Enhanced Assessment of Perioperative Mortality Risk in Adults With Congenital Heart Disease.

BACKGROUND: In-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment. OBJECTIVES: The purpose of this study was to assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery. METHODS: We assessed perioperative survival in patients aged >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable-derived PEACH (PErioperative ACHd) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. RESULTS: The development cohort comprised 1,782 procedures performed during the study period. Re-sternotomy was undertaken in 897 (50.3%). There were 31 (1.7%) in-hospital deaths. The PEACH score showed excellent discrimination ability (area under the curve [AUC]: 0.88; 95% CI: 0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC: 0.69; 95% CI: 0.6-0.78; P = 0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), and ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC: 0.75; 95% CI: 0.72-0.77) and was well calibrated (Hosmer-Lemeshow chi-square goodness-of-fit P = 0.55). There was agreement in expected and observed perioperative mortality between cohorts. CONCLUSIONS: The PEACH score is a simple, novel perioperative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery.

Added value of three-dimensional transthoracic echocardiography in assessment of an adult patient with atrioventricular septal defect.

Transthoracic echocardiography is the principal imaging modality for assessment of patients with atrioventricular septal defects. Three-dimensional echocardiography streamlines and simplifies data acquisition offering a unique realistic en-face display of heart valves and septal defects and enables accurate evaluation of the cardiac anatomy, dynamic, and function. We demonstrated an added value of three-dimensional echocardiography in assessment of an adult patient with atrioventricular septal defect and its advantages over conventional echocardiography.

Choice and competition between adult congenital heart disease centers: evidence of considerable geographical disparities and association with clinical or academic results.

BACKGROUND: Although concentrating adult congenital heart disease services at high-volume centers has been widely advocated, the potential beneficial effects of competition and patient choice have received relatively little attention. We aimed to assess the degree of patient choice and competition between adult congenital heart disease units and to investigate whether competition indices correlate with clinical quality or research output. METHODS AND RESULTS: Competition between the 10 major adult congenital heart disease units in England was evaluated based on the Herfindahl-Hirschman Index, representing the sum of squared market shares of individual units. In addition, to account for geography and feasible access, we calculated spatial indices of competition based on travel time by road. These indices were correlated with 30-day mortality postpulmonary valve replacement in adult patients (as obtained from the National Central Cardiac Audit Database) and the aggregate research impact factors of individual centers. On a national level, a high level of competition without obvious dominant players was found (Herfindahl-Hirschman Index between 0.107 and 0.013). When accounting for geography, however, important disparities in patient choice and competition faced by individual centers emerged. The degree of local competition was correlated significantly with clinical outcomes and research output. In contrast, no association between center volume and outcome could be established. CONCLUSIONS: Beyond the usual focus on concentrating services at high-volume centers, the potentially beneficial effects of competition should not be ignored. Therefore, policymakers should consider fostering a competitive environment for adult congenital heart disease centers or at least avoiding creating government-granted monopolies in the field.

Non-invasive assessment of pulmonary blood flow using an inert gas rebreathing device in fibrotic lung disease.

BACKGROUND AND AIMS: Pulmonary hypertension (PH) is increasingly recognised in patients with diffuse lung disease, and is associated with increased mortality. Cardiac output (CO) is a prognostic marker in PH. Non-invasive assessment of pulmonary blood flow (PBF(INNOCOR)) with the inert gas rebreathing Innocor device has been validated against CO in PH, but not in PH associated with parenchymal lung disease. PBF(INNOCOR) may be less accurate in patients with lung disease because of intrapulmonary shunting and/or incomplete gas mixing. Our aim was to determine the variability of PBF(INNOCOR) in normal subjects, before evaluating PBF(INNOCOR) in diffuse lung disease against CO measured by the indirect Fick method (CO(FICK)) at right heart catheterisation (RHC). METHODS AND RESULTS: 23 normal subjects had lung volume measurements by a constant-volume body plethysmograph and three consecutive PBF(INNOCOR) measurements on the same day. 20 subjects returned for repeat assessment. PBF(INNOCOR) had good intrasession repeatability (coefficient of variation (CV)=6.57%) and intersession reproducibility (mean CO difference=0.13; single determinant SD=0.49; CV=9.7%). 28 consecutive patients with lung fibrosis referred for RHC had PBF(INNOCOR) measured within 24 h of RHC. There was good agreement between CO(FICK) and PBF(INNOCOR), with no evidence of systematic bias (mean CO(FICK) 4.3+/-1.0; PBF(INNOCOR) 4.0+/-1.2l/ min; p=0.07). Bland-Altman analysis revealed a mean difference of -0.32 and limits of agreement of -2.10 to +1.45. CONCLUSION: Non-invasive PBF measured by the inert gas rebreathing Innocor device has good intrasession repeatability and intersession reproducibility. In diffuse lung disease, CO can be accurately and non-invasively measured by the Innocor device.

A single, comprehensive non-invasive cardiovascular assessment in pulmonary arterial hypertension: combined computed tomography pulmonary and coronary angiography.

BACKGROUND: Comprehensive assessment of pulmonary arterial hypertension (PAH) should identify structural causes and subsequent cardiopulmonary consequences of PAH. This currently requires the use of several imaging modalities. Computed tomography (CT) is routinely used for pulmonary angiography (CTPA). Our aim was to assess whether combined pulmonary and coronary angiography (CTPCA) using ECG-gated, multi-detector CT (MDCT) would allow satisfactory pulmonary angiography, coronary angiography and ventriculography to be combined into a single acquisition using a single imaging modality. METHODS: We assessed CTPCA in 30 consecutive adult patients (mean age 41+/-11 years) with a diagnosis of PAH. In addition to the standard assessment of lung parenchyma and pulmonary vasculature, we assessed the ability of CT to satisfactorily visualise coronary vessels and biventricular function. Functional analysis included: end-diastolic volume (EDV), end-systolic volume (EDV), stroke volume (SV) and ejection fraction (EF) and mass and these parameters were correlated with same day cardiovascular magnetic resonance (CMR). RESULTS: Lung parenchyma, pulmonary and coronary vessels were fully visualised in all cases. Ventriculography correlated well with same day CMR (RVEDV r=0.94, +19.5+/-49.2 ml, RVESV r=0.93, +11.1+/-46.4 ml, RVSV r=0.60, +8.5+/-41.6 ml, RVEF r=0.77, -0.5+/-21.3% and RV mass r=0.73, -17.3+/-60.4 g, LVEDV r=0.68, +12.2+/-110 ml, LVESV r=0.69, +7.5+/-59.7 ml, LVSV r=0.54, +2.5+/-40.6 ml, LVEF r=0.73, -1.9+/-20.8% and LV mass r=0.87, -20.5+/-22.5 g (all p<0.001)). Associated congenital cardiovascular malformations were characterised in 22/30 cases. CONCLUSIONS: A CTPCA protocol allows safe, fast, comprehensive, non-invasive assessment of the possible anatomical causes and cardiopulmonary sequelae of PAH in adult patients, demonstrating congenital heart abnormalities, coronary artery disease and cardiac function.

Adult congenital heart disease: a 2008 overview.

INTRODUCTION: During the past decades, health care of patients born with congenital heart disease (CHD) has improved substantially, leading to a growing population of adult survivors. SOURCE OF DATA: Using the recently published and relevant data on adult CHD (ACHD), we reviewed the most common congenital heart defects and discussed important related issues. AREAS OF AGREEMENT: Adults with CHD most often require specialized medical or surgical care in a tertiary centre. However, this population also need local follow-up; general practitioners and other specialists therefore have to face the complexity of their disease. AREAS OF CONTROVERSIES: Management of pregnancy, non-cardiac surgery, arrhythmias and endocarditis prophylaxis may be challenging in patients with CHD and should be adapted to their condition. GROWING POINTS: The present article summarizes key clinical information on ACHD for the benefit of physicians who are not specialized in this field. Areas timely for developing research Research efforts and education strategies are greatly needed in order to optimize the care of patients with ACHD.

Survey of specialized tertiary care facilities for adults with congenital heart disease.

BACKGROUND: Specialized tertiary care facilities developed in response to the increasing numbers of adults with congenital heart disease (CHD). Because this patient population comprises a relatively new area of specialized cardiovascular interest, the first facilities necessarily evolved without preexisting guidelines or interaction. OBJECTIVES: To characterize the major features of the six original and largest tertiary adults CHD facilities. METHODS: Written questionnaire sent to six participating facilities in North America and Europe. Information was analyzed centrally. RESULTS: All but one facilities was established over 20 years ago, and each cares for over 1500 patients. Hospital admissions ranged from 100 to 660 patients/unit/year. Of the total number of registered patients, 52-81% had undergone one or more reparative surgeries. Reoperations constituted 25-80% of the 50-170 operations/unit/year. Overall mean surgical mortality was 1.9%/year. Inpatient and outpatient care was provided in adult (n = 4) or both adult and pediatric (n = 2) settings. All six facilities enjoyed close collaboration between adult and pediatric cardiologists, cardiac surgeons, nurse specialists and cardiac and non-cardiac consultants. Training and research were pivotal activities. CONCLUSIONS: Provision of comprehensive care by multidisciplinary teams including adult and pediatric cardiologists, cardiac surgeons, specialized nurses and other cardiac and non-cardiac consultants was the unifying feature for all six tertiary care facilities reported here. There were minor differences among them based on available resources, local expertise and national health care policies. There appears to be a significant shortfall in tertiary care provision for the adult with CHD that requires further planning and resource allocation. These data may be useful for new and evolving adult CHD services.

Comparison of transthoracic echocardiography versus cardiovascular magnetic resonance imaging for the assessment of ventricular function in adults after atrial switch procedures for complete transposition of the great arteries.

Adult patients with a history of transposition of the great arteries and atrial switch operation were studied with transthoracic echocardiography and cardiovascular magnetic resonance imaging. Measurements of ventricular dimensions and function by these 2 techniques in 18 consecutive patients were well correlated.

Doppler-echocardiographic assessment of pulmonary regurgitation in adults with repaired tetralogy of Fallot: comparison with cardiovascular magnetic resonance imaging.

AIMS: The purpose of this study was to compare the noninvasive assessment of severity of pulmonary regurgitation with Doppler echocardiography versus cardiovascular magnetic resonance imaging (CMR) in adult patients with repaired tetralogy of Fallot (rTOF). METHODS: We studied 52 (22 females) consecutive patients (aged 32 +/- 2 years, 23 +/- 5 years after rTOF) using Doppler echocardiography and compared these findings with CMR. From the continuous-wave Doppler trace, the duration of pulmonary regurgitation and of total diastole was measured and the ratio between the 2 was defined as pulmonary regurgitation index (PRi). Pulmonary regurgitant fraction (PRF) was assessed with flow phase velocity mapping with CMR. RESULTS: Patients were divided into 2 groups according to the median value (24.5%) of PRF measured by CMR: Group I (26 patients) with PRF < or =24.5% and Group II with PRF >24.5%. There was no difference between patients' age, sex, or age at repair between the 2 groups. More patients from Group II had a right ventricular outflow or transannular patch repair compared to Group I (12/26 [46%] vs 6/26 [23%], P <.01). Mean pulmonary regurgitation time was shorter (340 +/- 60 vs 440 +/- 135 ms, P =.001) and PRi was lower (0.61 +/- 0.11 vs 0.91 +/- 0.11, P <.001) in Group II compared to Group I. Color Doppler regurgitant jet was also broader in Group II (1.4 +/- 0.4 vs 0.7 +/- 0.5 cm, P <.001), signifying more severe pulmonary regurgitation. Doppler-measured PRi correlated closely with CMR regurgitant fraction (r = -0.82, P <.001) and with color Doppler pulmonary regurgitant jet width (r = -0.66, P <.001); the latter correlated with PRF assessed with CMR (r = 0.72, P <.001). A PRi <0.77 had 100% sensitivity and 84.6% specificity for identifying patients with pulmonary regurgitant fraction >24.5%, with a predictive accuracy of 95%. Furthermore, echocardiographically-assessed right ventricular end-diastolic dimensions correlated with CMR end-diastolic volume index (r = 0.49, P <.001 ). CONCLUSIONS: Pulmonary regurgitation is common in asymptomatic adults with rTOF. Severity of pulmonary regurgitation and its effects on right ventricular dimensions in these patients can be assessed noninvasively by Doppler echocardiography and CMR, with reasonable agreement between the 2 techniques.

Risky business: insuring adults with congenital heart disease.

Accurate prognostication in congenital heart disease is vital for purposes of obtaining insurance, yet can be problematic for patients, physicians and insurers. This article discusses the scope of the problem, and describes the process of evaluating life insurance. Mortality data as well as predictors of adverse outcomes for individual congenital heart lesions are reviewed. Practical tips for patients and their physicians are given to aid in successful application for insurance. To expand the possibility of future patients obtaining insurance coverage, the ongoing reporting and constant updating of very long-term survival data in congenital heart disease is emphasized.