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.     

Adjunctive value of CT coronary angiography in the diagnostic work-up of patients with typical angina pectoris.

AIMS: To determine the adjunctive value of CT coronary angiography (CTCA) in the diagnostic work-up of patients with typical angina pectoris. METHODS AND RESULTS: CTCA was performed in 62 consecutive patients (45 male, mean age 58.8 +/- 7.7 years) with typical angina undergoing diagnostic work-up including exercise-ECG and conventional coronary angiography. Only patients with sinus heart rhythm and ability to breath hold for 20 s were included. Patients with initial heart rates >/=70 beats/min received beta-blockers. We determined the post-test likelihood ratios, to detect or exclude patients with significant (>/=50% lumen diameter reduction) stenoses, of exercise-ECG and CTCA separately, and of CT performed after exercise-ECG testing. The prevalence of patients with significant coronary artery disease (CAD) was 74%. Positive and negative likelihood ratios for exercise-ECG were 2.3 [95% confidence interval (CI): 1.0-5.3] and 0.3 (95% CI: 0.2-0.7) and for CTCA 7.5 (95% CI: 2.1-27.1) and 0.0 (95% CI: 0.0-8), respectively. CTCA increased the post-test probability of significant CAD after a negative exercise-ECG from 58 to 91%, and after a positive exercise-ECG from 89 to 99%, while CT correctly identified patients without CAD (probability 0%). CONCLUSION: Non-invasive CTCA is a potentially useful tool, in the diagnostic work-up of patients with typical angina pectoris, both to detect and to exclude significant CAD.     

The use of contrast echocardiography for the detection of cardiac shunts.

Recently, debate has erupted about the clinical significance of cardiovascular shunts. Several major health problems such as stroke and migraine have been associated with patent foramen ovale (PFO) with right-to-left shunt (RLS). The nature of the relationship between these syndromes and PFO is not clearly understood. Technical advances have led to more therapeutic options including device closure of PFO, hence prevention of such a PFO-related stroke has become feasible. Therefore, optimal diagnosis of PFO has become of greater clinical importance. Contrast echocardiography with non-transpulmonary contrast agents has been the cornerstone in diagnosis of PFO with RLS for over four decades. Despite being a relatively invasive procedure, transesophageal echocardiography (TEE) is considered the gold standard for detection of RLS. Several other echocardiographic techniques such as transthoracic echocardiography (TTE) with second harmonic imaging and transcranial Doppler ultrasonography (TCD) have shown increased sensitivity and specificity compared to TEE for the detection of PFO with RLS. Moreover, improvement of skills and techniques used for detection of these shunts has led to greater detection of small and large sized RLS in the echocardiographic laboratory. This review gives and overview of the echocardiographic techniques, contrast agents and manoeuvres used for detection of the major cardiovascular shunts and their clinical relevance to major health problems.     

Immune reconstitution in severe combined immunodeficiency disease after lectin-treated, T-cell-depleted haplocompatible bone marrow transplantation

We describe our 9-year experience with lectin-treated T-cell-depleted haplocompatible parental bone marrow transplantation (BMT) for 24 patients with severe combined immunodeficiency disease (SCID). Nineteen of 21 evaluable patients had T-cell engraftment; 2 of 11 patients tested had B-cell and monocyte engraftment. Fourteen of 24 (58%) patients are alive 7 months to 9.8 years post-BMT. Seventeen of 24 patients received pretransplant conditioning with chemotherapy and/or total body irradiation, and 8 of 24 received more than one transplant. Patients who received conditioning had a survival rate of 61% versus 57% for those who received no conditioning. None received graft-versus- host disease (GVHD) prophylaxis and no patient had acute or chronic GVHD greater than grade I. Kinetics and follow-up of immune recovery were analyzed in 14 patients who are greater than 1 year from transplant. Half of the patients showed evidence of T-cell function by 3 months and normal T-cell function by 4 to 7 months post-BMT. On average, T-cell numbers and subsets became normal 10 to 12 months posttransplant. Recovery of B-cell function was more delayed, although in most patients B-cell numbers and IgM levels were normal by 12 months post-BMT. B-cell function, as determined by isohemagglutinin titers or specific antibodies to pneumococcal polysaccharide, keyhole limpet hemocyanin, or tetanus toxoid, became normal in 10 of 14 patients 2 to 8 years post-BMT. Seven of the 14 are off gammaglobulin therapy. Production of isohemagglutinins tended to predict recovery of antibody response to pneumococcal polysaccharide (P < .064). Based on these results, we believe that haplocompatible BMT is an effective, curative treatment for patients with SCID who lack an HLA-matched related donor.     

Detection and viability of tumor cells in peripheral blood stem cell collections from breast cancer patients using immunocytochemical and clonogenic assay techniques [see comments]

Although peripheral blood stem cell collections (PBSC) are thought to have less tumor involvement than bone marrow (BM), the incidence of circulating tumor cells in patients with breast cancer has not been widely investigated. We prospectively investigated the incidence and viability of tumor cell involvement in PBSC and BM collections from breast cancer patients undergoing high-dose chemotherapy/hematopoietic stem cell transplantation. Paired samples of PBSC and BM from 48 patients were analyzed using an immunocytochemical technique that detects one epithelial-derived tumor cell per 5 x 10(5) mononuclear cells. Immunostained tumor cells were detected in 9.8% (13/133) PBSC specimens from 9/48 (18.7%) patients and in 62.3% (38/61) BM specimens from 32/48 (66.7%) patients, a significantly higher rate than in PBSC (P < .005). The geometric mean concentration of tumor cells in contaminated PBSC specimens was 0.8/10(5) mononuclear cells (range 0.33 to 2.0/10(5)) compared with 22.9/10(5) mononuclear cells in BM (range 1 to 3,000/10(5), P < .0001). In culture experiments, clonogenic tumor colonies grew in 21/26 immunocytochemically positive specimens. No tumor colony growth was detected in 30/32 immunocytochemically negative specimens. Immunocytochemical detection of tumor involvement in BM and PBSC correlated significantly with in vitro clonogenic growth (P < .0001). We conclude that PBSC contain fewer tumor cells than paired BM specimens from patients with advanced breast cancer and that these tumor cells appear to be capable of clonogenic growth in vitro.     

Protocols for the treatment of supraventricular tachycardias in the fetus]

The protocols mentioned are used by Utrecht University Hospital for the treatment of foetal supraventricular tachycardias. In the case of atrial flutters the pregnant woman is treated with sotalol administered orally and, if no sinus rhythm is obtained nor a reduced ventricular rhythm occurs, subsequently with digoxin. If there is no hydrops foetalis then this is also the treatment regimen for other forms of foetal supraventricular tachycardia. In the case of hydrops foetalis the treatment is more aggressive: digoxin intravenously or flecanide orally; if the rhythm does not convert into a sinus rhythm nor a reduced ventricular rhythm occurs then both of these medications are administered; if that also proves to be insufficient then direct foetal therapy can be considered.     

Pregnancy and the mechanical prosthetic valve: dilemmas about the choice of antithrombotic prophylaxis

Two pregnant women aged 32 and 34, both of whom had mechanical prosthetic mitral valves, for which they were using low molecular weight heparin. One developed orthopnoea and dyspnoea at 36 weeks amenorrhoea, and the other suffered an acute myocardial infarction at 18 weeks. Both had valvular thrombosis. Following effective treatment, both women delivered at term and one week later after an uncomplicated course, were discharged home from hospital. Clinicians caring for pregnantwomen with mechanical prosthetic valves are faced with a dilemma when trying to provide optimal treatment. Inadequate anticoagulant therapy can result in thrombosis of the mechanical prosthetic valve while, on the other hand, anticoagulant therapy is associated with foetal and maternal bleeding and teratogenic effects. Pregnant women with mechanical prosthetic valves should be thoroughly counselled before or shortly after the confirmation of pregnancy regarding the risks associated with available anticoagulant options, allowing them to make an informed decision concerning the best management plan. This is an algorithm for optimal care of these patients.     

Prediction of behavioural and emotional problems in children and adolescents with operated congenital heart disease

Aims The aim of the present study was to determine which medicalvariables were predictors of long-term behavioural/emotionaloutcome after surgical correction for congenital heart diseasein infancy and childhood. Methods The Child Behavior Checklist (CBCL) was used to predict parent-reportedbehavioural/emotional problems in 125 10–15 year-old congenitalheart disease children from: (1) biographical status (2) medicalhistory (3) heart surgery (4) short-term post-operative courseand (5) number of heart operations and (6) extra cardiac concomitantanomalies. Results Higher CBCL total problem scores at follow-up were associatedwith a greater number of heart oper-ations and deep hypothermiccirculatory arrest (<22°). ‘Internalizing problems’were associated with a greater number of heart operations, deephypothermic circulatory arrest, a short gestational age, lowsystemic oxygen saturation, and older age at surgical repair.‘Externalizing problems’ were associated with agreater number of heart operations only. Conclusion Several medical variables were significant predictors and canbe used to identify those congenital heart disease childrenwho are at risk of long-term behavioural/emotional maladjustment.     

Computed tomography coronary angiography accuracy in women and men at low to intermediate risk of coronary artery disease

Objectives

To investigate the diagnostic accuracy of CT coronary angiography (CTCA) in women at low to intermediate pre-test probability of coronary artery disease (CAD) compared with men.

Methods

In this retrospective study we included symptomatic patients with low to intermediate risk who underwent both invasive coronary angiography and CTCA. Exclusion criteria were previous revascularisation or myocardial infarction. The pre-test probability of CAD was estimated using the Duke risk score. Thresholds of less than 30?% and 30–90?% were used for determining low and intermediate risk, respectively. The diagnostic accuracy of CTCA in detecting obstructive CAD (≥50?% lumen diameter narrowing) was calculated on patient level. P?<?0.05 was considered significant.

Results

A total of 570 patients (46?% women [262/570]) were included and stratified as low (women 73?% [80/109]) and intermediate risk (women 39?% [182/461]). Sensitivity, specificity, PPV and NPV were not significantly different in and between women and men at low and intermediate risk. For women vs. men at low risk they were 97?% vs. 100?%, 79?% vs. 90?%, 80?% vs. 80?% and 97?% vs. 100?%, respectively. For intermediate risk they were 99?% vs. 99?%, 72?% vs. 83?%, 88?% vs. 93?% and 98?% vs. 99?%, respectively.

Conclusion

CTCA has similar diagnostic accuracy in women and men at low and intermediate risk.

Key Points

? Coronary artery disease (CAD) is increasingly investigated by computed tomography angiography (CTCA). ? CAD detection or exclusion by CTCA is not different between sexes. ? CTCA diagnostic accuracy was similar between low and intermediate risk sex-specific-groups. ? CTCA rarely misses obstructive CAD in low–intermediate risk women and men. ? CAD yield by invasive coronary angiography after positive CTCA is similar between sex-risk-specific groups.