Cardiac imaging and biomarkers for assessing myocardial fibrosis in children with hypertrophic cardiomyopathy.

BACKGROUND: Myocardial fibrosis, as diagnosed on cardiac magnetic resonance imaging (cMRI) by late gadolinium enhancement (LGE), is associated with adverse outcomes in adults with hypertrophic cardiomyopathy (HCM), but its prevalence and magnitude in children with HCM have not been established. We investigated: (1) the prevalence and extent of myocardial fibrosis as detected by LGE cMRI; (2) the agreement between echocardiographic and cMRI measurements of cardiac structure; and (3) whether serum concentrations of N-terminal pro hormone B-type natriuretic peptide (NT-proBNP) and cardiac troponin-T are associated with cMRI measurements. METHODS: A cross-section of children with HCM from 9 tertiary-care pediatric heart centers in the U.S. and Canada were enrolled in this prospective NHLBI study of cardiac biomarkers in pediatric cardiomyopathy (ClinicalTrials.gov Identifier: NCT01873976). The median age of the 67 participants was 13.8 years (range 1-18 years). Core laboratories analyzed echocardiographic and cMRI measurements, and serum biomarker concentrations. RESULTS: In 52 children with non-obstructive HCM undergoing cMRI, overall low levels of myocardial fibrosis with LGE >2% of left ventricular (LV) mass were detected in 37 (71%) (median %LGE, 9.0%; IQR: 6.0%, 13.0%; range, 0% to 57%). Echocardiographic and cMRI measurements of LV dimensions, LV mass, and interventricular septal thickness showed good agreement using the Bland-Altman method. NT-proBNP concentrations were strongly and positively associated with LV mass and interventricular septal thickness (P < .001), but not LGE. CONCLUSIONS: Low levels of myocardial fibrosis are common in pediatric patients with HCM seen at referral centers. Longitudinal studies of myocardial fibrosis and serum biomarkers are warranted to determine their predictive value for adverse outcomes in pediatric patients with HCM.

Comparison of tissue Doppler imaging and conventional echocardiography to discriminate rejection from non-rejection after pediatric heart transplantation.

TDI is considered superior to conventional echocardiography for detecting changes in graft function during rejection in adults but has not demonstrated after pediatric OHT. We retrospectively analyzed echocardiograms performed within 24 hours of biopsy in 122 recipients with median age of 8.7 years. Using biopsy findings as the gold standard, we compared paired rejection and non-rejection echocardiograms using each patient as their own control. We included pairs of LV dimensions, FS, volumes, mass, mass/volume, sphericity, wall stress, SSI, SVI, and TDI velocities in this comparison. C-statistic was used to assess discrimination for individual echo variables and combinations of variables. Overall, 647 non-rejection and 24 rejection biopsy-echo pairs were identified. There was a significant decline in TDI velocities and their Z-scores during rejection but not in conventional variables (P ≤ .005). The variable that best discriminated rejection from non-rejection was LV S', with C-statistic = 0.93. Conventional echo variables performed less well with C-statistic range 0.65-0.67 for LV EF, shortening fraction, and mass. TDI is superior to conventional echocardiography measures for discriminating rejection from non-rejection. The use of newer non-invasive parameters to detect myocardial dysfunction and shifting the paradigm of rejection surveillance to detection of non-rejection together provide a promising approach to reducing the need for biopsy in pediatric heart recipients.

PTB-XL, a large publicly available electrocardiography dataset.

Electrocardiography (ECG) is a key non-invasive diagnostic tool for cardiovascular diseases which is increasingly supported by algorithms based on machine learning. Major obstacles for the development of automatic ECG interpretation algorithms are both the lack of public datasets and well-defined benchmarking procedures to allow comparison s of different algorithms. To address these issues, we put forward PTB-XL, the to-date largest freely accessible clinical 12-lead ECG-waveform dataset comprising 21837 records from 18885 patients of 10 seconds length. The ECG-waveform data was annotated by up to two cardiologists as a multi-label dataset, where diagnostic labels were further aggregated into super and subclasses. The dataset covers a broad range of diagnostic classes including, in particular, a large fraction of healthy records. The combination with additional metadata on demographics, additional diagnostic statements, diagnosis likelihoods, manually annotated signal properties as well as suggested folds for splitting training and test sets turns the dataset into a rich resource for the development and the evaluation of automatic ECG interpretation algorithms.

Is Doppler echocardiography useful for estimating left ventricular filling pressures in pediatric heart transplant recipients?

LV E/E' ratio obtained using Doppler echocardiography is considered a surrogate for LV filling pressure in adults but has performed poorly in children. We hypothesized that LV E/E' ratio Z-score, adjusted for age, will relate more strongly to LV filling pressures than LV E/E' ratio in pediatric HT recipients. We analyzed 751 echocardiograms performed within 24 hours of a heart catheterization in 122 pediatric HT recipients (median age at HT 13 years, median 6 studies per patient). The primary end-point was PCWP, assessed both as a continuous and a binary variable. Associations with LV E/E' ratios and z-scores were assessed using generalized estimating equations models. PCWP, LV E/E' ratios (using E' from LV free wall, septum, and their average), and LV E/E' ratio Z-scores, all declined over time after HT. LV E/E' ratios and their Z-scores were significantly associated with PCWP assessed as a continuous variable (P < 0.001 for all); however, the relationship was weak (R2 range, 0.083 to 0.121). LV E/E' ratios and their Z-scores were also significantly associated with PCWP as a binary variable (P < 0.001 for all) but with only modest ability to discriminate PCWP ≥15 mm Hg (c-statistic range, 0.660 to 0.695). The association between LV E/E' ratio and PCWP in pediatric HT recipients is modest. Using a LV E/E' ratio Z-score did not result in significantly improved association with PCWP. Current Doppler echocardiographic methods are unreliable for estimating LV filling pressures in pediatric HT recipients.

Feasibility of exercise stress echocardiography and myocardial response in patients with repaired congenital heart disease.

BACKGROUND: Exercise stress echocardiography (ESE) can unmask ventricular dysfunction in asymptomatic patients with congenital heart disease (CHD), but its acquisition and interpretation is often challenging, and the method has not been validated in CHD. This study aimed to evaluate the feasibility of ESE using Doppler imaging and to assess myocardial response to exercise in patients with biventricular (BiV) and univentricular (UniV) circulation after CHD repair. METHODS: In this single-center prospective study, we recruited 55 participants (17 females), median age 14 years (8-22 years). Our analysis categorized participants in these three groups: with structurally normal hearts as controls (n=21), with BiV circulation (n=20) and with UniV circulation (n=14). We acquired ESE images of the systemic ventricle including pulsed-wave flow and spectral tissue Doppler imaging (TDI) of lateral free wall before and immediately after standard, symptom-limited exercise tests on an electronically braked cycle ergometer. RESULTS: During ESE we obtained inflow E-wave and TDI systolic (S') and early diastolic (E') velocities in 93% to 100% of participants at rest and in 90% to 100% of participants post exercise. Feasibility to obtain Doppler imaging parameter was the same across study groups. The myocardial response to exercise was increase in heart rate (HR), S' and inflow E-wave velocity in all participants. Patients with BiV circulation had preserved ventricular function at rest. While patients with UniV circulation had low S', E', and E-wave velocities at rest in comparison to controls and to BiV group (all P<.001), both patients with BiV and UniV circulation showed significant increases in HR, S' velocity and inflow E-wave velocity post exercise, with magnitudes of these increases higher in controls than in the BiV and UniV group. The S' and E' velocities were strongly associated with lower percent predicted peak oxygen consumption VO2 (rs=0.614 and rs=0.64, respectively, both P<.001). CONCLUSION: ESE with Doppler imaging is a practical noninvasive diagnostic method and sufficiently robust for the assessment of morphologic LV/systemic ventricles under exercise in patients after biventricular and univentricular CHD repair. Although patients with BiV and UniV circulation had both preserved myocardial response to exercise, the magnitude of this response was the lowest in patients with UniV circulation.

Progressive intermediate-term improvement in ventricular and atrioventricular interaction after transcatheter pulmonary valve replacement in patients with right ventricular outflow tract obstruction.

BACKGROUND: Relief of postoperative right ventricular outflow tract (RVOT) obstruction with transcatheter pulmonary valve replacement (TPVR) results in functional improvement in the short term which we investigated at baseline (BL), early follow-up (FU), and midterm FU after TPVR. METHODS: Echocardiography and cardiopulmonary exercise testing were performed at BL and at early (median 6 months) and midterm FU (median 2.5years) after TPVR. RESULTS: Patients with RVOT obstruction (n=22, median age 17years) were studied. The max RVOT Doppler gradient fell from BL to early FU (60±24 to 26±8mm Hg, P<.001). Left ventricular (LV) end-diastolic and stroke volume increased at early FU (both P<.001) without further change, whereas LV ejection fraction improved throughout FU (P<.001). LV end-systolic and diastolic eccentricity (leftward septal displacement) improved early (both P≤.003), and end-diastolic eccentricity improved further at midterm FU (P=.02). Furthermore, whereas mitral inflow A wave velocity increased (P=.003), the LV A' velocity declined early (P=.007) without further change at midterm. RV systolic and early diastolic function was impaired at BL. Whereas RV strain improved partially at early and midterm FU (P≤.02), RV E' velocity did not improve throughout FU. Mildly impaired LV strain at BL fully recovered by midterm FU (P≤.002). Peak oxygen uptake improved at early and midterm FU (all P≤.003). CONCLUSIONS: Patients with RVOT obstruction had biventricular systolic and diastolic dysfunction at BL. Relieving RVOT obstruction with TPVR reduced adverse ventricular and compensatory atrioventricular interaction, resulting in progressive biventricular functional improvement and remodeling at early and midterm FU.

Sexual violence from police and HIV risk behaviours among HIV-positive women who inject drugs in St. Petersburg, Russia - a mixed methods study.

INTRODUCTION: Police violence against people who inject drugs (PWID) is common in Russia and associated with HIV risk behaviours. Sexual violence from police against women who use drugs has been reported anecdotally in Russia. This mixed-methods study aimed to evaluate sexual violence from police against women who inject drugs via quantitative assessment of its prevalence and HIV risk correlates, and through qualitative interviews with police, substance users and their providers in St. Petersburg, Russia. METHODS: Cross-sectional analyses with HIV-positive women who inject drugs (N=228) assessed the associations between sexual violence from police (i.e. having been forced to have sex with a police officer) and the following behaviours: current drug use, needle sharing and injection frequency using multiple regression models. We also conducted in-depth interviews with 23 key informants, including PWID, police, civil society organization workers, and other stakeholders, to explore qualitatively the phenomenon of sexual violence from police in Russia and strategies to address it. We analyzed qualitative data using content analysis. RESULTS: Approximately one in four women in our quantitative study (24.1%; 95% CI, 18.6%, 29.7%) reported sexual violence perpetrated by police. Affected women reported more transactional sex for drugs or money than those who were not; however, the majority of those reporting sexual violence from police were not involved in these forms of transactional sex. Sexual violence from police was not significantly associated with current drug use or needle sharing but with more frequent drug injections (adjusted incidence rate ratio 1.43, 95% CI 1.04, 1.95). Qualitative data suggested that sexual violence and coercion by police appear to be entrenched as a norm and are perceived insurmountable because of the seemingly absolute power of police. They systematically add to the risk environment of women who use drugs in Russia. CONCLUSIONS: Sexual violence from police was common in this cohort of Russian HIV-positive women who inject drugs. Our analyses found more frequent injection drug use among those affected, suggesting that the phenomenon represents an underappreciated human rights and public health problem. Addressing sexual violence from police against women in Russia will require addressing structural factors, raising social awareness and instituting police trainings that protect vulnerable women from violence and prevent HIV transmission.

Global surgery in a postconflict setting--5-year results of implementation in the Russian North Caucasus.

BACKGROUND: Collaborations for global surgery face many challenges to achieve fair and safe patient care and to build sustainable capacity. The 2004 terrorist attack on a school in Beslan in North Ossetia in the Russian North Caucasus left many victims with complex otologic barotrauma. In response, we implemented a global surgery partnership between the Vladikavkaz Children's Hospital, international surgical teams, the North Ossetian Health Ministry, and civil society organizations. This study's aim was to describe the implementation and 5-year results of capacity building for complex surgery in a postconflict, mid-income setting. DESIGN: We conducted an observational study at the Children's Hospital in Vladikavkaz in the autonomous Republic of North Ossetia-Alania, part of the Russian Federation. We assessed the outcomes of 15 initial patients who received otologic surgeries for complex barotrauma resulting from the Beslan terrorism attack and for other indications, and report the incidence of intra- and postoperative complications. RESULTS: Patients were treated for trauma related to terrorism (53%) and for indications not related to violence (47%). None of the patients developed peri- or postoperative complications. Three patients (two victims of terrorism) who underwent repair of tympanic perforations presented with re-perforations. Four junior and senior surgeons were trained on-site and in Germany to perform and teach similar procedures autonomously. CONCLUSIONS: In mid-income, postconflict settings, complex surgery can be safely implemented and achieve patient outcomes comparable to global standards. Capacity building can build on existing resources, such as operation room management, nursing, and anesthesia services. In postconflict environments, substantial surgical burden is not directly attributable to conflict-related injury and disease, but to health systems weakened by conflicts. Extending training and safe surgical care to include specialized interventions such as microsurgery are integral components to strengthen local capacity and ownership. Our experience identified strategies for fair patient selection and might provide a model for potentially sustainable surgical system building in postconflict environments.

Left Ventricular Strain and Myocardial Fibrosis in Congenital Aortic Stenosis.

The aim of this study was to evaluate whether left ventricular (LV) systolic strain in children and young adults with congenital aortic stenosis (AS) and preserved ejection fraction was different from normal subjects and to determine whether any alterations in strain were related to myocardial fibrosis. In this retrospective study, 29 patients with congenital AS with a median age of 15.3 years (range 1.7 to 23.7), highest lifetime AS peak Doppler gradient of 73 mm Hg (22 to 110), most recent AS peak Doppler gradient of 49 mm Hg (0 to 90), and ejection fraction of 65 (55 to 79) were included. Strain was measured using 2-dimensional speckle-tracking echocardiography. Cardiac magnetic resonance was used to identify focal fibrosis by late gadolinium enhancement (LGE) and diffuse fibrosis by calculating the extracellular volume fraction (ECV) from T1 measurements. Compared to age-matched controls (n = 29), patients with AS had reduced LV longitudinal (17.0 ± 3.0% vs 20.6 ± 2.2%, p <0.001) and radial strain (28.8 ± 8.6% vs 34.9 ± 8.5%, p = 0.01), and similar circumferential strain (26.2 ± 5.9% vs 26.4 ± 3.9%, p = 0.79). Median ECV in patients with AS was 0.27 (0.22 to 0.38) and was not significantly correlated with systolic strain. Patients with LGE (n = 7) had lower longitudinal strain than those without LGE (n = 21; 15.0 ± 2.2 vs 17.7 ± 3.1, p = 0.036). In conclusion, in this cohort of children and young adults with congenital AS and preserved ejection fraction, longitudinal and radial strain were reduced, and decreased longitudinal strain was associated with LGE but not ECV.

Stigma and Human Rights Abuses against People Who Inject Drugs in Russia--A Qualitative Investigation to Inform Policy and Public Health Strategies.

INTRODUCTION: Drug policing practices in the Russian Federation (Russia) are often punitive and have been shown to be associated with HIV risk behaviors among people who inject drugs (PWID). Less is known about strategies to address the problem in that setting, where substance use stigma is highly persistent. A better understanding of forms, causes and consequences of drug policing in Russia could inform drug policy in a context of substantial policy resistance. This qualitative study's goal is to characterize the phenomenon of police involvement with Russian PWID and to explore strategies for drug policing in the Russian country context. METHODS: Using a semi-structured interview guide, we collected data from a purposive sample of 23 key informants including PWID, police officers, and experts from civil society and international organizations in Russia. We used a thematic analysis approach to inductively generate new insight into the phenomenon of police involvement and potential strategies to address it. RESULTS: Policing practices involving PWID include unjustified arrests, planting of false evidence and extrajudicial syringe confiscations, and often constitute human rights violations. Russian PWID personally experienced police violence as ubiquitous, taking on various forms such as beating, unjustified arrests, verbal harassment, and coercion. The persistent societal stigma dehumanizes PWID, and such stigmatization facilitates police abuse. To address stigma and overcome the PWID-police adversity, study participants suggested fostering a mutual understanding between the police and public health sectors. CONCLUSIONS: Participants describe substantial human rights violations as part of policing illicit drug use in Russia. Police should include principles of effective prevention of substance use and HIV risk reduction in their trainings. Alignment of public safety and public health goals could address drug use-related risks and HIV prevention among key populations in Russia.

Effects of transcatheter pulmonary valve replacement on the hemodynamic and ventricular response to exercise in patients with obstructed right ventricle-to-pulmonary artery conduits.

OBJECTIVES: This study sought to investigate the effects of exercise on the right ventricle in patients with an obstructed right ventricular outflow tract (RVOT) conduit before and after transcatheter pulmonary valve replacement (TPVR). BACKGROUND: Conventionally, assessment of the right ventricle in congenital heart disease patients with dysfunctional RVOT conduits is performed at rest. However, this does not reflect dynamic exercise changes. METHODS: Exercise stress echocardiography (ESE) before and 6 months after TPVR was performed. ESE protocol included measurement of rest and immediate post-exercise RVOT maximal instantaneous gradients (MIGs), right ventricular (RV) systolic pressure, 2-dimensional fractional area change, and global longitudinal strain (GLS). RESULTS: Twenty patients with RVOT conduit obstruction (median age, 18 years), the majority (n = 14) with tetralogy of Fallot, completed the study. Pre-TPVR, the median resting MIG across the RVOT was 53 mm Hg (23 to 95 mm Hg) and increased to 93 mm Hg (49 to 156 mm Hg; p < 0.001) with exercise. After TPVR, the median MIG at rest was 26 mm Hg (6 to 41 mm Hg, and after exercise, it was 45 mm Hg (9 to 102 mm Hg), both significantly lower than before TPVR (p ≤ 0.001), but there was still a substantial increase in gradient with exercise in many patients. The RV fractional area change, RV GLS, and left ventricular GLS, both at rest and after exercise, were significantly greater after TPVR than before. A greater pre-TPVR exercise-related increase in RV function was associated with improvement in peak Vo2 after TPVR (p = 0.01). CONCLUSIONS: In patients with obstructed RVOT conduits, TPVR resulted in significant improvement in conduit stenosis and RV function at both rest and at peak exercise and in exercise cardiopulmonary function. The ability to augment RV function at peak exercise before TPVR was associated with improved exercise capacity 6 months after TPVR.

Heterogeneity of regional function and relation to ventricular morphology in patients with fontan circulation.

The relation between underlying ventricular morphology and regional function in patients with Fontan circulation remains unclear. The aim of this study was to compare regional function and its heterogeneity in patients with tricuspid atresia (TA), biventricular apex-forming morphology (BiV), and controls. Nineteen patients (median age 12 years) with Fontan circulation who presented consecutively were prospectively enrolled and compared with age- and heart rate-matched controls. Most patients were in New York Heart Association class I (63%). Longitudinal systolic strain (S), systolic strain rate (SRsys), and early diastolic strain rate (SRdia) peaks were obtained from 6 ventricular segments, and a coefficient of variation by segment was calculated as a measure of regional heterogeneity. Systolic S, SRsys and SRdia peaks were decreased at the right and left lateral walls in both patient groups compared with controls (p ≤0.001 for all). Patients with TA had higher systolic S and SRsys in the middle of the right lateral wall than those with BiV morphology (p = 0.009 and p = 0.001, respectively). The mean coefficients of variation assessed by S and SRsys were similar in controls and patients with TA but lower in those with BiV than in controls and patients with TA (p <0.001 and p = 0.01, respectively). The mean coefficient of variation assessed by SRdia was greater only in patients with BiV than in controls (p = 0.001). In conclusion, patients with Fontan circulation have more heterogeneous systolic and early diastolic regional function than healthy control subjects, and patients with TA have better systolic regional function in the middle of the right lateral wall and less systolic heterogeneity than patients with BiV morphology.

Tissue Doppler imaging for rejection surveillance in pediatric heart transplant recipients.

BACKGROUND: Most transplant centers perform serial cardiac biopsies for rejection surveillance in pediatric heart transplant (HT) recipients. We sought to assess tissue Doppler imaging (TDI) findings during biopsy specimen-proven rejection in pediatric HT recipients and to develop TDI criteria for absence of rejection with high predictive accuracy. METHODS: We included the 122 HT recipients in follow-up at our center (median age at HT, 8.7 years). We identified all echocardiograms with adequate TDI data performed within 24 hours of a cardiac biopsy during 2005 to 2011. Rejection was defined as Grade ≥ 2R cellular rejection or antibody-mediated rejection. Paired comparisons of TDI velocities were made using patients' baseline velocities as the control. RESULTS: Overall, 647 specimen-pairs were identified where there was no rejection at baseline. In 24 of these, the second biopsy specimen demonstrated rejection. Using receiver operating characteristic curve analysis of percentage change from baseline, we identified < 15% decline in left ventricular (LV) S' velocity and < 5% decline in LV A' velocity to individually predict non-rejection with > 99% accuracy. When joint criteria were used, the predictive accuracy was 100%, and no rejection event was misclassified. More than 75% of TDI pairs met these criteria for non-rejection. CONCLUSIONS: Biopsy specimen-proven rejection is associated with a significant decline in biventricular TDI velocities from baseline in pediatric HT recipients. By using well-defined TDI criteria to predict non-rejection, a substantial proportion of planned biopsies may be deferred or avoided at minimal risk to pediatric HT recipients.

Cardiac allograft function during the first year after transplantation in rejection-free children and young adults.

BACKGROUND: Allograft dysfunction is a common finding early after heart transplant (HT). We sought to assess the recovery of left (LV) and right ventricular (RV) function during the first year after HT in children and young adults using pulsed-wave tissue Doppler imaging. METHODS AND RESULTS: We analyzed serially performed echocardiography in 44 pediatric HT recipients (median age: 7.3 years at HT) who remained rejection-free during the first year post-transplant. Age-based normative values for systolic (S'), early-diastolic (E'), and late-diastolic (A') velocities obtained using pulsed-wave tissue Doppler imaging in 380 healthy children were used to transform patient data into z scores. Pulsed-wave tissue Doppler imaging studies ≤10 days post-HT demonstrated biventricular systolic and diastolic dysfunction with most prominent impairment in RV systolic function (S' z score -2.7±0.8), RV early-diastolic filling (E' z score -2.3±1.1), and LV early-diastolic filling (E' z score -2.3±1.1). LV systolic function (S' z score) and late-diastolic filling (A' z score) improved to normal in 11 to 30 days, LV early-diastolic filling (E' z score) in 4 to 6 months, and RV early-diastolic filling in 6 to 9 months (P<0.001 for all on longitudinal analysis). However, RV systolic function (RV S' z score -1.2±1.1) remained impaired 1-year post-transplant. Analysis of serial cardiac catheterization studies showed that RV and LV filling pressures were elevated early post-HT and declined gradually during the first year post-transplant. CONCLUSIONS: Pediatric HT recipients have biventricular dysfunction using pulsed-wave tissue Doppler imaging early after HT with most significant impairment in RV systolic function and RV and LV early-diastolic filling. Although other aspects of LV and RV function normalize in 6 to 9 months, RV systolic function remains abnormal 1 year-post-transplant.

Addressing the burden of post-conflict surgical disease - strategies from the North Caucasus.

The 2004 terror attack on a school in Beslan, North Caucasus, with more than 1300 children and their families taken hostage and 334 people killed, ended after extreme violence. Following the disaster, many survivors with blast ear injuries developed complications because no microsurgery services were available in the region. Here, we present our strategies in North Ossetia to strengthen subspecialty surgical care in a region of instable security conditions. Disaster modifies disease burden in an environment of conflict-related health-care limitations. We built on available secondary care and partnered international with local stakeholders to reach and treat victims of a humanitarian disaster. A strategy of mutual commitment resulted in treatment of all consenting Beslan victims with blast trauma sequelae and of non disaster-related patients. Credible, sustained partnerships and needs assessments beyond the immediate phases after a disaster are essential to facilitate a meaningful transition from humanitarian aid to capacity building exceeding existing insufficient standards. Psychosocial impacts of disaster might constitute a barrier to care and need to be assessed when responding to the burden of surgical disease in conflict or post-conflict settings. Involving local citizen groups in the planning process can be useful to identify and access vulnerable populations. Integration of our strategy into broader efforts might strengthen the local health system through management and leadership.

Preserved regional atrial contractile function following extra-atrial rather than intra-atrial type Fontan operation: a tissue Doppler imaging study.

OBJECTIVE: To determine the univentricular and atrial function in patients following Fontan operation using tissue Doppler echocardiography (TDE). SETTING: Hospital-based outpatient clinic and inpatient unit. PATIENTS: Thirty-six patients (mean age 13 ys [2-34 ys]) after Fontan procedure and 30 healthy individuals matched for age and heart rate. MAIN OUTCOME MEASURES: Pulsed wave Doppler derived Tei index was determined for global univentricular function. Tissue-Doppler derived strain rate and strain were measured for regional ventricular function. Different planimetric approaches were used to evaluate the global atrial function. Strain rate was measured for regional atrial contractile function. RESULTS: The Tei index, reflecting global univentricular function, was significantly higher in Fontan patients than in the control group (p<0.001). The regional ventricular contractile function, which was determined by TDE derived strain and strain rate, was significantly lower in Fontan patients than in healthy controls (p<0.001). The global atrial function evaluated planimetrically, and the regional atrial function assessed using the TDE strain rate, were significantly lower in Fontan patients than in normal controls (p<0.001, p<0.001, respectively). A comparison of different types of Fontan operations with extra-atrial or intra-atrial baffle showed a better regional left atrial wall strain rate in patients with extra-atrial baffle than with intra-atrial baffle. CONCLUSION: Patients who have undergone the Fontan procedure may present with altered ventricular and atrial performance independent of whether the extra-atrial or intraatrial type Fontan operation was performed. However, the regional atrial contractile function seems to be better preserved following the extra-atrial type Fontan operation.