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Autonomic neuropathy: diagnosis and impact on health in adolescents with diabetes.

Symptomatic autonomic neuropathy and abnormal cardiovascular autonomic tests are associated with increased mortality due to vascular disease and increased risk of sudden deaths. Intensive therapy in the DCCT caused a significant risk reduction of developing autonomic nerve abnormalities at five years only in the Primary Prevention Group (4 vs. 9%). At the Royal Alexandra Hospital for Children, the prevalence of cardiovascular reflex abnormalities is 28%. Over 5 years, we have found no increase in the rate of single cardiovascular abnormalities and a low rate of persistence. Two or more abnormalities developed in 5%. Repeated measures of pupillary function showed a significant increase in abnormalities and a higher level of persistently abnormal tests. Spectral analysis and 24-hour BP monitoring may detect autonomic dysfunction at earlier stages but longitudinal studies are not yet available.

Adolescent↗

Cardiac involvement in autosomal-dominant polycystic kidney disease: a hypertensive heart disease.

BACKGROUND: There is little information regarding the occurrence and distribution of cardiovascular abnormalities during the course of autosomal-dominant polycystic kidney disease (ADPKD). We conducted a cross-sectional study in three different groups of ADPKD patients to determine the profile and prevalence of cardiac involvement in this population. METHODS: Doppler color echocardiography was performed in 130 ADPKD patients. Patients were divided into normotensive (Group I, n=60), hypertensive (Group II, n=32) and those undergoing hemodialysis (Group III, n=38). RESULTS: There was a progressive increase in left ventricular mass (LVM) index (88.6+/-19.7, 127.6+/-40.4 and 150.5+/-56.5 g/m2, p < 0.0001) and in the prevalence of left ventricular hypertrophy (LVH) (3%, 43%, 62%, p < 0.0001) in Groups I, II and III, respectively. E/A ratio < 1 was found in 2% of normotensives, 46% of hypertensives and 62% of hemodialysis patients (p < 0.0001). Prevalence ofmitral valve prolapse and aortic and/or mitral regurgitation was 4.3% and 8.6%, respectively, in non-dialysis patients. The majority of valvular abnormalities occurred in dialysis patients, and were generally related to annular mitral calcification (28%) or aortic valve calcification (38%). Age, sex, systolic blood pressure (BP) and hemoglobin were independent predictors of LVM index in the entire population, systolic BP and creatinine in non-dialysis patients and systolic BP in dialysis patients. Age, heart rate and diastolic BP in the entire group, and age, heart rate and LVM index in non-dialysis patients remained as independent predictors of abnormal diastolic function. CONCLUSIONS: Cardiac involvement in ADPKD patients is a continuous process that evolves during the course of this disease. It is characterized by a low prevalence of specific valvular abnormalities, a progressive increase in LVM, LVH, and diastolic dysfunction, which are greatest in the latter stages of the disease. This study confirms the major influence of BP on cardiovascular abnormalities of ADPKD patients.

Adult↗

The clinical problems in cardiovascular control following spinal cord injury: an overview.

On a daily basis, individuals with cervical and upper thoracic spinal cord injury face the challenge of managing their unstable blood pressure, which frequently results in persistent hypotension and/or episodes of uncontrolled hypertension. This chapter will focus on the clinical issues related to abnormal cardiovascular control in individuals with spinal cord injury, which include neurogenic shock, autonomic dysreflexia and orthostatic hypotension. Blood pressure control depends upon tonic activation of sympathetic preganglionic neurons by descending input from the supraspinal structures (Calaresu and Yardley, 1988). Following spinal cord injury, these pathways are disrupted, and thus spinal circuits are solely responsible for the generation of sympathetic activity (Osborn et al., 1989; Maiorov et al., 1997). This results in a variety of cardiovascular abnormalities that have been well documented in human studies, as well as in animal models (Osborn et al., 1990; Mathias and Frankel, 1992a, b; Krassioukov and Weaver, 1995; Maiorov et al., 1997, 1998; Teasell et al., 2000). However, the recognition and management of these cardiovascular dysfunctions following spinal cord injury represent challenging clinical issues. Moreover, cardiovascular disorders in the acute and chronic stages of spinal cord injury are among the most common causes of death in individuals with spinal cord injury (DeVivo et al., 1999).

Autonomic Dysreflexia↗

Pathophysiologic principles relevant for therapeutic procedures in persistent pulmonary hypertension of the newborn.

Though the various pathophysiologic mechanisms underlying persistent pulmonary hypertension of the newborn (PPHN) necessitate rather specific therapeutic concepts, in the majority of infants with PPHN management is mainly focused on the treatment of the symptoms respiratory failure and cardiovascular abnormalities. For the few neonates with severe PPHN who fail to respond to optimized conventional ventilatory support, high-frequency oscillatory ventilation and extracorporeal membrane oxygenation are available. Treatment of cardiovascular abnormalities should be based on knowledge of the pathophysiologic events in the heart and in the pulmonary and systemic circulation of the newborn with PPHN at least as far as these events are known or suspected based on animals studies. The primary goals of therapy in PPHN must be reduction of right ventricular afterload and support of myocardial function. In this context inhalational nitric oxide therapy may be effective as a new therapeutic strategy to decrease pulmonary vascular resistance.

Critical Care↗

Echocardiographic features of genetic diseases: part 1. Cardiomyopathy.

Introduction To The Series Genetic disorders have characteristic cardiovascular manifestations. These cardiovascular abnormalities often are a major determinant of the morbidity and mortality in this patient population. Some characteristics are unique and can be detected with echocardiography. Drs Alizad and Seward have compiled in this series a review of genetic disorders that have recognizable morphologic and/or functional cardiovascular abnormalities. The following topics will be explored, with examples from the database of the Mayo Clinic Echocardiography Laboratory:Cardiomyopathy Storage disease Shunts Connective tissue Tumors Complex cardiovascular defects Complex genetic disorders Organ system

Arrhythmogenic Right Ventricular Dysplasia↗

Structural evidence of injury or malformation in the brains of children with congenital heart disease.

Neurological and developmental deficits are common in children with congenital heart disease (CHD). These are due to multiple factors that include the etiology of the CHD, the effects of abnormal cardiovascular function, and the possible sequelae of open heart surgery. CHD is frequently part of a multiple malformation syndrome that includes the brain. The causes of these syndromes include known or putative genetic defects. Abnormal cardiovascular function may be associated with poor brain growth, embolic infarction, cerebrovascular thrombosis, and abscess formation. Perioperative neurological complications include diffuse hypoxic-ischemic injury (particularly in neonates who undergo more than 45 to 60 minutes of hypothermic circulatory arrest), cerebral macro- and micro-emboli, dural sinus thrombosis, and cerebral hemorrhage. Neuroimaging, especially magnetic resonance imaging, is a useful prognostic instrument, can easily display gross congenital and acquired lesions, and should be performed preoperatively in addition to genetic studies. In some instances poor brain function may not be predicted unless slow head growth or microcephaly is present and thorough preoperative neurodevelopmental evaluation is encouraged.

Abnormalities, Multiple↗

[Two cases of Marfan syndrome, surgically treated for complicating spontaneous pneumothorax].

Two cases of spontaneous pneumothorax in the patients of Marfan Syndrome were reported. Nine and 13-year-old girls were admitted to our hospital complaining of chest pain and dyspnea for the first time. Tension pneumothorax was confirmed by the chest radiograph. Both of them had the physical stigmata of Marfan Syndrome including cardiovascular abnormalities. The drainage tube was inserted into the thoracic cavity. As the drainage was ineffective, the surgical treatment was undertaken. The thoracic cavity was opened though axillary incision and bullae were resected. The patient of pneumothorax with the Marfan Syndrome should be examined preoperatively about cardiovascular abnormality using chest computed tomography and echocardiography. As far as the surgical intervention was concerned, the axillary approach is preferred to the median sternotomy, as such patient highly probably require cardiovascular operation in future.

Adolescent↗

A role for smad6 in development and homeostasis of the cardiovascular system.

Smad proteins are intracellular mediators of signalling initiated by Tgf-betasuperfamily ligands (Tgf-betas, activins and bone morphogenetic proteins (Bmps)). Smads 1, 2, 3, 5 and 8 are activated upon phosphorylation by specific type I receptors, and associate with the common partner Smad4 to trigger transcriptional responses. The inhibitory Smads (6 and 7) are transcriptionally induced in cultured cells treated with Tgf-beta superfamily ligands, and downregulate signalling in in vitro assays. Gene disruption in mice has begun to reveal specific developmental and physiological functions of the signal-transducing Smads. Here we explore the role of an inhibitory Smad in vivo by targeted mutation of Madh6 (which encodes the Smad6 protein). Targeted insertion of a LacZ reporter demonstrated that Smad6 expression is largely restricted to the heart and blood vessels, and that Madh6 mutants have multiple cardiovascular abnormalities. Hyperplasia of the cardiac valves and outflow tract septation defects indicate a function for Smad6 in the regulation of endocardial cushion transformation. The role of Smad6 in the homeostasis of the adult cardiovascular system is indicated by the development of aortic ossification and elevated blood pressure in viable mutants. These defects highlight the importance of Smad6 in the tissue-specific modulation of Tgf-beta superfamily signalling pathways in vivo.

Animals↗

[Modified presentation of a lung embolism by a rare variant of pulmonary vein anomalous connection].

Partial anomalous pulmonary venous return of the left lung is an extremely rare cardiovascular abnormality. Mostly, this rare entity is found incidentally when patients undergo angiography of the pulmonary artery because of suspected pulmonary embolism. In this case hemodynamic data obtained by a swan ganz catheter suggested a cardiovascular abnormality presenting as left-right shunt. Surgical therapy of anomalous venous return of the left lung is only required if volume overload of the left ventricle occurs.

Aged↗

Sudden death in young athletes.

The causes of sudden and unexpected death in 29 highly conditioned, competitive athletes, ages 13-30 years, are summarized. Sudden death occurred during or just after severe exertion on the athletic field in 22 of the 29 athletes. Structural cardiovascular abnormalities were identified at necropsy in 28 of the 29 athletes (97%), and in 22 (76%) were almost certainly the cause of death. The most common cause of death in this series was hypertrophic cardiomyopathy, which was present in 14 athletes. Other cardiovascular abnormalities that occurred in more than one athlete were anomalous origin of the left coronary artery from the right (anterior) sinus of Valsalva, idiopathic concentric left ventricular hypertrophy, coronary heart disease and ruptured aorta. Cardiac disease was suspected during life in only seven of the 29 patients, and in only two of the seven was the correct diagnosis made clinically. Hence, in this series of young athletes, sudden death was usually due to structural cardiovascular disease, and hypertrophic cardiomyopathy was a frequent cause of sudden death; atherosclerotic coronary heart disease was relatively uncommon.

Adolescent↗

[The autopsy findings in 51 cases of AIDS with cardiovascular damage].

We describe the cardiovascular abnormalities found at autopsy in patients with AIDS and a description of the opportunistic infections in these cases studied between January 1988 and August 1993. There were 51 cases with such diagnosis. Pericardial effusion appeared in 9, pleural effusion in 7, myocarditis in 5, 7 with pericarditis, endocarditis in 6, left ventricular hypertrophy in 20, right ventricular hypertrophy in 21 and evidence of atherosclerosis in 15. Thus, data of cardiovascular damage was present in 42.7% of our patients. The cardiovascular abnormalities in this group are common, in contrast to the paucity of clinical findings. Diagnosis of cardiac pathology was made in only 12% of them. So in every case with diagnosis of AIDS, a careful clinical examination and cardiac diagnostic oriented tests must be done for detection of these abnormalities.

Acquired Immunodeficiency Syndrome↗

Autonomic nervous system dysfunction in lightning and electrical injuries.

Autonomic nervous system (ANS) dysfunction is a serious complication of lightning and electrical trauma (L/ET). The ANS regulates the normal vegetative functioning of many organ systems. When ANS is compromised after lightning and electric trauma, patients are vulnerable to serious medical problems. Three conditions of ANS dysfunction of particular concern for L/ET patients are complex regional pain syndrome (CRPS), cardiovascular abnormalities, and keraunoparalysis (KP). The patient with CRPS presents with pain, hyperpathia, sweating, and edema hours to days after trauma. Neurorehabilitation is exceedingly important. A primary goal is to keep the affected extremity mobile and functional. Some patients benefit from sympathetic blockade. Cardiovascular abnormalities associated with lightning and electrical trauma can be life threatening. Care for these patients require a multidisciplinary team including a cardiologist. Keraunoparalysis is a frightening and distressing complication of lightning strikes. The syndrome consists of limb paralysis, sensory symptoms, pallor, coolness and absent pulses. Release of excessive catecholamines is said to be responsible for these findings. Fortunately, the condition is transient.

Arrhythmias, Cardiac↗

Prenatal diagnosis of cardiovascular diseases.

UNLABELLED: Fetal echocardiography is a safe means to obtain reliable anatomical and hemodynamic data of the fetal heart. The procedure is essential for prenatal diagnosis of cardiovascular abnormalities. In addition, fetal echocardiography is useful to follow-up the progression, monitoring during treatment and making a plan of treatment especially in life-threatening cardiac conditions. The objective of this study was to find the distribution of indications for fetal echocardiography, fetal cardiac anomalies detected, outcome of the fetuses and to assess the accuracy of the procedure. A retrospective study was done at Siriraj Hospital, Mahidol University, Bangkok, Thailand. Pregnant women whose fetuses had prenatal echocardiographic data and gave birth from January 1999 to December 2001 were included. The total number of pregnant women was 54 cases. The indications for fetal echocardiography were suspicion of fetal cardiovascular disease by the obstetrician 42.6 per cent, family history of congenital heart diseases 14.8 per cent, multiple organ system anomalies 14.8 per cent, chromosomal anomalies 11.1 per cent, hydrops fetalis 9.3 per cent, maternal systemic diseases 5.6 per cent and previous pregnancy with a dead fetus in utero 1.9 per cent. The gestational age at the time of the first fetal echocardiography ranged from 17 to 39 weeks (median 28 weeks). The number of fetal echocardiography done in each case ranged from 1 to 10 (median 1.4). Prenatal diagnosis of the abnormal cardiovascular system in fetuses was found in 19 cases (35.2%). There were abnormal cardiac anatomies in 42 per cent, cardiac tumors or abnormal masses in 37 per cent and rhythm disturbances in 21 per cent. When compared with postnatal echocardiography and/or autopsy finding, fetal echocardiography had a sensitivity of 100 per cent, specificity of 96.3 per cent and accuracy of 97.8 per cent. CONCLUSION: Fetal echocardiography has good accuracy in the diagnosis of cardiovascular diseases. Obstetrician's suspicion is important in the prenatal diagnosis of heart disease that would have affected the short and long-term outcome of the fetus.

Abortion, Therapeutic↗

The prevalence and incidence of medical conditions in healthy pharmaceutical company employees who volunteer to participate in medical research.

AIMS: Although clinical research in healthy volunteers is commonly performed there have been few studies of the value of the medical screening of subjects. The aim of this study was to investigate the prevalence and incidence of medical conditions found during the medical screening of 'healthy' subjects employed in a pharmaceutical company who volunteered to participate in medical research. METHODS: This was a retrospective study of the medical notes of all the subjects who volunteered for membership of the Zeneca Clinical Pharmacology Unit's healthy volunteer panel over a 4 year period from 1990 to 1994. The prevalence of medical conditions found at presentation was determined. The incidence of medical conditions during the 4 year observation period was also ascertained. Medical screening included a full medical history and examination, clinical chemistry, haematology and urinalysis screens, pulmonary function tests, ECGs, 24 h ambulatory cardiac monitoring and a request for information from the volunteer's General Practitioner. RESULTS: Prevalence-1293 subjects volunteered to join the panel of which 156 subjects (12%) were not accepted at presentation including 141 (10. 9%) for medical reasons. The most medical common reasons were; previously diagnosed medical conditions (3.3%), cardiovascular abnormalities (1.9%), abnormal liver function tests (1.9%), anaemia (1.2%), hyperlipidaemia (1.1%), excess alcohol intake (0.6%) and thyroid disease (0.5%). Incidence-36 of the 1137 volunteers (0.8% per year) accepted onto the panel subsequently developed medical conditions of which the most common were; anaemia (0.29% per year), cardiovascular abnormalities (0.13% per year) and vasovagal syncope (0.13% per year). CONCLUSIONS: This study demonstrates the importance of medical screening before healthy volunteers participate in clinical research.

Adult↗

Cardiovascular predictors of in-patient mortality after subarachnoid hemorrhage.

BACKGROUND AND PURPOSE: Whether cardiac dysfunction contributes to morbidity and mortality after subarachnoid hemorrhage (SAH) remains controversial. The objective of this study was to test the hypothesis that cardiovascular abnormalities are independently related to in-patient mortality after SAH. METHODS: This was a prospective cohort study of patients with aneurysmal SAH. Heart rate and blood pressure were measured, a blood sample was obtained, and echocardiography was performed on three study days, starting as soon after admission as possible. The cardiovascular predictor variables were heart rate, systolic blood pressure (SBP), cardiac troponin I (cTi) level, B-type natriuretic peptide (BNP) level, and left ventricular ejection fraction. The primary outcome measure was in-patient mortality. The association between each predictor variable and mortality was quantified by multivariate logistic regression, including relevant covariates and reporting odds ratios (OR) and 95% confidence intervals (CI). RESULTS: The study included 300 patients. An initial BNP level greater than 600 pg/mL was markedly associated with death (OR 37.7, p < 0.001). On the third study day (9.1 +/- 4.1 days after SAH symptom onset), a cTi level greater than 0.3 mg/L (OR 7.6, p = 0.002), a heart rate of 100 bpm or greater (OR 4.9, p = 0.009), and a SBP less than 130 mmHg (OR 6.7, p = 0.007) were significantly associated with death. CONCLUSIONS: Cardiovascular abnormalities are independent predictors of in-patient mortality after SAH. Though these effects may be explained by a reduction in cerebral perfusion pressure or other mechanisms, further research is required to determine whether or not they are causal in nature.

Adult↗

Cardiovascular consequences of clinical stroke.

In clinical stroke cardiovascular abnormalities are frequently neglected although they occur more often than it is generally assumed. However, cardiac arrhythmias, pathological ECG findings, and changes of circadian blood pressure patterns are significantly increased in patients with acute cerebrovascular lesions and are associated with an increased mortality. Several clinical studies have shown that cerebral infarctions may cause different cardiovascular abnormalities depending on the location and the size of the stroke. Hereby, the prolongation of the QT interval and the expansion of the QRS-complex as the most frequent ECG abnormalities are regarded as indicators of the electrical instability of the ventricular myocardium. Furthermore, cardiac enzyme increases are interpreted as an indicator of myocardial damage during the acute phase after cerebral ischaemia. Since the autonomic nervous system plays a major role in the regulation of blood pressure, alterations of sympatho-adrenergic activity can also affect the diurnal blood pressure profile. Some studies report frequent changes of the circadian blood pressure patterns with a decreased night-time blood pressure decline or a pathological night-time blood pressure elevation. Several studies proved the importance of infarct location. The insular cortex in particular has an important role in the genesis of the pathological activation of the sympathetic nervous system. Hence, a highly significant relationship between the extent of circadian blood pressure variation and percentage insular infarction could be found. Some findings implied that the mechanism of cardiovascular instability following stroke relates to the disinhibition of the insular cortex and a reacting augmentation of the sympathetic tone. A further important aspect is given by the strong evidence that sympathetic activation ] is lateralized following hemispheric brain infarction. Accordingly, patients with a right-sided hemispheric infarction showed a significantly diminished circadian blood pressure variation as compared with patients with left-sided hemispheric infarction. The results in patients with brain stem infarction were heterogeneous. On the one hand, patients with brain stem infarction had substantially higher mean plasma norepinephrine levels than did patients with hemispheric infarction; on the other hand, hemispheric lesions were associated with a significantly higher incidence of cardiac arrhythmias when compared to patients with brain stem infarction.

Animals↗

[Cardiological fitness examination of the athlete and prevention of sudden death].

Sudden death happening to an athlete is a frightening tragedy. Epidemiological data are quite controversial but sudden death occurring during sporting activity is probably not a rare occurrence. Beside drug abuse and accidental causes, cardiovascular abnormalities represent the principal origin of sudden death. Cardiomyopathy in young athletes and coronary artery disease in older athletes are the underlying pathologic substrates for most incidences. A certain proportion of sudden death in apparently healthy athletes was preceded by symptoms of exercise intolerance; pre participation evaluation directed to detect the cardiovascular abnormalities is probably of more value to reduce sudden cardiac death in athletes than detailed and routine sophisticated investigations.

Adolescent↗