe IVH with and without PHVD were significantly lower than those of the first CSF samples (P<0.05),while,there was no significant difference in MDA levels between the end and first CSF samples.Conclusion The non-protein-bound iron and MDA of CSF in severe IVH preterm infants were both significantly increased,non-protein-bound iron and oxygen free radicals may be involved in the preterm brain injury.Serial lumbar punctures can lower the levels of non-protein-bound iron in CSF.

Syringomyelia as a Sequelae of the 4th Ventricular Dilatation from Traumatic Hydrocephalus and Cerebellar Atrophy

A 32-year-old man with stuporous mental state was transferred to our hospital emergency room after a car accident. The brain computed tomography(CT) showed 4th ventricular hemorrhage. He woke up 2 weeks after admission and then discharged. However, he returned to the hospital 10 months after discharge because of both shoulder pain and weakness of both arms. His brain CT showed marked dilatation of the 4th ventricle. His MRI showed whole spinal syringomyelia without Chiari malformation. The patient then underwent ventriculo-peritoneal shunt. His symptoms dramatically improved on the immediate postoperative day, and the syringomyelia also disappeared after operation. The authors report a very rare case of syringomyelia that was developed as a sequelae, especially of the 4th ventricular dilatation without intracranial pressure elevation after traumatic hydrocephalus and cerebellar atrophy, favoring Gardner's hypothesis.

Follow up of neonata hypoxic ischemic encephalopathy and ventricular dilatation / 实用儿科临床杂志

Objective To investigate the prognostic sequelae in neontes with hypoxic-ischemic encephalopathy (HIE) and ven-tricnlar dilatation.Methods Seventy-six full term newborns infants with HIE were followed up at the age from 3 to 19 months after therapy. Twenty-five infants among them were followed up by telephone in the epidemic period of SARS.Results Among 76 infants of 88 newborn infants with HIE(84.6%), 73 infants were normal (96.1% ). 1 infant had cerebral palsy (1.3%), 2 infants died (2.6 %).Among 39 cases with mild HIE, none of them had cerebral sequelae; among moderate HIE. 1 infant had cerebral palsy (2.9%) 1 infant died (2. 9 %), interlenkin-4 among severe HIE 50 % died (P00.5 The poor outcome of HIE in those infants were related to intrauterine growth retardation,severe birth asphyxia;and inadequate treatment.Cranial ultra-sonography of 49 infants were done on follow-up,and 12 of them (24.5 % ) had ventricular dilatations, which appeared after birth with 6 infants. Others occurred on follow-up with 1 infant had cerobral palsy,all ventricular dilatations recovered to normal at 12- 19 months except the cerebral palsy.Conclusions The poor outcome of HIE depends on the infants with intranterine growth relarda-tion,severe birth asphyxia and inadequate treatment.The prognosis of transient ventrealar ddatation are good except cerebral palsy.J Appl Clin pediatr,2004,19(12) : 1045- 1047

Subdural Fluid Collection after Intracranial Aneurysmal Surgery

The clinical course of subdural fluid collection(SDGC) was studied in 32 cases of patients which underwent craniotomy for intracranial aneurysm and analysed regard to patients' age, sex, degree of initial subarachnid hemorrhage, the operating time of aneurysm, duration to appearance of SDFC from craniotomy, changes of clinical state, disappearance time, site, maximal thickness and changes of SDFC, management and prognosis. The results were summarized as followings. 1) SDFC was found in 33 of 85 patients(38.8%). 2) The older age group had higher incidence of SDFC than the younger age group. 3) The older age grop had thicker SDFC than the younger age group in the maximal thickness of SDFC, and that was statistically significant. 4) The early surgery group of aneurysm had lower incidence of SDFC compared with the delayed surgery group. 5) There were minimal changes of clinical states when SDFC was diagnosed by brain CT. 6) The frontal area was involved in all cases and bilateral involvement was noted in 15 cases(45.5%). 7) Surgical procedures were needed in 8 cases(24.2%) of SDFC, including 2 cases of chronic subdural hematoma. 8) Ventricular dilatation with spontaneous improvement of SDFC were noted in 8 cases(24.2%) and lumboperitoneal shunt for hydrocephalus was needed in only 2 cases of them.

Ventricular Dilatation by Early Surgery on Aneurysms with SAH

Among 202 aneurysmal patients with SAH who were admitted to Kyungpook National University Hospital from Jan. 1984 through Jan. 1988, the incidence of ventricular dilatation confirmed by postoperative follow-up brain CT scannings was 32.2%(65 cases). And 33 cases(19.3%) was in mild ventricular dilatation, 9 cases(4.5%) was in moderate and 23 cases(11.4%) in severe who needed the shunting procedure were analysed. The incidence of ventricular dilatation to the site of aneurysm was highest in A-com A. aneurysm(42.2%), followed by P-com A. aneurysm(36.4%) and MCA aneurysm(11.3%). The incidence of ventricular dilatation and the need for shunting was higher in multiple bleeding patients than the single. The good risk patients had meaningfully lower incidence of ventricular dilatation than the poor risk group. In the delayed surgical group showed significantly more numbers of ventricular dilatation needing the shunt than the cases in early group.

Factors Affecting the Prognosis in Hypertensive Thalamic Hemorrhage

The authors reviewed 114 patients with hypertensive thalamic hemorrhage diagnosed by CT scan, and analyzed the factors affecting the prognosis in acute stage of 76 patients who were selected by our criteria. The overall mortality was 26.3%. The isolated hemorrhage within thalamus represented 22.8% and showed 100% survivability of ADL(ability in daily life) 3 or above. The mortality of the other that were extended to internal capsule or midbrain, was 35.7%. The factors affecting poor prognosis were as follows : 1) poor conscious level on admission ; 2) the hematoma on left side in location ; 3) hemorrhagic groups that were extended to internal capsule with intraventricular hemorrhage, midbrain, or both ; 4) the presence of ventricular penetration or dilatation ; 5) the hematoma volume of 10cc or above ; 6) the maximum diameter of 33mm or above ; 7) the maximum height of 40mm or above. The prognosis had no significant relationship with the age.

An Experimental Study about the Effect of Urokinase Injected into the Ventricle on Intraventricular Hemorrhage

For the reduction of morbidity, such as an acute hydrocephalus, following intraventricular hemorrhage, we attempted to produce a lysis of the experimental intraventricular hemorrhage by a direct intraventricular injection of fibrinolytic agent. Urokinase was used as the fibrinolytic activator. Sixty-four adult rabbits were used in this study. The animals were divided into 5 groups to investigate the effect of urokinase in different time interval of urokinase injection. Intraventricular hemorrhage was made by an injection of 0.3cc of autogenous venous blood. Group I was the control group in which intraventricular injection of blood or urokinase was only done. In Group II and III urokinase was injected into the ventricule 30 minutes and 2 hours after the blood injection. In Group IV urokinase was injected into the ventricle at the same time of the blood injection. In Group V urokinase was injected into the ventricle at the same time of the blood injection, and then the urokinase injection was repeated 24 hours later. The animals of each group were sacrificed on the 1st, 2nd, 3rd and 7th day successively after the experimental procedures. The brains were examined to observe the outcome of intraventricular hematoma with urokinase injection and the consequent changes of the ventricular system grossly and microscopically. The results were as follows : The duration of the remaining hematoma in the ventricles and basal cisterns was 7 days in both the control and the urokinase injection groups equally. In the group of repeated urokinase injection the duration of remaining hematoma in the ventricular system was shortened to 5 days after the blood injection. Upon the ventricular dilatation, the blood injection control group showed moderate dilatation persistently for 1 week ; from minimal to moderate dilatations were found in the urokinase injection groups. In the microscopic examination there were no definite abnormal changes on the ventricular walls and leptomeninges throughout 1 week in the urokinase injection control group. The group of repeated urokinase injection revealed mild flattening and denudation of the ependyma of the ventricular system than the group of single urokinase injection.

CT Evaluation in Patients with Intraventricular Hemorrhage

Ninety-three patients who had evidence of intraventricular hemorrhage(IVH) were reviewed. Primary and secondary IVH could be well distinguished by computerized tomographic(CT) brain scan. 11 patients had primary pure IVH and remaining 82 patients were secondary IVH from mainly hypertensive intracerebral hemorrhage. Four patients died and 7 survived in the pure IVH. Three of the 4 who died were aneurysmal rupture. Remaining one was unknown etiology. In CT scan hemorrhage was most frequently found in the occipital horns according to the supine position of the patient during the examination and least found in the temporal horns probably due to narrow crescentic shape. In the secondary IVH the distribution of the blood within the lateral ventricle was mainly ipslateral to the intracerebral hematoma. The point of penetration of the intracerebral hematoma into the lateral ventricle was the posterior body in the basal ganglia or thalamic hemorrhage, the frontal horn in the aneurysmal rupture and the trigone in the arteriovenous malformation. Although the level of consciousness on admission related to the size and location of primary intracerebral hemorrhage or IVH, the ventricular dilatation itself also seemed to contribute to the consciousness state. The degree of temporal horn dilatation correlated more with the clinical grade than the frontal horn dilatation.