Management of immunosuppression and antiviral treatment before and after heart transplant for HIV-associated dilated cardiomyopathy.

Infection with HIV may lead to the development of cardiomyopathy as improved antiretroviral regimens continue to prolong patient life. However, advanced therapeutic options, such as heart transplant, have until recently been precluded to HIV-positive persons. A favorable long-term outcome has been obtained after kidney or liver transplant in HIV-positive recipients fulfilling strict virological and clinical criteria. We recently reported the first heart transplant in a HIV-infected patient carried out in our center. In this article, we detail the major challenges we faced with the management of antiretroviral and immunosuppressive treatments over the first 3 years post-transplant. The patient had developed dilated cardiomyopathy while on antiretroviral treatment with zidovudine, lamivudine and efavirenz. He was in WHO Stage 1 of HIV infection and had normal CD4+ count and persistently undetectable HIV-RNA. In spite of cardiac resynchronization therapy and maximal drug therapy, the patient progressed to end stage heart failure, requiring heart transplant. He was placed on a standard immune suppressive protocol including cyclosporine A and everolimus. Despite its potential pharmacokinetic interaction with efavirenz, everolimus was chosen to reduce the long-term risk of opportunistic neoplasia. Plasma levels of both drugs were monitored and remained within the target range, although high doses of everolimus were needed. There were no infectious, neoplastic or metabolic complications during a 3-year follow-up. In summary, our experience supports previous data showing that cardiac transplantation should not be denied to carefully selected HIV patients. Careful management of drug interactions and adverse events is mandatory.

Heart failure: molecular, genetic and epigenetic features of the disease.

Factors that compete to establish heart failure (HF) are not completely known. In the last years the several technological improvements allowed us to deeply study the molecular and genetic aspects of this complex syndrome. This new approach to HF based on molecular biology new discoveries shows us more clearly the pathophysiological bases of this disease, and a future scenery where the genetics may be useful in the clinical practice, as screening of high risk populations, as well as in the diagnosis and therapy of underlying myocardial diseases. The purpose of this review was to analyse the molecular, genetic and epigenetic factors of HF. We described the molecular anatomy of the sarcomere and the pathogenesis of the heart muscle diseases, abandoning the previous monogenic theory for the concept of a polygenic disease. Different actors play a role to cause the illness by themselves, modifying the expression of the disease and, eventually, the prognosis of the patient.

Novel insights into the role of cardiotrophin-1 in cardiovascular diseases.

Cardiotrophin-1 (CT-1), a member of interleukin (IL)-6 family, was originally isolated for its ability to induce a hypertrophic response in neonatal cardiac myocytes. This cytokine mediates a pleiotropic set of growth and differentiation activities through a unique receptor system, consisting of IL-6 receptor (IL-6R) and a common signal transducer, the glycoprotein 130 (gp130). Both in humans and in mice, CT-1 mRNA has been detected in several tissues, such as liver tissue, adipose tissue, and tissues in the respiratory and nervous systems; in each of these tissues it performs different functions. Predominant actions of CT-1 are on the heart, where it is synthesized and where it provides first myocardial protection by promoting cell survival and proliferation, it carries on its haemodynamic effects and endocrine properties, and finally, it predisposes the heart to pathological conditions. The aim of this review is to describe the pathophysiological mechanisms through which CT-1 carries out its activities, especially on the heart, and its potential contribution as a disease marker in clinical cardiology. Recent studies have confirmed its active role in promoting structural changes typical of most common cardiovascular disease, such as hypertension, valve diseases, congestive heart failure, and coronary artery disease. In fact, CT-1 induces myocyte hypertrophy and collagen synthesis, thereby participating in the progression of ventricular remodelling, which results in cardiac muscle failure at the latest stage. CT-1 plasma levels are elevated in patients with hypertension and coronary artery diseases, and they are also correlated with the severity of valve diseases and heart failure. Therefore, CT-1 may represent a diagnostic, staging, and prognostic biomarker of cardiovascular diseases.

A child cohort study from southern Italy enlarges the genetic spectrum of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiovascular disorder worldwide. It is the leading cause of sudden cardiac-related death in young people and a major cause of cardiac failure and death in elderly people. However, HCM frequently goes undiagnosed until the appearance of overt signs and symptoms, thereby delaying prophylactic and therapeutic measures. We screened patients for sarcomeric genes associated with HCM to obtain information that could be useful for an early diagnosis and so limit the severe consequences of silent HCM. We recruited 39 families with HCM from southern Italy and found mutations in 41% of families (12 with familial HCM and 4 with sporadic HCM). The remaining 23 families (59%) were negative for myofilament gene mutations. Of the 12 mutations identified, 8 were novel. Screening of the other family members available revealed that 27 had mutations; 11 of these individuals had no signs or symptoms suggestive of HCM. This study, besides characterizing the spectrum of mutations in another childhood population, and revealing an even greater genetic heterogeneity than formerly recognized, may increase genotype-phenotype correlations, and thus may help to identify asymptomatic candidates for early preventive or therapeutic measures.

[The role of natriuretic peptides in heart failure]. / Ruolo dei peptidi natriuretici nello scompenso cardiaco.

Over the last decades, there has been a significant increase in incidence and prevalence of heart failure, a major cause of cardiac morbidity and mortality. Measurements of neurohormones, in particular B-type natriuretic peptide (BNP), can significantly improve diagnostic accuracy, and also correlate with long-term morbidity and mortality in patients with chronic heart failure presenting to the emergency department. BNP is secreted by cardiac ventricles mainly in response to wall stress and neurohormonal factors like the sympathetic nervous system, endothelins, and the rennin-angiotensin-aldosterone system. BNP increases myocardial relaxation and oppose the vasoconstrictive, sodium retaining, and natriuretic effects caused by vasoconstrictive factors. BNP is the first biomarker to prove its clinical value for the diagnosis of left ventricular systolic and diastolic dysfunction but also for the right ventricular dysfunction, guiding prognosis and therapy management. Emerging clinical data will help further refine biomarker-guided therapeutic and monitoring strategies involving BNP.

Evaluation of outcomes in SPA-treated osteoarthrosic patients.

INTRODUCTION: Osteoarthrosis is the most prevalent joints disorder and it is also the most frequent cause of physical disability in the elderly. When surgery is not indicated, symptomatic drugs are generally used. These treatments are frequently associated to balneotherapy. In fact, balneotherapy or spa therapy has been widely used in classical medicine as a cure for such diseases. The aim and significance of this study is to evaluate the impact of thermalism in subjects suffering from osteoarthrosis. METHODS: We randomly selected 220 osteoarthrosic subjects (STs = spa treatment subjects), aged from 40 to 90, that usually undergone mud pack therapy and balneotherapy at least once a year. They were enrolled in thermal establishments in the Euganean Basin. We also recruited, as control group, 172 osteoarthrosic subjects (NCs = normal care subjects) that never underwent any spa therapy. A questionnaire, comprehensive of a disability score, was administered by physicians to each subject. RESULTS: STs reported to suffer from osteoarthrosis for more years than NCs. Furthermore STs significantly suffered more than NCs from pain in several joints, and they also showed a more elevated average number of painful joints. In spite of that, STs used less drugs than NCs, and showed a higher degree of disability due to osteoarthrosis (p < 0.001). CONCLUSION: The regular use of mudpack and balneotherapy seem to improve the wellness, and the spa treatment seems to help the achievement of this goal. In this regard it might be important to encourage new investigations in order to assess in which measure thermal therapy contribute to the wellness improvement.

Strain rate imaging: data acquisition and postrocessing.

Several studies already demonstrated the clinical relevance of strain rate imaging. Unfortunately, so far only few echolaboratories are using this technique in their clinical practice. This is mainly due to the lack of information on how to perform a standard strain rate imaging study. Thus, the aim of the present review is to provide the bases and methodology to perform a correct strain rate study.

Atenolol vs enalapril in young hypertensive patients after successful repair of aortic coarctation.

Late arterial hypertension has been identified as a major predictor for morbidity and mortality in aortic coarctation (AoC) patients. Few data are available about efficacy and tolerability of angiotensin converting enzyme inhibitors vs beta-blockers in young AoC patients. This study aimed to evaluate the tolerability and efficacy on 24-h blood pressure (BP) and left ventricular mass/height(2.7) (LVMI), of atenolol vs enalapril. We enrolled consecutive AoC hypertensive patients with (a) no history of BP treatment or after >48 h of withdrawn, (b) aged 6-20 years, (c) body mass index (BMI) <90th percentile for age and sex, (d) >12 months from a successful AoC repair and (e) no major associated cardiovascular abnormalities. All patient were evaluated with 24-h ambulatory BP monitoring, standard echocardiography, strain-strain rate imaging, at enrolment, 3, 6 and 12 months of treatment. We studied 51 AoC patients (13±3.9 years, BMI: 21.4±4.3 kg m(-2)). Patients were randomly assigned at atenolol treatment (n=26), or enalapril treatment (n=25). The mean follow-up duration was 11±2 months. Both drugs were able to significantly reduce 24-systolic BP (SBP; atenolol: 133±11 mm Hg vs 124±16 mm Hg, P=0.016; enalapril: 135±6 mm Hg vs 127±7 mm Hg, P=0.001). Only enalapril was able to significantly reduce LVMI (47±12 vs 39.6±10 g m(-)(2.7), P=0.016). Only in atenolol group in two cases (7.7%) drug withdrawal was needed because of adverse events. Enalapril and atenolol are similarly effective in reducing SBP. However, only enalapril demonstrated a significant reduction of LVMI. In no case, enalapril was stopped because of adverse events.

Complete transposition of the great arteries: patterns of congenital heart disease in familial precurrence.

BACKGROUND: Transposition of the great arteries (TGA) is considered to be associated only rarely with genetic syndromes and to have a low risk of precurrence among relatives of affected patients. Because most family studies have involved a relatively small number of patients and evaluated all types of TGA as a single group, we performed a large, prospective study investigating the precurrence of congenital heart disease in families of children with complete, nonsyndromic TGA. METHODS AND RESULTS: From January 1997 through December 2000, 370 patients with nonsyndromic, complete TGA were consecutively evaluated and enrolled in the study. The occurrence of cardiac and noncardiac anomalies among relatives of the probands was investigated. Relatives with congenital heart disease were found in 37 of 370 families (10%), including 5 of 37 families (13.5%) with more than one affected relative. TGA itself was the most common precurrent malformation: complete TGA occurred in 6 families and congenitally corrected TGA occurred in 5 families. Precurrence risks for congenital heart disease were calculated at 1.8% (8 of 436) for siblings, 0.5% (4 of 740) for parents, 0.5% (16 of 3261) for first cousins, 0.2% (4 of 2101) for uncles/aunts, and 0.06% (1 of 1480) for grandparents. CONCLUSIONS: The present study shows that TGA is not always sporadic in families. Precurrence of concordant cardiac defects within affected family members supports monogenic or oligogenic inheritance of TGA in certain kindreds. Moreover, the occurrence of complete TGA and congenitally corrected TGA among first-degree relatives in several different families strongly suggests an underlying pathogenetic link between these 2 malformations that has been previously unrecognized.

Echocardiographic evaluation of left ventricular systolic function in the Down syndrome.

Left ventricular systolic function was evaluated by echo-Doppler in 22 Down syndrome patients without congenital heart disease. Although they had evident left ventricular hyperkinesia, this did not appear to reflect intrinsic abnormalities of myocardial properties but a reduced afterload.

Left ventricular remodeling and mechanics after successful repair of aortic coarctation.

Forty normotensive patients (mean age 12.3 +/- 6.5 years) followed up after a successful repair of aortic coarctation (mean age at coarctectomy 5.1 +/- 4.8 yrs) were studied by echo-Doppler to (1) evaluate left ventricular (LV) remodeling and endocardial and midwall mechanics, and (2) identify factors that might predispose to persistent abnormalities. Sex- and age-specific cutoff levels for LV mass/height2.7 and relative wall thickness were defined to assess LV geometry. To adjust for age-and growth-related changes in ventricular mechanics, all echocardiographic variables were expressed as a Z-score relative to the normal distribution. In addition, the smallest diameter of the aorta was assessed by magnetic resonance imaging and calculated as percent narrowing compared with the diameter of the aorta at the diaphragmatic level. In the study group, 24 of 40 patients (60%) had normal LV geometry. Among the 16 patients (40%) with abnormal LV geometry, 5 (12.5%) had a pattern of concentric remodeling and 11 (27.5%) an eccentric hypertrophy. LV hypertrophy was marked (LV mass index >51 g/m2.7) in 5 of these patients. No patient had a pattern of concentric hypertrophy. LV contractility was increased (Z-score >95th percentile) in 28 patients (70%) as assessed using the endocardial stress-velocity index. In contrast, LV contractility assessed using midwall stress-velocity index remained elevated (Z-score >95th percentile) in 15 patients (37.5%). The stepwise multiple logistic regression analysis was not able to detect any significant independent predictor of abnormal LV remodeling, including sex, age at surgical repair, length of postoperative follow-up, heart rate, body mass index, systolic and diastolic blood pressure, and smallest diameter of the aorta, as well as indexes of LV geometry (shape, mass, volume, mass/ volume ratio) and function (preload, afterload, pump function, and myocardial contractility). Thus, normotensive patients after surgical repair of aortic coarctation may be in an LV hyperdynamic cardiovascular state (more frequent in those who have undergone late repair) and have multiple patterns of LV geometry.

Repeat syncopal attacks due to postsurgical right ventricular pseudoaneurysm.

Pseudoaneurysm of the right ventricular outflow tract is a rare lesion caused by disruption of the ventricular wall that allows the blood to leak into the surrounding space. It often complicates surgery involving right ventriculotomy and progressively increases in size, therefore causing airway compression, pulmonary perfusion asymmetry, thromboembolism, and rupture. We report on a patient who developed right ventricular pseudoaneurysm early after surgery for atrio-ventricular septal defect with tetralogy of Fallot and needed emergency surgical repair due to low cardiac output and repeat syncopal attacks.

Left ventricular midwall mechanics in healthy children and adolescents.

The assessment of left ventricular function in pediatric age generally has been made by measuring the extent and velocity of fiber shortening at the endocardium and relating these parameters to systolic wall stress. It has been suggested from animal and human studies that this "endocardial method" can result in an overestimation of myocardial function when healthy hearts and those with hypertrophy are compared. Thus an assessment of left ventricular pump function and contractility that takes into account the epicardial migration of the midwall circumferential fibers during systole (midwall analysis) is warranted. Although the normal range for midwall indexes of left ventricular mechanics has been established in adult subjects, up to now it has not been studied in pediatric subjects. To establish normal values for left ventricular midwall mechanics, 70 healthy children ranging in age from 3 days to 18 years were evaluated with 2-dimensional and M-mode echocardiography. Midwall fractional shortening (FSmw), rate-corrected mean velocity of circumferential fiber shortening (VCFcmw), and the relation between these indexes and left ventricular end-systolic wall stress (ESS) were calculated separately for the children younger than 2 (group 1, 20 subjects) and older than 2 (group 2, 50 subjects) years of age. Group 1 had significantly higher FSmw and VCFcmw compared with group 2. An inverse linear relation between FSmw and ESS and between VCFcmw and ESS was found in both age groups. The y-intercept was higher in group 1, and the slope of the mean regression line was steeper than in group 2 for both the relationships, suggesting an age-dependent midwall left ventricular pump function and contractility. These normative data can be used to assess the left ventricular midwall mechanics in pediatric patients with pressure or volume overload.

Arrhythmogenic substrate in young patients with repaired tetralogy of Fallot: role of an abnormal ventricular repolarization.

Ventricular repolarization analysis has been shown to be effective in the identification of electrical myocardial instability leading to ventricular arrhythmias. The aim of the present study was to examine ventricular repolarization time indexes, in terms of both absolute measures and dispersion across the myocardium, in young patients with repaired tetralogy of Fallot (41 pts; 28M/13F, age 11.7+/-3.6 years), assessing, furthermore, the possible influence of known negative prognostic factors relative to the surgical operation and residual haemodynamic abnormalities. The data of the study group were compared with those of 33 aged-matched asymptomatic control subjects (22M/11F, age 11.7+/-2.3 years). Ventricular depolarisation, as expressed by QRS duration, resulted significantly longer in total Fallot group than in the Control group (P<0.0001). Particularly, patients operated through a right ventricular approach showed higher values of QRS interval (P<0.0001) than those operated through a combined transatrial-transpulmonary approach. All the patients operated on for tetralogy of Fallot exhibit, with respect to control subjects, an inhomogeneous prolongation of ventricular repolarization across the myocardium, as showed by the significant increase in the absolute indexes of ventricular repolarization, JTc (P<0.001), QT (P<0.0001) and QTc (P<0.0001) with a concomitant prolongation of the indexes of dispersion of ventricular recovery time, QTcD (P<0.0001), JTcD (P<0.0001), 'adjusted' QTcD (P<0.001) and Tp-Te interval (P<0.0001). A temporal and regional variation in the ventricular repolarization across the myocardium in patients with repaired tetralogy of Fallot, could create the pathophysiological substrate for an increased cardiac electrical instability. The presence of negative prognostic factors, relative to the surgical intervention or residual haemodynamic abnormalities, even if not influencing the arrhythmic substrate, invariably present, could determine 'trigger' conditions essential for the development of ventricular arrhythmias.

Hypertrophic cardiomyopathy in pediatric patients: effect of verapamil on regional and global left ventricular diastolic function.

OBJECTIVE: To assess the effects of treatment with verapamil on regional and global left ventricular (LV) diastolic function in paediatric patients with hypertrophic cardiomyopathy (HCM). DESIGN: Twelve patients (age range 5.1 to 12.3 years, median 8.6) with HCM were evaluated during ongoing chronic oral treatment with verapamil (4 mg/kg/day) and four days after withdrawal of therapy. Twelve age- and body surface area-matched normal children served as controls. In an echocardiographic study, global LV diastolic function was evaluated by assessing isovolumic relaxation time (IVRT) and mitral flow indexes, including peak filling rate normalized to mitral stroke volume (PFR/SV). In addition, regional LV diastolic function was assessed by pulsed-wave Doppler tissue imaging at the subendocardial portion of the middle region of the anterior and posterior interventricular septum, and anterolateral and inferior walls to measure the peak velocities and the velocity-time integrals of myocardial excursion in both early diastole and atrial systole. In addition, as an index of diastolic asynchrony (AsyI), the variation in time to peak filling rate, measured as the time from the peak of the R wave on the electrocardiogram to the peak of the regional E wave, among the four myocardial regions was defined by subtracting the smallest value from the greatest and expressing the difference as a percentage of the smallest value. RESULTS: Compared with the controls, patients with HCM without therapy showed a longer IVRT (P<0.01) and a decrease in PFR/SV (P<0.01) without a compensatory increase in filling during atrial systole. Oral administration of verapamil induced a significant shortening of the IVRT (P=0.003) and an increase in PFR/SV (P=0.02). Furthermore, patients with HCM without therapy showed a significantly longer time to peak filling rate (P<0.01) associated with a decreased peak velocity in early diastole without a concomitant increase in peak velocity during atrial systole in each of the myocardial regions. Furthermore, the AsyI was higher in the HCM group than in controls (19% versus 6%, respectively), and this index was inversely correlated with the PFR/SV (r=-0.86, P<0.001). The regional diastolic velocity of the myocardium at each of the four analyzed regions was not significantly different with verapamil, but the AsyI was significantly lower (P<0.05). CONCLUSIONS: Children with HCM show abnormalities of both global and regional LV diastolic function. In these patients, chronic administration of verapamil plays a crucial role in the improvement in global LV filling and, as a consequence, in clinical manifestations. The beneficial effects of verapamil seem to be related to a reduction in diastolic asynchrony more than to significant changes in diastolic velocities of the myocardial fibres.

Truncus arteriosus and double aortic arch associated with DiGeorge syndrome.

We report the 1st known case in which truncus arteriosus and double aortic arch have been associated with DiGeorge syndrome. The association of these 2 cardiovascular anomalies lends support, by itself, to speculation that truncus arteriosus and double aortic arch have a common embryonic pathogenesis; and the presence of these anomalies in a patient with DiGeorge syndrome strengthens the contention that the common causative factor is pathologic development of the neural crest cells.

Myocardial dysplasia in a 3rd-trimester fetus. An ultrasound and pathologic study.

Arrested myocardial development, often described as spongiosum heart, has been reported in association with obstructive semilunar valve disease and, much more rarely, as a primary disease in adolescents and adults. To our knowledge, this condition has never been diagnosed in utero. We describe the echocardiographic and pathoanatomic findings of the 1st case of myocardial dysplasia detected in utero by ultrasound. A 28-year-old woman, gravida 2, para 1, was referred to our unit at 34 weeks of gestation due to severe fetal hydrops. On echocardiography, we observed gross fetal cardiomegaly (particularly of the septal and ventricular myocardium), an unusually bright myocardial echostructure, thick trabeculations in both ventricular chambers, and severe loss of myocardial contraction. There were normal ventriculoarterial connections and no signs of obstructive semilunar valve disease. After fetal death, necropsy confirmed the presence of spongiosum heart and the diagnosis of myocardial dysplasia--which term best describes this disorder in its various temporal expressions. Because this condition has never before been observed prenatally, no consideration has been given to intrauterine management. We recommend that fetal cardiac function be monitored echocardiographically whenever a pregnant patient has a positive family history of this disease. There is a possibility that the life of the affected fetus might be prolonged beyond the gestational period by avoiding intrauterine cardiac decompensation, through early delivery. We recommend further that the parents of these children be advised of the risks associated with future pregnancies. Little is known about the pattern of inheritance of myocardial dysplasia, but the disorder appears to be familial. Therefore, the possibility that it may recur within the same generation must be taken into account.

New insights in the pathophysiology of mitral and aortic regurgitation in pediatric age: role of angiotensin-converting enzyme inhibitor therapy.

This review has been focused on the new insights in the pathophysiology of mitral and aortic regurgitation and on the role of ACE-inhibitor therapy in children with chronic volume overload due to left-sided valvular lesions. Recent clinical studies show that these drugs have favorable effects when administered orally in chronic mitral and aortic regurgitation. Interestingly, the beneficial effects of ACE-inhibition regard the basic anatomic, hemodynamic and adaptive pathologic conditions related to volume overload, namely, the regurgitant orifice area and volume and ventricular remodeling. The heart is a plastic structure, constantly being altered in size, shape and composition in response to chronic volume overload. Thus, modulation of cardiac plasticity by ACE-inhibition raises the possibility of using new therapeutic strategies specifically designed to prevent and/or antagonize the mechanical disadvantages secondary to volume overload-induced cardiac remodeling. The beneficial effects of ACE-inhibition have also been observed in growing children with asymptomatic valvular regurgitation; thus, it appears that the unloading therapy has the potential of influencing the natural history of both mitral and aortic regurgitation and possibly delays surgical valve repair or replacement. These data justify early inhibition of the renin-angiotensin system in children with left ventricular volume overload due to mitral and aortic regurgitation.

[Epidemiologic surveillance of ischemic heart disease in the population of Pavia in 1986-1995]. / Sorveglianza epidemiologica delle malattie ischemiche del cuore nella popolazione di Pavia nel decennio 1986-1995.

BACKGROUND: In Italy mortality for coronary heart disease is continuously decreasing in males and females. However, it is not yet clear how much of this decline is attributable to a longer survival of coronary heart disease patients or to a real decreased incidence in the population. The aim of this paper was to analyze coronary heart disease mortality trends in the population of Pavia, case-fatality rates and acute myocardial infarction attack rates. METHODS: Mortality surveillance was carried out by the Epidemiological Unit of the ASL of Pavia; acute myocardial infarction attack rates were estimated from regional admission data for the Pavia population. The target population (1991 Census) was represented by two groups: the first was equal to 49,326 residents (23,627 males and 25,699 females) 45-64 years of age, the second was equal to 17,208 residents (7236 males and 9972 females) 65-74 years of age. RESULTS: The decline in mortality was mainly observed in males aged 45-64. Acute myocardial infarction attack rates showed a decline in 45-64 men and an increase in the oldest age group. CONCLUSIONS: The surveillance of coronary heart disease epidemiological data from 1986 to 1995 in this population showed a decreased mortality mainly attributable to the decline of attack rates in the youngest and only to case-fatality rates in the oldest age group.

Masked hypertension in young patients after successful aortic coarctation repair: impact on left ventricular geometry and function.

Life expectancy is still reduced in aortic coarctation (AoC) patients despite a successful repair because of late arterial hypertension and atherosclerosis. Masked hypertension (MH) consists of an elevated daytime or awake ambulatory blood pressure (BP) in the presence of a normal BP on conventional measurement at the office. To assess the prevalence of MH among AoC normotensive young patients successfully treated and to evaluate the impact of MH on left ventricular (LV) geometry and function.We studied 76 AoC patients (mean age 14.5±5.7 years, male 64%). According to 24 h ambulatory BP monitoring (ABPM) our sample was divided in real normotensive patients (Group RN, n=40) and MH patients (Group MH, n=36). There was an increased pressure gradient in the aortic arch (15 mm Hg±4 vs 13 mm Hg±4.7, P<0.05), increased LV mass (51 g m(-2.7)±13 vs 46 g m(-2.7)±12, P<0.05), in MH AoC patients. Regional longitudinal deformation properties of the basal septal segment (-15%±2.4 vs -20%±5, P<0.01) and LV twist values (14°±1.6 vs 12°±1.9, P<0.0001) were reduced in the MH group. There is a high prevalence of MH in young patients with repaired AoC, which is associated with abnormal LV structure and function. Clinicians should consider 24 h ABPM measurements in apparently normotensive patients followed up for AoC repair.