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PubMed1997

Outcome of pregnancy in women with Marfan's syndrome.

OBJECTIVE: To improve life expectancy and prevent premature mortality in women with Marfan's syndrome. METHODS: During the development of a regional genetic register for Marfan's Syndrome the outcome of 91 pregnancies in 36 women with this condition was established retrospectively and the cardiovascular and obstetric complications documented. RESULTS: No patient had a significant cardiovascular abnormality limiting function before her pregnancy. Of 36 women, four had an aortic dissection relating to pregnancy and two others required aortic surgery following delivery. Thirty women had uncomplicated gestational histories. The incidence of obstetric complications did not exceed expectation. CONCLUSIONS: Women with Marfan's syndrome are at significant risk of aortic dissection in pregnancy even in the absence of preconceptional cardiovascular abnormality. Aortic root dilatation may be a predictor of risk but dissection may occur without significant dilatation. Guidelines for obstetric care are suggested and preconceptional assessment recommended.

Adult↗
PubMed1994

[Multiple heart valve involvement in renal polycystic disease in the adult].

Adult polycystic kidney disease is associated with frequent extrarenal manifestations. Previous studies have reported a increased incidence of cardiovascular abnormalities, in particular of mitral and tricuspid-valve prolapse, and multivalvular incompetence. These findings lends support to the hypothesis that autosomal dominant polycystic kidney disease is a disorder of connective tissue. A mitral-valve prolapse, and multivalvular incompetence was detected in a 75-year old patient with a polycystic kidney disease. The cardiovascular abnormalities published in the literature are review.

Aged↗
PubMed1981

Comparative features of newly acquired left and right bundle branch block in the general population: the Framingham study.

Cardiovascular abnormalities were prospectively identified in all 55 persons who acquired left bundle branch block and all 70 persons who acquired right bundle branch block during 18 years of follow-up of the Framingham Study cohort. Those with left and right bundle branch block did not differ from each other in the overall prevalence of either hypertension, clinical coronary disease or diabetes. In men, but not in women, left bundle branch block was associated with a significantly greater prevalence of cardiac enlargement and congestive failure than was right bundle branch block. A trend suggesting a higher mortality rate from cardiovascular disease in those with left than in those with right bundle branch block was more apparent in men than in women. It is concluded that in the general adult population, men who acquire left bundle branch block are more likely to have or subsequently acquire advanced cardiovascular abnormalities than are men who acquire right bundle branch block. In women, however, the clinical correlates of the two conduction abnormalities are similar.

Adult↗
PubMed2004

Older age is a risk factor for the development of cardiovascular sequelae in Kawasaki disease.

OBJECTIVES: To clarify the characteristics of Kawasaki disease (KD) in children 6 years and older and to determine whether age is a risk factor for cardiovascular abnormalities. METHODS: Patients who had KD and were reported between 1999 and 2000 in the 16th nationwide survey of KD in Japan (n = 15,314) were analyzed. Patients who were aged 6 years or older (older group) were matched with patients who were aged 6 months to 3 years and were treated at the same hospital (younger groups). The total number of analyzed patients was 1498 (749 matched pairs). RESULTS: The proportion of complete KD in the older group was similar to that in the younger group. Recurrent cases in the older group were significantly more common than those in the younger group (9% vs 2%). The proportion of patients who were treated with intravenous gamma-globulin in the older group was significantly lower than that in the younger group (82% vs 87%). The proportion of older group patients who were treated with intravenous gamma-globulin at or after 7 days of illness was significantly higher than that in the younger group (35% vs 14%). There was a higher prevalence of cardiovascular abnormalities in the older group than in the younger group (20% vs 15%). Multivariate logistic regression analysis showed that older age was an independent risk factor for cardiovascular sequelae (odds ratio: 1.58; 95% confidence interval: 1.01-2.46). CONCLUSIONS: In children older than 6 years, age is an independent risk factor for cardiovascular sequelae in KD.

Age Factors↗
PubMed1984

Comparative value of peripheral and central venous pCO2 in predicting normal paCO2 during anaesthesia.

The ability of venous pCO2 to predict arterial pCO2 within the normal range was tested by measuring pCO2 in blood sampled simultaneously from a large forearm vein (PER), from the superior vena cava (SVC), and from an artery in 35 anaesthetized patients. The relationship between arterial and both venous pCO2's were studied in a first series of 15 patients (ASA physical status class I-II) anaesthetized with methohexitone, fentanyl, pancuronium and nitrous oxide/oxygen, and in a second series of 20 patients scheduled for cardiac surgery anaesthetized with flunitrazepam, fentanyl, pancuronium and nitrous oxide. A marked correlation was found between arterial and both venous pCO2's samples in the normal patients (a/PER: r = 0.922; a/SVC: r = 0.940); in the patients with abnormal cardiovascular status the correlation observed was less pronounced (a/PER: r = 0.501; a/SVC: r = 0.507). In view of the similar correlation coefficients observed from the PER or SVC blood sampling sites, we conclude that the degree of accuracy of the prediction of paCO2 from the venous pCO2's is not modified by the origin of the venous blood. The differences between the coefficients of correlation found in the normal patients and in those with abnormal cardiovascular function indicate that venous pCO2 as estimate of paCO2 appears useful only in subjects with normal haemodynamic status.

Anesthesia, General↗
PubMed2003

Clinical impact of HIV-related lipodystrophy and metabolic abnormalities on cardiovascular disease.

Metabolic complications and altered fat distribution associated with HIV infection and antiretroviral therapy may lead to accelerated coronary artery disease (CAD). The high prevalence of multiple cardiovascular risk factors in a significant number of HIV patients is a cause for concern in both patients and physicians. Non-invasive strategies to measure subclinical CAD have been inconclusive. Long-term studies are underway to determine cardiac event rates, intervention strategies and consequences for the clinical management of HIV disease. In the present paper, we summarize the most prevalent risk factors in individuals with HIV infection receiving highly active antiretroviral therapy by focusing on the clinical implications of metabolic abnormalities and HIV-related lipodystrophy on CAD.

Adipose Tissue↗
PubMed1996

Hypertension, cardiac hypertrophy, and neurohumoral activity in a new animal model of obesity.

Although obesity is a major risk factor for morbidity and mortality, the mechanisms mediating cardiovascular abnormalities in response to weight gain are unclear. One reason for the paucity of information in this area is the lack of appropriate animal models for the study of human obesity. Therefore, the goal of the present study was to develop a small animal model of dietary-induced obesity that mimics many of the characteristics of human obesity. We studied female New Zealand White rabbits fed either a normal (n = 17) or high-fat diet (n = 15) and examined the cardiovascular consequences of obesity, including changes in blood pressure, humoral activation, and end-organ effects such as cardiac hypertrophy. After 12 wk, rabbits on the high-fat diet were 46% heavier than their lean counterparts (5.49 +/- 0.09 vs. 3.77 +/- 0.06 kg, respectively; P = 0.0001). Obese rabbits had higher resting heart rates than lean rabbits (220 +/- 7 vs. 177 +/- 6 beats/min; P = 0.0001) and developed hypertension (96 +/- 2 vs. 85 +/- 1 mmHg; P = 0.0001), hyperinsulinemia (32.5 +/- 3.4 vs. 15.5 +/- 1.0 microU/ml; P = 0.0001), hyperglycemia (162.4 +/- 2.9 vs. 141.9 +/- 2.7 mg/dl; P = 0.0001), and elevated triglycerides (102.3 +/- 9.1 vs. 48.5 +/- 4.0 mg/dl; P = 0.0001). Obese rabbits also developed cardiac hypertrophy, as evidenced by left ventricular (LV) dry weights that were 52% greater in obese than in lean rabbits (P = 0.0003). In addition, LV total protein was increased in proportion to the increase in LV weight. The results of this study suggest that rabbits fed a high-fat diet for a period of 12 wk develop many of the characteristics of human obesity. The obese rabbit should provide a small and relatively inexpensive animal model to investigate mechanisms of obesity-related cardiovascular abnormalities.

Animals↗
PubMed1995

[Abnormal autonomic cardiovascular responses in patients with sickle cell anemia].

PURPOSE: To evaluate the presence of anomalies of the autonomic reflex cardiovascular response in patients with chronic sickle-cell anaemia. PATIENTS AND METHODS: The study was extended to 30 patients with sickle-cell anaemia, 10 patients with iron-lack anaemia and 30 healthy subjects. Age and sex distribution was similar in each group. To be included in the study, patients should have had no painful crisis or blood transfusion in the 6 months previous to the assay. Clinico-laboratory survey, chest x-ray and EKG were performed in every case. Blood cell count and abnormal haemoglobin study on cellulose acetate were carried out as well. The evaluation of reflex autonomic responses was performed by means of active orthostatism, cold pressor test, Valsalva maneuver and urine catecholamine output. The statistical analysis was performed with the variance analysis (ANOVA) for multiple groups. RESULTS: The following abnormalities were found: 12 patients had haemoglobin SS, 8 had haemoglobin SS and F, 3 had haemoglobin SC, 2 had haemoglobin S and beta-thalassaemia, and 5 had combined haemoglobin SS,F and A2. Systolic pressure and heart frequency in the supine position were similar in all groups. Diastolic pressure was lower in the sickle-cell anaemia group with respect to the normals. Patients with sickle-cell disease had lower heart frequency in the active orthostatism test with regard to the other groups, along with paradoxal changes in systolic pressure and lesser increase of the diastolic pressure. Significantly lower response to the cold pressor test was seen in the sickle-cell patients as compared with the iron-lack cases and the normal controls. Reduced sympathetic tachycardia was seen with the Valsalva maneuver, whereas the bradycardia was similar to the other groups. The urine noradrenaline in output was significantly lower in the sickle-cell patients, it was normal in the other groups (p < 0.01). CONCLUSION: These results suggest a defective sympathetic activity of heart and arteries in patients with sickle-cell anaemia.

Adolescent↗
PubMed1996

Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis.

OBJECTIVE: To assess cardiovascular abnormalities in patients with limited systemic sclerosis (SSc), using noninvasive cardiac techniques. METHODS: Sixty-three patients with limited SSc were prospectively evaluated with Doppler echocardiography and thallium-201 perfusion scintigraphy after a cold-stress test and radionuclide ventriculography. RESULTS: In the patients with limited SSc, there was a significantly high prevalence of abnormal left- and right-diastolic function parameters (P = 0.001 and P = 0.0002, respectively), thickening of papillary muscles (46%; P = 0.003), and mild mitral regurgitation (49%; P < 0.0001), compared with controls. Systolic pulmonary arterial hypertension was detected in 9 patients (14%), and pericardial effusion in 11 patients (18%). In 64% of patients with limited SSc, an ischemic response was detected on the thallium cold-stress scan; similarly, an ischemic response was detected in 57% of patients with primary Raynaud's phenomenon (P < 0.0001 versus controls). CONCLUSION: Although the frequency of cardiovascular symptoms was low in patients with limited SSc, a significant rate of cardiovascular abnormalities was found by noninvasive cardiac techniques.

Adult↗
PubMed1996

[Cerebrovascular complications of Takayasu's disease].

We report four young women with Takayasu's arteritis who developed an ischemic stroke. In three of these patients, this complication heralded the onset of the disease. Three patients also had seizures. In all cases, the diagnosis was suspected on the basis of an abnormal cardiovascular examination showing absent carotid or radial pulses, carotid bruits or significant asymmetries in blood pressure determinations. The aortogram showed that three patients had Takayasu's arteritis type I, with affection confined to the aortic arch and supra-aortic trunks, and the other patient had Takayasu's arteritis type III with additional involvement of the descending aorta. Three patients had an elevated erythrocyte sedimentation rate and were treated with corticosteroids. Takayasu's arteritis should be suspected in young patients, especially women, presenting with a cerebral infarct or seizures who also have an abnormal cardiovascular examination. The diagnosis is usually confirmed by aortography, following Ishikawa's diagnostic criteria. Corticosteroid therapy may be of value in patients with an elevated erythrocyte sedimentation rate.

Adolescent↗
PubMed2004

Cardiovascular status in long-term survivors of Hodgkin's disease treated with chest radiotherapy.

PURPOSE: Cardiovascular status was assessed in 48 Hodgkin's disease (HD) survivors at a median of 14.3 years (range, 5.9 to 27.5 years) after diagnosis because they may be at increased risk for cardiovascular abnormalities. PATIENTS AND METHODS: Patients completed the Short-Form 36 quality-of-life instrument and were screened by echocardiography, exercise stress testing, and resting and 24-hour ECG. RESULTS: All patients received mediastinal irradiation (median, 40.0 Gy; range, 27.0 to 51.7 Gy) at a median age of 16.5 years (range, 6.4 to 25.0 years). Four patients received an anthracycline. Although every patient described their health as good or better, and none had symptomatic heart disease at screening, all but one had cardiac abnormalities on screening. Restrictive cardiomyopathy was suggested by reduced average left ventricular (LV) dimension (P < .001) and mass (P < .001), without increased LV wall thickness. Significant valvular defects were present in 42%; 75% had conduction defects. One survivor developed complete heart block shortly after the study visit. Autonomic dysfunction was suggested by a monotonous heart rate in 57%, persistent tachycardia in 31%, and blunted hemodynamic responses to exercise in 27%. Peak oxygen uptake (VO2max) during exercise, a predictor of mortality in heart failure, was significantly reduced (< 20 mL/kg/m2) in 30% of survivors. VO2max was correlated with increasing fatigue, increasing shortness of breath (both, r = -0.35; P =. 02), and decreasing physical component score on the SF-36 (r = 0.554; P = .00017). CONCLUSION: A variety of unsuspected, clinically significant cardiovascular abnormalities are common in long-term survivors of HD who are treated at a young age with mediastinal irradiation. We recommend serial, comprehensive cardiac screening of HD survivors who fit this profile.

Adolescent↗
PubMed2006

Cardiac and vascular changes in cirrhosis: pathogenic mechanisms.

Cardiovascular abnormalities accompany both portal hypertension and cirrhosis. These consist of hyperdynamic circulation, defined as reduced mean arterial pressure and systemic vascular resistance, and increased cardiac output. Despite the baseline increased cardiac output, ventricular inotropic and chronotropic responses to stimuli are blunted, a condition known as cirrhotic cardiomyopathy. Both conditions may play an initiating or aggravating pathogenic role in many of the complications of liver failure or portal hypertension including ascites, variceal bleeding, hepatorenal syndrome and increased postoperative mortality after major surgery or liver transplantation. This review briefly examines the major mechanisms that may underlie these cardiovascular abnormalities, concentrating on nitric oxide, endogenous cannabinoids, central neural activation and adrenergic receptor changes. Future work should address the complex interrelationships between these systems.

Blood Circulation↗
PubMed1987

Chest pain, dyspnea on exertion, and exercise induced asthma in children and adolescents.

UNLABELLED: The contribution of maximal exercise tests to the evaluation of 180 patients with chest pain associated with exercise (n = 147) or dyspnea on exertion (DOE, n = 33) was examined. The ages ranged from 5 to 22 (mean 13.2) years, and 68 patients were females. All patients had a normal cardiovascular examination, electrocardiogram, chest x-ray, and 2D-echocardiogram. Maximal exercise tests were performed on a treadmill or bicycle ergometer, and flow volume loops were performed before and after exercise (n = 65). Exercise tests did not reveal any cardiovascular abnormalities, but 14 patients with chest pain (9.5%) and seven patients with DOE (21.2%) developed exercise-induced asthma. Postexercise decrease in peak expiratory flow rate was 26.2 +/- 3.7 percent in patients with chest pain and 39.4 +/- 8.9 percent in those with DOE. Only five patients had a personal history and four others had a family history of asthma. Seven patients had a personal or family history of allergies. IMPLICATIONS: exercise-induced asthma should be considered in pediatric patients with symptoms of chest pain or dyspnea on exertion; when exercise tests are performed, flow volume loops should be included before and after exercise; maximal exercise tests are unlikely to unmask any cardiovascular abnormalities in such patients.

Adolescent↗
PubMed2004

[Significance of the cardiovascular function tests in patients with collagen diseases, especially for SSc (systemic scleroderma)].

The purpose of this study is to evaluate the cardiovascular function in patients with incomplete type of SSc (SSSD; Scleroderma Sjögren syndrome associated Spectrum Disorders), SSc (Systemic Scleroderma) and SLE (Systemic Lupus Erythematosus) by using ECG and digital plethysmograph. We also preformed ambulatory ECG monitoring for cases of SSSD and SSc with QT interval prolongation. 1) In ECG findings, ischemic myocardial damage, left ventricular hypertrophy or QT interval prolongation was observed in 33% cases of SSSD and 32% cases of SSc, respectively. In addition, parasympathetic disorders were observed in approximately 20% cases of SSSD, SSc and SLE. 2) Digital plethysmogram findings suggested that the peripheral vascular damage was present in both cases of SSSD and SSc, and its severity was lower in SSSD than SSc. The incidence of abnormal cardiovascular function tests in SSSD was relatively less than that in SSc. The cardiovascular function tests are useful to find cardiovascular abnormalities in these collagen diseases.

Adult↗
PubMed1983

Cardiovascular manifestations of mixed connective tissue disease in adults.

To assess the nature and distribution of cardiovascular abnormalities associated with mixed connective tissue disease, we studied 38 patients with overlapping clinical manifestations of systemic lupus erythematosus, progressive systemic sclerosis and polymyositis, and circulating antibodies to nuclear ribonucleoprotein. The protocol included taking a medical history and a physical echocardiogram, and pulmonary function tests. Cardiac catheterization was performed on 17 patients. Postmortem examination was performed on four of the five patients who died during follow-up. Acute pericarditis and/or pericardial effusion was detected in 11 patients (29%) and mitral valve prolapse was identified in 10 patients (26%). Marked intimal hyperplasia of coronary arteries was observed in all four hearts that were autopsied and perivascular and myocardial leukocytic aggregates were present in two hearts. Pulmonary vascular resistance was elevated in 11 of the 17 patients who underwent cardiac catheterization. In summary, cardiovascular abnormalities associated with mixed connective tissue disease include acute pericarditis and/or effusion, mitral valve prolapse, intimal hyperplasia of coronary arteries, perivascular and myocardial leukocytic infiltrates, and pulmonary hypertension.

Adolescent↗
PubMed1994

[Abnormalities of cardiovascular baroreflex control in renal failure and cardiac insufficiency. Experimental study and brief critical review of the literature].

We attempted to determine the relevance and relative prevalence of autonomic dysfunction in 7 patients with end-stage renal disease (ESRD) but no heart disease, 8 patients with left ventricular dysfunction (LVD) with no renal disease and 8 patients with ESRD + LVD. In each group, the assessment of autonomic function and location of the lesion was performed by using the baroreflex sensitivity tests, based upon heart rate (ie Valsalva, deep breathing, lying to standing) to study the parasympathetic system as well as based upon blood arterial pressure (ie standing and sustained handgrip) to evaluate the sympathetic system. The same tests were performed in 7 healthy volunteers enrolled as control group. Separate studies were performed on the efferent parasympathetic arc (atropine test) and the efferent sympathetic arc (cold pressor test). There was evidence of parasympathetic damage in 100% of ESRD patients, in 87.5% of ESRD + LVD patients, and in 62.5% of LVD. Only 1 of ESRD patients showed defective response to atropine whereas the remaining had a normal response, suggesting an afferent lesion alone. No abnormalities were found in the response to cold pressor test, thereby suggesting a lack of involvement of the efferent sympathetic arc. The response to handgrip was higher in LVD patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed2004

Population screening of fetal cardiac abnormalities through prenatal echocardiography in low-risk pregnancies in the municipality of Porto Alegre.

OBJECTIVE: To study the morphological and functional abnormalities of the fetal cardiovascular system detectable through ultrasonography. METHODS: The study comprised 3,980 fetuses of pregnant women with no obstetrical or cardiological risk in the municipality of Porto Alegre, who, from July 1996 to November 2000, underwent echocardiography for screening fetal cardiovascular malformations. The examinations consisted of 4-chamber and right and left ventricular outflow tract echocardiographic views. RESULTS: One hundred and three diagnoses of fetal cardiovascular abnormalities were established, corresponding to 2.58% (103/3,980) of the population studied, or 25.8/1000, of which 47 represented morphological and functional changes in the fetal heart with an overall prevalence of 11.8/1000 (47/3,980), and 56 showed changes in refringence (golf ball). Three false negative and no false positive results increased the overall prevalence to 12.5/1000 (50/3,980), or 26.6/1000 (106/3,980), when the refringence changes were also included. CONCLUSION: The detection of cardiovascular malformations still in the intrauterine period, aiming at perinatal planning, is possible, which confirms the international experience. Implantation of cardiovascular malformation screening into the routine care network of obstetrical ultrasonography is suggested.

Adolescent↗
PubMed2006

HRAS mutation analysis in Costello syndrome: genotype and phenotype correlation.

Costello syndrome is a rare condition comprising mental retardation, distinctive facial appearance, cardiovascular abnormalities (typically pulmonic stenosis, hypertrophic cardiomyopathy, and/or atrial tachycardia), tumor predisposition, and skin and musculoskeletal abnormalities. Recently mutations in HRAS were identified in 12 Japanese and Italian patients with clinical information available on 7 of the Japanese patients. To expand the molecular delineation of Costello syndrome, we performed mutation analysis in 34 North American and 6 European (total 40) patients with Costello syndrome, and detected missense mutations in HRAS in 33 (82.5%) patients. All mutations affected either codon 12 or 13 of the protein product, with G12S occurring in 30 (90.9%) patients of the mutation-positive cases. In two patients, we found a mutation resulting in an alanine substitution in position 12 (G12A), and in one patient, we detected a novel mutation (G13C). Five different HRAS mutations have now been reported in Costello syndrome, however genotype-phenotype correlation remains incomplete.

Abnormalities, Multiple↗