Sex Differences in Outcomes of Adults With Isolated Coarctation of the Aorta.

Background: Data are limited about the effect (or lack thereof) of sex on clinical outcomes in adults with coarctation of the aorta (COA). The purpose of this study was to compare atherosclerotic cardiovascular disease (ASCVD) risk profile, blood pressure (BP) data, echocardiographic indices, and mortality between men and women with COA. Methods: Retrospective study of adults with COA, and no associated left-sided obstructive lesions, who received care at Mayo Clinic (2003-2022). ASCVD risk profile was assessed as the prevalence of hypertension, hyperlipidemia, type 2 diabetes, obesity, smoking history, and coronary artery disease. A 24-hour BP monitor was used to assess daytime and nighttime BP and calculate nocturnal dipping. Results: Of 621 patients with isolated COA, 375 (60%) were men, and 246 (40%) were women. Women had similar ASCVD risk profile and daytime BP as men. However, women had less nocturnal dipping (7 ± 5 mm Hg vs 16 ± 7 mm Hg, P < 0.001), higher pulmonary artery mean pressure (23 mm Hg [interquartile range: 16-31] vs 20 mm Hg [interquartile range: 15-28], P = 0.04), and higher pulmonary vascular resistance index (3.41 ± 1.14 WU · m2 vs 3.02 ± 0.76 WU · m2, P = 0.006). Female sex was associated with all-cause mortality (adjusted hazard ratio 1.26, 95% confidence interval 1.04-1.94) and cardiovascular mortality (adjusted hazard ratio 1.38, 95% confidence interval 1.09-2.18). Conclusions: Women had a higher risk of both cardiovascular mortality and all-cause mortality compared to the risks in men. This difference may be related to the higher-than-expected ASCVD risk factors, abnormal nocturnal blood pressure, and pulmonary hypertension observed in women in this cohort. Further studies are required to identify optimal measures to address these risk factors.

Contexte: Il existe peu de données sur l'issue clinique en fonction du sexe chez les adultes présentant une coarctation de l'aorte (CoA). Le but de cette étude consistait donc à comparer le profil de risque de maladie cardiovasculaire athéroscléreuse (MCVAS), les données relatives à la pression artérielle (PA), les indices échocardiographiques et le taux de mortalité chez des hommes et des femmes présentant une CoA. Méthodologie: Il s'agissait d'une étude rétrospective réalisée chez des adultes présentant une CoA en l'absence de lésions obstructives gauches, soignés à la clinique Mayo entre 2003 et 2022. Le profil de risque de MCVAS a été évalué en fonction de la prévalence de l'hypertension, de l'hyperlipidémie, du diabète de type 2, de l'obésité, des antécédents tabagiques et de la coronaropathie. Une surveillance sur 24 heures a été utilisée pour évaluer la PA diurne et nocturne, en plus de calculer la chute nocturne de la PA. Résultats: Parmi les 621 patients présentant une CoA isolée, 375 (60 %) étaient des hommes et 246 (40 %) étaient des femmes. Les femmes présentaient une PA diurne et un profil de risque de MCVAS semblables aux hommes. Elles présentaient néanmoins une chute nocturne de la PA moins prononcée (7 ± 5 mmHg vs 16 ± 7 mmHg, p < 0,001), une pression artérielle pulmonaire moyenne plus haute (23 mmHg [max.-min. : 16-31] vs 20 mmHg [max.-min. : 15-28], p = 0,04) et un indice de résistance vasculaire pulmonaire plus élevé (3,41 ± 1,14 UW · m2 vs 3,02 ± 0,76 UW · m2, p = 0,006). Le sexe féminin a été associé à un plus fort taux de mortalité toutes causes confondues (rapport de risques ajusté : 1,26; intervalle de confiance à 95 % : 1,04-1,94) et de mortalité cardiovasculaire (rapport de risques ajusté : 1,38; intervalle de confiance à 95 % : 1,09-2,18). Conclusions: Les femmes sont exposées à un risque de mortalité cardiovasculaire et de mortalité toutes causes confondues plus élevé que les hommes. Cette différence pourrait être attribuable au rôle plus important que prévu joué par les facteurs de risque de MCVAS ainsi qu'à la pression artérielle nocturne anormale et à l'hypertension pulmonaire chez les femmes de cette cohorte. D'autres études sont nécessaires pour savoir quels seraient les paramètres optimaux qui permettraient d'évaluer ces facteurs de risque.

Superiorly Based Posterior Pharyngeal Flaps: Using A Care Pathway to Optimize Speech and Airway Outcomes.

BACKGROUND: Pharyngeal flap (PF) surgery is effective at improving velopharyngeal sufficiency, but historical literature shows a concerning prevalence rate of obstructive sleep apnea (OSA), reported as high as 20%. Our institution has developed a protocol to minimize risk of postoperative obstructive complications and increase safety of PF surgery. We hypothesize that (1) preoperative staged removal of significant adenotonsillar tissue along with (2) multiview videofluoroscopy to guide patient-specific surgical approach via appropriately sized PFs can result in excellent speech outcomes while limiting occurrence of OSA. METHODS: This was a retrospective chart review of all patients with velopharyngeal insufficiency (VPI) (aged 2-20 years) seen at the University of Rochester from 2015 to 2022 undergoing PF surgery to correct VPI. Nasopharyngoscopy was used for surgical planning and airway evaluation. Patients with tonsillar and adenoid hypertrophy underwent staged adenotonsillectomy at least 2 months before PF. Multiview videofluoroscopy was used to identify anatomic causes of VPI and to determine PF width. Patients underwent polysomnography and speech evaluation before and at least 6 months after PF surgery. RESULTS: Forty-one children aged 8.5 ± 4.1 years (range, 4 to 18 years) who underwent posterior PF surgery for VPI were identified. This included 10 patients with 22q11.2 deletion and 4 patients with Pierre Robin sequence. Thirty-nine patients had both pre- and postoperative speech data and underwent both a pre- and postoperative sleep study. Polysomnography showed no significant difference in obstructive apnea-hypopnea index after posterior PF surgery (obstructive apnea-hypopnea index preop, 1.3 ± 1.2 events per hour; postop, 1.7 ± 2.1 events per hour; P = 0.111). Significant improvements in speech outcome were seen in patients who underwent PF (modified Pittsburgh score preop, 11.52 ± 1.37; postop, 1.09 ± 2.35; P < 0.05). CONCLUSIONS: Use of preoperative staged adenotonsillectomy as well as patient-specific PF dimensions results in effective resolution of VPI and a low risk of OSA.

Coarctation of Aorta With Tricuspid Aortic Valve Is Not Associated With Ascending Aortic Aneurysm.

BACKGROUND: Aortic aneurysm is common in patients with coarctation of aorta (COA), but it is unclear whether the risk of aortic aneurysms is due to COA or related to the presence of other risk factors such as bicuspid aortic valve (BAV) and hypertension. OBJECTIVES: The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms. METHODS: A total of 867 patients with COA (COA group) were matched 1:1:1 to 867 patients with isolated BAV (BAV group) and 867 patients without structural heart disease (SHD) (no-SHD group). The COA group was further subdivided into a COA+BAV subgroup (n = 304 [35%]), and COA with tricuspid aortic valve (TAV) (COA+TAV subgroup [n = 563 (65%)]). Aortic dimensions were assessed at baseline and at 3, 5, and 7 years. RESULTS: Compared with the no-SHD group, the COA+BAV subgroup had larger aortic root diameter (37 mm [Q1-Q3: 30-43 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001) and mid ascending aorta dimeter (34 mm [Q1-Q3: 29-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P = 0.008). Similarly, the BAV group had larger aortic root diameter (37 mm [Q1-Q3: 30-42 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001), and mid ascending aorta dimeter (35 mm [Q1-Q3: 30-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P < 0.001). Compared with the COA+TAV subgroup, the COA+BAV subgroup and BAV group were associated with larger aortic root and mid ascending aorta diameter at baseline and follow-up. The risk of acute aortic complications was low in all groups. CONCLUSIONS: These findings suggest that BAV (and not COA) was associated with ascending thoracic aorta dimensions, and that patients with COA+TAV were not at a greater risk of developing ascending aortic aneurysms as compared with patients without SHD.

Heart Failure Staging and Indications for Advanced Therapies in Adults with Congenital Heart Disease.

Heart failure (HF) is common in adults with congenital heart disease (CHD), and it is the leading cause of death in this population. Adults with CHD presenting with stage D HF have a poor prognosis, and early recognition of signs of advanced HF and referral for advanced therapies for HF offer the best survival as compared with other therapies. The indications for advanced therapies for HF outlined in this article should serve as a guide for clinicians to determine the optimal time for referral. Palliative care should be part of the multidisciplinary care model for HF in patients with CHD.

Nuclear Abnormalities in LMNA p.(Glu2Lys) Variant Segregating with LMNA-Associated Cardiocutaneous Progeria Syndrome.

The LMNA gene encodes lamin A and lamin C, which play important roles in nuclear organization. Pathogenic variants in LMNA cause laminopathies, a group of disorders with diverse phenotypes. There are two main groups of disease-causing variants: missense variants affecting dimerization and intermolecular interactions, and heterozygous substitutions activating cryptic splice sites. These variants lead to different disorders, such as dilated cardiomyopathy and Hutchinson-Gilford progeria (HGP). Among these, the phenotypic terms for LMNA-associated cardiocutaneous progeria syndrome (LCPS), which does not alter lamin A processing and has an older age of onset, have been described. Here, we present the workup of an LMNA variant of uncertain significance, NM_170707.2 c. 4G>A, p.(Glu2Lys), in a 36-year-old female with severe calcific aortic stenosis, a calcified mitral valve, premature aging, and a family history of similar symptoms. Due to the uncertainty of in silico predictions for this variant, an assessment of nuclear morphology was performed using the immunocytochemistry of stable cell lines to indicate whether the p.(Glu2Lys) had a similar pathogenic mechanism as a previously described pathogenic variant associated with LCPS, p.Asp300Gly. Indirect immunofluorescence analysis of nuclei from stable cell lines showed abnormal morphology, including lobulation and occasional ringed nuclei. Relative to the controls, p.Glu2Lys and p.Asp300Gly nuclei had significantly (p < 0.001) smaller average nuclear areas than controls (mean = 0.10 units, SD = 0.06 for p.Glu2Lys; and mean = 0.09 units, SD = 0.05 for p.Asp300Gly versus mean = 0.12, SD = 0.05 for WT). After functional studies and segregation studies, this variant was upgraded to likely pathogenic. In summary, our findings suggest that p.Glu2Lys impacts nuclear morphology in a manner comparable to what was observed in p.Asp300Gly cells, indicating that the variant is the likely cause of the LCPS segregating within this family.

Thoracic Aortic Aneurysmal Disease: Comprehensive Recommendations for the Primary Care Physician.

Thoracic aortic aneurysm (TAA) is a commonly encountered disease that is defined as aortic dilation with an increase in diameter of at least 50% greater than the expected age- and sex-adjusted size. Thoracic aortic aneurysms are described by their size, location, morphology, and cause. Primary care clinicians and other noncardiologists are often the first point of contact for patients with TAA. This review is intended to provide them with basic information on the differential diagnosis, diagnostic evaluation, and medical and surgical management of TAAs. Management decisions depend on having as precise a diagnosis as possible. Fortunately, this can often be achieved with a stepwise diagnostic approach that incorporates imaging and targeted genetic testing. Our review includes recommendations. In this review, we discuss these issues at a basic level and include recommendations for patients considering pregnancy.

Safety and Efficacy of Surgical and Percutaneous Cardiac Interventions for Adults With Down Syndrome.

Objective: To assess risks and benefits of cardiac intervention in adults with Down syndrome (DS). Patients and Methods: A retrospective review was conducted using data from a study we published in 2010. Patients aged 18 years or older with DS who underwent cardiac operation or percutaneous intervention from February 2009 through April 2022 (new cohort) were compared with patients in the previous study (January 1969 through November 2007; remote cohort) at Mayo Clinic. Results: In total, 81 adults (43 men; 38 women) with DS underwent 89 cardiac interventions (84 surgical; 5 percutaneous) at a mean age of 33 years. Twenty-six patients presented with complete atrioventricular canal defect (17%) or tetralogy of Fallot (15%). The most common adult procedures were valve interventions: mitral (31%), tricuspid (15%), and pulmonary (12%). Of pulmonary valve interventions in the new cohort, 33% were performed percutaneously. The postoperative mortality rate was low (1% total). The mean time between last operation and death was 16 years. Conclusion: Adults with DS can undergo cardiac operation and percutaneous intervention with low morbidity and mortality risk and good long-term survival.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Right Heart Reverse Remodeling and Prosthetic Valve Function After Transcatheter vs Surgical Pulmonary Valve Replacement.

BACKGROUND: There are limited data about postprocedural right heart reverse remodeling and long-term prosthesis durability after transcatheter pulmonary valve replacement (TPVR) and how these compare to surgical pulmonary valve replacement (SPVR). OBJECTIVES: This study sought to compare right heart reverse remodeling, pulmonary valve gradients, and prosthetic valve dysfunction after TPVR vs SPVR. METHODS: Patients with TPVR were matched 1:2 to patients with SPVR based on age, sex, body surface area, congenital heart lesion, and procedure year. Right heart indexes (right atrial [RA] reservoir strain, RA volume index, RA pressure, right ventricular [RV] global longitudinal strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline (preintervention), 1 year postintervention, and 3 years postintervention. Pulmonary valve gradients were assessed at 1, 3, 5, 7, and 9 years postintervention. RESULTS: There were 64 and 128 patients in the TPVR and SPVR groups, respectively. Among patients with TPVR, 46 (72%) and 18 (28%) received Melody (Medtronic) vs SAPIEN (Edwards Lifesciences) valves, respectively. The TPVR group had greater postprocedural improvement in RA reservoir strain and RV global longitudinal strain at 1 and 3 years. The TPVR group had a higher risk of prosthetic valve dysfunction mostly because of a higher incidence of prosthetic valve endocarditis compared to SPVR but a similar risk of pulmonary valve reintervention because some of the patients with endocarditis received medical therapy only. Both groups had similar pulmonary valve mean gradients at 9 years postintervention. CONCLUSIONS: These data suggest a more favorable right heart outcome after TPVR. However, the risk of prosthetic valve endocarditis and prosthetic valve dysfunction remains a major concern.

Determinants and prognostic implications of left atrial reverse remodelling after coarctation of aorta repair in adults.

AIMS: Left atrial (LA) dysfunction and atrial fibrillation are also relatively common in adults with coarctation of aorta (COA), and the severity of LA dysfunction is associated with a higher risk of atrial fibrillation in this population. The purpose of this study was to determine whether LA function improved after COA repair (LA reverse remodelling), and the relationship between LA reverse remodelling and atrial fibrillation. METHODS AND RESULTS: Retrospective cohort study of adults undergoing COA repair (2003-20). LA reservoir strain was assessed pre intervention and 12-24 months post intervention, using speckle tracking echocardiography. Incident atrial fibrillation was assessed from COA repair to last follow-up. Of 261 adults who underwent COA repair [age 37 ± 13 years; males 148 (57%)], 124 (47%) and 137 (53%) presented with native vs. recurrent COA, respectively. Of 261 patients, 231 (82%) and 48 (18%) underwent surgical and transcatheter COA repair, respectively. The LA reservoir strain increased from 32 ± 8% (pre intervention) to 39 ± 7% (post intervention), yielding a relative increase of 21 ± 5%. Older age [ß ± standard error (SE) -0.16 ± 0.09 per 5 years, P = 0.02], higher systolic blood pressure (ß ± SE -0.12 ± 0.04 per 5 mmHg, P = 0.005), and higher residual COA mean gradient (ß ± SE -0.17 ± 0.06 per 5 mmHg, P = 0.002) post intervention were associated with less LA reverse remodelling, after adjustment for sex, hypertension diagnosis, and left ventricular indices. LA reverse remodelling (hazard ratio 0.97, 95% confidence interval 0.96-0.98 per 1% increase from pre-intervention LA function, P = 0.006) was associated with a lower risk of atrial fibrillation after adjustment for age, sex, pre-intervention LA reservoir strain, and history of atrial fibrillation. CONCLUSION: COA repair resulted in improved LA function and decreased risk for atrial fibrillation, especially in patients without residual hypertension or significant residual COA gradient.

Trends in cardiac implantable electronic device utilization in adults with congenital heart disease: a US nationwide analysis.

BACKGROUND: Adults with congenital heart disease (ACHD) have increased risk of arrhythmias warranting implantation of cardiac implantable electronic devices (CIEDs), which may parallel the observed increase in survival of ACHD patients over the past few decades. We sought to characterize the trends and outcomes of CIED implantation in the inpatient ACHD population across US from 2005 to 2019. METHODS: A retrospective analysis of the Nationwide Inpatient Sample (NIS) identified 1,599,519 unique inpatient ACHD admissions (stratified as simple (85.1%), moderate (11.5%), and complex (3.4%)) using the International Classification of Diseases 9/10-CM codes. Hospitalizations for CIED implantation (pacemaker, ICD, CRT-p/CRT-d) were identified and the trends analyzed using regression analysis (2-tailed p < 0.05 was considered significant). RESULTS: A significant decrease in the hospitalizations for CIED implantation across the study period [3.3 (2.9-3.8)% in 2005 vs 2.4 (2.1-2.6)% in 2019, p < 0.001] was observed across all types of devices and CHD severities. Pacemaker implantation increased with each age decade, whereas ICD implantation rates decreased over 70 years of age. Complex ACHD patients receiving CIED were younger with a lower prevalence of age-related comorbidities, however, had a greater prevalence of atrial/ventricular tachyarrhythmias and complete heart block. The observed inpatient mortality rate was 1.2%. CONCLUSIONS: In a nationwide analysis, we report a significant decline in CIED implantation between 2005 and 2019 in ACHD patients. This may either be due to a greater proportion of hospitalizations resulting from other complications of ACHD or reflect a declining need for CIED due to advances in medical/surgical therapies. Future prospective studies are needed to elucidate this trend further.

Cardiac Reverse Remodeling and Changes in Heart Failure Indices After Transcatheter Tricuspid Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: There are limited data about changes in cardiac function (cardiac reverse remodeling) and heart failure indices after transcatheter tricuspid valve-in-valve replacement (TT-VIVR). The purpose of this study was to evaluate cardiac reverse remodeling and temporal changes in heart failure indices after TT-VIVR in adults with congenital heart disease. METHODS: Retrospective cohort study of adults with congenital heart disease that underwent TT-VIVR and had >6 months of follow-up (January 1, 2011, to April 30, 2023). Echocardiographic indices of cardiac remodeling and heart failure indices (New York Heart Association class, NT-proBNP (N-terminal pro-brain natriuretic peptide), glomerular filtration rate, and model for end-stage liver disease excluding international normalized ratio score) were assessed preintervention and at 1-, 3-, and 5-year postintervention. RESULTS: Of 39 patients (age 39 [32-46] years), 14 (36%) and 25 (64%) received Melody valve and Sapien valve prosthesis, respectively. At 1-year post-TT-VIVR, there was a temporal improvement in right atrial reservoir strain (17±8% versus 22±8%, P<0.001), right atrial volume (81 [59-108] versus 63 [48-82] mL/m2, P<0.001), right atrial pressure (12±4% versus 6±4%, P<0.001), and right ventricular global longitudinal strain (-15±7% versus -20±7%, P<0.001). Similarly, there was a temporal improvement in NT-proBNP, glomerular filtration rate, model for end-stage liver disease excluding international normalized ratio score, and New York Heart Association class. The temporal improvements in heart failure indices and valve function were maintained at 3- and 5-year post-TT-VIVR. CONCLUSIONS: Considering the significant mortality risk associated with reoperations for tricuspid valve replacement, these data suggest favorable outcomes after TT-VIVR, and support TT-VIVR as a viable alternative to surgical tricuspid valve replacement, especially in high-risk patients.

Diagnostic and Prognostic Role of Left Ventricular Strain Imaging in Adults with Coarctation of aorta.

The relative diagnostic and prognostic performance of left ventricular (LV) global longitudinal strain (LVGLS) compared with LV ejection fraction (LVEF) and the role of LVGLS for detecting the early stages of LV systolic dysfunction in adults with repaired coarctation of the aorta are unknown. This study aimed to address these knowledge gaps. We used a retrospective cohort study of adults with repaired coarctation of the aorta who underwent transthoracic echocardiogram (2003 to 2020). LV systolic function was assessed using LVEF (derived from volumetric analysis) and LVGLS (derived from speckle-tracking echocardiography). Of the 795 patients (age 36 ± 14 years), the mean LVEF and LVGLS were 62 ± 11% and 21 ± 4%, respectively. The prevalence of LV systolic dysfunction was higher when assessed using LVGLS than using LVEF (20% vs 6%, p <0.001). Of 795 patients, 94 (12%) patients died, of which 75 (9%) died from cardiovascular causes. LVGLS provided more robust prognostic power in predicting the all-cause mortality than LVEF, as evidenced by a higher C-statistic (0.743, 95% confidence interval 0.730 to 0.755 vs 0.782, 95% confidence interval 0.771 to 0.792, p <0.001). Furthermore, patients with normal LVEF in the setting of reduced LVGLS had a higher risk of all-cause mortality (than patients with normal LVGLS and LVEF) and were at risk for a temporal decrease in LVEF during follow-up. These findings suggest that the use of LVGLS for risk stratification can help identify high-risk patients and provide opportunities for interventions, which would, in turn, improve clinical outcomes. Further studies are required to empirically test these postulates.

Cardiac Catheterization in Adults with Ebstein Anomaly: Pathophysiologic Insights Regarding Surgical Repair and Prognosis.

The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA has not been well studied. Retrospective cohort of 52 adults with repaired and 36 with unrepaired EA undergoing right heart catheterization at Mayo Clinic, MN between 1993 and 2021. "Repaired" EA was defined as prior tricuspid valve repair and/or replacement (83% post-tricuspid valve replacement). Repaired patients were younger than those with unrepaired EA (41.3 ± 16.0 versus 50.6 ± 15.6 years, p = 0.008) and had a lower prevalence of ≥ moderate native or prosthetic tricuspid regurgitation (67% versus 81%, p = 0.01). Right atrial (RA) pressure was higher among patients with repaired EA than in unrepaired disease [13 (11; 18) versus 10 (8; 15) mmHg; p = 0.02], but these differences were no longer present when adjusting for ≥ moderate right ventricular systolic dysfunction and ≥ moderate tricuspid regurgitation. Cardiac index (Qs) was lower among those with unrepaired EA than in repaired EA (1.9 ± 0.7 versus 2.3 ± 0.6 l/min/m2, p = 0.01), even after adjusting for similar confounders. During a follow-up of 8.6 (3.2-13.3) years, 16 (18%) patients died. Systolic pulmonary artery pressure was independently associated with all-cause mortality. In summary, higher cardiac indices were found in those with repaired EA compared to those with unrepaired disease. RA hypertension was prevalent in both groups and no differences in right filling pressures were found between groups after adjusting for potential confounders. Elevation in pulmonary pressures was independently associated with survival. The use of pulmonary vasomodulators in EA requires further investigation.