Anomalous Aortic Origin of the Right Coronary Artery: Invasive Haemodynamic Assessment in Adult Patients With High-Risk Anatomic Features.

Background: Anomalous aortic origin of a right coronary artery (AAORCA) with an interarterial course merits further evaluation; however, robust risk assessment strategies for myocardial ischemia and sudden cardiac death are currently lacking. The aim of this study is to explore the potential role of fractional flow reserve (FFR), instantaneous wave-free ratio (iFR), and intravascular ultrasound (IVUS) in patients with AAORCA. Methods: Consecutive adult patients with AAORCA with an interarterial course were included. Computed tomography angiography, noninvasive ischemia detection, and FFR, iFR, and IVUS were performed at baseline and during adrenaline-induced stress. External compression was evaluated with IVUS. Results: Eight patients (63% female, mean age: 53 ± 9.5 years) were included. Five patients (63%) were symptomatic, and computed tomography angiography revealed high-risk anatomy of the AAORCA in all patients. Only in 1 (12.5%) patient FFR and iFR were positive; however, this was attributed at large to concomitant diffuse atherosclerosis. In 2 of 8 (25%), IVUS revealed external compression; however, the ostial coronary surface area remained unchanged. In all patients, a conservative treatment strategy was pursued. During a mean follow-up of 29.3 months (standard deviation ±2.6 months), symptoms spontaneously disappeared in 4 of 5 (80%) and no adverse cardiac events occurred in any of the patients. Conclusions: Despite the presence of high-risk anatomy in all patients, none had proven ischemia prompting a conservative treatment strategy. No adverse cardiac events occurred during follow-up, and in the majority of patients, symptoms spontaneously disappeared. Therefore, FFR, iFR, and IVUS with pharmacologic stress merit further investigation and might contribute to ischemia-based risk stratification and management strategies in adult patients with AAORCA.

Contexte: L'anomalie de naissance de l'artère coronaire droite à partir de l'aorte (AAORCA, anomalous aortic origin of a right coronary artery) combinée à un trajet interartériel mérite un examen plus approfondi. Cependant, on observe à l'heure actuelle des lacunes en ce qui a trait à l'emploi de stratégies fiables d'évaluation du risque d'ischémie myocardique et de mort subite d'origine cardiaque. L'objectif de cette étude est d'examiner le rôle potentiel de la mesure de la réserve coronarienne (MRC), de l'évaluation du rapport instantané sans onde (iFR, instantaneous wave-free ratio) et de l'échographie intravasculaire chez des patients présentant une AAORCA. Méthodologie: Des cas de patients adultes consécutifs présentant une AAORCA combinée à un trajet interartériel ont été inclus à l'étude. Une angiographie par tomodensitométrie (TDM), une détection non invasive de la présence d'une ischémie, la MRC, l'évaluation de l'iFR et l'échographie intravasculaire ont été effectuées au début de l'étude ainsi que lors d'un stress induit par l'adrénaline. La compression externe a également été évaluée au moyen d'une échographie intravasculaire. Résultats: Huit patients (63 % de sexe féminin; âge moyen de 53 ans ± 9,5 ans) ont participé à l'étude. Cinq patients (63 %) présentaient des symptômes, et l'angiographie par TDM a révélé une AAORCA à risque élevé chez tous les patients. Les résultats de la MRC et de l'évaluation de l'iFR étaient positifs chez seulement un patient (12,5 %), ce qui est attribuable en majeure partie à une athérosclérose diffuse concomitante. Chez deux patients (25 %), l'échographie intravasculaire a montré une compression externe de l'artère coronaire droite même si l'aire de l'ostium de l'artère n'avait pas changé. Une stratégie thérapeutique prudente a été employée pour tous les patients. Pendant la période de suivi qui a duré en moyenne 29,3 mois (écart-type : ± 2,6 mois), les symptômes se sont résorbés de manière spontanée chez quatre des cinq patients (80 %), et aucun événement cardiaque indésirable n'est survenu. Conclusion: Malgré une anatomie à risque élevé chez tous les patients, aucun d'entre eux ne présentait une ischémie connue, ce qui justifiait une stratégie thérapeutique prudente. Aucun événement cardiaque indésirable n'est survenu durant la période de suivi, et les symptômes se sont résorbés de manière spontanée chez la majorité des patients. À la lumière de ces renseignements, la MRC, l'évaluation de l'iFR et l'échographie intravasculaire lors d'un stress pharmacologique devraient faire l'objet d'autres études et pourraient éventuellement être utiles dans la stratification du risque d'ischémie et dans le choix des stratégies de prise en charge des patients adultes présentant une AAORCA.

Validation and Feasibility of Echocardiographic Assessment of Systemic Right Ventricular Function: Serial Correlation With MRI.

Background: Inherent to its geometry, echocardiographic imaging of the systemic right ventricle (RV) is challenging. Therefore, echocardiographic assessment of systemic RV function may not always be feasible and/or reproducible in daily practice. Here, we aim to validate the usefulness of a comprehensive range of 32 echocardiographic measurements of systemic RV function in a longitudinal cohort by serial assessment of their correlations with cardiac magnetic resonance (CMR)-derived systemic RV ejection fraction (RVEF). Methods: A single-center, retrospective cohort study was performed. Adult patients with a systemic RV who underwent a combination of both CMR and echocardiography at two different points in time were included. Off-line analysis of echocardiographic images was blinded to off-line CMR analysis and vice versa. In half of the echocardiograms, measurements were repeated by a second observer blinded to the results of the first. Correlations between echocardiographic and CMR measures were assessed with Pearson's correlation coefficient and interobserver agreement was quantified with intraclass correlation coefficients (ICC). Results: Fourteen patients were included, of which 4 had congenitally corrected transposition of the great arteries (ccTGA) and 10 patients had TGA late after an atrial switch operation. Eight patients (57%) were female. There was a mean of 8 years between the first and second imaging assessment. Only global systemic RV function, fractional area change (FAC), and global longitudinal strain (GLS) were consistently, i.e., at both time points, correlated with CMR-RVEF (global RV function: r = -0.77/r = -0.63; FAC: r = 0.79/r = 0.67; GLS: r = -0.73/r = -0.70, all p-values < 0.05). The ICC of GLS (0.82 at t = 1, p = 0.006, 0.77 at t = 2, p = 0.024) was higher than the ICC of FAC (0.35 at t = 1, p = 0.196, 0.70 at t = 2, p = 0.051) at both time points. Conclusion: GLS appears to be the most robust echocardiographic measurement of systemic RV function with good correlation with CMR-RVEF and reproducibility.

Prospective validation and assessment of cardiovascular and offspring risk models for pregnant women with congenital heart disease.

OBJECTIVES: Adequate prepregnancy prediction of maternal cardiovascular and offspring risk is important for counselling and management of pregnancy in women with congenital heart disease (CHD). Therefore we performed a study to identify the optimal assessment strategy for estimating the risk of pregnancy in women with CHD. METHODS: In this prospective study, we determined the outcomes of 213 pregnancies in 203 women with CHD. The ZAHARA I (Zwangerschap bij Aangeboren HARtAfwijkingen I) and CARPREG (CARdiac disease in PREGnancy) risk scores were calculated for each pregnancy, as was the total number of cardiovascular (TPc) or offspring risk predictors (TPo) from these and other studies combined. Pregnancies were also classified according to the modified WHO classification of maternal cardiovascular risk and according to disease complexity (DC). RESULTS: Maternal cardiovascular events occurred during 22 pregnancies (10.3%). Offspring events occurred during 77 pregnancies in 81 children (37.3%). Cardiovascular and offspring event rates increased with higher risk scores, higher TPc or TPo, higher WHO class and greater DC. The highest area under the curve (AUC) for maternal cardiovascular risk was achieved by the WHO class (AUC: 0.77, p<0.0001). AUC for the ZAHARA I risk score was 0.71 (p=0.001), and for the CARPREG risk score 0.57 (p=0.32). All models performed insufficiently in predicting offspring events (AUC≤0.6). CONCLUSIONS: The WHO classification is the best available risk assessment model for estimating cardiovascular risk in pregnant women with CHD. None of the offspring prediction models perform adequately in our cohort.

Illness perceptions of adults with congenital heart disease and their predictive value for quality of life two years later.

BACKGROUND: To improve patients' quality of life (QoL) we need to identify modifiable determinants, such as illness perceptions. Patients' illness perceptions are known to regulate emotional responses and health-behaviour. Illness perceptions comprise several components: consequences, control, coherence, changeability and emotional representations. AIMS: To examine (a) the relation between patient characteristics and illness perceptions, and (b) the independent predictive value of illness perceptions for future QoL. METHODS: A longitudinal study in 845 patients with congenital heart disease was conducted. Patients completed three questionnaires: the IPQ-R (illness perceptions) and two years later the SF-36 and TAAQOL-CHD (QoL). Linear regression analyses were performed relating illness perceptions to patient characteristics (sex, age, disease complexity and functional status) and QoL. RESULTS: Patients with a complex defect or poor functional status reported poor illness perceptions. Independent of patient characteristics, poor illness perceptions (i.e. a strong belief that the illness has severe consequences; a weak belief that you have a coherent illness understanding and that the illness can be controlled by treatment; and a strong belief that the illness is changeable and causes negative emotions) were predictive of future QoL. CONCLUSION: Illness perceptions independently predict QoL, suggesting that QoL may be improved by altering patients' beliefs about their illness. For example, increasing patients' knowledge regarding their disease and informing them about treatment opportunities may enhance their QoL.

The perspective of patients with congenital heart disease: does health care meet their needs?

OBJECTIVE: A first step in the delivery of tailored care is answering the following question: does health care meet the needs of patients? Therefore patients' perspective on health care use and their needs was examined. The design used was cross-sectional questionnaire study. PATIENTS: A total of 1109 adult congenital heart defect (CHD) patients attending one of eight Dutch hospitals were randomly selected from a national database (10% of all registered patients). MAIN OUTCOME MEASURES: Patient reported questionnaires on in- and outpatient health care use during the past year and need for additional care. RESULTS: A total of 66% and 40% of patients had contact with their cardiologist and general practitioner, respectively. Six to 10 percent were hospitalized, operated upon, or visited the emergency room. For the majority, the amount of contact was sufficient. Most patients indicated that the communication skills and expertise of the cardiologist and general practitioner were sufficient, and health care improvements were not necessary. Frequent health care users had a poor functional status and frequent contact with their cardiologist and general practitioner. Patients who want more contact with their cardiologist rated the communication skills of the cardiologist as insufficient. CONCLUSIONS: For most patients, the amount and quality of care are both sufficient. Patients who rate the communication skills of the cardiologist as insufficient have need more contact. In addition to the recommended training program as described in the American College of Cardiology/American Heart Association (ACC/AHA) and European Society of Cardiology (ESC) guidelines, we recommend the incorporation of communication training. This is the first study to provide insight into health care use and needs of CHD patients in countries with a compulsory health insurance system from the patient perspective.

Global and regional left ventricular function assessment with 16-detector row CT: comparison with echocardiography and cardiovascular magnetic resonance.

AIMS: To compare multidetector row computed tomography (MDCT) global and regional left ventricular (LV) function assessment with echocardiography and cardiovascular magnetic resonance (CMR). METHODS AND RESULTS: In 25 patients, who were referred for noninvasive angiography with 16-detector row CT, LV function assessment was also performed. A subsequent echocardiogram was performed, and in a subgroup of patients, CMR examination was completed to evaluate LV function. For global function assessment, the LV ejection fraction (LVEF) was calculated. Regional LV function was scored using a 17-segment model and a 4-point scoring system. MDCT agreed well with echocardiography for the assessment of LVEF (r=0.96; bias 0.54%; p<0.0001) and regional LV function (kappa=0.78). Eight patients had no contra-indications and gave informed consent for CMR examination. A fair correlation between MDCT and CMR was demonstrated in the assessment of LVEF (r=0.86; bias -1.5%; p<0.01). Regional LV function agreement between MDCT and CMR was good (kappa=0.86). CONCLUSION: MDCT agreed well with both echocardiography and CMR in the assessment of global and regional LV function. Global and regional LV function may accurately be evaluated by 16-detector row CT, and can be added to a routine CT image analysis protocol without need for additional contrast or imaging time.

Comprehensive assessment of patients after coronary artery bypass grafting by 16-detector-row computed tomography.

BACKGROUND: Multidetector-row computed tomography (MDCT) is a versatile modality to evaluate stenoses in native coronary arteries and bypass grafts. Acquired MDCT data can additionally be used to assess left ventricular ejection fraction (LVEF). The purpose was to use MDCT for the assessment of bypass graft and coronary artery disease combined with evaluation of LVEF. METHODS: Twenty-five patients underwent 16-detector-row CT examination and coronary angiography. Bypass grafts and nongrafted coronary artery segments at MDCT were evaluated on eligibility, patency, and > or = 50% stenosis. The MDCT data set was used to calculate LVEF and was divided into patients with no/subendocardial/transmural myocardial infarctions (MIs). RESULTS: Ninety vessels were evaluated: 14 arterial grafts/53 vein grafts/23 nongrafted vessels. Of 225 segments, 17 were ineligible for evaluation because of metal clips. With MDCT, patency in segments of arterial grafts/vein grafts/nongrafted vessels could be evaluated with high accuracy in 100%/100%/97% of segments. In arterial grafts, stenoses > or = 50% did not occur at angiography, which was for all eligible segments correctly diagnosed at MDCT. Stenosis > or = 50% could be correctly detected by MDCT with a sensitivity/specificity of 100%/94% for vein grafts and 100%/89% for nongrafted vessels. Negative predictive value was 100% for vein grafts and nongrafted vessels. In patients with transmural MI, MDCT revealed a significant lower LVEF as compared with patients without or with subendocardial MI (P < .05). CONCLUSION: Comprehensive assessment of bypass grafts, nongrafted vessels, and LVEF is feasible with MDCT. Owing to the high negative predictive value this noninvasive approach may be used as gatekeeper before coronary angiography.

Noninvasive angiography and assessment of left ventricular function using multislice computed tomography in patients with type 2 diabetes.

OBJECTIVE: Early identification of coronary artery disease (CAD) in patients with diabetes is important because these patients are at increased risk for CAD and have worse outcome than nondiabetic patients after CAD is diagnosed. Recently, noninvasive coronary angiography and assessment of left ventricular function has been demonstrated with multislice computed tomography (MSCT). The purpose of the present study was to validate this approach in patients with type 2 diabetes. RESEARCH DESIGN AND METHODS: MSCT was performed in 30 patients with confirmed type 2 diabetes. From the MSCT images, coronary artery stenoses (> or =50% luminal narrowing) and left ventricular function (left ventricular ejection fraction, regional wall motion) were evaluated and compared with results of conventional angiography and two-dimensional echocardiography. RESULTS: Two hundred twenty of 256 coronary artery segments (86%) were interpretable with MSCT. In these segments, sensitivity and specificity for detection of coronary artery stenoses were 95%. Including the uninterpretable segments, sensitivity and specificity were 81 and 82%, respectively. Bland-Altman analysis in the comparison of left ventricular ejection fractions demonstrated a mean difference of -0.48 +/- 3.8% for MSCT and echocardiography, which was not significantly different from 0. Agreement between the two modalities for assessment of regional contractile function was excellent (91%, kappa statistic 0.81). CONCLUSIONS: Accurate noninvasive evaluation of both the coronary arteries and left ventricular function with MSCT is feasible in patients with type 2 diabetes. This noninvasive approach may allow optimal identification of high-risk patients.

Feasibility of assessment of coronary stent patency using 16-slice computed tomography.

Intracoronary stent implantation is a frequently performed procedure in the treatment of stenoses in coronary arteries, but in-stent restenosis occurs in approximately 10% to 15% of patients. A noninvasive diagnostic procedure to evaluate in-stent restenosis would therefore be of great benefit. We investigated the feasibility of assessing stent patency with 16-slice computed tomography. Multislice computed tomography (MSCT) was performed in 22 patients with previously implanted stents. For each stent, assessability was determined and related to stent type and diameter. Subsequently, the presence of significant restenosis was determined in the evaluable stents. In addition, peristent lumina (5 mm proximal and distal to the stent) were evaluated. Conventional angiography in combination with quantitative coronary angiography served as the standard of reference. MSCT was performed successfully in all but 1 patient. Of 65 stents, 50 (77%) were determined assessable. Uninterpretable stents tended to have a thicker strut and/or a smaller diameter. In the evaluable stents, 7 of 9 stenoses were detected and the absence of restenosis was correctly identified in all 41 patent stents, resulting in a sensitivity and specificity of 78% and 100%, respectively. Sensitivity and specificity for the detection of peristent stenosis were 75% and 96%, respectively. In conclusion, MSCT may be useful in the assessment of stent patency and may function as a gatekeeper before invasive diagnostic procedures.

Employment in adults with congenital heart disease.

OBJECTIVE: To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. DESIGN: Cross-sectional study. SETTING: Patients were randomly selected from the archives of the Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. PATIENTS AND MAIN OUTCOME MEASURES: In total, 76 patients with complex CHD and 80 with mild CHD (age range, 17-32 years) completed a self-reported questionnaire on employment and handicaps, with reference data available (response rate, 70%). RESULTS: In the study groups, 45 (59%) of 76 patients with complex CHD had a paid job compared with 61 (76%) of 80 patients with mild CHD. Patients older than 25 years with complex CHD had significantly lower job participation (64%) than the general population (83%). Multiple logistic regression showed that type of CHD and level of education were significantly and independently related to job participation (odds ratio, 4.8; 99% confidence interval, 1.2-19.6; and odds ratio, 4.7; 99% confidence interval, 1.3-17.2, respectively). Of the 76 patients with complex CHD, 42 (55%) experienced disease-related career problems, in contrast to only 1 patient with mild CHD. Both CHD groups had more job-related mobility handicaps than did the reference group. However, in the mild CHD group, handicaps could be attributed to additional noncardiac diseases. CONCLUSIONS: Patients with complex CHD have reduced job participation compared with patients with mild CHD and the general population. Many receive disability benefits or experience career problems or job handicaps. Career counseling focusing on physical abilities and level of education may help prevent or reduce these job-related problems.

Exercise MR imaging in the assessment of pulmonary regurgitation and biventricular function in patients after tetralogy of fallot repair.

PURPOSE: To assess the responses of pulmonary regurgitation (PR) and biventricular function to submaximal exercise by using a magnetic resonance (MR) imaging exercise protocol with young adult patients who underwent tetralogy of Fallot repair at a young age. MATERIALS AND METHODS: Fifteen patients with corrected tetralogy of Fallot (mean age, 17.5 years +/- 2.5 [SD]) underwent MR imaging at rest and during exercise for the evaluation of PR and biventricular function. Results were compared with findings from 16 control subjects (mean age, 17.5 years +/- 2.3). Mean age at tetralogy of Fallot repair was 2.1 years +/- 1.6, and mean follow-up time after repair was 15.4 years +/- 2.6. Exercise level at MR imaging was calculated individually and corresponded to 60% of peak oxygen uptake. The parameters of cardiac function obtained at rest and during exercise were compared by using a paired t test. An unpaired t test was used to compare parameters of cardiac function between patients and control subjects. RESULTS: PR decreased during exercise (from 27 mL/m(2) +/- 17 to 23 mL/m(2) +/- 15; P =.012). At rest, right ventricular (RV) ejection fraction was normal (>47%) in 80% of patients. RV response to exercise in the patient group was abnormal compared with response in the control group, as demonstrated by an increase in RV end-diastolic volume index (132 mL/m(2) +/- 36 to 137 mL/m(2) +/- 38; P =.041) and no significant change in end-systolic volume index or ejection fraction. In only one patient, RV ejection fraction increased by more than 5%. Left ventricular response was not different between patients and control subjects. CONCLUSION: MR imaging is well suited to assess cardiac response to exercise, and findings revealed a decrease in PR and an abnormal RV response to exercise in patients with corrected tetralogy of Fallot.