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1.
Eur Heart J Acute Cardiovasc Care ; 11(7): 584-594, 2022 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-35767583

RESUMO

Cardiogenic shock from left ventricular failure is a common presentation in the intensive care unit. In contrast, right ventricular (RV)-predominant heart failure (HF) causing shock is less well recognized. We review the epidemiology and mechanisms of RV-predominant HF and discuss pharmacologic and device-based approaches for the management of this challenging clinical problem.


Assuntos
Insuficiência Cardíaca , Disfunção Ventricular Direita , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/terapia , Ventrículos do Coração , Humanos , Unidades de Terapia Intensiva , Choque Cardiogênico/epidemiologia , Choque Cardiogênico/etiologia , Choque Cardiogênico/terapia , Disfunção Ventricular Direita/complicações , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/terapia
2.
Eur Rev Med Pharmacol Sci ; 25(20): 6300-6310, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34730210

RESUMO

OBJECTIVE: Understanding changes of right ventricular (RV) geometry and function in repaired Tetralogy of Fallot (rToF) patients can improve decision-making for pulmonary valve replacement. Therefore, we aimed to assess the magnitude and clinical correlations of RV changes in rToF patients. PATIENTS AND METHODS: Clinical and MRI data of rToF patients who underwent repeated cardiac magnetic resonance imaging (MRI) at two centers between December 2003 and September 2020 were analyzed together with anatomical factors, including RV outflow tract obstruction, pulmonary artery branch stenosis, and tricuspid regurgitation. Adverse cardiac events and/or NYHA class worsening were documented and correlated with MRI changes. QRS length was reported at each MRI. RESULTS: Two-hundred-and-nineteen rToF patients (53% males, aged 20.2 ± 10.1 years) were enrolled. An increase of ventricular dimensions, except LVEDVi, and worsening of right and left ejection fractions were found over an average period of 5 years of follow-up. These changes were statistically significant but within 10% of the initial value. No significant changes were reported on a year-to-year basis, except in a small group of patients (6%) in whom no predictive factors were identified. Despite similar RV dimensions at the first examination, younger patients had a higher RV ejection fraction and a different annual rate of change of ventricular dimensions compared to older ones. Patients with arrhythmias (20%) were more frequently older and had larger RV dimensions but showed no significant correlations with MRI changes/years. CONCLUSIONS:  Changes in RV dimensions and function occur rarely and very slowly in rToF patients. A small percentage of patients experience a significant worsening in a short time interval without any recognized risk factors. Arrhythmias appear to occur in a small percentage of cases in the late follow-up.


Assuntos
Arritmias Cardíacas/epidemiologia , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/epidemiologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Fatores Etários , Criança , Progressão da Doença , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Fatores de Risco , Volume Sistólico/fisiologia , Disfunção Ventricular Direita/diagnóstico por imagem , Adulto Jovem
3.
Open Heart ; 8(1)2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33504630

RESUMO

AIMS: It was predicted internationally that transthoracic echocardiography (TTE) would be vital during the SARS-CoV-2 outbreak. We therefore, designed a study to report the demand for TTE in two large District General Hospitals during the rise in the first wave of the SARS-CoV-2 pandemic in the UK. A primary clinical outcome of 30-day mortality was also assessed. METHODS: The TTE service across two hospitals was reconfigured to maximise access to inpatient scanning. All TTEs of suspected or confirmed SARS-CoV-2 patients over a 3-week period were included in the study. All patients were followed up until at least day 30 after their scan at which point the primary clinical outcome of mortality was recorded. Comparative analysis based on mortality was conducted for all TTE results, biochemical markers and demographics. RESULTS: 27 patients with confirmed SARS-CoV-2 had a TTE within the inclusion window. Mortality comparative analysis showed the deceased group were significantly older (mean 68.4, SD 11.9 vs 60.5, SD 13.0, p=0.03) and more commonly reported fatigue in their presenting symptoms (29.6% vs 71.4%, p=0.01). No other differences were identified in the demographic or biochemical data. Left ventricular systolic dysfunction was noted in 7.4% of patients and right ventricular impairment or dilation was seen in 18.5% patients. TTE results were not significantly different in mortality comparative analysis. CONCLUSION: This study demonstrates an achievable approach to TTE services when under increased pressure. Data analysis supports the limited available data suggesting right ventricular abnormalities are the most commonly identified echocardiographic change in SARS-CoV-2 patients. No association can be demonstrated between mortality and TTE results.


Assuntos
COVID-19/mortalidade , Doenças Cardiovasculares/mortalidade , Ecocardiografia/métodos , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , COVID-19/diagnóstico , COVID-19/diagnóstico por imagem , COVID-19/virologia , Doenças Cardiovasculares/diagnóstico por imagem , Doenças Cardiovasculares/fisiopatologia , Doenças Cardiovasculares/virologia , Ecocardiografia/estatística & dados numéricos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , SARS-CoV-2/genética , Reino Unido/epidemiologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/fisiopatologia
5.
Indian Heart J ; 71(5): 406-411, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-32035524

RESUMO

BACKGROUND: Few studies have evaluated the right ventricle systolic function in different categories of heart failure despite its effect on outcomes. METHODS AND RESULTS: Single-centre, cross-sectional study included 150 patients, 50 patients in each category of HF: group I, preserved; group II, mid-range; group III, reduced ejection fraction. Left ventricular systolic function was assessed by 3D echo, and right ventricular systolic function was assessed by fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler image (TDI), and global longitudinal strain (GLS). There was no significant difference among the three groups regarding sex, the prevalence of risk factors, but patients in group III were significantly older (p < 0.001) and had a higher prevalence of coronary artery disease (p = 0.004) than were found in the other two groups. In group I, the prevalence of RV systolic dysfunction was 18%, 22%, 14% and 26% by TAPSE, FAC, S wave velocity, and GLS, respectively. Their prevalence was higher in group II and much higher in group III than in group I. There were significant positive correlations among TAPSE, S wave velocity, GLS, and ejection fraction in groups II and III (p < 0.001). CONCLUSION: The prevalence and severity of RV systolic dysfunction were positively correlated with LV systolic dysfunction, and the degree of RV dysfunction in mid-range was closer to reduce than preserved ejection fraction. STUDY REGISTRATION AT CLINICAL TRIAL.GOV: NCT03641599.


Assuntos
Insuficiência Cardíaca/fisiopatologia , Disfunção Ventricular Direita/fisiopatologia , Fatores Etários , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/epidemiologia , Doença da Artéria Coronariana/fisiopatologia , Estudos Transversais , Ecocardiografia Doppler , Ecocardiografia Tridimensional , Egito/epidemiologia , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Volume Sistólico , Sístole , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/epidemiologia
6.
Mayo Clin Proc ; 93(7): 895-903, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29891343

RESUMO

OBJECTIVE: To investigate differences in invasive hemodynamic parameters and outcomes in patients with and without heart failure (HF) symptoms after left ventricular assist device (LVAD) implantation. PATIENTS AND METHODS: We performed a single-center retrospective analysis of 51 symptomatic patients and 50 patients with resolved HF symptoms who underwent right-sided heart catheterization (RHC) after LVAD implantation from March 1, 2007, through June 30, 2016. Patient characteristics and outcomes including all-cause mortality and right ventricular (RV) failure were compared between groups. RESULTS: Fifty-one patients had development of HF symptoms after LVAD implantation and underwent RHC a mean ± SD of 243.7±288 days postoperatively. Fifty asymptomatic LVAD recipients underwent routine RHC 278.6±205 days after implantation. Compared with patients who had resolved HF symptoms, symptomatic patients were older, more likely to be male, and more likely to have ischemic cardiomyopathy. Symptomatic patients had higher right atrial pressure (P<.001), mean pulmonary arterial pressure (P<.001), and pulmonary capillary wedge pressure (P<.001). Improvements in right atrial pressure, mean pulmonary arterial pressure, and pulmonary capillary wedge pressure before and after LVAD implantation were less remarkable in symptomatic patients. The frequency of RV dysfunction was significantly higher among symptomatic patients than patients with resolved HF symptoms (P=.001). Symptomatic patients displayed significantly higher risk of all-cause mortality (hazard ratio, 3.0; 95% CI, 1.3-6.5; P=.007) and RV failure (hazard ratio, 6.2; 95% CI, 1.3-29.7; P=.02) independent of other predictors of outcome. CONCLUSION: Patients with recurrent HF symptoms after LVAD implantation display more profound hemodynamic derangements, greater burden of RV failure, and increased rates of all-cause mortality compared with LVAD recipients with resolved HF symptoms.


Assuntos
Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Coração Auxiliar , Complicações Pós-Operatórias/epidemiologia , Disfunção Ventricular Direita/epidemiologia , Idoso , Pressão Sanguínea , Cateterismo Cardíaco , Débito Cardíaco , Feminino , Insuficiência Cardíaca/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resistência Vascular
7.
Int J Cardiol ; 243: 544-549, 2017 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-28526545

RESUMO

BACKGROUND: Little is known on right ventricular (RV) involvement in obstructive sleep apnoea (OSA). This study aimed at evaluating early RV dysfunction by standard and advanced echocardiography in OSA. METHODS: Fifty-nine OSA patients without heart failure and 29 age-matched controls underwent standard, speckle tracking and real time 3D echocardiography of right ventricle. OSA patients performed lung function tests and overnight cardio-respiratory monitoring with evaluation of apnea-hypopnea index (AHI). RESULTS: OSA had significantly higher body mass index and systolic blood pressure (BP) than controls. RV diameters and systolic pulmonary arterial pressure (sPAP) were significantly higher in OSA, in presence of comparable tricuspid annular plane systolic excursion (TAPSE). OSA showed marginally lower RV global longitudinal strain (GLS) (p<0.05) and RV lateral wall strain (RV LLS) (p=0.04). Three-dimensional RV ejection fraction did not differ between the two groups. By stratifying patients according to sPAP, 18 OSA patients with sPAP≥30mmHg had lower TAPSE (p<0.05), RV GLS and RV LLS (both p<0.001) than 37 patients with normal sPAP. By separate multivariate analyses, RV GLS and RV LLS were independently associated with sPAP (both p<0.0001), AHI (p=0.035 and p=0.015 respectively) and BMI (p<0.05 and p=0.034) but not with age and systolic BP in OSA. CONCLUSIONS: A subclinical RV dysfunction is detectable by speckle tracking in OSA. The impairment of RV GLS and RV LLS is more prominent than that of TAPSE and is evident when RVEF is still normal. GLS is independently associated with sPAP and OSA severity.


Assuntos
Ecocardiografia Doppler/métodos , Ecocardiografia Tridimensional/métodos , Apneia Obstrutiva do Sono/diagnóstico por imagem , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular Direita/fisiologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Apneia Obstrutiva do Sono/epidemiologia , Apneia Obstrutiva do Sono/fisiopatologia , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/fisiopatologia
8.
Am Heart J ; 185: 123-129, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28267465

RESUMO

The optimal approach to assess right ventricular (RV) function in patients with acute symptomatic pulmonary embolism (PE) lacks clarity. METHODS: This study aimed to evaluate the optimal approach to assess RV function in normotensive patients with acute symptomatic PE. Outcomes assessed through 30-days after the diagnosis of PE included all-cause mortality and complicated course. RESULTS: Eight hundred forty-eight patients were enrolled. Multidetector computed tomography (MDCT) and transthoracic echocardiography agreed on the presence or absence of RV overload in 449 (53%) patients. The combination of the simplified Pulmonary Embolism Severity Index (sPESI) and MDCT showed a negative predictive value for 30-day all-cause mortality of 100%. Of the 43% that had an sPESI of >0 points and MDCT RV enlargement, 41 (11.3%) experienced a complicated course that included 24 (6.6%) deaths. One hundred twenty-nine patients (15%) had an sPESI of >0 points, MDCT, and echocardiographic RV overload. Of these, 21 (16.3%) experienced a complicated course within the first 30days, and 10 (7.7%) of them died. CONCLUSIONS: Incorporation of echocardiographic RV overload to the sPESI and MDCT did not improve identification of low-risk PE patients, whereas it improved identification of those at intermediate-high risk for short-term complications.


Assuntos
Mortalidade , Embolia Pulmonar/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem , Doença Aguda , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Comorbidade , Ecocardiografia , Feminino , Insuficiência Cardíaca/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Prognóstico , Estudos Prospectivos , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Embolia Pulmonar/complicações , Embolia Pulmonar/epidemiologia , Medição de Risco , Índice de Gravidade de Doença , Disfunção Ventricular Direita/complicações , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/fisiopatologia , Função Ventricular Direita
9.
J Am Soc Echocardiogr ; 29(9): 861-70, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27297619

RESUMO

BACKGROUND: Echocardiography-derived linear dimensions offer straightforward indices of right ventricular (RV) structure but have not been systematically compared with RV volumes on cardiac magnetic resonance (CMR). METHODS: Echocardiography and CMR were interpreted among patients with coronary artery disease imaged via prospective (90%) and retrospective (10%) registries. For echocardiography, American Society of Echocardiography-recommended RV dimensions were measured in apical four-chamber (basal RV width, mid RV width, and RV length), parasternal long-axis (proximal RV outflow tract [RVOT]), and short-axis (distal RVOT) views. For CMR, RV end-diastolic volume and RV end-systolic volume were quantified using border planimetry. RESULTS: Two hundred seventy-two patients underwent echocardiography and CMR within a narrow interval (0.4 ± 1.0 days); complete acquisition of all American Society of Echocardiography-recommended dimensions was feasible in 98%. All echocardiographic dimensions differed between patients with and those without RV dilation on CMR (P < .05). Basal RV width (r = 0.70), proximal RVOT width (r = 0.68), and RV length (r = 0.61) yielded the highest correlations with RV end-diastolic volume on CMR; end-systolic dimensions yielded similar correlations (r = 0.68, r = 0.66, and r = 0.65, respectively). In multivariate regression, basal RV width (regression coefficient = 1.96 per mm; 95% CI, 1.22-2.70; P < .001), RV length (regression coefficient = 0.97; 95% CI, 0.56-1.37; P < .001), and proximal RVOT width (regression coefficient = 2.62; 95% CI, 1.79-3.44; P < .001) were independently associated with CMR RV end-diastolic volume (r = 0.80). RV end-systolic volume was similarly associated with echocardiographic dimensions (basal RV width: 1.59 per mm [95% CI, 1.06-2.13], P < .001; RV length: 1.00 [95% CI, 0.66-1.34], P < .001; proximal RVOT width: 1.80 [95% CI, 1.22-2.39], P < .001) (r = 0.79). CONCLUSIONS: RV linear dimensions provide readily obtainable markers of RV chamber size. Proximal RVOT and basal width are independently associated with CMR volumes, supporting the use of multiple linear dimensions when assessing RV size on echocardiography.


Assuntos
Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/epidemiologia , Ecocardiografia/métodos , Imagem Cinética por Ressonância Magnética/métodos , Volume Sistólico , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/epidemiologia , Causalidade , Comorbidade , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , New York/epidemiologia , Tamanho do Órgão , Reprodutibilidade dos Testes , Fatores de Risco , Sensibilidade e Especificidade
10.
Scand J Rheumatol ; 44(5): 385-8, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25928303

RESUMO

OBJECTIVES: Systemic sclerosis (SSc) leads to pulmonary circulation dysfunctionand there are some indications of systemic circulation impairment. We evaluated the influence of SSc on the elastic properties of large systemic arterial walls and potential correlations between systemic and pulmonary circulation involvement. METHOD: We examined 75 consecutive women (mean age 53.13±10.1 years) with confirmed SSc [mean disease duration (DD) 7.1±9.1 years] and 21 age-matched female volunteers (mean age 52.6±8.3 years, ns). Pulse wave velocity (PWV) and transthoracic echocardiography were performed. SSc patients were divided into two groups according to the median of DD: ≤3 years (39 patients) and >3 years (36 patients). RESULTS: Patients with DD>3 years had higher PWV than those with DD≤3 years and controls (log PWV: 2.23±0.23 vs. 2.13±0.16 and vs. 2.11±0.16 m/s; p=0.028 and 0.029, respectively). In addition, echocardiographic indices showed impaired right ventricular (RV) function in the patients with DD>3 years. Also in these SSc patients, PWV correlated with clinical and echocardiographic parameters of pulmonary circulation: age (r=0.64, p<0.0001), acceleration time of pulmonary ejection (AcT; r=-0.38, p=0.021), and tricuspid regurgitation peak gradient (TRPG; r=0.34, p=0.04). Multiple linear regression analysis showed that PWV was independently associated with DD (ß=0.22, p==0.02), AcT (ß=-0.215, p=0.03), and age (ß=0.44, p<0.001). CONCLUSIONS: In patients with SSc lasting more than 3 years, the disease is characterized by increased stiffness of the large systemic arteries. Longer duration of SSc leads simultaneously to the increased stiffness of the large systemic arteries and to the progressive impairment of RV function and its coupling to the pulmonary arterial bed.


Assuntos
Artérias/fisiopatologia , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/fisiopatologia , Remodelação Vascular/fisiologia , Adulto , Artérias/diagnóstico por imagem , Estudos de Casos e Controles , Ecocardiografia , Elasticidade/fisiologia , Feminino , Humanos , Hipertensão/epidemiologia , Incidência , Pessoa de Meia-Idade , Artéria Pulmonar/diagnóstico por imagem , Escleroderma Sistêmico/diagnóstico por imagem , Disfunção Ventricular Direita/epidemiologia
11.
Pacing Clin Electrophysiol ; 31(11): 1456-62, 2008 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-18950303

RESUMO

OBJECTIVES: Evaluation of left ventricular (LV) dyssynchrony in patients undergoing short-term right ventricular apical (RVA) pacing and correlation with baseline echocardiographic and clinical characteristics. BACKGROUND: RVA pacing causes abnormal ventricular depolarization that may lead to mechanical LV dyssynchrony. The relationships between pacing-induced LV dyssynchrony and baseline echocardiographic and clinical variables have not been fully clarified. METHODS: Tissue Doppler echocardiography was performed in 153 patients before and after RVA pacing. LV dyssynchrony was measured by the time between the shortest and longest electromechanical delays in the five basal LV segments (intra-LV). The prevalence and degree of LV dyssynchrony after RVA pacing was evaluated in three groups: baseline LV ejection fraction (LVEF) <35%, 35-55%, and >or=55%. The intrapatient effect of RVA pacing was determined as the percent increase in intra-LV value (Deltaintra-LV%). The pacing-induced intra-LV was correlated with baseline variables. RESULTS: The prevalence and degree of LV dyssynchrony after RVA pacing was significantly higher in patients with lower LVEF (P < 0.001). DeltaIntra-LV% was inversely correlated with baseline intra-LV and LVEF (B =-2.6, B =-4.2, P < 0.001). Baseline intra-LV and LV end-systolic volume correlated positively with intra-LV after RVA pacing (B = 0.49, B = 0.6, P < 0.001), whereas LVEF showed an inverse correlation. CONCLUSIONS: The degree of LV dyssynchrony induced by RVA is variable. Patients with higher baseline LV dyssynchrony, more dilated LV, and more depressed LVEF showed a higher degree of LV dyssynchrony during pacing. These findings may assume importance in predicting the risk of heart failure in pacemaker patients.


Assuntos
Estimulação Cardíaca Artificial/estatística & dados numéricos , Medição de Risco/métodos , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/prevenção & controle , Idoso , Comorbidade , Feminino , Humanos , Incidência , Itália/epidemiologia , Masculino , Reprodutibilidade dos Testes , Fatores de Risco , Sensibilidade e Especificidade , Resultado do Tratamento
12.
Clin Transplant ; 21(3): 321-9, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17488380

RESUMO

INTRODUCTION: The aim of this paper was to review the outcomes of cardiac transplantation with regards to short- and long-term survival, focusing particularly on patients who receive organs with long ischemic times and the resource utilization necessary to support such patients through their postoperative period. METHODS: A retrospective review of 420 consecutive cardiac transplants in a single institution was undertaken. RESULTS: The five- and 10-yr survival rates for the entire group were 0.76 (95% CI: 0.72-0.80) and 0.60 (0.54-0.66). There was no decrease in mid- or long-term survival in patients who received organs with ischemic times over 300 min. Longer donor organ ischemic time was not associated with increased 30 d mortality but was significantly associated with longer intensive care bed stay, increased incidence of primary graft failure, need for mechanical support, and complications such as acute renal failures. CONCLUSIONS: Although using donor organs with longer ischemic times for cardiac transplantation does not impact on survival, there is a significantly increased utilization of resources to ensure these patients survive the postoperative period.


Assuntos
Transplante de Coração/mortalidade , Adulto , Ponte Cardiopulmonar , Feminino , Transplante de Coração/economia , Humanos , Imunossupressores/uso terapêutico , Tempo de Internação , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Análise de Sobrevida , Fatores de Tempo , Transplante Homólogo , Disfunção Ventricular Direita/epidemiologia , Vitória
13.
J Am Coll Cardiol ; 38(5): 1477-84, 2001 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-11691526

RESUMO

OBJECTIVES: The purpose of this study was to examine the genotype-phenotype relation with respect to penetrance, age and severity of expression, disease progression and prognosis in a recessively inherited arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND: Naxos disease is a recessively inherited ARVC caused by a mutation in the gene encoding plakoglobin (cell adhesion protein) in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. METHODS: Twelve families with Naxos disease underwent cardiac and molecular genetic investigation. Serial cardiac assessment with annual resting 12-lead and 24-h ambulatory electrocardiogram (ECG) and two-dimensional echocardiography was performed during 1 to 16 years, median 7 +/- 6 years in all 78 surviving members. RESULTS: Twenty-eight surviving members were homozygous and 40 were heterozygous for the mutation. All adults who were homozygous (n = 26) fulfilled the diagnostic criteria for ARVC, the youngest by the age of 13 years. In eight who were heterozygous, minor ECG or echocardiographic abnormalities were observed. Of the 26 subjects who were affected homozygotes, 92% showed ECG abnormalities, 92% ventricular arrhythmias, 100% right ventricular structural alterations and 27% left ventricular involvement. During follow-up (10 +/- 6 years), 16 (62%) developed structural progression, 12 (46%) arrhythmic events and 7 (27%) heart failure. The annual disease-related and sudden death mortality was 3% and 2.3%, respectively. CONCLUSIONS: Autosomal recessive ARVC caused by a mutation in plakoglobin was 100% penetrant by adolescence. Affected subjects who were homozygous experienced progressive disease with adverse prognosis. A minority of subjects who were heterozygous showed minor ECG/echocardiographic changes, but clinically significant disease did not develop.


Assuntos
Anormalidades Múltiplas/genética , Arritmias Cardíacas/genética , Cardiomiopatia Hipertrófica Familiar/genética , Proteínas do Citoesqueleto/genética , Deleção de Genes , Genes Recessivos/genética , Cabelo/anormalidades , Ceratodermia Palmar e Plantar/genética , Penetrância , Disfunção Ventricular Direita/genética , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idade de Início , Idoso , Análise de Variância , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/epidemiologia , Cardiomiopatia Hipertrófica Familiar/diagnóstico , Cardiomiopatia Hipertrófica Familiar/epidemiologia , Criança , Pré-Escolar , Morte Súbita Cardíaca/etiologia , Desmoplaquinas , Progressão da Doença , Ecocardiografia , Eletrocardiografia , Feminino , Testes Genéticos , Genótipo , Heterozigoto , Homozigoto , Humanos , Lactente , Ceratodermia Palmar e Plantar/diagnóstico , Ceratodermia Palmar e Plantar/epidemiologia , Masculino , Ilhas do Mediterrâneo/epidemiologia , Pessoa de Meia-Idade , Linhagem , Fenótipo , Valor Preditivo dos Testes , Prognóstico , Índice de Gravidade de Doença , Análise de Sobrevida , Síncope/etiologia , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/epidemiologia , gama Catenina
14.
Cardiovasc Surg ; 8(1): 1-9, 2000 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-10661697

RESUMO

Isolated acute refractory right ventricular failure is extremely uncommon. There are greater prospects of seeing a right dominant biventricular failure, as the two ventricular chambers are contiguous. The overall clinical spectrum is determined by the relative ischemic involvement of the right or left ventricle. The postoperative acute refractory right ventricular failure that develops after cardiotomy, heart transplant, or during a left ventricular assist device support, may have somewhat dissimilar elements of origin, but the resultant clinical picture and the management are essentially similar. In this collective review, the authors have summarized the incidence, pathogenesis, management and prognosis of postoperative acute refractory right ventricular failure, in adult cardiac surgical practice. The incidence of post-cardiotomy acute refractory right ventricular failure ranges from 0.04 to 0.1%. Acute refractory right ventricular failure has also been reported in 2-3% patients after a heart transplant and in almost 20-30% patients who receive a left ventricular assist device support. The main contributor to this problem is a disproportionate ischemic involvement of the right ventricle. Other pertinent contributors to this problem are pulmonary hypertension and an altered interventricular balance. The latter component is predominant in recipients of a left ventricular assist device support. Postoperative acute refractory right ventricular failure has been successfully managed with conventional pulmonary vasodilators, mechanical support with a pulmonary artery balloon pump, a right ventricular assist device, or cavopulmonary diversion. Unfortunately, the reported initial salvage rate is only 25-30%. This problem is often underestimated. Support measures are often started late or terminated prematurely. These factors have contributed to a poor initial salvage rate in this group of patients.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Insuficiência Cardíaca/etiologia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/terapia , Doença Aguda , Adulto , Gerenciamento Clínico , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/terapia , Humanos , Incidência , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/terapia , Prognóstico , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/epidemiologia
15.
J Card Surg ; 13(1): 32-6, 1998 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-9892483

RESUMO

UNLABELLED: The impact of continuous retrograde cardioplegia (RCP) on right ventricular (RV) function was evaluated prospectively with intraoperative transesophageal echocardiography (TEE) in 36 patients (23 males, 13 females) with a mean age of 60.4 years (ages 24-82). Operative procedures included 12 aortic valve replacements, 16 mitral valve repair/replacements, both with or without an associated cardiac operation, and 8 Ross procedures. Mean cardiopulmonary bypass (CPB) time was 123.3 minutes (66-280 minutes) with an average cross-clamp time of 88.9 minutes (43-199 minutes). The amount of cold blood RCP ranged from 3160-18,500 mL (mean = 7382.5), and the average pulmonary artery pressure was 35/18 mmHg. The coronary sinus was distally snared in 11 patients. TEE documented biventricular global dysfunction in two patients and post-CPB with preservation of the left ventricular function in all other patients. Localized akinesis of the RV apex and outflow tract were noted in three patients, and isolated worsening tricuspid insufficiency of moderate to severe intensity in six patients. Two of the six patients with worsening tricuspid insufficiency belonged to the snared coronary sinus group (11 patients). All of the documented RV dysfunctions were new and showed no correlation with the perfusion data. IN CONCLUSION: (1) RV apex, RV outflow tract and tricuspid valve were particularly subject to important dysfunction post-RCP; (2) RCP did not protect RV adequately in 11 patients (31%); and (3) TEE is a convenient intraoperative technique in evaluating RV dysfunction.


Assuntos
Ecocardiografia Transesofagiana , Parada Cardíaca Induzida/métodos , Complicações Pós-Operatórias/diagnóstico por imagem , Disfunção Ventricular Direita/diagnóstico por imagem , Procedimentos Cirúrgicos Cardíacos , Feminino , Parada Cardíaca Induzida/efeitos adversos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Disfunção Ventricular Direita/epidemiologia , Disfunção Ventricular Direita/etiologia , Função Ventricular Direita/fisiologia
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