Evaluation of new predictive scores for sudden cardiac death in childhood hypertrophic cardiomyopathy in a French cohort.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is rare in children, and sudden cardiac death (SCD) is difficult to predict. Two prognostic scores - HCM Risk-Kids and Precision Medicine for Cardiomyopathy (PRIMaCY) - were developed to assess the risk of SCD in the next 5 years in children with HCM. AIMS: To test the ability of these scores to predict SCD in children with HCM. Also, to identify factors associated with a severe cardiac rhythmic event (SCRE) (ventricular fibrillation, sustained ventricular tachycardia, heart transplant for rhythmic reasons or SCD). METHODS: Retrospective, multicentre, observational study at 10 medical centres in the Nord-Pas-de-Calais region, France. RESULTS: This study included 72 paediatric patients with HCM during 2009-2019 who were followed for a median (interquartile range [IQR]) of 8.5 (5.0-16.2) years. Eleven patients (15.3%) presented with SCRE. HCM Risk-Kids was high, with a median (IQR) score of 6.2% (2.1-12.8%; significant threshold≥6.0%) and the PRIMaCY median (IQR) score was 7.1% (2.6-15.0%; significant threshold≥8.3%). The positive predictive value was only 27.1% (95% confidence interval [CI] 21.5-32.5%) for HCM Risk-Kids (with a threshold of≥6.0%) and 33.2% (95% CI 27.1-38.9%) for the PRIMaCY score (with a threshold of≥8.3%). The negative predictive values were 95.4% (95% CI 92.3-97.7%) and 93.0% (95% CI 89.8-96.2%), respectively. Three of 28 patients with an implantable cardioverter defibrillator (ICD) experienced complications (including inappropriate shocks). CONCLUSION: HCM Risk-Kids and the PRIMaCY score have low positive predictive values to predict SCD in paediatric patients. If used alone, they could increase the rate of ICD implantation and thus ICD complications. Therefore, the scores should be used in combination with other data (genetic and magnetic resonance imaging results).

Management and outcomes of pregnant women with cardiovascular diseases in a cardio-obstetric team.

BACKGROUND: Cardiovascular diseases (CVDs) are currently the leading cause of maternal death in Western countries. Although multidisciplinary cardio-obstetric teams are recommended to improve the management of pregnant women with CVD, data supporting this approach are scarce. AIMS: To describe the characteristics and outcomes of pregnant patients with CVD managed within the cardio-obstetric programme of a tertiary centre. METHODS: We included every pregnant patient with history of CVD managed by our cardio-obstetric team between June 2017 and December 2019, and collected all major cardiovascular events (death, heart failure, acute coronary syndromes, stroke, endocarditis and aortic dissection) that occurred during pregnancy, peripartum and the following year. RESULTS: We included 209 consecutive pregnancies in 202 patients. CVDs were predominantly valvular heart diseases (37.8%), rhythm disorders (26.8%), and adult congenital heart diseases (22.5%). Altogether, 47.4% were classified modified World Health Organization (mWHO)>II, 66.5% had CARdiac disease in PREGnancy score (CARPREG II)≥2 and 80 pregnancies (38.3%) were delivered by caesarean section. Major cardiovascular events occurred in 16 pregnancies (7.7%, 95% confidence interval [CI] 4.5-12.2) during pregnancy and in three others (1.5%, 95% CI 0.3-4.1) during 1-year follow-up. Most events (63.1%) occurred in the 16.3% of patients with unknown CVD before pregnancy. CONCLUSIONS: The management of pregnant patients with CVD within a cardio-obstetric team seems encouraging as we found a relatively low rate of cardiovascular events compared to the high-risk profile of our population. However, most of the remaining events occurred in patients without cardiac monitoring before pregnancy.

Transcatheter Closure of Perimembranous Ventricular Septal Defects Including Multifenestrated and Gerbode-Type Defects Using the Lifetech Konar Device.

(1) Transcatheter closure of perimembranous ventricular septal defects (PmVSD) is becoming more attractive and effective with the development of new occluders. The aim of this study was to report a single-center experience in PmVSD closure using the Lifetech Konar-multifunctional occluder (MFO). (2) From March 2019 to October 2022, 43 consecutive patients were enrolled in the study. Among them, 13 had multifenestrated PmVSD including 5 Gerbode-type defects. (3) There were 23 males/20 females, and the median age was 17 years (range 2-68 years). Trivial aortic regurgitation was noticed in 19 patients. Implantation was successful in all patients under general anesthesia. A retrograde approach was used in 35 patients (81%). The retrograde approach was associated with a lower radiation dose (p = 0.042) and shorter fluoroscopy time (p = 0.002) compared to the antegrade approach. Full occlusion was observed immediately in 12 patients (28%) and in 33 patients (77%) at a median follow-up of 11 months. There were no complications such as embolization, complete atrioventricular block, device dislocation, new onset above grade I, or progression of tricuspid or aortic valve regurgitation. Seven of the thirteen patients with a multifenestrated defect had no residual shunt. The persistent shunts were all trivial intra-prosthetic leaks. (4) MFO is effective and safe for PmVSD closure including multifenestrated/Gerbode-type defects with no complication. However, a longer follow-up remains warranted to establish the safety of this technique.

Efficiency and Impact of Hypnoanalgesia for Cardiac Catheterisation in Paediatric Population.

Hypnoanalgesia is a promising non-pharmacologic adjunct technique in paediatric interventions. Its safety, efficiency, and impacts on paediatric cardiac catheterisation (CC) are unknown. METHODS: In a prospective study, patients aged <16 years who underwent CC under hypnoanalgesia from January to December 2021 were included. Pain and anxiety were assessed using the analgesia nociception index (ANI) and the visual analogue scale (VAS). RESULTS: Sixteen patients were included; the mean age was 10.5 years, and the mean weight was 37 kg. Catheterisations were interventional in 10 patients (62.5%). Hypnoanalgesia indications were general anaesthesia (GA) contraindication in four patients (25.0%), the need for accurate pressure measurements in three patients (18.7%), and interventionist/patient preferences in nine (56.3%). CC was accomplished in 15 patients (93.7%), even in complicated cases. In one case, pulmonary artery pressures were normalised compared to previous catheterisation under local anaesthesia alone. The VAS score was under 5/10 for all patients. The ANI remained above 50 (no painful zone) for all but one patient. There was no significant decrease in the ANI during the intervention compared to the baseline (p = 0.62). No complications were reported. CONCLUSION: Paediatric CC is feasible and safe under hypnoanalgesia, even in complicated cases. Hypnoanalgesia was efficient in managing pain and stress, and it ensures more reliable pressure measurements.

Health-Related Quality of Life and Its Association With Outcomes in Adults With Congenital Heart Disease and Heart Failure: Insight From FRESH-ACHD Registry.

Background Quality of Life (QoL) is a prognostic factor in heart failure (HF) of patients with acquired cardiac disease. The aim of this study was to determine the predictive value of QoL on outcomes in adults with congenital heart disease (ACHD) and HF. Methods and Results Quality of life of 196 adults with congenital heart disease with clinical heart failure (HF) (mean age: 44.3±13.8 years; 51% male; 56% with complex congenital heart disease; 47% New York Heart Association class III/IV) included in the prospective multicentric registry FRESH-ACHD (French Survey on Heart Failure-Adult with Congenital Heart Disease) was assessed using the 36-Item Short Form Survey (SF-36), a patient-reported survey. The primary end point was defined by all-cause death, HF-related hospitalization, heart transplantation, and mechanical circulatory support. At 12 months, 28 (14%) patients reached the combined end point. Patients with low quality of life experienced major adverse events more frequently (logrank P=0.013). On univariate analysis, lower score at physical functioning (hazard ratio [HR], 0.98 [95% CI, 0.97-0.99]; P=0.008), role limitations related to physical health (HR, 0.98 [95% CI, 0.97-0.99]; P=0.008), and general health dimensions of the SF-36 (HR, 0.97 [95% CI, 0.95-0.99]; P=0.002) were significantly predictive of cardiovascular events. However, after multivariable analysis, SF-36 dimensions were no longer significantly associated with the primary end point. Conclusions Patients with congenital heart disease with HF and poor quality of life experience severe events more frequently, making quality of life assessment and rehabilitation programs essential to alter their trajectory.

Impact of time to diagnosis on the occurrence of cardiogenic shock in MIS-C post-COVID-19 infection.

BACKGROUND: In multisystem inflammatory syndrome in children (MIS-C), diagnostic delay could be associated with severity. This study aims to measure the time to diagnosis in MIS-C, assess its impact on the occurrence of cardiogenic shock, and specify its determinants. METHODS: A single-center prospective cohort observational study was conducted between May 2020 and July 2022 at a tertiary care hospital. Children meeting the World Health Organization MIS-C criteria were included. A long time to diagnosis was defined as six days or more. Data on time to diagnosis were collected by two independent physicians. The primary outcome was the occurrence of cardiogenic shock. Logistic regression and receiver operating characteristic curve analysis were used for outcomes, and a Cox proportional hazards model was used for determinants. RESULTS: Totally 60 children were assessed for inclusion, and 31 were finally analyzed [52% males, median age 8.8 (5.7-10.7) years]. The median time to diagnosis was 5.3 (4.2-6.2) days. In univariable analysis, age above the median, time to diagnosis, high C-reactive protein, and high N-terminal pro-B-type natriuretic peptide (NT-proBNP) were associated with cardiogenic shock [odds ratio (OR) 6.13 (1.02-36.9), 2.79 (1.15-6.74), 2.08 (1.05-4.12), and 1.70 (1.04-2.78), respectively]. In multivariable analysis, time to diagnosis ≥ 6 days was associated with cardiogenic shock [adjusted OR (aOR) 21.2 (1.98-227)]. Time to diagnosis ≥ 6 days had a sensitivity of 89% and a specificity of 77% in predicting cardiogenic shock; the addition of age > 8 years and NT-proBNP at diagnosis ≥ 11,254 ng/L increased the specificity to 91%. Independent determinants of short time to diagnosis were age < 8.8 years [aHR 0.34 (0.13-0.88)], short distance to tertiary care hospital [aHR 0.27 (0.08-0.92)], and the late period of the COVID-19 pandemic [aHR 2.48 (1.05-5.85)]. CONCLUSIONS: Time to diagnosis ≥ 6 days was independently associated with cardiogenic shock in MIS-C. Early diagnosis and treatment are crucial to avoid the use of inotropes and limit morbidity, especially in older children.

Obstetrical outcomes in cases of maternal heart disease with a risk of cardiac decompensation: A retrospective study since the establishment of a multidisciplinary consultation meeting "heart and pregnancy".

BACKGROUND: Pregnant women with chronic heart failure (CHF) are at increased risk for cardiac complications. However, the frequency of obstetrical and neonatal complications in pregnant women with CHF remains unclear. OBJECTIVE: The objective of our study was to describe obstetrical and neonatal outcomes in pregnant with CHF. METHOD: This single-center retrospective cohort study involves pregnant women with a CHF who delivered at Jeanne de Flandre, the Lille's university hospital, from 2017 to 2021. The frequency of obstetrical, neonatal, and cardiovascular complications was collected. RESULT: During this period, we identified 26 pregnant women with a CHF. The main cardiac diseases responsible for CHF were cardiomyopathies (53.8%) and congenital heart disease (46.2%). Acute heart failure occurred in 30.8% of the cases and mainly concerned patients with no follow-up of their heart disease. The main obstetrical complications were fetal growth restriction (38.5%) and premature rupture of fetal membranes (19.2%). The 26 pregnancies comprised 25 live births and 1 stillbirth. Newborn infants were delivered via cesarean in 69.2%. Of the live births, 60% were preterm at a median gestational age of 36 (34-38) weeks. CONCLUSION: Pregnant women with CHF had a higher risk for obstetrical and neonatal outcomes.

Prevalence and Associated Factors of Long-term Growth Failure in Infants with Congenital Heart Disease Who Underwent Cardiac Surgery Before the Age of One.

Long-term growth failure can have negative impact on health (by increasing morbidity and mortality) and on neurodevelopmental outcomes. Its prevalence among children with congenital heart disease (CHD) is not well described. The aim of our study was to evaluate the prevalence of growth failure in a population of infants with CHD away from cardiac surgery and identify associated factors. We conducted a retrospective and multicentric study that included infants from the North of France who underwent cardiac surgery before the age of one, between January 2013 and December 2017. 331 infants were included among which 48% had a prenatal diagnosis, 15% had a genetic syndrome, and 15% were premature infants. Mean birth weight was 3 ± 0.6 kg. At surgery, 35% presented feeding difficulties (need for enriched formula and/or feeding tube) and 14% had growth failure (defined by Z-score weight for age < -2SD). 6-12 months after surgery, 16% still presented growth failure. Several associated factors were identified: prenatal diagnosis, genetic syndrome association, birth weight ≤ 3 kg, complex CHD (≥ 2 significative lesions, or double outlet right ventricle or single ventricle physiology), surgery after 30 days, and need for diuretic drug before surgery and/or still needed 1 month after surgery. Growth failure persists between 6 and 12 months after surgery in 16% of infants with CHD. More studies are needed to link growth failure and neurodevelopment, which is the new challenge for this aging population.

Treatment of two infants with PIK3CA-related overgrowth spectrum by alpelisib.

PIK3CA-related overgrowth spectrum (PROS) includes rare genetic conditions due to gain-of-function mutations in the PIK3CA gene. There is no approved medical therapy for patients with PROS, and alpelisib, an approved PIK3CA inhibitor in oncology, showed promising results in preclinical models and in patients. Here, we report for the first time the outcome of two infants with PROS having life-threatening conditions treated with alpelisib (25 mg) and monitored with pharmacokinetics. Patient 1 was an 8-mo-old girl with voluminous vascular malformation. Patient 2 was a 9-mo-old boy presenting with asymmetrical body overgrowth and right hemimegalencephaly with West syndrome. After 12 mo of follow-up, alpelisib treatment was associated with improvement in signs and symptoms, morphological lesions and vascular anomalies in the two patients. No adverse events were reported during the study. In this case series, pharmacological inhibition of PIK3CA with low-dose alpelisib was feasible and associated with clinical improvements, including a smaller size of associated complex tissue malformations and good tolerability.

Prognostic values of exercise echocardiography and cardiopulmonary exercise testing in patients with primary mitral regurgitation.

AIMS: To compare the clinical significance of exercise echocardiography (ExE) and cardiopulmonary exercise testing (CPX) in patients with ≥moderate primary mitral regurgitation (MR) and discrepancy between symptoms and MR severity. METHODS AND RESULTS: Patients consulting for ≥moderate discordant primary MR prospectively underwent low (25 W) ExE, peak ExE, and CPX within 2 months in Lille and Rennes University Hospital. Patients with Class I recommendation for surgical MR correction were excluded. Changes in MR severity, systolic pulmonary artery pressure (SPAP), left ventricular ejection fraction (LVEF), and tricuspid annular plane systolic excursion were evaluated during ExE. Patients were followed for major events (ME): cardiovascular death, acute heart failure, or mitral valve surgery. Among 128 patients included, 22 presented mild-to-moderate, 61 moderate-to-severe, and 45 severe MR. Unlike MR variation, SPAP and LVEF were successfully assessed during ExE in most patients. Forty-one patients (32%) displayed reduced aerobic capacity (peak VO2 < 80% of predicted value) with cardiac limitation in 28 (68%) and muscular or respiratory limitation in the 13 others (32%). ME occurred in 61 patients (47.7%) during a mean follow-up of 27 ± 21 months. Twenty-five Watts SPAP [hazard ratio (HR) (95% confidence interval, CI) = 1.03 (1.01-1.06), P = 0.003] and reduced aerobic capacity [HR (95% CI) = 1.74 (1.03-2.95), P = 0.04] were independently predictive of ME, even after adjustment for MR severity. The cut-off of 55 mmHg for 25 W SPAP showed the best accuracy to predict ME (area under the curve = 0.60, P = 0.05). CONCLUSION: In patients with ≥moderate primary MR and discordant symptoms, 25 W exercise pulmonary hypertension, defined as an SPAP ≥55 mmHg, and poor aerobic capacity during CPX are independently associated with adverse events.

Case Report: Persistent Pulmonary Hypertension of the Newborn and Narrowing of the Ductus Arteriosus After Topical Use of Non-Steroidal Anti-Inflammatory During Pregnancy.

Background: The use of non-steroidal anti-inflammatory drugs (NSAIDs) during the third trimester of pregnancy can cause premature constriction of the ductus arteriosus. This report describes a case of in utero narrowing of the ductus arteriosus (DA) diagnosed postnatally in a baby with Persistent Pulmonary Hypertension of the Newborn (PPHN), after maternal use of Diclofenac-Epolamine 140 mg patch during the second and third trimester. Case Presentation: A fetal ultrasounds revealed an enlarged hypertrophic right ventricle at 32 weeks of gestation. Detailed questioning of the mother highlighted that topical Diclofenac (FLECTOR®) had been used at 26 and at 31 weeks of gestation. An echocardiography performed 8 h postnatally showed supra-systemic pulmonary hypertension, a restrictive ductus arteriosus and a dilated right ventricle. The newborn was treated by inhaled nitric oxide and oral Sildenafil and was discharged from hospital on day 24. He had a complete normalization of his pulmonary vascular resistance on day 48. Conclusion: This case illustrates the potential fetal and neonatal complications associated with maternal topical Diclofenac medication during pregnancy resulting in antenatal closure of the DA.

The natural history of a family with aortic dissection associated with a novel ACTA2 variant.

Disease-causing heterozygous variants in the ACTA2 gene cause an autosomal dominant heritable thoracic aortic disease (HTAD) with thoracic aortic aneurysm and dissection as main phenotype, and occasional extravascular abnormalities such as livedo reticularis. ACTA2-HTAD accounts for an important part of non-syndromic HTAD, with detection rates varying between 1.5-21% according to different studies. A consensus statement for the screening and management of patients with pathogenic ACTA2 variants has been recently published by the European reference network for rare vascular diseases (VASCERN). However, management of ACTA2 patients is often challenged by extremely variable inter- and intra-familial clinical courses of the disease. Here we report a family harboring a disease-causing ACTA2 variant. The proband and two siblings presented with acute type A aortic dissection and rupture involving nondilated aortic segments before the age of 30. Their mother died at 49 years-old from type B aortic dissection and rupture. Genetic testing revealed the heterozygous novel p.(Pro335Arg) variant in the ACTA2 gene in the proband and in the affected siblings. The clinical history of this family highlights the difficulty of adopting effective prevention strategies in ACTA2 patients.

Prognostic value of aerobic capacity and exercise oxygen pulse in postaortic dissection patients.

BACKGROUND: Although recommendations encourage daily moderate activities in post aortic dissection, very little data exists regarding cardiopulmonary exercise testing (CPET) to personalize those patient's physical rehabilitation and assess their cardiovascular prognosis. DESIGN: We aimed at testing the prognostic insight of CPET regarding aortic and cardiovascular events by exploring a prospective cohort of patients followed-up after acute aortic dissection. METHODS: Patients referred to our department after an acute (type A or B) aortic dissection were prospectively included in a cohort between September 2012 and October 2017. CPET was performed once optimal blood pressure control was obtained. Clinical follow-up was done after CPET for new aortic event and major cardio-vascular events (MCE) not directly related to the aorta. RESULTS: Among the 165 patients who underwent CPET, no adverse event was observed during exercise testing. Peak oxygen pulse was 1.46(1.22-1.84) mlO2/beat, that is, 97 (83-113) % of its predicted value, suggesting cardiac exercise limitation in a population under beta blockers (92% of the population). During a follow-up of 39(20-51) months from CPET, 42 aortic event recurrences and 22 MCE not related to aorta occurred. Low peak oxygen pulse (<85% of predicted value) was independently predictive of aortic event recurrence, while low peak oxygen uptake (<70% of predicted value) was an independent predictor of MCE occurrence. CONCLUSION: CPET is safe in postaortic dissection patients should be used to not only to personalize exercise rehabilitation, but also to identify those patients with the highest risk for new aortic events and MCE not directly related to aorta.

Is the new Occlutech duct occluder an appropriate device for transcatheter closure of patent ductus arteriosus?

AIM: To describe our initial experience with the Occlutech Duct Occluder (ODO) for percutaneous closure of patent ductus arteriosus (PDA). METHODS: Retrospective review of patients undergoing transcatheter PDA closure with the ODO in 2 academic centers. RESULTS: From April 2013 to September 2017, 42 patients underwent PDA closure. Median age at implantation was 34 months (range 4 months-68 years) and median weight was 12 kg (range 4.1-57 kg). Ducts were Krichenko type A duct (n = 34), type E (n = 6), and type C (n = 2). The mean duct diameter was 3.76 mm (range 1.69 to 9.95 mm, median 3.1 mm). Implantation succeeded in all. There was neither device embolization nor hemolysis. At device release, immediate angiogram showed a small residual shunt in 54.7%. During follow-up, Doppler echocardiography demonstrated 71% of full occlusion at day one, rising to 95% at one month and 100% at one year and half after implantation. The mean maximal systolic pressure gradient in left pulmonary artery was 4.2 ±â€¯4.3 mm and across the distal aortic arch 5.4 ±â€¯4.7 mm Hg. No patient had any significant stenosis with clinical relevance. CONCLUSIONS: ODO is safe and effective in transcatheter closure of PDA including relatively large sized ducts. The results are satisfactory with a high level of full occlusion and a low rate of complications. Further evaluation with larger studies and longer follow-up will be required to confirm these preliminary good results.

[Pulmonary revalvulation and rhythmologenic risks in patients with repaired tetralogy of Fallot]. / Revalvulation pulmonaire et risque rythmique chez les patients opérés d'une tétralogie de Fallot.

Tetralogy of Fallot is a frequent congenital heart disease that has been repaired since the mid-1950s. The follow-up after repair is good despite a persistent risk of sudden death. The risk factors in long-term follow-up are advanced age at repair, hemodynamic status of the right ventricle, QRS duration≥180ms, left ventricular dysfunction, and existence of sustained or not ventricular tachycardia. In the presence of significant pulmonary regurgitation, it is necessary to perform revalvulation either by classic surgery or cardiac catheterization. To correct the risk of ventricular arrhythmia, some have proposed radiofrequency ablation of critical isthmus, or cryo-application during surgery. However, the use of implantable cardioverter defibrillator is another therapeutic option that is more and more employed as secondary or primary prevention in patients at risk of sudden death.

Cardiopulmonary exercise testing is a better outcome predictor than exercise echocardiography in asymptomatic aortic stenosis.

BACKGROUND: Objective assessment of maximal aerobic capacity using peak oxygen consumption (peak VO2) can be helpful in the management of patients with asymptomatic aortic stenosis (AS). The relationship between peak VO2 and AS severity criteria derived from rest and supine exercise echocardiography (SEE) has never been explored. OBJECTIVES: We aimed to determine whether low peak VO2 (<85% of predicted value) is associated with severity parameters in SEE, and poor clinical outcome. METHODS: Fifty one asymptomatic patients (mean age of 54±21years) with moderate to severe aortic stenosis (Vmax>3m/s) and left ventricle ejection fraction>50% prospectively underwent resting and SEE and cardiopulmonary exercise testing (CPX). RESULTS: Peak VO2 was lower than expected (21.9±7.4mL/kg/min), i.e. <85% of predicted value in 57% patients, secondary to cardiac limitation in most of them (69%). In multiple regression analysis, age, BMI and female gender were the only independent determinants of peak VO2. Interestingly no parameter derived from SEE was associated with peak VO2. After 21±7month follow-up, no patient died, 20 underwent cardiac surgery. Peak VO2<85% of predicted value was associated with lower event free survival compared to normal peak VO2 (57%±11% vs 93±6%, p=0.036) whereas no exercise echocardiographic parameter could predict such events. Peak VO2≥85% had a negative predictive value of 97%. CONCLUSION: CPX detects a high proportion of false asymptomatic AS patients with poorer outcome that cannot be predicted by SEE markers of AS severity. Assessment of aerobic capacity should be part of current approach within a "watchful waiting" strategy.

Post aortic dissection: Gap between activity recommendation and real life patients aerobic capacities.

BACK GROUND: Regular exercise at a safe level, i.e. 3-5 metabolic equivalents, is recommended to improve blood pressure control and quality of life even after aortic dissection, although aerobic exercise capacities in these patients are unexplored yet. METHODS: We prospectively collected data from 105 patients with a history of post aortic dissection referred for a cardiopulmonary exercise testing (CPX) aiming to guide exercise rehabilitation. RESULTS: The population was composed of 76% of male, with a mean age of 57.9±12.4years. There were an equal distribution between the two type of dissection (47% of type A and 53% of type B aortic dissection). No cardiac event occurred during or after CPX. One third of patients have normal aerobic exercise capacity defined as peak oxygen uptake upper than 85% of their predicted capacity. Mean oxygen uptake peak was quite low 19.2±5.2ml/kg/min (5.5±1.5 metabolic equivalents). Aerobic capacity was limited by cardiac chronotropic incompetence in 42% or peripheral deconditioning in 45%. Blood pressure remained in an acceptable range during the exercise. Systolic and diastolic blood pressures were respectively 151±20 and 77±13mmHg at first ventilatory threshold. CONCLUSIONS: CPX is a safe exploration in patients with post aortic dissection syndrome. Given the fact that most of these patients are faced with significant alteration of aerobic capacities, the recommended daily practice of moderate exercise at 3-5 METS should be adapted and personalized to each patient thanks to CPX.