Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey.

AIMS: The increasing number of adults with congenital heart disease (CHD) has prompted the development of recommendations for the management of these patients and for the organization of their healthcare. The aim of this report is to describe the delivery of care in Europe for adults with congenital cardiac anomalies. METHODS AND RESULTS: As part of the Euro Heart Survey on Adult Congenital Heart Disease, we obtained data from 71 voluntarily participating centres that detailed their care practices for these patients. Forty-eight of these centres were specialist centres and 23 were non-specialist centres. We found that only 19% of the specialist centres complied with defined standards for optimal care structure. The criteria that appeared to be most difficult for all centres to achieve were performing 50 congenital heart operations or more per year and involving nurse specialists in the care of these patients. CONCLUSION: This survey indicated that the provision of care in Europe for adults with congenital heart defects is suboptimal. To fully realize the benefits of cardiac surgery performed in infants and children, continuous effort must be applied by healthcare professionals in order to implement the recommendations on the organization of care for these patients.     

Euro Heart Survey on adult congenital heart disease: concern for the complexity of care.

Management of adults with congenital heart defects Progress in paediatric cardiac surgery over the last decadesresulted in a growing number of adult patients with (surgicalcorrected) congenital heart defects. Most of these patientsrequire lifelong care, as the long-term outcome of the complexsurgical procedures is often complicated by heart failure, atrialor ventricular arrhythmias, pulmonary hypertension, or endocarditis.¹In addition, surgical re-interventions are frequently necessaryin case of, for example, obstruction of conduits or residualdefects. Taking into account the complexity of the late post-operativesequelae, adequate monitoring and care for the adult patient     

Adherence to guidelines in the clinical care for adults with congenital heart disease: the Euro Heart Survey on adult congenital heart disease.

AIMS: To investigate the role of guidelines in structuring the clinical care for adult patients with congenital heart disease (CHD), and to assess adherence to the guidelines in Europe. METHODS AND RESULTS: A selected number of current guidelines were chosen pertaining to operative procedures, investigations, and the use of medication ('interventions'). The source for this analysis was the database of the Euro Heart Survey on adult CHD, which contains retrospectively collected data on 4110 patients followed-up for a median of 5.1 years. For each guideline investigated, patients were selected from the database for whom the particular guideline was relevant. The selected cases were classified according to two criteria: was there an indication for the particular intervention and did the intervention take place? In this manner, cases of 'undue treatment' and 'insufficient treatment' were identified. Adherence to guidelines was found to be good in the case of operative procedures and prophylactic drug treatment. However, regarding diagnostic procedures there had been adherence to guidelines in only slightly more than half of the cases. CONCLUSION: Guidelines have an important role in the actual clinical care of adults with CHD. However, large outcome studies are needed to develop more precise guidelines.     

A prospective survey of patients with valvular heart disease in Europe: The Euro Heart Survey on Valvular Heart Disease.

AIMS: To identify the characteristics, treatment, and outcomes of contemporary patients with valvular heart disease (VHD) in Europe, and to examine adherence to guidelines. METHODS AND RESULTS: The Euro Heart Survey on VHD was conducted from April to July 2001 in 92 centres from 25 countries; it included prospectively 5001 adults with moderate to severe native VHD, infective endocarditis, or previous valve intervention. VHD was native in 71.9% of patients and 28.1% had had a previous intervention. Mean age was 64+/-14 years. Degenerative aetiologies were the most frequent in aortic VHD and mitral regurgitation while most cases of mitral stenosis were of rheumatic origin.Coronary angiography was used in 85.2% of patients before intervention. Of the 1269 patients who underwent intervention, prosthetic replacement was performed in 99.0% of aortic VHD, percutaneous dilatation in 33.9% of mitral stenosis, and valve repair in 46.5% of mitral regurgitation; 31.7% of patients had > or =1 associated procedure. Of patients with severe, symptomatic, single VHD, 31.8% did not undergo intervention, most frequently because of comorbidities. In asymptomatic patients, accordance with guidelines ranged between 66.0 and 78.5%. Operative mortality was <5% for single VHD. CONCLUSIONS: This survey provides unique contemporary data on characteristics and management of patients with VHD. Adherence to guidelines is globally satisfying as regards investigations and interventions.     

The prevalence of abnormal glucose regulation in patients with coronary artery disease across Europe. The Euro Heart Survey on diabetes and the heart.

AIM: The objective behind the Euro Heart Survey on diabetes and the heart was to study the prevalence of abnormal glucose regulation in adult patients with coronary artery disease (CAD). METHODS AND RESULTS: The survey engaged 110 centres in 25 countries recruiting 4196 patients referred to a cardiologist due to CAD out of whom 2107 were admitted on an acute basis and 2854 had an elective consultation. Patient data were collected via a web-based case record form. An oral glucose tolerance test (OGTT) was used for the characterisation of the glucose metabolism. Thirty-one per cent of the patients had diabetes. An OGTT was performed on the 1920 patients without known diabetes, of whom 923 had acute and 997 had a stable manifestation of CAD, respectively. In patients with acute CAD, 36% had impaired glucose regulation and 22% newly detected diabetes. In the stable group these proportions were 37% and 14%. CONCLUSION: This survey demonstrates that normal glucose regulation is less common than abnormal glucose regulation in patients with CAD. OGTT easily discloses the glucometabolic state and should be a routine procedure. The knowledge of glucometabolic state among these patients should influence their future management because it has great potential to improve the outcome.     

Prognosis, disease progression, and treatment of atrial fibrillation patients during 1 year: follow-up of the Euro Heart Survey on atrial fibrillation.

AIMS: To gain insight in the prognosis and treatment of atrial fibrillation (AF) patients during 1-year follow-up in the Euro Heart Survey (EHS) on AF. METHODS AND RESULTS: The EHS enrolled 5333 AF patients in 2003--2004. One-year follow-up data were available for 80%. Of first detected AF patients, 46% did not have a recurrence during 1 year, paroxysmal AF largely remained paroxysmal AF (80%), and 30% of persistent AF progressed to permanent AF. Many treatment changes occurred since baseline. Oral anticoagulation was started in 19% and discontinued in 16% of all patients. Of patients initially on rhythm control 27% did not receive rhythm control during follow-up, whereas 15% of patients initially on rate control received rhythm control. Mortality was highest in permanent AF (8.2%), but also substantial in first detected AF (5.7%). In multivariable analysis, sinus rhythm at baseline was associated with lower mortality, but no significant effect was observed regarding the application of either rhythm or rate control. CONCLUSION: The EHS on AF provides unique prospective observational data on AF progression, long-term treatment, prognosis, and determinants of adverse outcome of the total clinical spectrum of AF in a European cardiology-based patient cohort.     

Noninvasive evaluation of progression of ischemic heart disease: 5 year follow-up.

Left ventricular (LV) apexcardiogram can be used for the assessment of LV diastolic abnormalities during isometric handgrip stress. In the present study, the clinical value of handgrip-apexcardiographic test (HAT) for identifying patients with new ischemia by the assessment of LV diastolic abnormalities during HG was prospectively investigated. After 5 years follow-up, 9 at entry asymptomatic patients with prior infarction developed a slight effort angina pectoris as a sign of progression of the disease. The data of HAT obtained at entry were compared with those within one month after the onset of angina. As apexcardiographic indices of LV diastolic function the relative A-wave to total height (A/H) and the total apexcardiographic relaxation time (TART) of the apex tracing were used. In 243 controls A/H averaged at rest 10 +/- 4% and TART 107 +/- 9 ms and changed only slightly during and after handgrip stress. Both indices were in patients at rest and during HAT within normal limits at entry. At 5 years, TART was longer (123 +/- 13 ms) and A/H unchanged at rest. During and after handgrip, however, A/H increased significantly (29 +/- 9% and 24 +/- 6%, respectively, p less than 0.01), whereas TART showed no significant change. Furthermore, all patients had an abnormal A-wave response to isometric stress (A/H during or/and after handgrip higher than 21%, which is the largest individual value in the controls).(ABSTRACT TRUNCATED AT 250 WORDS)     

Heart failure in the adult patient with congenital heart disease

BACKGROUND: Adult survival is now the rule for most congenital heart defects. However, despite surgical "repair," heart failure remains a common long-term complication of congenital heart disease. Heart failure specialists are seeing an increasing number of patients with congenital defects as the underlying etiology of their heart failure. METHODS AND RESULTS: Familiarity with the anatomy, physiology, and long-term sequelae of surgical correction are essential for providing care to this complex group of patients. CONCLUSION: This review discusses the diagnosis, pathophysiology, and management of the adult congenital heart patient with concomitant heart failure.     

Psychosocial functioning of the adult with congenital heart disease: a 20-33 years follow-up.

AIMS: Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups. METHODS AND RESULTS: Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results. CONCLUSIONS: Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.     

Heart transplantation for congenital heart disease in the first year of life

Successful infant heart transplantation has now been performed for over 25 years. Assessment of long term outcomes is now possible. We report clinical outcomes for322 patients who received their heart transplant during infancy. Actuarial graft survival for newborn recipients is 59% at 25 years. Survival has improved in the most recent era. Cardiac allograft vasculopathy is the most important late cause of death with an actuarial incidence at 25 years of 35%. Post-transplant lymphoma is estimated to occur in 20% of infant recipients by25 years. Chronic kidney disease grade 3 or worse is present in 31% of survivors. The epidemiology of infant heart transplantation has changed through the years as the results for staged repair improved and donor resources remained stagnant. Most centers now employ staged repair for hypoplastic left heart syndrome and similar extreme forms of congenital heart disease. Techniques for staged repair, including the hybrid procedure, are described. The lack of donors is described with particular note regarding decreased donors due to newer programs for appropriate infant sleep positioning and infant car seats. ABO incompatible donors are a newer resource for maximizing donor resources, as is donation after circulatory determination of death and techniques to properly utilize more donors by expanding the criteria for what is an acceptable donor. An immunological advantage for the youngest recipients has long been postulated, and evaluation of this phenomenon may provide clues to the development of accommodation and/or tolerance.