Diagnostic efficacy of ECG-derived ventricular gradient for the detection of chronic thromboembolic pulmonary hypertension in patients with acute pulmonary embolism.

INTRODUCTION: Application of the chronic thromboembolic pulmonary hypertension (CTEPH) rule out criteria (manual electrocardiogram [ECG] reading and N-terminal pro-brain natriuretic peptide [NTproBNP] test) can rule out CTEPH in pulmonary embolism (PE) patients with persistent dyspnea (InShape II algorithm). Increased pulmonary pressure may also be identified using automated ECG-derived ventricular gradient optimized for right ventricular pressure overload (VG-RVPO). METHOD: A predefined analysis of the InShape II study was performed. The diagnostic performance of the VG-RVPO for the detection of CTEPH and the incremental diagnostic value of the VG-RVPO as new rule-out criteria in the InShape II algorithm were evaluated. RESULTS: 60 patients were included; 5 (8.3%) were ultimately diagnosed with CTEPH. The mean baseline VG-RVPO (at time of PE diagnosis) was -18.12 mV·ms for CTEPH patients and - 21.57 mV·ms for non-CTEPH patients (mean difference 3.46 mV·ms [95%CI -29.03 to 35.94]). The VG-RVPO (after 3-6 months follow-up) normalized in patients with and without CTEPH, without a clear between-group difference (mean Δ VG-RVPO of -8.68 and - 8.42 mV·ms respectively; mean difference of -0.25 mV·ms, [95%CI -12.94 to 12.44]). The overall predictive accuracy of baseline VG-RVPO, follow-up RVPO and Δ VG-RVPO for CTEPH was moderate to poor (ROC AUC 0.611, 0.514 and 0.539, respectively). Up to 76% of the required echocardiograms could have been avoided with VG-RVPO criteria replacing the InShape II rule-out criteria, however at cost of missing up to 80% of the CTEPH diagnoses. CONCLUSION: We could not demonstrate (additional) diagnostic value of VG-RVPO as standalone test or as on top of the InShape II algorithm.

Non-invasive early exclusion of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: the InShape II study.

BACKGROUND: The current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is unacceptably long, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies for early CTEPH diagnosis are lacking. Echocardiographic screening among all PE survivors is associated with overdiagnosis and cost-ineffectiveness. We aimed to validate a simple screening strategy for excluding CTEPH early after acute PE, limiting the number of performed echocardiograms. METHODS: In this prospective, international, multicentre management study, consecutive patients were managed according to a screening algorithm starting 3 months after acute PE to determine whether echocardiographic evaluation of pulmonary hypertension (PH) was indicated. If the 'CTEPH prediction score' indicated high pretest probability or matching symptoms were present, the 'CTEPH rule-out criteria' were applied, consisting of ECG reading and N-terminalpro-brain natriuretic peptide. Only if these results could not rule out possible PH, the patients were referred for echocardiography. RESULTS: 424 patients were included. Based on the algorithm, CTEPH was considered absent in 343 (81%) patients, leaving 81 patients (19%) referred for echocardiography. During 2-year follow-up, one patient in whom echocardiography was deemed unnecessary by the algorithm was diagnosed with CTEPH, reflecting an algorithm failure rate of 0.29% (95% CI 0% to 1.6%). Overall CTEPH incidence was 3.1% (13/424), of whom 10 patients were diagnosed within 4 months after the PE presentation. CONCLUSIONS: The InShape II algorithm accurately excluded CTEPH, without the need for echocardiography in the overall majority of patients. CTEPH was identified early after acute PE, resulting in a substantially shorter diagnostic delay than in current practice.

Clinical Characteristics of Patients Undergoing Right Heart Catheterizations in Community Hospitals.

Background Recognition of precapillary pulmonary hypertension (PH) has significant implications for patient management. However, the low a priori chance to find this rare condition in community hospitals may create a barrier against performing a right heart catheterization (RHC). This could result in misclassification of PH and delayed diagnosis/treatment of precapillary PH. Therefore, we investigated patient characteristics and echocardiographic parameters associated with the decision whether to perform an RHC in patients with incident PH in 12 Dutch community hospitals. Methods and Results In total, 275 patients were included from the OPTICS (Optimizing PH Diagnostic Network in Community Hospitals) registry, a prospective cohort study with patients with incident PH; 157 patients were diagnosed with RHC (34 chronic thromboembolic PH, 38 pulmonary arterial hypertension, 81 postcapillary PH, 4 miscellaneous PH), while 118 patients were labeled as probable postcapillary PH without hemodynamic confirmation. Multivariable analysis showed that older age (>60 years), left ventricular diastolic dysfunction grade 2-3, left atrial dilatation were independently associated with the decision to not perform an RHC, while presence of prior venous thromboembolic events or pulmonary arterial hypertension-associated conditions, right atrial dilatation, and tricuspid regurgitation velocity ≥3.7 m/s favor an RHC performance. Conclusions Older age and echocardiographic parameters of left heart disease were independently associated with the decision to not perform an RHC, while presence of prior venous thromboembolic events or pulmonary arterial hypertension-associated conditions, right atrial dilation, and severe PH on echocardiography favored an RHC performance. As such, especially elderly patients may be at an increased risk of diagnostic delays and missed diagnoses of treatable precapillary PH, which could lead to a worse prognosis.

Noninvasive Prediction of Elevated Wedge Pressure in Pulmonary Hypertension Patients Without Clear Signs of Left-Sided Heart Disease: External Validation of the OPTICS Risk Score.

Background Although most newly presenting patients with pulmonary hypertension (PH) have elevated pulmonary artery wedge pressure, identification of so-called postcapillary PH can be challenging. A noninvasive tool predicting elevated pulmonary artery wedge pressure in patients with incident PH may help avoid unnecessary invasive diagnostic procedures. Methods and Results A combination of clinical data, ECG, and echocardiographic parameters was used to refine a previously developed left heart failure risk score in a retrospective cohort of pre- and postcapillary PH patients. This updated score (renamed the OPTICS risk score) was externally validated in a prospective cohort of patients from 12 Dutch nonreferral centers the OPTICS network. Using the updated OPTICS risk score, the presence of postcapillary PH could be predicted on the basis of body mass index ≥30, diabetes mellitus, atrial fibrillation, dyslipidemia, history of valvular surgery, sum of SV1 (deflection in V1 in millimeters) and RV6 (deflection in V6 in millimeters) on ECG, and left atrial dilation. The external validation cohort included 81 postcapillary PH patients and 66 precapillary PH patients. Using a predefined cutoff of >104, the OPTICS score had 100% specificity for postcapillary PH (sensitivity, 22%). In addition, we investigated whether a high probability of heart failure with preserved ejection fraction, assessed by the H2FPEF score (obesity, atrial fibrillation, age >60 yrs, ≥2 antihypertensives, E/e' >9, and pulmonary artery systolic pressure by echo >35 mmHg), similarly predicted the presence of elevated pulmonary artery wedge pressure. High probability of heart failure with preserved ejection fraction (H2FPEF score ≥6) was less specific for postcapillary PH. Conclusions In a community setting, the OPTICS risk score can predict elevated pulmonary artery wedge pressure in PH patients without clear signs of left-sided heart disease. The OPTICS risk score may be used to tailor the decision to perform invasive diagnostic testing.

The pivotal role of cardiac computed tomography angiogram and 18F-fluorodeoxyglucose positron emission tomography-computed tomography in the diagnosis of right sided endocarditis: a case report.

BACKGROUND: Infective endocarditis (IE) poses many clinical and diagnostic challenges. Echocardiography is regarded as the imaging modality of choice for the diagnosis of IE, and plays a key role in both the diagnosis and management of endocarditis. We report on a case in which one could have overlooked an endocarditis of a pulmonary homograft if one had relied on echocardiography alone. CASE SUMMARY: A 38-year-old man presented with intermittent fever and fatigue for 1 month. He had undergone a Ross procedure for a bicuspid aortic valve stenosis at the age of 17 years. At the age of 36 years a valve-sparing aortic root replacement was performed because of aortic root dilatation. Besides a systolic murmur 3/6 noted at the left sternal border, physical examination was normal. Multiple blood cultures grew Streptococcus mitis. Both transthoracic and transoesophageal echocardiogram could not detect any signs of endocarditis. As endocarditis can be overlooked due to reverberations and acoustic shadowing, we performed 18F-fluorodeoxyglucose positron emission tomography-computed tomography (FDG-PET/CT) and cardiac computed tomography angiogram (cardiac CTA). Both imaging modalities showed large vegetations attached to the pulmonary homograft. DISCUSSION: Endocarditis poses diagnostic challenges. While echocardiography is the cornerstone of imaging, one may overlook a pulmonary homograft endocarditis due to reverberations and acoustic shadowing. Therefore, if clinical suspicion of endocarditis is strong, one should consider additional imaging by means of cardiac CTA and/or 18F-fluorodeoxyglucose positron emission tomography-computed tomography imaging to assess valves in pulmonary position, especially in those whom have had prior surgical intervention at this location.

Advantages of mobile health in the management of adult patients with congenital heart disease.

BACKGROUND: Adults with congenital heart disease (ACHD) often suffer from deterioration related to cardiac arrhythmias, hypertension (HT) or heart failure (HF), frequently occurring between planned visits. Mobile health (mHealth) could improve management through remote monitoring by enabling swift therapeutic response and detecting new diagnoses. METHODS: We performed a prospective study employing mHealth in ACHD patients, weekly monitoring heart rhythm, weight and blood pressure. In case of consecutive threshold exceeding measurements or in case of new diagnosis, patients were contacted and if needed the treating physician was consulted. Inclusion criteria were: palpitations within the last three years (with or without arrhythmia diagnosis) or HF NYHA class ≥ II. We evaluated the detection of recurrences and new diagnosis of arrhythmias, HT and HF, adherence and patient experience (Net Promotor Score (NPS)). RESULTS: In total, 109 of the 268 invited ACHD patients were enrolled, 80 with palpitations, 13 with HF, 16 experienced both, mean age 45 (±13) years, 33% male. Median follow-up was 12 (Q1-Q3;9-14) months, 91 patients initiated all measurements (heart rhythm, weight and blood pressure). In 25% of the patients with diagnosed arrhythmias (14/56) recurrences of arrhythmias were detected; 13% of the patients with undiagnosed palpitations (4/32) were diagnosed with novel arrhythmias. In 38% of the patients with HT at baseline (6/16), treatment adjustment was necessary, 4% of the patients without HT (4/76) received novel HT diagnosis. Diuretics were adjusted in 7% of the patients with HF (2/29). Adherence was > 70% in 77% of the patients that started weekly measurements (70/91). Patients that were female, older of age and experienced palpitations at inclusion were more likely to acquire an adherence of > 70%. NPS was completed by 68 patients, 57 patients (84%) were promotors or neutral, and 11 patients (16%) were critics. CONCLUSIONS: mHealth offers advantages in the management of selected ACHD patients; it enabled early detection of recurrences and new diagnosis of arrhythmias, hypertension and heart failure, which lead to swift therapeutic response or remote reassurance. Furthermore, mHealth was well accepted with high adherence and positive patient experience.

Increased carotid and femoral intima-media thickness in patients after repair of aortic coarctation: influence of early repair.

BACKGROUND: In patients, after repair of aortic coarctation, abnormal function of the proximal precoarctation arterial conduits has been demonstrated, but data on arterial wall structure of proximal and distal arteries in patients are scarce. The aim of our study is to compare intima-media thickness (IMT) of the carotid and femoral arteries in controls and patients after coarctation repair and to identify independent predictors of carotid and femoral IMT. METHODS: A total of 137 patients after repair of aortic coarctation (89 male, median age 29.7 years, range 17-74 years, and median age at repair 6.3 years, range 0.01-46 years) and 46 age-matched controls underwent ambulatory blood pressure monitoring and B-mode ultrasound of the carotid and femoral arteries. RESULTS: Twenty (15%) of the 137 patients were operated on before the age of 1 year ("early repair"). The patients with an early repair were younger compared to the controls and compared with the patients with a late repair (24.5 [10.5] vs 31.6 [8.4] years, P = .005, and vs 32.1 [10.5] years, P < .001, respectively). Eighty patients (59%) had hypertension on the basis of the results of the ambulatory blood pressure monitoring (mean daytime blood pressure > or = 135/85 mm Hg). The prevalence of hypertension was not significantly different between patients with an early or late repair. Although patients with an early repair were younger, both carotid and femoral IMT were increased in these patients compared to controls (common carotid artery 0.57 [0.11] vs 0.49 [0.07] mm, P < .001; common femoral artery 0.48 [0.10] vs 0.44 [0.05] mm, P = .033). In multivariable regression analyses, age at repair was only an independent predictor of femoral and not of carotid IMT. CONCLUSIONS: Patients, after repair of aortic coarctation, have increased carotid and femoral IMT despite normal ambulatory blood pressures and successful previous repair. Early repair seems to preserve postcoarctation arterial wall structure, but seems to have only limited effect on precoarctation vessel walls, which supports the theory of a developmental defect of the proximal aorta and its branches.

Non-sustained ventricular tachycardia in patients with congenital heart disease: An important sign?

BACKGROUND: Sustained ventricular tachycardia (susVT) and ventricular fibrillation (VF) are observed in adult patients with congenital heart disease (CHD). These dysrhythmias may be preceded by non-sustained ventricular tachycardia (NSVT). The aims of this study are to examine the 1] time course of ventricular tachyarrhythmia (VTA) in a large cohort of patients with various CHDs and 2] the development of susVT/VF after NSVT. METHODS: In this retrospective study, patients with VTA on ECG, 24-hour Holter or ICD-printout or an out-of-hospital-cardiac arrest due to VF were included. In patients with an ICD, the number of shocks was studied. RESULTS: Patients (N=145 patients, 59% male) initially presented with NSVT (N=103), susVT (N=25) or VF (N=17) at a mean age of 40 ± 14 years. Prior to VTA, 58 patients had intraventricular conduction delay, 14 an impaired ventricular dysfunction and 3 had coronary artery disease. susVT/VF rarely occurred in patients with NSVT (N=5). Fifty-two (36%) patients received an ICD; appropriate and inappropriate shocks, mainly due to supraventricular tachycardia (SVT), occurred in respectively 15 (29%) (NSVT: N=1, susVT: N=9, VF: N=5) and 12 (23%) (NSVT: N=4, susVT: N=5, VF: N=3) patients. CONCLUSIONS: VTA in patients with CHD appear on average at the age of 40 years. susVT/VF rarely developed in patients with only NSVT, whereas recurrent episodes of susVT/VF frequently developed in patients initially presenting with susVT/VF. Hence, a wait-and-see treatment strategy in patients with NSVT and aggressive therapy of both episodes of VTA and SVT in patients with susVT/VF seems justified.

Late complications in patients after repair of aortic coarctation: implications for management.

Survival of patients with aortic coarctation has dramatically improved after surgical repair became available and the number of patients who were operated and reach adulthood is steadily increasing. However, life expectancy is still not as normal as in unaffected peers. Cardiovascular complications are frequent and require indefinite follow-up. Concern falls chiefly in seven categories: recoarctation, aortic aneurysm formation or aortic dissection, coexisting bicuspid aortic valve, endocarditis, premature coronary atherosclerosis, cerebrovascular accidents and systemic hypertension. In this review, these complications, with particular reference to late hypertension, are discussed and strategies for the clinical management of post-coarctectomy patients are described.

Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects.

BACKGROUND: The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the age of onset and initial treatment of AF, coexistence of atrial tachyarrhythmia and (2) progression of paroxysmal to (long-standing) persistent/permanent AF during long-term follow-up. METHODS AND RESULTS: Patients (n=199) with 15 different CHD and documented AF episodes were studied. AF developed at 49±17 years. Regular atrial tachycardia (AT) coexisting with AF occurred in 65 (33%) patients; 65% initially presented with regular AT. At the end of a follow-up period of 5 (0-24) years, the ECG showed AF in 81 patients (41%). In a subgroup of 114 patients, deterioration from paroxysm of AF to (long-standing) persistent/permanent AF was observed in 29 patients (26%) after only 3 (0-18) years of the first AF episode. Cerebrovascular accidents/transient ischemic attacks occurred in 26 patients (13%), although a substantial number (n=16) occurred before the first documented AF episode. CONCLUSIONS: Age at development of AF in patients with CHD is relatively young compared with the patients without CHD. Coexistence of episodes of AF and regular AT occurred in a considerable number of patients; most of them initially presented with regular AT. The fast and frequent progression from paroxysmal to (long-standing) persistent or permanent AF episodes justifies close follow-up and early, aggressive therapy of both AT and AF.

Relation between exercise-induced hypertension and sustained hypertension in adult patients after successful repair of aortic coarctation.

OBJECTIVES: To investigate whether exercise-induced hypertension in successfully repaired adult post-coarctectomy patients is associated with hypertension on 24-h blood pressure measurement and increased left ventricular mass. METHODS: One hundred and forty-four consecutive post-coarctectomy patients (mean age 31.5 years, range 17-74 years; mean age at repair 7.9 years, range 0-45 years) from three tertiary referral centres were studied using ambulatory blood pressure monitoring, treadmill exercise testing and echocardiography. RESULTS: Of the 144 patients, 27 (19%) were known to have sustained hypertension, based on their history, and all were on antihypertensive medication. However, 32 (27%) of the remaining 117 patients showed elevated mean daytime systolic blood pressure readings at 24-h ambulatory blood pressure monitoring (systolic blood pressure > or = 140 mmHg). Of the remaining 85 patients with normal mean daytime systolic blood pressure, 18 patients (21%) had exercise-induced hypertension (maximal exercise systolic blood pressure > 200 mmHg). Mean daytime systolic blood pressure was higher in the exercise-induced hypertensive patients compared to the normotensive patients with normal exercise blood pressure (134 +/- 5 versus 129 +/- 7 mmHg, P = 0.008). By multivariate analysis, both maximal exercise systolic blood pressure (P = 0.007) and resting systolic blood pressure (P < 0.0001) were independently associated with mean daytime systolic blood pressure. Maximal exercise systolic blood pressure had no independent predictive value for left ventricular mass (P = 0.132). CONCLUSIONS: In adult post-coarctectomy patients, maximal exercise systolic blood pressure is independently associated with mean daytime systolic blood pressure at ambulatory blood pressure monitoring. In this study no independent predictive value of maximal exercise systolic blood pressure for left ventricular mass could be demonstrated.

An unusual cause of hypertension in pregnancy.

Aortic coarctation is an unusual cause of hypertension in pregnancy. We report the case of a 34-year-old woman with severe hypertension after surgical repair of aortic coarctation in childhood. An MRI showed a residual stenosis of the aortic arch and a small aneurysm. Pregnant postcoarctectomy patients are at an increased risk for developing hypertension during pregnancy due to residual aortic gradients and abnormal vascular reactivity of the precoarctation vessels. Women after repair of aortic coarctation should be closely monitored for blood pressure during pregnancy.

Increased carotid intima-media thickness predicts cardiovascular events in aortic coarctation.

BACKGROUND: Adult post-coarctectomy patients (CoA) demonstrate increased cardiovascular morbidity and mortality. The carotid intima-media thickness (CIMT), a marker for atherosclerosis, is increased in CoA. The aim was to evaluate the predictive value of CIMT for cardiovascular events. METHODS AND RESULTS: Consecutive CoA patients were prospectively studied during 10.1±0.7 years follow-up. At baseline and follow-up echocardiography, MRI imaging and CIMT imaging were performed, while cardiovascular events were registered. CIMT data were compared with controls. The composite endpoint included: myocardial infarction, cerebrovascular events (CVAs), and (sudden) cardiac death. 160 CoA patients were studied (median age 31.7 (18-74 years), 64% male). Events occurred in 11 patients (7%), five (3%) with myocardial infarction, four (2.5%) with an ischemic CVA and two (1%) died suddenly. An increased CIMT (≥0.8 mm) (HR=15.44, P=<0.001) was predictive for the occurrence of cardiovascular events. Baseline CIMT was increased in CoA compared to controls (0.64±0.12 mm vs 0.57±0.07 mm, P=0.005). CIMT progression rates were similar (0.0091±0.016 mm/year vs 0.0097±0.018 mm/year, P=0.84). Signs of atherosclerosis occurred significantly earlier in CoA patients. CONCLUSION: The contemporary cardiovascular event rate in CoA is 11% in 10 years. Atherosclerosis seems to appear earlier in CoA patients as compared to controls. CoA patients with a CIMT exceeding 0.8mm have a fifteen fold higher cardiovascular risk. CIMT seems to be a useful tool for cardiovascular risk assessment in CoA.

Increased risk for ascending aortic dilatation in patients with complex compared to simple aortic coarctation.

AIMS: Aortic coarctation (CoA) occurs as a "simple" isolated disorder, and in a more "complex" form, combined with associated congenital cardiac abnormalities. Long term outcome of all CoA patients may be complicated by dilatation of the thoracic aorta. The aim of this study was to quantify progressive aortic dilatation, and identify determinants for progressive aortic dilatation. METHODS AND RESULTS: Cardiovascular Magnetic Resonance Imaging (CMR) and echocardiographic data of 93 CoA patients were analyzed retrospectively on the progression, and determinants, of progressive thoracic aortic dilatation. Outcome of simple- versus complex CoA patients were compared. 93 CoA patients (mean age 39 ± 12 years, male 59%) were followed with CMR (follow-up 5.3 ± 1.8 years). Twenty-eight patients were classified as simple- and 68 as complex CoA. The mean progression rate of thoracic aortic dilatation was highest in the ascending aorta with 2.2 ± 2.0mm/5 years (range 0-7.2mm/5 years). History of VSD (ß=1.77, P=0.004) and an increased left ventricular mass index (ß=0.02, P=0.04) were associated with progressive ascending aortic dilatation. Complex CoA patients show an increased progression rate compared to simple CoA patients with 2.4mm/5 years versus 1.5mm/5 years respectively. (P=0.03). CONCLUSION: Adult post-coarctectomy patients show an increased mean progression rate of ascending aortic dilatation with 2.2mm/5 years. The progression rate of ascending aortic dilatation is increased in complex CoA patients, as compared to simple CoA patients. These findings point towards a more comprehensive genetic subset of patients with an increased risk for progressive ascending aortic dilatation.

Rates and determinants of progressive aortic valve dysfunction in aortic coarctation.

PURPOSE: Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA. METHODS: 96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥ 20 mmHg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥ 37 mm. RESULTS: All patients (median age 28.0 years, range 17-61 years; male 57%) were followed with a median follow-up of 7.0 years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV). Median AS progression was 1.1 mmHg/5 years (range - 13-28). Determinants for AS at follow-up were age (ß=0.20, P=0.01), aortic dilatation (ß=4.6, P=0.03), and baseline aortic valve gradient (ß=0.93, P<0.001). BAV was predictive for AR. (ß=0.91, P=0.049). CONCLUSION: Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA.

Rationale and design of a trial on the effect of high dose statins on cardiovascular risk in adults after successful coarctation repair.

BACKGROUND: HMG-coA-reductase-inhibitors (statins) have been proven to reduce atherosclerosis progression as observed by carotid intima-media thickness in patients with known coronary heart disease, independent of lipid lowering. Cardiovascular complications are common in patients after successful coarctation repair. The effect of statins on cardiovascular risk in adults after successful coarctation repair has not yet been established. METHODS: We designed a multicentre, prospective, randomised, open label trial to evaluate the effect of the HMGcoA-reductase-inhibitor (Atorvastatin) on atherosclerotic progression in adult post-coarctectomy patients. The primary endpoint in this study is the carotid intima-media thickness as measured by Bmode ultrasonography of the carotid arteries. CONCLUSION: This large prospective, randomised, open label trial will establish the effect of HMG-coA-reductase inhibitors (Atorvastatin) on cardiovascular risk in adult patients after successful coarctation repair.

Usefulness of exercise-induced hypertension as predictor of chronic hypertension in adults after operative therapy for aortic isthmic coarctation in childhood.

Chronic hypertension is a major concern in adults who have undergone resection of coarctation of the aorta (CoA) in childhood. In otherwise healthy subjects, exercise-induced hypertension is prognostic for chronic hypertension; however, the prognostic value in patients with CoA remains unknown. The aim of the present study was to evaluate the predictive value of exercise-induced hypertension for chronic hypertension in these patients. In the present prospective follow-up study, 74 patients with CoA (58% men, age 30.9 ± 9.5 years) underwent ambulatory blood pressure (BP) monitoring and exercise testing twice from 2001 to 2009 with a follow-up period of 6.3 ± 0.8 years. Hypertension was defined as a mean systolic BP ≥140 mm Hg and/or mean diastolic BP ≥90 mm Hg or the need for antihypertensive treatment. Exercise-induced hypertension was defined as a mean systolic BP of <140 mm Hg and peak exercise systolic BP of ≥200 mm Hg. At baseline, 27 patients (36%) were hypertensive, 11 (15%) had exercise-induced hypertension, and 36 (49%) were normotensive. At follow-up, all 27 hypertensive patients remained hypertensive. Of the 11 with exercise-induced hypertension, 7 (64%) had developed chronic hypertension, and 4 (36%) continued to have exercise-induced hypertension. Of the 36 normotensive patients, 7 (19%) had developed hypertension, 12 (33%) had developed exercise-induced hypertension, and 17 (47%) remained normotensive. On multivariate analysis, baseline maximum exercise systolic BP was independently associated with the mean systolic BP at follow-up (ß = 0.13, p = 0.005). In conclusion, the maximum exercise systolic BP was a predictor for chronic hypertension in patients with CoA. These findings demonstrate the clinical importance of exercise-induced hypertension and warrant additional study into the long-term consequences of exercise-induced hypertension and the potential beneficial role of early antihypertensive treatment in adult patients after CoA repair with exercise-induced hypertension.