RESUMO
BACKGROUND: Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. AIMS: To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK. METHODS: Records of all patients with a univentricular (Fontan) circulation at the Queen Elizabeth Hospital were reviewed. Employment status was categorized according to the Standard Occupational Classification criteria (2010). RESULTS: A total of 210 patient records were reviewed. There was the same proportion of professionals in our cohort compared to the rest of the UK (20% versus 20%). There were greater proportions working in the caring, leisure and other service occupations (15% versus 9%), the elementary occupations (17% versus 11%), sales and customer service occupations (14% versus 8%) and administrative and secretarial occupations (12% versus 11%). The reverse trend was observed for associate professions and technical occupations (7% versus 14%), skilled trades (10% versus 11%), process, plant and machine operatives (3% versus 6%) and managers, directors and senior officials (2% versus 10%). CONCLUSIONS: The data show that ACHD patients with a single ventricle are engaged in a diverse range of occupations. It is essential that early education and employment advice are given to this cohort to maximize future employment potential.
Assuntos
Emprego/estatística & dados numéricos , Cardiopatias Congênitas , Ocupações/estatística & dados numéricos , Adulto , Estudos de Coortes , Feminino , Técnica de Fontan , Humanos , Masculino , Reino Unido/epidemiologiaRESUMO
OBJECTIVE: Eisenmenger syndrome is characterized by severe and lifelong hypoxemia and pulmonary hypertension. Despite this, patients do surprisingly well and report a reasonable quality of life. The aim of this study was to investigate whether these patients undergo adaptation of their skeletal and cardiac muscle energy metabolism which would help explain this paradox. DESIGN AND SETTING: Ten patients with Eisenmenger syndrome and eight age- and sex-matched healthy volunteers underwent symptom-limited treadmill cardiopulmonary exercise testing, transthoracic echocardiography and (31) P magnetic resonance spectroscopy of cardiac and skeletal muscle. Five subjects from each group also underwent near infrared spectroscopy to assess muscle oxygenation. RESULTS: Despite having a significantly lower peak VO2 , patients with Eisenmenger syndrome have a similar skeletal muscle phosphocreatine (PCr) recovery, a measure of oxidative capacity, when compared to healthy controls (34.9 s ± 2.9 s vs. 35.2 s ± 1.7 s, P = .9). Furthermore their intracellular pH falls to similar levels during exercise suggesting they are not reliant on early anaerobic metabolism (0.3 ± 0.06 vs. 0.28 ± 0.04, P = .7). While their right ventricular systolic function remained good, the Eisenmenger group had a lower cardiac PCr/ATP ratio compared to the control group (1.55 ± 0.10 vs. 2.17 ± 0.15, P < .05). CONCLUSIONS: These results show that adult patients with Eisenmenger syndrome have undergone beneficial physiological adaptations of both skeletal and cardiac muscle. This may, in part, explain their surprisingly good survival despite a lifetime of severe hypoxemia and adverse cardiopulmonary hemodynamics.
Assuntos
Complexo de Eisenmenger/complicações , Metabolismo Energético , Hipóxia/etiologia , Músculo Esquelético/metabolismo , Miocárdio/metabolismo , Adaptação Fisiológica , Adulto , Biomarcadores/metabolismo , Estudos de Casos e Controles , Ecocardiografia , Complexo de Eisenmenger/diagnóstico , Complexo de Eisenmenger/metabolismo , Complexo de Eisenmenger/fisiopatologia , Teste de Esforço , Tolerância ao Exercício , Feminino , Humanos , Concentração de Íons de Hidrogênio , Hipóxia/diagnóstico , Hipóxia/metabolismo , Hipóxia/fisiopatologia , Espectroscopia de Ressonância Magnética/métodos , Masculino , Músculo Esquelético/fisiopatologia , Consumo de Oxigênio , Fosfocreatina/metabolismo , Espectroscopia de Luz Próxima ao Infravermelho , Função Ventricular DireitaRESUMO
Heart disease is the leading cause of maternal mortality in the UK. Deaths from acquired conditions such as ischaemic heart disease are increasing and often occur in patients with no history of heart disease, thus emphasising the need for vigilance for risk factors in women of childbearing age. All women with known heart disease should have pre-pregnancy counselling to assess for maternal and fetal risk. Women deemed to be at moderate or high risk should be under the care of a specialist antenatal team with experience of managing women with heart disease in pregnancy. Conditions that are considered particularly high risk (mortality >10%) include Marfan syndrome with dilated aortic root, severe left heart obstructive lesions, pulmonary hypertension, and severe left ventricular dysfunction. This article reviews the management of women with heart disease during pregnancy, labour and in the puerperium.