Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BACKGROUND: Patients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. METHODS: A survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. RESULTS: In Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. CONCLUSIONS: Over the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey.

Background: The COVID-19 pandemic significantly impacted health care access across Canada with the reduction in in-person evaluations. The aim of the study was to examine the effects of the COVID-19 pandemic on access to health care services among the Canadian population with adult congenital heart disease (ACHD). Methods: All Canadian adult congenital heart affiliated centres were contacted and asked to collect data on outpatient clinic and procedural volumes for the 2019 and 2020 calendar years. A survey was sent detailing questions on clinic and procedural volumes and wait times before and after pandemic restrictions. Descriptive statistics were used with the Student t-test to compare groups. Results: In 2019, there were 19,326 ACHD clinic visits across Canada and only 296 (1.5%) virtual clinic visits. However, during the first year of the pandemic, there were 20,532 clinic visits and 11,412 (56%) virtual visits (P < 0.0001). There were no differences in procedural volumes (electrophysiology, cardiac surgery, and percutaneous intervention) between 2019 and 2020. The mean estimated wait times (months) before the pandemic vs the pandemic were as follows: nonurgent consult 5.4 ± 2.6 vs 6.6 ± 4.2 (P = 0.65), ACHD surgery 6.0 ± 3.5 vs 7.0 ± 4.6 (P = 0.47), electrophysiology procedures 6.3 ± 3.3 vs 5.7 ± 3.3 (P = 0.72), and percutaneous intervention 4.6 ± 3.9 vs 4.4 ± 2.3 (P = 0.74). Conclusions: During the pandemic and restrictions of social distancing, the use of virtual clinic visits helped to maintain continuity in ACHD clinical care, with 56% of ACHD visits being virtual. The procedural volumes and wait times for consultation and percutaneous and surgical interventions were not delayed.

Contexte: La pandémie de COVID-19 a eu des répercussions sur l'accès aux soins de santé partout au Canada, y compris une diminution des évaluations en personne. La présente étude visait à évaluer l'effet de la pandémie de COVID-19 sur l'accès aux soins de santé chez les adultes atteints de cardiopathie congénitale. Méthodologie: Nous avons communiqué avec tous les centres canadiens de prise en charge de la cardiopathie congénitale chez l'adulte et nous leur avons demandé de recueillir des données sur les consultations externes et le volume des interventions pour les années 2019 et 2020. Un sondage détaillé leur a été transmis sur les volumes de consultations et d'interventions et sur les temps d'attentes avant et après la mise en place de restrictions liées à la pandémie. Les groupes ont été comparés lors d'une analyse statistique descriptive utilisant le test t de Student. Résultats: En 2019, 19 326 consultations pour cause de cardiopathie congénitale chez l'vadulte ont été enregistrées au Canada, dont seulement 296 (1,5 %) ont eu lieu en mode virtuel. Au cours de la première année de la pandémie, 20 532 consultations ont été relevées; 11 412 (56 %) ont été menées virtuellement (p < 0,0001). Aucune différence n'a été observée dans le volume d'interventions (interventions en électrophysiologie, interventions chirurgicales et interventions percutanées) entre 2019 et 2020. Les temps d'attente moyens estimés en mois, avant et pendant la pandémie, étaient les suivants : consultations non urgentes, 5,4 ± 2,6 vs 6,6 ± 4,2 (p = 0,65); interventions chirurgicales, 6,0 ± 3,5 vs 7,0 ± 4,6 (p = 0,47); interventions en électrophysiologie, 6,3 ± 3,3 vs 5,7 ± 3,3 (p = 0,72); et interventions percutanées, 4,6 ± 3,9 vs 4,4 ± 2,3 (p = 0,74). Conclusion: Au cours de la pandémie et de la période où les restrictions de distanciation sociale étaient en vigueur, le recours aux consultations virtuelles dans les cliniques a contribué à la continuité des soins offerts aux adultes atteints de cardiopathie congénitale, puisque 56 % des visites se sont déroulées virtuellement. Le volume d'interventions n'a pas été touché et les temps d'attentes pour les consultations, les interventions percutanées et les interventions chirurgicales ne se sont pas allongés.

Arterial Age and Early Vascular Aging, But Not Chronological Age, Are Associated With Faster Thoracic Aortic Aneurysm Growth.

Background Aneurysm size is an imperfect risk assessment tool for those with thoracic aortic aneurysm (TAA). Assessing arterial age may help TAA risk stratification, as it better reflects aortic health. We sought to evaluate arterial age as a predictor of faster TAA growth, independently of chronological age. Methods and Results We examined 137 patients with TAA. Arterial age was estimated according to validated equations, using patients' blood pressure and carotid-femoral pulse wave velocity. Aneurysm growth was determined prospectively from available imaging studies. Multivariable linear regression assessed the association of chronological age and arterial age with TAA growth, and multivariable logistic regression assessed associations of chronological and arterial age with the presence of accelerated aneurysm growth (defined as growth>median in the sample). Mean±SD chronological and arterial ages were 62.2±11.3 and 54.2±24.5 years, respectively. Mean baseline TAA size and follow-up time were 45.9±4.0 mm and 4.5±1.9 years, respectively. Median (interquartile range) TAA growth was 0.31 (0.14-0.52) mm/year. Older arterial age (ß±SE for 1 year: 0.004±0.001, P<0.0001) was independently associated with faster TAA growth, while chronological age was not (P=0.083). In logistic regression, each 5-year increase in arterial age was associated with a 23% increase in the odds of accelerated TAA growth (95% CI, 1.085-1.394; P=0.001). Conclusions Arterial age is independently associated with accelerated aneurysm expansion, while chronological age is not. Our results highlight that a noninvasive and inexpensive assessment of arterial age can potentially be useful for TAA risk stratification and disease monitoring as compared with the current clinical standard (chronological age).

Aortic Stenosis Progression: A Systematic Review and Meta-Analysis.

BACKGROUND: Aortic valve stenosis is a progressive disorder with variable progression rates. The factors affecting aortic stenosis (AS) progression remain largely unknown. OBJECTIVES: This systematic review and meta-analysis sought to determine AS progression rates and to assess the impact of baseline AS severity and sex on disease progression. METHODS: The authors searched Medline, Embase, and the Cochrane Central Register of Controlled Trials from inception to July 1, 2020, for prospective studies evaluating the progression of AS with the use of echocardiography (mean gradient [MG], peak velocity [PV], peak gradient [PG], or aortic valve area [AVA]) or computed tomography (calcium score [AVC]). Random-effects meta-analysis was performed to evaluate the rate of AS progression for each parameter stratified by baseline severity, and meta-regression was performed to determine the impact of baseline severity and of sex on AS progression rate. RESULTS: A total of 24 studies including 5,450 patients (40% female) met inclusion criteria. The pooled annualized progression of MG was +4.10 mm Hg (95% CI: 2.80-5.41 mm Hg), AVA -0.08 cm2 (95% CI: 0.06-0.10 cm2), PV +0.19 m/s (95% CI: 0.13-0.24 m/s), PG +7.86 mm Hg (95% CI: 4.98-10.75 mm Hg), and AVC +158.5 AU (95% CI: 55.0-261.9 AU). Increasing baseline severity of AS was predictive of higher rates of progression for MG (P < 0.001), PV (P = 0.001), and AVC (P < 0.001), but not AVA (P = 0.34) or PG (P = 0.21). Only 4 studies reported AS progression stratified by sex, with only PV and AVC having 3 studies to perform a meta-analysis. No difference between sex was observed for PV (P = 0.397) or AVC (P = 0.572), but the level of confidence was low. CONCLUSIONS: This study provides progression rates for both hemodynamic and anatomic parameters of AS and shows that increasing hemodynamic and anatomic baseline severity is associated with faster AS progression. More studies are needed to determine if sex differences affect AS progression. (Aortic Valve Stenosis Progression Rate: A Systematic Review and Meta-Analysis; CRD42021207726).

Combining Aortic Size With Arterial Hemodynamics Enhances Assessment of Future Thoracic Aortic Aneurysm Expansion.

BACKGROUND: Thoracic aortic aneurysm (TAA) is a deadly disease whose current method for risk stratification (aneurysm size) is imperfect. We sought to evaluate whether combining aortic size with hemodynamic measures that reflect the aorta's function was superior to aortic size alone in the assessment of TAA expansion. METHODS: One hundred thirty-seven nonoperated participants with TAA were followed prospectively. Aortic stiffness and pulsatile hemodynamics were noninvasively assessed at baseline with a combination of arterial tonometry with echocardiography using validated methodology. Aneurysm growth was calculated from standard imaging modalities. Multivariable linear regression models adjusted for potential confounders evaluated the association of aneurysm size and arterial hemodynamics, alone and in combination, with TAA growth. RESULTS: Sixty-nine percent of participants were male. Mean ± SD age, baseline aneurysm size, follow-up, and aneurysm expansion were, respectively, 62.2 ± 11.4 years, 45.9 ± 4.0 mm, 4.5 ± 1.9 years, and 0.41 ± 0.46 mm/year. In the linear regression models, the standardised ß (ß∗) for the association of aneurysm size with aneurysm expansion was 0.178 (P = 0.044). This was improved by combining aortic size with most measures of aortic function, with ß∗ ranging from 0.192 (for aneurysm size combined with central diastolic blood pressure) to 0.484 (for aneurysm size combined with carotid-femoral pulse-wave velocity) (P ≤ 0.05 for each). CONCLUSIONS: Combining aneurysm size with measures of arterial function improves assessment of aneurysm growth over TAA size alone, which is the standard for clinical decisions in TAA. Thus, combining aneurysm size with measures of aortic function provides a clinical advantage in the assessment of TAA disease activity.

Estimated Aortic Pulse Wave Velocity Is Associated With Faster Thoracic Aortic Aneurysm Growth: A Prospective Cohort Study With Sex-Specific Analyses.

BACKGROUND: Thoracic aortic aneurysm (TAA) is associated with high morbidity and mortality, and there is a critical need for improved tools for risk assessment and prognostication. We have previously shown that aortic stiffness, measured from arterial tonometry (carotid-femoral pulse wave velocity [cfPWV]), is independently associated with TAA expansion. To increase clinical applicability, we sought to determine the association of mathematically estimated aortic pulse wave velocity (e-PWV) with TAA expansion. METHODS: One-hundred and five consecutive unoperated subjects with TAA were recruited. We used arterial tonometry to measure cfPWV and used mean arterial pressure and age to calculate e-PWV according to validated equations. Multivariable linear regression assessed associations of baseline e-PWV with future aneurysm growth. Given sex differences in TAA outcomes, sex-stratified analyses were performed. RESULTS: Seventy-eight percent of subjects were men. Mean ± standard deviation (SD) age, baseline aneurysm size, and follow-up time were 62.6 ± 11.4 years, 46.2 ± 3.8 mm, and 2.9 ± 1.0 years, respectively. Aneurysm growth was 0.43 ± 0.37 mm per year; e-PWV was independently associated with future aneurysm expansion (ß ± SE: 0.240 ± 0.085, P = 0.006). In sex-specific analyses, e-PWV was associated with aneurysm growth in both men (ß ± standard error (SE) : 0.076 ± 0.022, P = 0.001) and women (ß ± SE : 0.145 ± 0.050, P = 0.012), but the strength of association nearly twice as strong in women as in men. CONCLUSIONS: Greater aortic stiffness reflects worse aortic health and provides novel insights into disease activity; e-PWV is independently associated with TAA growth. This finding increases clinical applicability, as e-PWV can be estimated simply, quickly, and free of cost without the need for specialized equipment.

Natural History of Mitral Annular Calcification and Calcific Mitral Valve Disease.

BACKGROUND: The natural history of mitral annular calcification (MAC) and risk for developing calcific mitral valve disease (CMVD) have been poorly defined. The aim of this study was to evaluate the progression rate of MAC and of the development of CMVD. METHODS: Patients with MAC and paired echocardiograms ≥1 year apart between 2005 and 2019 were included. Progression rates from mild or moderate to severe MAC and to CMVD (defined as severe MAC and significant mitral stenosis and/or regurgitation) were assessed, along with potential association with sex. RESULTS: A total of 11,605 patients (mean age, 73 ± 10 years; 51% men) with MAC (78% mild, 17% moderate, 5% severe) were included and underwent follow-up echocardiography at 4.2 ± 2.7 years. Among patients with mild or moderate MAC, 33% presented with severe MAC at 10 years. The rate of severe MAC was higher in women than in men (41% vs 24% [P < .001]; hazard ratio, 1.3; P < .001) and in patients with moderate versus mild MAC (71% vs 22% [P < .001]; hazard ratio, 6.1; P < .001). At 10 years, 10% presented with CMVD (4%, 23%, and 60% in patients with mild, moderate, and severe MAC, respectively), which was predicted by female sex (15% vs 5%; P < .0001), even after adjustment for MAC severity (hazard ratio, 1.9; P < .001). CONCLUSION: In this large cohort of patients with MAC, progression to severe MAC was common and frequently resulted in CMVD. Female sex was associated with higher progression rates. MAC and CMVD are expected to dramatically increase as the population ages, highlighting the importance of a better understanding of the pathophysiology of MAC to develop effective preventive medical therapies.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Central Hypertension in Patients With Thoracic Aortic Aneurysms: Prevalence and Association With Aneurysm Size and Growth.

BACKGROUND: Hypertension (HTN) has the greatest population-attributable risk for aortic dissection and is highly prevalent among patients with thoracic aortic aneurysms (TAAs). Although HTN is diagnosed based on brachial blood pressure (bBP), central HTN (central systolic blood pressure [cSBP] ≥130 mm Hg) is of interest as it better reflects blood pressure (BP) in the aorta. We aimed to (i) evaluate the prevalence of central HTN among TAA patients without a diagnosis of HTN, and (ii) assess associations of bBP vs. central blood pressure (cBP) with aneurysm size and growth. METHODS: One hundred and five unoperated subjects with TAAs were recruited. With validated methodology, cBP was assessed with applanation tonometry. Aneurysm size was assessed at baseline and follow-up using imaging modalities. Aneurysm growth rate was calculated in mm/year. Multivariable linear regression adjusted for potential confounders assessed associations of bBP and cBP with aneurysm size and growth. RESULTS: Seventy-seven percent of participants were men and 49% carried a diagnosis of HTN. Among participants without diagnosis of HTN, 15% had central HTN despite normal bBP ("occult central HTN"). In these patients, higher central systolic BP (cSBP) and central pulse pressure (cPP) were independently associated with larger aneurysm size (ß ± SE = 0.28 ± 0.11, P = 0.014 and cPP = 0.30 ± 0.11, P = 0.010, respectively) and future aneurysm growth (ß ± SE = 0.022 ± 0.008, P = 0.013 and 0.024 ± 0.009, P = 0.008, respectively) while bBP was not (P > 0.05). CONCLUSIONS: In patients with TAAs without a diagnosis of HTN, central HTN is prevalent, and higher cBP is associated with larger aneurysms and faster aneurysm growth.

Single vs Serial Assessments of Arterial Hemodynamics to Predict Thoracic Aortic Aneurysm Expansion.

BACKGROUND: Thoracic aortic aneurysm (TAA) is a highly morbid disease. We have previously shown that baseline hemodynamic measures reflecting aortic function are associated with future TAA expansion. However, whether serial arterial hemodynamic assessment further improves TAA growth assessment remains unknown. Therefore, we aimed to compare single vs serial arterial hemodynamic assessments in the evaluation of future TAA growth. METHODS: Eighty-six unoperated participants with TAA underwent noninvasive arterial hemodynamic assessment using arterial tonometry and echocardiography at baseline and after 1 year. Aortic diameter was measured serially with the use of standard imaging modalities. Stepwise multivariable linear regression was used to assess associations of baseline and 1-year change (Δ) in arterial hemodynamic measures with TAA growth. RESULTS: Mean age was 62.7 ± 11.0 years; 79% were male. Mean aneurysm growth was 0.48 ± 0.54 mm/year after a follow-up of 2.96 ± 1.03 years. Yearly changes in arterial hemodynamic measures ranged from -3.2% to +4.2%. Linear regression results showed that while baseline arterial hemodynamic measures were independently associated with aneurysm growth (carotid-femoral pulse wave velocity: ß ± SE = 0.038 ± 0.013; aortic characteristic impedance: ß ± SE = 0.002 ± 0.001; proximal aortic compliance: ß ± SE = -0.011 ± 0.006; forward pressure wave amplitude: ß ± SE 0.009 ± 0.002; reflected pressure wave amplitude: ß ± SE = 0.017 ± 0.006; P < 0.05 for each), the 1-year Δ in these measures did not incrementally add to aneurysm growth assessment (P > 0.05 for each Δ). CONCLUSIONS: Although baseline measures of aortic function independently predict TAA expansion, 1-year changes in these measures do not improve this prediction. Thus, for TAA risk assessment purposes, a baseline assessment of aortic function may suffice, which simplifies its use for potential predictive algorithms.

Clinical implications of left atrial size adjustment: Impact of obesity.

BACKGROUND: American and European societies recommend using left atrial (LA) volume adjusted to body surface area (BSA) as the means of indexing LA volume to the patient's body size irrespective of morphometric characteristics. AIM: To evaluate the impact of obesity on LA volume indexation to BSA on the presence and degree of LA enlargement. METHODS: From our echocardiography database, we extracted all consecutive adults referred for a transthoracic echocardiography in 2019 (n=28,725; 64±17 years; 55% male; 31% obese [body mass index≥30kg/m2]). LA volume indexed to BSA was calculated using measured weight (LAMeas) and ideal weight (LAIdeal) calculated using the Devine Formula. RESULTS: LAMeas and LAIdeal were 35±17mL/m2 and 40±19mL/m2, respectively (P<0.0001); 13% were classified as having a normal LAMeas but LAIdeal enlargement overall, 25% in obese patients and 7% in non-obese patients (P<0.0001). The percentages of patients with no, mild, moderate and severe LA dilatation were 57%, 19%, 9% and 16%, respectively, using LAMeas, and 45%, 20%, 11% and 24%, respectively, using LAIdeal (kappa=0.57). Degree of LA enlargement differed in 8194 patients (29%); 96% of the disagreement was related to underestimation of the degree of LA enlargement using LAMeas. Agreement for the degree of LA enlargement was poor in obese and good in non-obese patients (kappa=0.28 and 0.71, respectively). As illustrative clinical implications, diastolic function grade was modified in 8.3% of patients with preserved ejection fraction and 10.8% of patients with reduced left ventricular ejection fraction/myocardial disease, and timing for intervention was potentially different in 12.9% of patients with primary mitral regurgitation. CONCLUSIONS: Indexing LA volume to measured BSA versus ideal BSA markedly underestimates the presence and severity of LA enlargement, especially in obese patients, with potential important clinical implications.

Risk prediction models for heart failure admissions in adults with congenital heart disease.

BACKGROUND: Heart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH. METHODS: Data source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18-64 (1995-2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18-64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios. RESULTS: The cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance. CONCLUSIONS: One eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH.

Cardiovascular outcomes of pregnancy in Turner syndrome.

OBJECTIVES: Women with Turner syndrome (TS) are frequently counselled against pregnancy due to lack of data and unclear aortic dissection risk. However, with advances in fertility therapy, more women with TS are contemplating pregnancy. This study compared rates of adverse cardiovascular (CV) outcomes among: (1) pregnant and non-pregnant women with TS and (2) pregnant women with TS with/without structural heart disease. METHODS: Retrospective analysis of pregnant and age-matched non-pregnant controls with TS (2005-2017) across 10 CV centres was done. Data were collected at initial evaluation in pregnancy and outcomes were assessed to 6 months postpartum. Adverse CV events were defined as CV death, aortic dissection/rupture and/or aortic intervention. Non-pregnant age-matched controls were followed over the same time period. RESULTS: Sixty-eight pregnancies were included (60 women, mean age 33 years, 48% primigravid, 49% fertility therapy, 80% structurally normal heart, 25% XO karyotype). Based on American Society of Reproductive Medicine criteria, 10 pregnancies occurred in women stratified to high-risk category. There were no CV events in the pregnant women or in the non-pregnant women with TS. Obstetric events complicated 12 (18%) pregnancies with 9 (13%) attributed to hypertensive disorder of pregnancy. Fetal events included small for gestational age neonates (18%), preterm delivery (15%) and fetal death (3%). CONCLUSIONS: This study helps to refine the approach to pregnancy in women with TS. Among women with TS without structural heart disease, pregnancy does not impose an increased risk of CV outcomes. Among women with TS with structural heart disease, the risk of pregnancy is not as prohibitive as previously described but does require ongoing evaluation.

Size-adjusted aortic valve area: refining the definition of severe aortic stenosis.

AIMS: Severe aortic valve stenosis (AS) is defined by an aortic valve area (AVA) <1 cm2 or an AVA indexed to body surface area (BSA) <0.6 cm/m2, despite little evidence supporting the latter approach and important intrinsic limitations of BSA indexation. We hypothesized that AVA indexed to height (H) might be more applicable to a wide range of populations and body morphologies and might provide a better predictive accuracy. METHODS AND RESULTS: In 1298 patients with degenerative AS and preserved ejection fraction from three different countries and continents (derivation cohort), we aimed to establish an AVA/H threshold that would be equivalent to 1.0 cm2 for defining severe AS. In a distinct prospective validation cohort of 395 patients, we compared the predictive accuracy of AVA/BSA and AVA/H. Correlations between AVA and AVA/BSA or AVA/H were excellent (all R2 > 0.79) but greater with AVA/H. Regressions lines were markedly different in obese and non-obese patients with AVA/BSA (P < 0.0001) but almost identical with AVA/H (P = 0.16). AVA/BSA values that corresponded to an AVA of 1.0 cm2 were markedly different in obese and non-obese patients (0.48 and 0.59 cm2/m2) but not with AVA/H (0.61 cm2/m for both). Agreement for the diagnosis of severe AS (AVA < 1 cm2) was significantly higher with AVA/H than with AVA/BSA (P < 0.05). Similar results were observed across the three countries. An AVA/H cut-off value of 0.6 cm2/m [HR = 8.2(5.6-12.1)] provided the best predictive value for the occurrence of AS-related events [absolute AVA of 1 cm2: HR = 7.3(5.0-10.7); AVA/BSA of 0.6 cm2/m2 HR = 6.7(4.4-10.0)]. CONCLUSION: In a large multinational/multiracial cohort, AVA/H was better correlated with AVA than AVA/BSA and a cut-off value of 0.6 cm2/m provided a better diagnostic and prognostic value than 0.6 cm2/m2. Our results suggest that severe AS should be defined as an AVA < 1 cm2 or an AVA/H < 0.6 cm2/m rather than a BSA-indexed value of 0.6 cm2/m2.

Aortic Stiffness, Central Blood Pressure, and Pulsatile Arterial Load Predict Future Thoracic Aortic Aneurysm Expansion.

Thoracic aortic aneurysm is a disease associated with high morbidity and mortality. Clinically useful strategies for medical management of thoracic aortic aneurysm are critically needed. To address this need, we sought to determine the role of aortic stiffness and pulsatile arterial load on future aneurysm expansion. One hundred five consecutive, unoperated subjects with thoracic aortic aneurysm were recruited and prospectively followed. By combining arterial tonometry with echocardiography, we estimated measures of aortic stiffness, central blood pressure, steady, and pulsatile arterial load at baseline. Aneurysm size was measured at baseline and follow-up with imaging; growth was calculated in mm/y. Stepwise multivariable linear regression assessed associations of arterial stiffness and load measures with aneurysm growth after adjusting for potential confounders. Mean±SD age, baseline aneurysm size, and follow-up time were 62.6±11.4 years, 46.24±3.84 mm, and 2.92±1.01 years, respectively. Aneurysm growth rate was 0.43±0.37 mm/y. After correcting for multiple comparisons, higher central systolic (ß±SE: 0.026±0.009, P=0.007), and pulse pressures (ß±SE: 0.032±0.009, P=0.0002), carotid-femoral pulse wave velocity (ß±SE: 0.032±0.011, P=0.005), amplitudes of the forward (ß±SE: 0.044±0.012, P=0.0003) and reflected (ß±SE: 0.060±0.020, P=0.003) pressure waves, and lower total arterial compliance (ß±SE: -0.086±0.032, P=0.009) were independently associated with future aneurysm growth. Measures of aortic stiffness and pulsatile hemodynamics are independently associated with future thoracic aortic aneurysm growth and provide novel insights into disease activity. Our findings highlight the role of central hemodynamic assessment to tailor novel risk assessment and therapeutic strategies to patients with thoracic aortic aneurysm.

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). METHODS: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. RESULTS: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. CONCLUSIONS: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.