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.

Very long-term follow-up after aortic stenting for coarctation of the aorta.

INTRODUCTION AND OBJECTIVES: Stent implantation is the preferred treatment in older children and adults with aortic coarctation (CoA). We aimed to determine the incidence of very late events after CoA stenting. METHODS: We analyzed a cohort of CoA patients who underwent stent implantation at our center between 1993 and 2018. Patients were periodically followed up in outpatient clinics, including computed tomography (CT) and fluoroscopy assessment. RESULTS: A total of 167 patients with CT and fluoroscopy data were included: 83 (49.7%) were aged ≤ 12 years and 46 (28%) were female. The mean clinical follow-up time was 17±8 (range 4-30) years and the mean time to CT/fluoroscopy was 11±7 years. Aortic aneurysm was present in 13% and was associated with the PALMAZ stent (OR, 3.09; 95%CI, 1.11-9.49; P=.036) and the stented length (OR, 0.94; 95%CI, 0.89-0.99; P=.039). Stent fracture was frequent (34%), but was not related to the presence of aneurysm. Stent fracture was associated with young age (OR, 3.57; 95%CI, 1.54-8.33; P=.003), male sex (OR, 4.00; 95%CI, 1.51-12.5, P=.008) and inversely with the PALMAZ stent (OR, 0.29; 95%CI, 0.12-0.67, P=.005). Reintervention was lower in adults (10%), mainly related to aneurysms. Those treated when aged ≤ 12 years had higher reintervention rates (43%) due to recoarctation somatic growth. CONCLUSIONS: This long-term follow-up study of CoA patients treated with stenting revealed a significant incidence of late events. Reintervention rates were higher in patients treated at younger ages. Periodic imaging surveillance appears to be advisable.

Prenatal diagnosis of coarctation: Impact on early and late cardiovascular outcome.

BACKGROUND: Prenatal diagnosis (PND) of aortic coarctation (AoCo) has been associated with a significant improvement in early results, but there is limited information on the long-term cardiovascular outcome. METHODS: We studied 103 patients with simple AoCo, operated in the neonatal period, with a median follow-up of 8,5 years (2 to 23,7 years), with 47% followed for over 10 years. PND was made in 35%. The primary aim was to determine the short and long-term cardiovascular impact of PND of AoCo. RESULTS: Neonates with PND had less preoperative neonatal complications, with only 2,8% incidence of a composite preoperative severe morbidity course, compared to 28% in the postnatal group. PND patients underwent surgery 8 days earlier and had a shorter length of stay in ICU. PND did not impact the incidence of post-operative complications. On the long-term, prevalence of hypertension, left ventricular hypertrophy and rate of recoarctation were not influenced by PND. The PND group had mean 24 h diastolic BP 9 mmHg lower and mean daytime diastolic BP 11 mmHg lower. In the final multivariable model, PND was the single independent variable correlating with daytime diastolic BP. CONCLUSION: PND of AoCo effectively leads to a better pre-operative course with less pre-operative morbidity. We found no significant differences in immediate post-operative cardiovascular outcomes. A better initial course of patients with PND does not have a major long-term impact on cardiovascular outcomes, nevertheless, at late follow-up PND patients had lower diastolic BP values on ambulatory monitoring, which may have an impact on long-term cardiovascular risk.

Outcomes of Heart Failure Hospitalizations in Adult Patients With Coarctation of Aorta: Report From National Inpatient Sample.

Coarctation of aorta (CoA) is a common congenital anomaly which portends patients to early diastolic and systolic heart failure. In this retrospective cohort study, we aimed to evaluate the impact of CoA on heart failure hospitalization. Using the national inpatient sample, the study compared the outcomes of heart failure hospitalization between patients with and without CoA. We noted increasing prevalence of CoA related heart failure admissions over the last decade. Heart failure patients with CoA were younger (mean age 57 vs 71.6 years, P < 0.001), had a longer length of stay (7.4 vs 5.4 days, P < 0.001), and a higher incidence of cardiogenic shock (6.5% vs 2.1%, P = 0.001). However, there was no statistically significant difference in in-hospital mortality (OR 1.45, 95% CI: 0.58, 3.62, P = 0.421) between both groups. These findings demonstrate that CoA increase healthcare resource utilization in patients admitted with heart failure without any significant increase in in-hospital mortality.

Prevalence and descriptive epidemiology of Turner syndrome in the United States, 2000-2017: A report from the National Birth Defects Prevention Network.

The lack of United States population-based data on Turner syndrome limits assessments of prevalence and associated characteristics for this sex chromosome abnormality. Therefore, we collated 2000-2017 data from seven birth defects surveillance programs within the National Birth Defects Prevention Network. We estimated the prevalence of karyotype-confirmed Turner syndrome diagnosed within the first year of life. We also calculated the proportion of cases with commonly ascertained birth defects, assessed associations with maternal and infant characteristics using prevalence ratios (PR) with 95% confidence intervals (CI), and estimated survival probability. The prevalence of Turner syndrome of any pregnancy outcome was 3.2 per 10,000 female live births (95% CI = 3.0-3.3, program range: 1.0-10.4), and 1.9 for live birth and stillbirth (≥20 weeks gestation) cases (95% CI = 1.8-2.1, program range: 0.2-3.9). Prevalence was lowest among cases born to non-Hispanic Black women compared to non-Hispanic White women (PR = 0.5, 95% CI = 0.4-0.6). Coarctation of the aorta was the most common defect (11.6% of cases), and across the cohort, individuals without hypoplastic left heart had a five-year survival probability of 94.6%. The findings from this population-based study may inform surveillance practices, prenatal counseling, and diagnosis. We also identified racial and ethnic disparities in prevalence, an observation that warrants further investigation.

Cardiovascular considerations in management of patients with Turner syndrome.

Turner syndrome (TS) is a chromosomal disorder that affects 25-50 per 100,000 live born females. Patients with TS face a heavy burden of cardiovascular disease (congenital and acquired) with an increased risk of mortality and morbidity compared to the general population. Cardiovascular diseases are a major cause of death in females with TS. Approximately 50% of TS patients have a congenital heart abnormality, with a high incidence of bicuspid aortic valve, coarctation of the aorta and generalised arteriopathy. Frequently, females with TS have systemic hypertension, which is also a risk factor for progressive cardiac dysfunction and aortopathy. This paper aims to provide an overview of the cardiovascular assessment, management and follow up strategies in this high-risk population.

Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: The nevada experience.

OBJECTIVE: We reviewed our center's experience with neonatal and infant hypoplastic aortic arch, unassociated with intracardiac malformations, and investigated changes in prenatal detection rates over time for those requiring therapeutic procedures. METHODS: We identified all prenatal diagnoses of hypoplastic aortic arch with situs solitus, unassociated with intracardiac malformations, made in Nevada between May 2017 and April 2022. In addition, we identified all those 0-180 days old, with prenatal care, that underwent a surgical or interventional cardiac catheterization aortic arch procedure, whether prenatally or postnatally diagnosed. We excluded those with ventricular septal defects, functionally univentricular hearts, interrupted aortic arches, or any associated malformation requiring an additional surgical or interventional procedure ≤6 months old. Additionally, we calculated prenatal detection rates for those undergoing a surgical or interventional catheterization procedure for each of the 5 years. RESULTS: We identified 107 patients prenatally and postnatally. Of the 107 patients, 56 (34 prenatally diagnosed and 22 postnatally diagnosed) underwent an aortic arch procedure, and 51 additionally prenatally diagnosed, live-born infants did not undergo a procedure. Of the 56 procedures, 2 were by interventional catheterization, and 54 underwent a surgical repair. Prenatal detection for those undergoing a procedure statistically significantly increased over the 5 years from 38% to 82%, rho = 0.95 (p = .04). CONCLUSIONS: Currently in Nevada, our prenatal detection rate is >80% in the general population for those between 0 and 6 months old who require a therapeutic procedure for aortic arch obstruction without intracardiac malformations.

Computed tomography study of bovine arch in patients with coarctation of aorta: A retrospective report analysis.

The objective of this study was to define the frequency of the bovine aortic arch in patients with coarctation using the tomographic studies. This is a descriptive retrospective study involving analysis of reports of computed tomographic angiography done at the Prince Sultan Cardiac center for 700 children with congenital heart diseases over a span of about 10 years from April 1, 2008, to August 1, 2018. Cases with coarctation of aorta were chosen to determine the frequency of the bovine aortic arch using the tomographic studies. From a total of 700 cases which underwent computed tomographic angiography, 117 (16.71%) were diagnosed with coarctation and 19 (2.71%) had bovine arch. Among the 117 patients with coarctation, the total number of patients with bovine arch was 7 patients representing 5.98%, while in patients without coarctation we found a total number of bovine arches of 12 out of 583 patients representing 2.06%. Patients having coarctation with normal branching pattern were at a slight increased incidence of atrial septal defect, ventricular septal defect and anomalous pulmonary venous return than patients having coarctation with bovine arch. In the face of increased incidence of bovine aortic arch in patients with coarctation, we recommend multislice computed tomographic angiography as a noninvasive and potentially safe and accurate method to precisely delineate the branching pattern of the arch of aorta in patients with coarctation before the interventional procedures and surgeries.

Incidence and risk factors for late complications after the arterial switch operation.

OBJECTIVE: The purpose of this study was to evaluate our institution's 16-year arterial switch operation (ASO) experience and to determine early and late mortality and late morbidity, as well as the need for reoperation and catheter intervention, and finally, to explore risk factors for late complications and reintervention. METHODS: The clinical data of 185 transposition of the great arteries (TGA) patients who received ASO treatment in our center from January 2006 to January 2022 were continuously included for retrospective study. RESULTS: There were 13 early deaths (7.03%), 5 late deaths (3.01%), and 6 lost to follow-up. The median follow-up time for the 166 hospitalized survivors was 88.5 (2190) months. Moderate or above new aortic valve regurgitation (NAR; in this article, NAR represents moderate or greater reflux unless otherwise specified) occurred in 19 cases (11.45%), and aortic root dilation (ARD) occurred in 28 cases (16.87%). Late right ventricular outflow tract obstruction (RVOTO) occurred in 33 cases (19.88%). There were 18 patients (10.84%) who underwent late re-intervention, and the most common indication for intervention was RVOTO, followed by recurrent aortic coarctation in patients undergoing concurrent arch repair and NAR or ARD. Receiver operating characteristics analysis found that NAR had the strongest predictive power for ARD, followed by RVOTO, followed by bicuspid native pulmonary valve (BPV), and aorto-pulmonary diameter mismatch (APDMM) was the weakest. Multivariate analysis showed that APDMM, previous pulmonary artery banding (PAB), and mild NAR at discharge were independent risk factors for late NAR and ARD. Low surgical weight was an independent risk factor specific to NAR, and BPV was an independent risk factor specific to ARD. Older surgical age and ARD were independent risk factors for late RVOTO. Older surgical age, operation before 2014, late RVOTO, and late ARD were independent risk factors for late intervention. No reintervention events for coronary dysfunction were found in the late stage, but one patient occurred myocardial infarction due to coronary embolism after reoperation. CONCLUSION: Early and late survival rates after ASO in TGA patients have been remarkably improved in recent decades. Increased rates of NAR, ARD, recurrent coarctation of the aorta, and RVOTO as children age are major future outcomes of concern and may imply more late reinterventions. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with APDMM, previous PAB, mild NAR at discharge, low surgical weight, and BPV structures.

Prevalence of aortic coarctation in Bogotá-Colombia from 2001 to 2018. The diagnostic needs to improve. / Prevalencia de coartación aórtica en Bogotá-Colombia de 2001 a 2018. El diagnóstico debe mejorar

OBJECTIVES: Aortic coarctation is the most frequent structural anomaly out of congenital heart diseases. This congenital defect is an important cause of death worldwide. We sought to determine the prevalence of aortic coarctation in Colombia and whether new policies have had an impact on its diagnosis. METHODS: In this study information from the Bogotá birth defect surveillance program during the years 2001-2018 from 63 hospitals was used. 537,026 live births of any weight and stillbirths of any weight were analyzed. The information was stored in a database on the servers of the Health Secretariat and the Pontificia Universidad Javeriana. We analyzed the presence of aortic coarctation according to the newborn's sex, weight, size, mother's age, and gestational age at the time of birth and when coarctation is accompanied by other types of congenital malformations. RESULTS: The prevalence of aortic coarctation in Bogotá during the years 2001-2018 found in this study was 1.25 in 10,000 live births. We also found that prevalence of aortic coarctation in Bogotá changes throughout the years having a significant increase in the year 2018 with 6.57 cases in 10,000 live births. CONCLUSIONS: This prevalence is higher than the one found in a study with data from 2001 to 2014, which suggests an improvement in the country's epidemiological surveillance and medical training. However, the prevalence found in Bogotá is still lower compared to the prevalence worldwide and from other continents, the prevalence for Latinamerica was significantly lower as compared to those in Asia, Europe, and United States so we emphasize the importance of continuing with improvements, such as standardizing screening methods and sensitivity of said methods in a local scale as well as a continental scale.

OBJETIVOS: La coartación aórtica es la anomalía estructural más frecuente de las cardiopatías congénitas. Este defecto congénito es una causa importante de muerte en todo el mundo. Buscamos determinar la prevalencia de la coartación aórtica en Colombia y si las nuevas políticas han tenido un impacto en su diagnóstico. MÉTODOS: En este estudio se utilizó información del programa de vigilancia de defectos de nacimiento de Bogotá durante los años 2001-2018 en 63 hospitales. Se analizaron 537,026 nacidos vivos de cualquier peso y nacidos muertos de cualquier peso. La información fue almacenada en una base de datos de los servidores de la Secretaría de Salud y de la Pontificia Universidad Javeriana. Se analizó la presencia de coartación aórtica de acuerdo al sexo del recién nacido, peso, tamaño, edad de la madre y edad gestacional en el momento del nacimiento y cuando la coartación se acompañó de otros tipos de malformaciones congénitas. RESULTADOS: La prevalencia de la coartación aórtica en Bogotá durante los años 2001-2018 encontrados en este estudio fue de 1.25 en 10,000 nacidos vivos. También encontramos que la prevalencia de coartación aórtica en Bogotá ha cambiado a lo largo de los años, teniendo un aumento significativo en el año 2018 con 6.57 casos en 10,000 nacidos vivos. CONCLUSIONES: Esta prevalencia es mayor que la encontrada en un estudio con datos de 2001 a 2014, lo que sugiere una mejora en la vigilancia epidemiológica y la formación médica del país. Si bien, la prevalencia encontrada en Bogotá es menor en comparación con la prevalencia a nivel mundial y de otros continentes, la prevalencia para el continente de Latinoamérica también es significativamente menor con respecto a Asia, Europa y Estados Unidos, por lo que enfatizamos la importancia de continuar con las mejoras, como la estandarización de los métodos de detección y la sensibilidad de dichos métodos tanto a nivel local como a nivel del continente.

Paradoxical increase in ambulatory SBP in coarctation of aorta a compared to essential hypertension.

BACKGROUND: The relationship between office vs. ambulatory blood pressure (BP) indices are well-studied in patients with essential hypertension and based on these data, it is known that the average 24-h ambulatory BP is typically lower than office BP. However, emerging data show that office SBP underestimates arterial afterload in patients with coarctation of aorta (COA), and a minimal increase in stroke volume during low-intensity exercise results in an exaggerated rise in SBP as compared to those with essential hypertension. We hypothesized that COA patients will have higher ambulatory SBP and a higher prevalence of masked hypertension compared to patients with essential hypertension. METHODS: Case-control study of 118 COA patients and 118 patients with essential hypertension matched by age, sex, BMI and office SBP. RESULTS: Although both groups had similar office SBP (132 ± 17 mmHg) by design, the COA group had paradoxical increases in 24-h ambulatory SBP (135 ± 14 vs. 126 ± 13; P < 0.001) and daytime ambulatory SBP (142 ± 16 vs. 130 ± 13; P < 0.001), and less nocturnal dipping (-3 ± 5 vs. -9 ± 4; P < 0.001). The COA group also had a higher prevalence of masked hypertension [36 (31%) vs 14 (12%); P < 0.001), and worse arterial function indices. CONCLUSION: These findings underscore the potential limitations of relying on office SBP for screening/monitoring of hypertension in COA and potential pitfalls in extrapolating essential hypertension guidelines recommendations to the treatment of COA. It also provides the rationale for further studies to determine if pharmacologic BP interventions guided by ambulatory BP data will improve clinical outcomes in the COA population.

Late complication rates after aortic coarctation repair in patients with or without a bicuspid aortic valve.

OBJECTIVE: Patients with previously repaired aortic coarctation (CoA) are at risk of developing late surgical complications. Many patients with CoA also have a bicuspid aortic valve (BAV). We sought to determine in patients with repaired CoA whether the presence of BAV is associated with more cardiovascular reinterventions during follow-up. METHODS: Adults with previously repaired simple CoA were recruited from our Adult Congenital Heart Disease database (Sydney, Australia). The incidence of complications relating to the 'CoA-site' (descending aortic aneurysm or dissection, or recoarctation) and the 'AV/AscAo' (aortic valve or ascending aortic pathology) that required intervention was compared between those with BAV ('CoA-BAV') and without BAV ('CoA-only'). RESULTS: Of 146 patients with repaired CoA, 101 (69%) had BAV. Age at CoA repair was similar (median 6.0 (IQR 0.5-14.0) years vs 5.0 (IQR 0.5-11.0) years, p=0.44), as was the distribution of repair types, with end-to-end repair the most common in both groups (45.9% vs 45.6%). At a median of 28 years following initial repair, a significantly higher proportion of patients with CoA-BAV required cardiovascular reintervention (45.5% vs 20.0%, p=0.003). Whereas 'CoA-site' complications were more common than 'AV/AscAo' complications in patients with CoA only (13.3% and 0%, respectively), patients with CoA-BAV had a high prevalence of both 'CoA-site' as well as 'AV/AscAo' complications (19.8% and 21.8%, respectively). Overall survival was similar (p=0.42). CONCLUSION: In adults with repaired CoA, patients with CoA-BAV are more than twice as likely to require cardiovascular reintervention by early-to-mid-adult life compared with those with CoA alone. Despite this, no difference in survival outcomes was observed.

Right Heart Dysfunction in Adults With Coarctation of Aorta: Prevalence and Prognostic Implications.

BACKGROUND: Chronic elevation of left heart filling pressure causes pulmonary vascular remodeling, pulmonary hypertension, and right heart dysfunction. Although diastolic dysfunction is relatively common in patients with coarctation of aorta, there are limited data about the prevalence and prognostic implications of pulmonary hypertension and right heart dysfunction in this population. The purpose of the study was to assess right heart function and hemodynamics in patients with coarctation of aorta and to determine the relationship between right heart indices and cardiovascular events defined as heart failure hospitalization, heart transplant, or cardiovascular death. METHODS: Right heart structure, function, and hemodynamics were assessed with these indices: right atrial volume, right atrial pressure, right atrial reservoir strain, right ventricular global longitudinal strain, right ventricular end-diastolic area, right ventricular systolic pressure, and tricuspid regurgitation severity. Right heart hemodynamic score, range 0 to 5, was generated based on the correlation between the right heart indices and cardiovascular events, using half of the cohort (derivation cohort, n=411), and then tested on the validation cohort (n=410). The goodness of fit and discrimination power was compared using C statistics and risk score. RESULTS: The median follow-up in the derivation cohort was 8.2 (4.0-11.1) years, and 59 (14%) patients had cardiovascular events during this period. Right heart hemodynamic score was independently associated with cardiovascular events (hazard ratio, 1.64 [95% CI, 1.38-2.17]) for every unit increase in right heart hemodynamic score after adjustment for clinical and echocardiographic indices (C statistic, 0.718 [95% CI, 0.682-0.746]). The right heart hemodynamic score was also independently associated with cardiovascular events in the validation cohort (C statistic, 0.711 [95% CI, 0.679-0.741]). The C statistic difference (0.007 [95% CI, 0.014-0.022]) and risk score (0.86 [95% CI, 0.54-1.17]) suggest a good model fit. CONCLUSIONS: The current study underscores the prognostic importance of right heart dysfunction in patients with coarctation of aorta and suggests that right heart indices should be used for risks stratification in this population.

Recoartación de la aorta en pacientes sometidos a angioplastia percutánea con o sin implantación de stent / Recoarctation of the aorta in patients subjected to ballon angioplasty with or without implementation of stent.

OBJECTIVE: Coarctation of the aorta can be treated surgically or with balloon angioplasty. The objective of our study was to describe the results after percutaneous balloon angioplasty with or without stent implantation for coarctation of the aorta and establish the incidence of recovery during follow-up. METHOD: Cohort study. 89 patients of any age where included in a follow up period of nine years. RESULTS: Of the 89 patients included in the study, 69.0% were male. The mean follow-up for all participants was 33.66 months. 32.5% of the patients had a stent implanted during the angioplasty procedure; of which 24.1% suffered recoarctation during follow-up. In the group without stent implantation, 36.6% suffered recoarctation. There was not significant difference in the survival curves of the two groups (p = 0.899). CONCLUSIONS: Stent implantation during balloon angioplasty to treat aortic coarctation did not influence in the incidence of aortic recoarctation; but factors such as preangioplasty arterial hypertension and the final angioplasty gradient > 20 mmHg is associated with aortic recoarctation.

OBJETIVO: La coartación de la aorta puede ser tratada quirúrgicamente o con angioplastia con balón. El objetivo de nuestro estudio fue describir los resultados postangioplastia percutánea con balón con o sin implantación de stent para coartación de la aorta y establecer la incidencia de recoartación en el seguimiento. MÉTODO: Estudio de cohorte, se incluyeron pacientes de cualquier edad en un periodo de seguimiento de nueve años. RESULTADOS: Se incluyeron 89 pacientes, un 69.0% hombres. La media de seguimiento para todos los participantes fue de 33.66 meses. Al 32.5% se les implantó un stent en angioplastia; de los cuales el 24.1% se recoartaron durante el seguimiento. En el grupo sin stent se recoartaron un 36.6%. No hubo diferencia significativa en las curvas de supervivencia de los dos grupos (p = 0.889). CONCLUSIONES: La implantación de stent durante la angioplastia con balón para tratar coartación de aorta no influyó en la incidencia de recoartación de la aorta; pero factores como la hipertensión arterial preangioplastia y el gradiente final de angioplastia > 20 mmHg se asoció con recoartación de la aorta.

Cardiovascular Morbidity and Mortality in Adult Patients With Repaired Aortic Coarctation.

Background The long-term burden of cardiovascular disease after repair of coarctation of the aorta (CoA) has not been elucidated. We aimed to determine the incidence of and risk factors for cardiovascular events in adult patients with repaired CoA. Additionally, mortality rates were compared between adults with repaired CoA and the general population. Methods and Results Using the Dutch Congenital Corvitia (CONCOR) registry, patients aged ≥16 years with previous surgical or transcatheter CoA repair from 5 tertiary referral centers were included. Cardiovascular events were recorded, comprising coronary artery disease, stroke/transient ischemic attack, aortic complications, arrhythmias, heart failure hospitalizations, endocarditis, and cardiovascular death. In total, 920 patients (median age, 24 years [range 16-74 years]) were included. After a mean follow-up of 9.3±5.1 years, 191 patients (21%) experienced at least 1 cardiovascular event. A total of 270 cardiovascular events occurred, of which aortic complications and arrhythmias were most frequent. Older age at initial CoA repair (hazard ratio [HR], 1.017; 95% CI, 1.000-1.033 [P=0.048]) and elevated left ventricular mass index (HR, 1.009; 95% CI, 1.005-1.013 [P<0.001]) were independently associated with an increased risk of cardiovascular events. The mortality rate was 3.3 times higher than expected based on an age- and sex-matched cohort from the Dutch general population (standardized mortality ratio, 3.3; 95% CI, 2.3-4.4 [P<0.001]). Conclusions This large, prospective cohort of adults with repaired CoA showed a high burden of cardiovascular events, particularly aortic complications and arrhythmias, during long-term follow-up. Older age at initial CoA repair and elevated left ventricular mass index were independent risk factors for the occurrence of cardiovascular events. Mortality was 3.3-fold higher compared with the general population. These results advocate stringent follow-up after CoA repair and emphasize the need for improved preventive strategies.

The clinical presentation and outcome of aortic coarctation associated with left ventricular inflow and outflow tract lesion in adult patients: Shone syndrome and beyond.

BACKGROUND: Aortic coarctation (AoCoa) is a congenital stenosis of aorta, which often co-exists with other congenital heart defects. Many studies have shown the importance of regular follow-up in these patients however there is scarcity of knowledge relating to the impact of left ventricle inflow lesions (LVIT) and left ventricle outflow track lesions (LVOT). The aim of this study is to evaluate the impact of isolated AoCoa with LVIT and/or LVOT on haemodynamic, morbidity and mortality. METHODS: We have retrospectively analysed clinical data of all adult AoCoa patients who underwent echocardiography between 2010-2018 in our centre. Outcome measures included death, number of hospitalisations for cardiac causes, development of cardiac arrhythmia, new prescription of HF medication. RESULTS: A total of 406 AoCoa patients were included and were followed for a median 4.2 years. At baseline, 38% patients had AoCoa alone, 54% patients had LVOT, 3% patients had LVIT, and 5% patients had mixed LVIT and LVOT, including patients with Shone syndrome. Patients with mixed LVIT and LVOT had the highest mortality of the four groups and the highest heart failure-related morbidity. Moreover, they were the most prone to have a higher indexed LA volume compared to patients with no LVOT (p=0.0001). During follow-up, 13 patients died, of which 21% patients were from the mixed LVIT and LVOT group. CONCLUSIONS: AoCoa patients with a combination of LVIT and LVOT including Shone complex are associated with a significantly higher morbidity and mortality compared to AoCoa alone.

LV non-compaction in patients with coarctation of the aorta: prevalence and effects on cardiac function.

BACKGROUND: Left ventricular non-compaction has been associated with heart failure, arrhythmia, thromboembolism and sudden death. The prevalence of non-compaction in patients with coarctation of the aorta and its clinical significance remains unknown, although obstructive left heart disease is common in patients with non-compaction. We sought to evaluate the prevalence of left ventricular non-compaction in patients with repaired aortic coarctation as well as its effect on left ventricular size and systolic function. METHODS AND RESULTS: In total, 268 patients (Mean age 26 (inter-quartile range 21-37) years, 63% male) undergoing cardiac magnetic resonance imaging for clinical follow-up were included from three tertiary centres for adult congenital heart disease. Clinical data was obtained from medical records and correlated with ventricular volumes and function. Left ventricular non-compaction was defined as a diastolic non-compacted:compacted dimension ratio >2.3 in the worst affected segment on a long-axis view. Left ventricular non-compaction was present in 8.2% of patients with repaired coarctation. Left ventricular end-diastolic volumes and stroke volumes were significantly higher in patients with non-compaction compared to those without. There were no significant differences in ventricular mass or ejection fraction in these two groups. CONCLUSIONS: Left ventricular non-compaction is relatively common in patients with repaired coarctation of the aorta and correlates with increased left ventricular end-diastolic volumes.