Functional Capacity Assessment in Adults After Fontan Palliation: A Cardiopulmonary Exercise Test-Invasive Exercise Hemodynamics Correlation Study.

Although cardiopulmonary exercise testing (CPET) parameters have known prognostic value in adults after Fontan palliation, there are limited data correlating treadmill CPET with invasive exercise hemodynamics. Furthermore, the invasive hemodynamic underpinnings of exercise limitations have not been thoroughly investigated. This is retrospective analysis of 55 adults (≥18 years) after Fontan palliation who underwent treadmill CPET before invasive exercise hemodynamic testing by way of supine cycle protocol between November 2018 and April 2023. The median age was 32.2 (24.1 to 37.2) years. The peak heart rate (HR) was 139.7 ± 28.1 beats per minute and the peak oxygen consumption (VO2) was 19.1 ± 5.7 ml/kg/min (47.4 ± 13.5% predicted). VO2/HR was directly related to exercise stroke volume index (r = 0.50, p = 0.0002), whereas no association was seen with exercise arterio-mixed venous O2 content difference (r = 0.14, p = 0.32). Peak HR was inversely related to exercise pulmonary artery (PA) pressures (r = -0 61, p <0.0001) and PA wedge pressures (PAWP) (r = -0.61, p <0.0001). Moreover, %predicted VO2 was inversely related to exercise PA pressures (r = -0.50, p <0.0001) and PAWP (r = -0.55, p <0.0001). Peak VO2 ≤19.1 ml/kg/min had a sensitivity of 81% and a specificity of 76% (area under the curve = 0.82) for predicting a ΔPAWP/ΔQs ratio >2 mm Hg/L/min and/or a ΔPA:ΔQp >3 mm Hg/L/min, whereas a predicted peak VO2 ≤48% had a sensitivity of 74% and a specificity of 81% (area under the curve = 0.79) for the same parameters. In summary, lower peak HR and peak VO2 were associated with higher exercise PAWP and PA pressure. Peak VO2 ≤48% predicted provided the optimal cutoff for predicting increased indexed exercise PAWP or PA pressures; therefore, low peak VO2 should alert clinicians of abnormal underlying hemodynamics.

Fontan hemodynamics in adults with obesity compared to overweight and normal body mass index: a retrospective invasive exercise study.

AIMS: The effects of obesity on Fontan hemodynamics are poorly understood. Accordingly, we assessed its impact on exercise invasive hemodynamics and exercise capacity. METHODS: Seventy-seven adults post-Fontan undergoing exercise cardiac catheterization (supine cycle protocol) were retrospectively identified using an institutional database and categorized according to the presence of obesity (body mass index [BMI] >30 kg/m2) and overweight/normal BMI (BMI≤30 kg/m2). RESULTS: There were 18 individuals with obesity (BMI 36.4±3 kg/m2) and 59 (BMI 24.1±3.6 kg/m2) with overweight/normal BMI. Peak oxygen consumption (VO2) on noninvasive cardiopulmonary exercise testing was lower in patients with obesity (15.6±3.5 vs 19.6±5.8 ml/kg/min, p=0.04). At rest, systemic flow (Qs) (7.0 [4.8; 8.3] vs 4.8 [3.9; 5.8] l/min, p=0.001), pulmonary artery (PA) pressure (16.3±3.5 vs 13.1±3.5 mmHg, p=0.002), and PA wedge pressure (PAWP) (11.7±4.4 vs 8.9±3.1 mmHg, p=0.01) were higher, while arterial O2 saturation was lower (89.5% [86.5; 92.3] vs 93% [90; 95]) in obesity compared to overweight/normal BMI. Similarly, patients with obesity had higher exercise PA pressure (29.7±6.5 vs 24.7±6.8 mmHg, p=0.01) and PAWP (23.0±6.5 vs 19.8±7.3 mmHg, p=0.047), but lower arterial O2 saturation (82.4±7.0% vs 89% [85; 92], p=0.003). CONCLUSION: Adults post-Fontan with obesity have worse aerobic capacity, increased Qs, higher filling pressures, and decreased arterial O2 saturation compared to those with overweight/normal BMI, both at rest and during exercise, mirroring the findings observed in the obesity phenotype of heart failure with preserved ejection fraction. Whether treating obesity and its cardiometabolic sequelae in Fontan patients will improve hemodynamics and outcomes requires further study.

Seventy-seven adults post-Fontan undergoing exercise cardiac catheterization (supine cycle protocol) at Mayo Clinic, MN were categorized according to according to the presence of obesity (body mass index [BMI] >30 kg/m2) and overweight/normal BMI (BMI≤30 kg/m2). Adults post-Fontan with obesity have worse aerobic capacity, increased cardiac output, higher filling pressures, decreased arterial O2 saturation, and lower systemic vascular resistance compared to individuals with overweight or normal BMI, both at rest and during exercise.These findings mirror the observations in the obesity phenotype of heart failure with preserved ejection fraction. Whether treating obesity and its cardiometabolic sequelae in Fontan patients will improve hemodynamics and outcomes requires further study.

Relationship between obesity, cardiac hemodynamics, and heart failure in adults with coarctation of aorta.

Objectives: Patients with coarctation of aorta (COA) have arterial stiffening and left ventricular (LV) diastolic dysfunction similar to patients with heart failure with preserved ejection fraction (HFpEF) and obese subjects. However, the relationship between obesity, cardiac hemodynamics, and HF in adults with COA is unknown. The purpose of this study was to compare cardiac hemodynamics and prevalence of HFpEF between COA patients with vs without obesity, and to assess the relationship between obesity and HFpEF in this population. Methods: Adults with COA who underwent right heart catheterization were divided into an obese group (body mass index, BMI > 30 kg/m2) or a non-obese group (BMI ≤ 30 kg/m2). We also selected a control group of subjects without structural heart disease and with normal invasive hemodynamics at rest (n = 36). HFpEF was defined as having clinical symptoms of HF (exertional dyspnea or fatigue), LV ejection fraction of at least 50%, and pulmonary artery wedge pressure (PAWP) greater than 15 mm Hg at rest. Results: Of 99 COA patients, 29 (29%) had obesity. The obese COA group had higher right atrial pressure and PAWP, and worse pulmonary and systemic vascular function compared with the non-obese COA group and the control group. The overall prevalence of HFpEF in adults with COA was 32%, and the prevalence was higher in COA patients with obesity (55%) compared with those without obesity (23%). Obesity was associated with HFpEF after adjustment for demographic indices, comorbidities, and vascular function. Conclusions: The abnormal hemodynamics and higher prevalence of HFpEF in COA patients with obesity underscores the need for intervention to address obesity in this population.

Cardiac remodelling during pregnancy in women with congenital heart disease and systemic left ventricle.

AIMS: Women with congenital heart disease (CHD) are at risk of pregnancy-related adverse outcomes (PRAO). The purpose of this study was to assess temporal changes in cardiac structure and function (cardiac remodelling) during pregnancy, and the association with PRAO in women with CHD. METHODS AND RESULTS: Retrospective study of pregnant women with CHD and serial echocardiograms (2003-2021). Cardiac structure and function were assessed at pre-specified time points: prepregnancy, early pregnancy, late pregnancy, and postnatal period. PRAO was defined as the composite of maternal cardiovascular, obstetric, and neonatal complications. The study comprised 81 women with CHD (age, 29 ± 5 years). Compared to the baseline echocardiogram, there was a relative increase in right ventricular systolic pressure (RVSP) (relative change 13 ± 5%, P < 0.001, in early pregnancy; and 18 ± 5%, P < 0.001, in late pregnancy). There was a relative decrease in right ventricle free wall strain (RVFWS) (relative change -11 ± 3%, P < 0.001, in late pregnancy; and -11 ± 4%, P = 0.003, in postnatal period), and a relative decrease in RVFWS/RVSP (relative change, -10 ± 5%, P = 0.02 in early pregnancy, -26 ± 7%, P < 0.001, in late pregnancy, and -14 ± 5%, P < 0.001, in postnatal period). Baseline right ventricular to pulmonary arterial (RV-PA) coupling, and temporal change in RV-PA coupling were associated with PRAO, after adjustment for maternal age and severity of cardiovascular disease. CONCLUSION: Women with CHD had a temporal decrease in RV systolic function and RV-PA coupling, and these changes were associated with PRAO. Further studies are required to delineate the aetiology of deterioration in RV-PA coupling during pregnancy, and the long-term implications of right heart dysfunction observed in the postnatal period.

Differences in Right Heart Function After Pulmonary Valve Replacement in Patients With Pulmonary Valve Stenosis Versus Tetralogy of Fallot.

BACKGROUND: There are limited data about the impact of timing of pulmonary valve replacement (PVR) on right heart reverse remodeling in patients with pulmonary regurgitation following intervention for isolated pulmonary valve stenosis (PS). This study compared differences in postprocedural right heart reverse remodeling after early versus late PVR (defined as PVR before versus after attainment of the conservative consensus criteria proposed by Bokma et al, 2018) in patients with prior intervention for PS, using patients with tetralogy of Fallot as the reference group. METHOD AND RESULTS: Right atrial reservoir strain and right ventricular free wall strain was measured at baseline, 1 and 3 years after PVR. There were 114 patients with PS (early PVR, 87 [76%]; late PVR, 27 [24%]) and 291 patients with tetralogy of Fallot (early PVR, 197 [67%]; late PVR, 96 [33%]). The PS group had greater improvement in right atrial reservoir strain at 1 year (12%±4% versus 8%±4%; P<0.001) and 3 years (15%±6% versus 9%±6%; P<0.001), and a greater improvement in right ventricular free wall strain at 1 year (12%±4% versus 7%±3%, P=0.008) and 3-years (16%±6% versus 12%±5%; P=0.01) after PVR compared with the tetralogy of Fallot group. There was no difference in right heart reverse remodeling between patients who underwent early versus later PVR within the PS group. In contrast, late PVR was associated with less right heart reverse remodeling within the tetralogy of Fallot group. CONCLUSIONS: These data suggest that patients with palliated PS presenting pulmonary regurgitation have a more benign clinical course, and hence delaying PVR in this population may be appropriate.

Outcomes of Esophageal Varices in Adults With Fontan Palliation and Liver Cirrhosis.

Background: The purpose of this study was to define the risk and outcomes of esophageal varices in adults with Fontan palliation and liver cirrhosis undergoing esophagogastroduodenoscopy (EGD). Method: The results of EGD, abdominal ultrasound, and liver biopsy, as well as clinic notes from the hepatologist, were reviewed to determine the diagnosis of cirrhosis and esophageal varices. The incidence of acute gastrointestinal bleeding complication was assessed among patients with esophageal varices using the time of EGD as the baseline. Results: Of 149 patients with Fontan palliation and liver cirrhosis, the prevalence of esophageal varices at baseline EGD was 34% (51 of 149). Of 98 patients without esophageal varices at baseline EGD, 27 (27%) underwent subsequent EGD, of whom 11 showed a new diagnosis of esophageal varices. The incidence of a new diagnosis of esophageal varices was 9% per year. Of 62 patients with esophageal varices, 9 (15%) had acute gastrointestinal bleeding complications during 45 (37-62) months of follow-up, yielding an incidence of 5% per year. Of the 9 patients, 8 underwent EGD and variceal banding during the hospitalization for bleeding and 1 patient died of septicaemia. Of the 8 patients who survived to hospital discharge, 2 patients were readmitted for esophageal bleeding within 12 months from the index hospitalization. Higher hepatic vein wedge pressure and hepatic vein pressure gradient were associated with esophageal varices and bleeding complications. Conclusions: In this selected sample of adults with Fontan palliation and liver cirrhosis, esophageal varices were relatively common, and patients with esophageal varices had risk of bleeding complications.

Contexte: Cette étude visait à déterminer le risque de varices œsophagiennes et leur issue clinique chez les adultes ayant fait l'objet d'une intervention de Fontan et présentant une cirrhose hépatique chez qui une œsophagogastroduodénoscopie (OGD) a été réalisée. Méthodologie: Les résultats de l'OGD, de l'échographie abdominale et de la biopsie du foie, ainsi que les notes cliniques de l'hépatologue ont été consultés pour établir les diagnostics de cirrhose et de varices œsophagiennes. L'incidence des complications hémorragiques gastro-intestinales aiguës a été évaluée chez les patients présentant des varices œsophagiennes en utilisant l'OGD initiale comme référence de départ. Résultats: Chez les 149 patients ayant fait l'objet d'une intervention de Fontan et présentant une cirrhose hépatique, la prévalence des varices œsophagiennes lors de l'OGD initiale était de 34 % (51/149). Parmi les 98 patients sans varices œsophagiennes lors de l'OGD initiale, 27 (27 %) ont subi une OGD ultérieure, et 11 d'entre eux ont alors reçu un diagnostic de varices œsophagiennes. Le taux d'incidence des nouveaux diagnostics de varices œsophagiennes était de 9 % par année. Sur les 62 patients présentant des varices œsophagiennes, 9 (15 %) ont subi des complications hémorragiques gastro-intestinales aiguës au cours d'une période de suivi de 45 (37 à 62) mois, ce qui correspond à un taux d'incidence de 5 % par année. Huit des 9 patients ont subi une OGD et une ligature des varices par bande élastique durant leur hospitalisation en raison des complications hémorragiques, et un patient est décédé des suites d'une septicémie. Deux des 8 patients en vie au moment du congé de l'hôpital ont été réhospitalisés pour une hémorragie de l'œsophage dans les 12 mois suivant la première hospitalisation. Une pression d'occlusion plus élevée de la veine hépatique ainsi qu'un plus grand gradient de pression de la veine hépatique ont été associés à la survenue de varices œsophagiennes et de complications hémorragiques. Conclusions: Dans cet échantillon d'adultes ayant fait l'objet d'une intervention de Fontan et présentant une cirrhose hépatique, la fréquence des varices œsophagiennes était relativement élevée, et les patients présentant des varices œsophagiennes étaient exposés à un risque de complications hémorragiques.

Hybrid management of descending thoracic pseudoaneurysm in a patient with DiGeorge syndrome.

We present a case of a 42-year-old man with DiGeorge syndrome and congenital cardiac anomalies including a type B interrupted aortic arch who had previously undergone two bypasses between the ascending and descending thoracic aorta in childhood. He was found to have a 7.4-cm pseudoaneurysm of the descending thoracic aorta with the left subclavian artery arising from the aneurysm. The patient was treated with a single stage hybrid repair including left common carotid to subclavian bypass followed by thoracic endovascular aortic aneurysm repair.

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.

Atrial and Ventricular Arrhythmia in Adults With Shone Complex: A Single-Center Cohort Study.

Background: Multilevel obstruction in left ventricular inflow and outflow predisposes to arrhythmias in Shone's complex (SC). Objectives: The purpose of this study was to study the prevalence and outcomes (heart failure [HF] hospitalization, cardiac transplant, death) of cardiac arrhythmias in adults with SC. Methods: Adults with SC (defined as ≥2 lesions out of supramitral ring, parachute mitral valve, subvalvular/valvular aortic stenosis (AS), and aortic coarctation) seen at Mayo Clinic between January 1999 and March 2020 were identified and evaluated for the presence of sustained atrial fibrillation, atrial flutter, and ventricular arrhythmias (VA). Kaplan-Meier survival analysis was used to calculate the occurrence of these arrhythmias. Results: Seventy-three patients with SC (mean age at first visit 33 ± 13 years) were identified. Most common anomalies were valvular AS (88%), coarctation (85%), parachute mitral valve (44%), subvalvular AS (44%), and supramitral ring (25%). Atrial arrhythmias were diagnosed in 24 patients (33%) at a mean age of 34.6 ± 12.7 years. Patients with atrial fibrillation and atrial flutter had higher number of surgeries, left atrial size, right ventricular systolic pressure, and HF hospitalizations. A rhythm control approach was used in majority of patients (75% on antiarrhythmic drugs and 50% underwent catheter ablation). Sustained VA occurred in 6 of 73 patients of whom 4 had an ejection fraction <40%. Death and cardiac transplantation occurred in 11 and 3 patients, respectively, during a median follow-up of 7.3 ± 6.0 years. Conclusions: In adults with SC, atrial arrhythmias occurred in one-third of patients, were associated with more HF hospitalizations, and frequently required rhythm control. Prevalence of sustained VA was 8% and implantable cardioverter-defibrillator implantation should be considered in those with reduced ejection fraction.

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.

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.

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.