Safety and feasibility of a functional electrical stimulation cycling-based muscular dysfunction diagnostic method in mechanically ventilated patients.

BACKGROUND: A nonvolitional diagnostic method based on FES-Cycling technology has recently been demonstrated for mechanically ventilated patients. This method presents good sensitivity and specificity for detecting muscle dysfunction and survival prognosis, even in unconscious patients. As the clinical relevance of this method has already been reported, we aimed to evaluate its safety and feasibility. METHODS: An observational prospective study was carried out with 20 critically ill, mechanically ventilated patients. The FES-cycling equipment was set in a specific diagnostic mode. For safety determination, hemodynamic parameters and peripheral oxygen saturation were measured before and immediately after the diagnostic protocol, as well as venous oxygen saturation and blood lactate. The creatine phosphokinase level (CPK) was measured before and 24, 48, and 72 h after the test. The time taken to carry out the entire diagnostic protocol and the number of patients with visible muscle contraction (capacity of perceptive muscular recruitment) were recorded to assess feasibility. RESULTS: Heart rate [91 ± 23 vs. 94 ± 23 bpm (p = 0.0837)], systolic [122 ± 19 vs. 124 ± 19 mm Hg (p = 0.4261)] and diastolic blood pressure [68 ± 13 vs. 70 ± 15 mm Hg (p = 0.3462)], and peripheral [98 (96-99) vs. 98 (95-99) % (p = 0.6353)] and venous oxygen saturation [71 ± 14 vs. 69 ± 14% (p = 0.1317)] did not change after the diagnostic protocol. Moreover, blood lactate [1.48 ± 0.65 vs. 1.53 ± 0.71 mmol/L (p = 0.2320)] did not change. CPK did not change up to 72 h after the test [99 (59-422) vs. 125 (66-674) (p = 0.2799) vs. 161 (66-352) (p > 0.999) vs. 100 (33-409) (p = 0.5901)]. The time taken to perform the diagnostic assessment was 11.3 ± 1.1 min. In addition, 75% of the patients presented very visible muscle contractions, and 25% of them presented barely visible muscle contractions. CONCLUSIONS: The FES cycling-based muscular dysfunction diagnostic method is safe and feasible. Hemodynamic parameters, peripheral oxygen saturation, venous oxygen saturation, and blood lactate did not change after the diagnostic protocol. The muscle damage marker (CPK) did not increase up to 72 h after the diagnostic protocol.

Effectiveness of chlorthalidone/amiloride versus losartan in patients with stage I hypertension and diabetes mellitus: results from the PREVER-treatment randomized controlled trial.

AIMS: To compare the blood pressure (BP)-lowering efficacy of a chlorthalidone/amiloride combination pill with losartan, during initial management of JNC 7 Stage I hypertension in patients with type 2 diabetes mellitus. METHODS: In an a priori subgroup analysis of a randomized, double-blind, controlled trial, volunteers aged 30-70 years, with stage I hypertension and diabetes mellitus, were randomized to 12.5/2.5 mg of chlorthalidone/amiloride (N = 47) or 50 mg of losartan (N = 50), and followed for 18 months in 21 clinical centers. If BP remained uncontrolled after three months, study medication dose was doubled, and if uncontrolled after six months, amlodipine (5 and 10 mg) and propranolol (40 and 80 mg BID) were added as open label drugs in a progressive fashion. RESULTS: Systolic BP decreased to a greater extent in participants allocated to diuretics compared to losartan (P < 0.001). After 18 months of follow-up, systolic BP was 128.4 ± 10.3 mmHg in the diuretic group versus 133.5 ± 8.0 in the losartan group (P < 0.01). In the diuretic group, 36 out of 43 participants (83.7%) had a JNC 7 normal BP, compared to 31/47 (66%) in the losartan group (P = 0.089). Serum cholesterol was higher in the diuretic arm at the end of the trial. Other biochemical parameters and reports of adverse events did not differ by treatment. CONCLUSIONS: Treatment of hypertension based on a combination of chlorthalidone and amiloride is more effective for BP lowering compared to losartan in patients with diabetes mellitus and hypertension. TRIAL REGISTRATION: Clinical trials registration number: NCT00971165.

Schistosomiasis-associated pulmonary arterial hypertension: survival in endemic area in Brazil.

BACKGROUND: The survival of schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) patients in endemic areas is unknown, but can be estimated using predictive equations. METHODS: We retrospectively analyzed all consecutive patients diagnosed with Sch-PAH referred to the Pronto SocorroCardiologico de Pernambuco between 2004 and 2010 using specific therapy and measured laboratory, diagnostic imaging, and baseline hemodynamic parameters. Observed and predicted survivals according to the National Institutes of Health (NIH) and Pulmonary Hypertension Connection (PHC) registry equations were compared by the Kaplan-Meier method, log-rank test and Cox proportional hazards model. RESULTS: Sixty-eight patients (47 [69.1%] women) observed for a mean of 3.1 years (range, 7-72 months), median survival was 74 months, and 42 (61.7%) survived. The sex and age distributions were similar for functional class I/II and III/IV patients. Hemodynamic abnormalities were severe: mean right atrial pressure, 12.6 ± 6.2 mmHg; mean pulmonary artery pressure, 60.3 ± 13.69 mmHg; pulmonary vascular resistance, 14.62 ± 7.04 Wood units; and cardiac index, 2.3 ± 0.8 L/min/m2. The usual idiopathic PAH predictors were not prognostic in Sch-PAH patients. The 1-, 3- and 5-year survival rates were 92.1%, 75.2%, and 50.8%, respectively, and those estimatedby the NIH and PHC registry equations were 68%, 45% and 32% (p = 0.001), and 93%, 79% and 68% (p = 0.340), respectively. CONCLUSIONS: Sch-PAH patients in endemic areas have severe hemodynamic profiles and reduced long-term survivaldespite treatment. The PHC registry equation may be a useful tool to estimate survival in Sch-PAH.

Effects of Resistance Training on Cardiovascular Function in Patients With Peripheral Artery Disease: A Randomized Controlled Trial.

Gomes, APF, Correia, MA, Soares, AHG, Cucato, GG, Lima, AHRA, Cavalcante, BR, Sobral-Filho, DC, and Ritti-Dias, RM. Effects of resistance training on cardiovascular function in patients with peripheral artery disease: A randomized controlled trial. J Strength Cond Res 32(4): 1072-1080, 2018-The aim of this study was to analyze the effects of resistance training on cardiovascular function of patients with peripheral artery disease (PAD). In total, 30 patients with PAD were invited to participate in this randomized controlled trial, randomly allocated to a control (n = 15, 66 ± 2 years; stretching and relaxation exercises) or resistance training group (n = 15, 60 ± 3 years; 3 sets of 10 repetitions of 8 whole-body exercises, with a 2-minute interval between sets). Resting and 24-hour blood pressure (BP), cardiac output, systemic vascular resistance, and autonomic variables were obtained before and after 12 weeks of intervention. A blinded investigator performed all analyses. After 12 weeks of intervention there was maintenance of resting systolic, diastolic, and mean BP (p > 0.18), cardiac output (p = 0.46), and systemic vascular resistance (p = 0.55) in both groups. There was a time effect reduction in heart rate (p = 0.02), accompanied by changes in cardiac autonomic modulation (p = 0.03). There were no changes in 24-hour systolic, diastolic, and mean BP, heart rate, or rate pressure product (p > 0.05). The BP variability decreased in systolic (asleep, p = 0.003), diastolic (24 hours and awake, p = 0.001), and mean (24 hours and asleep, p < 0.02) only in the resistance training (RT) group. Twelve weeks of RT did not change resting and 24-hour BP, or their hemodynamic and autonomic determinants in patients with PAD; however, there were decreases in BP variability, indicating that it could be considered as an alternative to reducing cardiovascular risk in patients with PAD.

Carbohydrate antigen 125: a promising tool for risk stratification in heart diseases.

All patients with heart diseases should undergo risk stratification to predict those who are at high risk for short- and long-term adverse outcomes. Carbohydrate antigen 125 (CA125) is a glycoprotein produced by mesothelium that has clinical role in ovarian cancer monitoring. However, as it is not specific for ovarian cells, CA125 could also be used in heart diseases to monitor congestion and inflammation. Pericarditis, atrial fibrillation, heart failure and coronary artery disease are some scenarios in which this biomarker was studied.CA125 identifies patients at high risk of rehospitalizations and death, in addition to being associated with hemodynamic data (ejection fraction and right atrial pressure). Hence, CA125 is a tool for risk stratification in heart diseases.

Effectiveness of Chlorthalidone Plus Amiloride for the Prevention of Hypertension: The PREVER-Prevention Randomized Clinical Trial.

BACKGROUND: Prehypertension is associated with higher cardiovascular risk, target organ damage, and incidence of hypertension. The Prevention of Hypertension in Patients with PreHypertension (PREVER-Prevention) trial aimed to evaluate the efficacy and safety of a low-dose diuretic for the prevention of hypertension and end-organ damage. METHODS AND RESULTS: This randomized, parallel, double-blind, placebo-controlled trial was conducted in 21 Brazilian academic medical centers. Participants with prehypertension who were aged 30 to 70 years and who did not reach optimal blood pressure after 3 months of lifestyle intervention were randomized to a chlorthalidone/amiloride combination pill or placebo and were evaluated every 3 months during 18 months of treatment. The primary outcome was incidence of hypertension. Development or worsening of microalbuminuria, new-onset diabetes mellitus, and reduction of left ventricular mass were secondary outcomes. Participant characteristics were evenly distributed by trial arms. The incidence of hypertension was significantly lower in 372 study participants allocated to diuretics compared with 358 allocated to placebo (hazard ratio 0.56, 95% CI 0.38-0.82), resulting in a cumulative incidence of 11.7% in the diuretic arm versus 19.5% in the placebo arm (P=0.004). Adverse events; levels of blood glucose, glycosylated hemoglobin, creatinine, and microalbuminuria; and incidence of diabetes mellitus were no different between the 2 arms. Left ventricular mass assessed through Sokolow-Lyon voltage and voltage-duration product decreased to a greater extent in participants allocated to diuretic therapy compared with placebo (P=0.02). CONCLUSIONS: A combination of low-dose chlorthalidone and amiloride effectively reduces the risk of incident hypertension and beneficially affects left ventricular mass in patients with prehypertension. CLINICAL TRIAL REGISTRATION: URL: http://www.ClinicalTrials.gov, www.ensaiosclinicos.gov. Unique identifiers: NCT00970931, RBR-74rr6s.

Recommendations of the Brazilian Society of Cardiac Arrhythmias for holter monitoring services.

BACKGROUND: There are innumerous indicators to assure the quality of a service. However, medical competence and the proper performance of a procedure determine its final quality. The Brazilian Society of Cardiac Arrhythmias recommends minimum parameters necessary to guarantee the excellence of ambulatory electrocardiographic monitoring services. OBJECTIVE: To recommend minimum medical competences and the information required to issue a Holter monitoring report. METHODS: This study was grounded in the concept of evidence-based medicine and, when evidence was not available, the opinion of a writing committee was used to formulate the recommendation. That committee consisted of professionals with experience on the difficulties of the method and management in providing services in that area. RESULTS: The professional responsible for the Holter monitoring analysis should know cardiovascular pathologies and have consistent formation on electrocardiography, including cardiac arrhythmias and their differential diagnoses. The report should be written in a clear and objective way. The minimum parameters that comprise a Holter report should include statistics of the exam, as well as quantification and analysis of the rhythm disorders observed during monitoring. CONCLUSION: Ambulatory electrocardiographic monitoring should be performed by professionals knowledgeable about electrocardiographic analysis, whose report should comprise the minimum parameters mentioned in this document.

Relação da pressão sistólica em artéria pulmonar com gênero, menopausa e gravidez em portadores de hipertensão arterial pulmonar associada à esquistossomose / Pulmonary artery pressure, gender, menopause, and pregnancy in schistosomiasis-associated pulmonary hypertension

FUNDAMENTO: A hipertensão arterial pulmonar associada à esquistossomose (HPAE) é uma grande preocupação no mundo todo. No entanto, o papel de fatores contribuintes específicos do gênero em HPAE é desconhecido. OBJETIVO: Investigamos os valores da pressão arterial pulmonar sistólica (PAPS) e a presença de elevação grave na PAPS relacionado ao gênero, presença de menopausa e histórico de gravidez em pacientes com HPAE. MÉTODOS: Setenta e nove pacientes diagnosticados com HPAE de 2000 a 2009 foram avaliados e 66 foram incluídos no estudo. As informações referentes à idade, status da menopausa, gravidez, PAPS derivada da ecocardiografia, e pressão arterial pulmonar média invasiva (PAPm) foram coletadas de registros médicos. A relação entre os valores de PAPS e PAPm e a correlação para doença grave foram avaliados. Os modelos de regressão avaliaram a associação de gênero, status da menopausa e histórico de gravidez com valores de PAPS e a presença de PAPS severa. RESULTADOS: Houve correlação moderada entre PAPm e PAPS, com boa concordância para classificação de doença grave. Os valores de PAPS foram semelhantes para homens e mulheres. Uma tendência a valores maiores de PAPS foi encontrada para mulheres não menopausadas em comparação a homens. Valores superiores de PAPS foram encontrados para mulheres menopausadas em comparação a mulheres não menopausadas; os valores não foram significativos após o ajuste de idade. O histórico de gravidez não teve relação com a PAPS. Presença de menopausa e passado de gravidez não mostraram associação com valores de PAPS. CONCLUSÃO: Em pacientes com HPAE, nem o gênero, nem o status da menopausa nem o histórico de gravidez apresentou uma correlação independente com valores de HPAE avaliados pela ecocardiografia.

BACKGROUND: Schistosomiasis-associated pulmonary arterial hypertension (SPAH) is a major concern worldwide. However, the role of gender-specific contributing factors in SPAH is unknown. OBJECTIVE: We investigated how systolic pulmonary artery pressure (SPAP) values and the presence of severe SPAP relate to gender, menopausal status, and pregnancy history in SPAH patients. METHODS: Seventy-nine patients diagnosed with SPAH from 2000 to 2009 were assessed and 66 were enrolled in the study. Information about age, menopausal status, pregnancy, echocardiography-derived SPAP, and invasive mean pulmonary artery pressure (mPAP) was collected from medical records. The relation between values of SPAP and mPAP and their agreement for severe disease were assessed. Regression models assessed the association of gender, menopausal status, and pregnancy history with SPAP values and the presence of severe SPAP. RESULTS: Moderate correlation and good agreement for severe disease were found between mPAP and SPAP. Mean SPAP values were similar for men and women. A trend toward higher values of SPAP was found for non-menopausal women compared to men. Higher SPAP values were found for menopausal compared to non-menopausal women; the values were non-significant after adjustment for age. Pregnancy history had no association with SPAP. Menopause and positive pregnancy had no association with severe SPAP. CONCLUSION: In SPAH patients, neither gender, nor menopausal status, nor pregnancy history showed independent correlation with SPAP values assessed by echocardiography.

Recomendações da sociedade brasileira de arritmias cardíacas para serviços de holter / Recommendations of the Brazilian society of cardiac arrhythmias for holter monitoring services

FUNDAMENTOS: Inúmeros indicadores são utilizados para assegurar a qualidade de um serviço; entretanto, a competência médica e o adequado fluxo de realização de um procedimento são determinantes da qualidade final. Nesse contexto, a Sociedade Brasileira de Arritmias Cardíacas pretende recomendar parâmetros mínimos necessários para garantir a excelência dos serviços de monitorização eletrocardiográfica ambulatorial. OBJETIVOS: Recomendar competências médicas mínimas e as informações necessárias para emissão do laudo de Holter. MÉTODOS: O documento foi fundamentado no conceito de medicina baseada em evidência, e nas circunstâncias em que a evidência não estava disponível a opinião de uma comissão de redação foi utilizada para a formulação da recomendação. Essa comissão foi formada por profissionais que apresentam vivência nas dificuldades do método e gestão na prestação de serviços nessa área. RESULTADOS: O profissional responsável pela análise de Holter deve conhecer as patologias cardiovasculares e ter formação consistente em eletrocardiografia, incluindo arritmias cardíacas e seus diagnósticos diferenciais. O laudo deve ser redigido de forma clara e objetiva. Os parâmetros mínimos que devem constar no laudo incluem as estatísticas do exame, assim como quantificação e análise dos distúrbios do ritmo observados durante a monitorização. CONCLUSÃO: A monitorização eletrocardiográfica ambulatorial deve ser realizada por profissionais com vivência em análise eletrocardiográfica e o laudo deve conter os parâmetros mínimos mencionados nesse documento.

BACKGROUND: There are innumerous indicators to assure the quality of a service. However, medical competence and the proper performance of a procedure determine its final quality. The Brazilian Society of Cardiac Arrhythmias recommends minimum parameters necessary to guarantee the excellence of ambulatory electrocardiographic monitoring services. OBJECTIVE: To recommend minimum medical competences and the information required to issue a Holter monitoring report. METHODS: This study was grounded in the concept of evidence-based medicine and, when evidence was not available, the opinion of a writing committee was used to formulate the recommendation. That committee consisted of professionals with experience on the difficulties of the method and management in providing services in that area. RESULTS: The professional responsible for the Holter monitoring analysis should know cardiovascular pathologies and have consistent formation on electrocardiography, including cardiac arrhythmias and their differential diagnoses. The report should be written in a clear and objective way. The minimum parameters that comprise a Holter report should include statistics of the exam, as well as quantification and analysis of the rhythm disorders observed during monitoring. CONCLUSION: Ambulatory electrocardiographic monitoring should be performed by professionals knowledgeable about electrocardiographic analysis, whose report should comprise the minimum parameters mentioned in this document.

Pulmonary artery pressure, gender, menopause, and pregnancy in schistosomiasis-associated pulmonary hypertension.

BACKGROUND: Schistosomiasis-associated pulmonary arterial hypertension (SPAH) is a major concern worldwide. However, the role of gender-specific contributing factors in SPAH is unknown. OBJECTIVE: We investigated how systolic pulmonary artery pressure (SPAP) values and the presence of severe SPAP relate to gender, menopausal status, and pregnancy history in SPAH patients. METHODS: Seventy-nine patients diagnosed with SPAH from 2000 to 2009 were assessed and 66 were enrolled in the study. Information about age, menopausal status, pregnancy, echocardiography-derived SPAP, and invasive mean pulmonary artery pressure (mPAP) was collected from medical records. The relation between values of SPAP and mPAP and their agreement for severe disease were assessed. Regression models assessed the association of gender, menopausal status, and pregnancy history with SPAP values and the presence of severe SPAP. RESULTS: Moderate correlation and good agreement for severe disease were found between mPAP and SPAP. Mean SPAP values were similar for men and women. A trend toward higher values of SPAP was found for non-menopausal women compared to men. Higher SPAP values were found for menopausal compared to non-menopausal women; the values were non-significant after adjustment for age. Pregnancy history had no association with SPAP. Menopause and positive pregnancy had no association with severe SPAP. CONCLUSION: In SPAH patients, neither gender, nor menopausal status, nor pregnancy history showed independent correlation with SPAP values assessed by echocardiography.

Low-intensity resistance exercise does not affect cardiac autonomic modulation in patients with peripheral artery disease.

OBJECTIVE: To analyze the effect of a single bout of resistance exercise on cardiac autonomic modulation in patients with peripheral artery disease. METHODS: Fifteen patients with peripheral artery disease (age: 58.3±4.0 years) underwent the following sessions in a random order: resistance exercise (three sets of 10 repetitions of the six resistance exercises with a workload of 5-7 in the OMNI-RES scale) and control (similar to the resistance session; however, the resistance exercises were performed with no load). The frequency domain (low frequency, high frequency and sympathovagal balance) and symbolic analysis (0V, 1V and 2V patterns) of heart rate variability were obtained before and until one hour after the interventions. RESULTS: After the resistance exercise and control sessions, similar increases were observed in the consecutive heartbeat intervals (control: 720.8±28.6 vs. 790.9±34.4 ms; resistance exercise: 712.9±30.1 vs. 756.8±37.9 ms; p<0.05) and in the pattern of the symbolic analysis with no variation (0V) (control: 25.1±3.5 vs. 33.4±4.1%; resistance exercise: 26.1±3.2 vs. 29.7±3.5%; p<0.05) until 50 min after both interventions. The pattern of two variations (2V) decreased similarly (control: 11.2±2.1 vs. 8.3±2.1%; resistance exercise: 9.5±1.7 vs. 7.8±1.7%; p<0.05). In contrast, the pattern of one variation (1V), the low and high frequency bands and sympathovagal balance did not change after the interventions (p>0.05). CONCLUSION: A single bout of resistance exercise did not alter cardiac autonomic modulation in patients with peripheral artery disease.

Low-intensity resistance exercise does not affect cardiac autonomic modulation in patients with peripheral artery disease

OBJECTIVE: To analyze the effect of a single bout of resistance exercise on cardiac autonomic modulation in patients with peripheral artery disease. METHODS: Fifteen patients with peripheral artery disease (age: 58.3±4.0 years) underwent the following sessions in a random order: resistance exercise (three sets of 10 repetitions of the six resistance exercises with a workload of 5-7 in the OMNI-RES scale) and control (similar to the resistance session; however, the resistance exercises were performed with no load). The frequency domain (low frequency, high frequency and sympathovagal balance) and symbolic analysis (0V, 1V and 2V patterns) of heart rate variability were obtained before and until one hour after the interventions. RESULTS: After the resistance exercise and control sessions, similar increases were observed in the consecutive heartbeat intervals (control: 720.8±28.6 vs. 790.9±34.4 ms; resistance exercise: 712.9±30.1 vs. 756.8±37.9 ms; p<0.05) and in the pattern of the symbolic analysis with no variation (0V) (control: 25.1±3.5 vs. 33.4±4.1%; resistance exercise: 26.1±3.2 vs. 29.7±3.5%; p<0.05) until 50 min after both interventions. The pattern of two variations (2V) decreased similarly (control: 11.2±2.1 vs. 8.3±2.1%; resistance exercise: 9.5±1.7 vs. 7.8±1.7%; p<0.05). In contrast, the pattern of one variation (1V), the low and high frequency bands and sympathovagal balance did not change after the interventions (p>0.05). CONCLUSION: A single bout of resistance exercise did not alter cardiac autonomic modulation in patients with peripheral artery disease. .

A comparison between diuretics and angiotensin-receptor blocker agents in patients with stage I hypertension (PREVER-treatment trial): study protocol for a randomized double-blind controlled trial.

BACKGROUND: Cardiovascular disease is the leading cause of death in Brazil, and hypertension is its major risk factor. The benefit of its drug treatment to prevent major cardiovascular events was consistently demonstrated. Angiotensin-receptor blockers (ARB) have been the preferential drugs in the management of hypertension worldwide, despite the absence of any consistent evidence of advantage over older agents, and the concern that they may be associated with lower renal protection and risk for cancer. Diuretics are as efficacious as other agents, are well tolerated, have longer duration of action and low cost, but have been scarcely compared with ARBs. A study comparing diuretic and ARB is therefore warranted. METHODS/DESIGN: This is a randomized, double-blind, clinical trial, comparing the association of chlorthalidone and amiloride with losartan as first drug option in patients aged 30 to 70 years, with stage I hypertension. The primary outcomes will be variation of blood pressure by time, adverse events and development or worsening of microalbuminuria and of left ventricular hypertrophy in the EKG. The secondary outcomes will be fatal or non-fatal cardiovascular events: myocardial infarction, stroke, heart failure, evidence of new subclinical atherosclerosis and sudden death. The study will last 18 months. The sample size will be of 1200 participants for group in order to confer enough power to test for all primary outcomes. The project was approved by the Ethics committee of each participating institution. DISCUSSION: The putative pleiotropic effects of ARB agents, particularly renal protection, have been disputed, and they have been scarcely compared with diuretics in large clinical trials, despite that they have been at least as efficacious as newer agents in managing hypertension. Even if the null hypothesis is not rejected, the information will be useful for health care policy to treat hypertension in Brazil. CLINICAL TRIALS REGISTRATION NUMBER: ClinicalTrials.gov: NCT00971165.

Autonomic modulation in patients with congenital generalized lipodystrophy (Berardinelli-Seip syndrome).

AIMS: This study was designed to assess cardiac autonomic regulation in congenital generalized lipodystrophy (CGL) patients using 24 h heart rate variability (HRV). METHODS AND RESULTS: A cross-sectional study was carried out to evaluate 18 patients with CGL and 19 healthy controls matched by sex and age. We measured blood pressure, plasma concentrations of glucose, triglycerides, cholesterol, high-density lipoprotein-cholesterol, insulin resistance by the homeostatic model assessment (HOMA-R), left ventricular mass (LVM) (by two-dimensional echocardiography), and 24 h HRV (by the time domain indices MeanRR, SDNN, and rMSSD). Compared with controls, CGL patients had higher blood pressure (systolic, 131.1 vs. 106.3 mmHg, P < 0.05; diastolic, 85.0 vs. 68.2 mmHg, P < 0.05) and 10 patients met criteria for arterial hypertension and concentric left ventricular hypertrophy (LVM index > or =115 g/m(2)and relative left ventricular wall thickness > or =0.42). Patients with CGL had higher levels of glucose, triglycerides, cholesterol, and HOMA-R and 12 met criteria for type 2 diabetes mellitus. Compared with controls, CGL patients had lower MeanRR (639.8 vs. 780.5 ms, P < 0.001), SDNN (79.2 vs. 168.5 ms, P < 0.001), and rMSSD (15.8 vs. 59.6 ms, P < 0.001). In CGL patients, the reduction in HRV was independent of the metabolic and haemodynamic disturbances. CONCLUSION: Congenital generalized lipodystrophy patients have abnormal autonomic modulation, reflected by increased heart rate and pronounced reduction in HRV, independent of the metabolic and haemodynamic disturbances observed in this disorder.