Effect of anthracycline therapy on myocardial function and markers of fibrotic remodelling in childhood cancer survivors.

AIMS: Anthracyclines are a cornerstone of paediatric cancer treatment. We aimed to quantify myocardial cardiac magnetic resonance (CMR) native T1 (NT1) and extracellular volume fraction (ECV) as markers of fibrosis in a cohort of childhood cancer survivors (CCS). METHODS AND RESULTS: A cohort of CCS in remission underwent CMR T1 mapping. Diastolic function was assessed by echocardiography. Results were compared to a cohort of normal controls of similar age and gender. Fifty-five CCS and 46 controls were included. Both groups had similar mean left ventricular (LV) NT1 values (999 ± 36 vs. 1007 ± 32 ms, P = 0.27); ECV was higher (25.6 ± 6.9 vs. 20.7 ± 2.4%, P = 0.003) and intracellular mass was lower (37.5 ± 8.4 vs. 43.3 ± 9.9g/m2, P = 0.02) in CCS. The CCS group had lower LV ejection fraction (EF) and LV mass index with otherwise normal diastolic function in all but one patient. The proportion of subjects with elevated ECV compared to controls did not differ between subgroups with normal or reduced LV EF (22% vs. 28%; P = 0.13) and no correlations were found between LVEF and ECV. While average values remained within normal range, mitral E/E' (6.6 ± 1.6 vs. 5.9 ± 0.9, P = 0.02) was higher in CCS. Neither NT1 nor ECV correlated with diastolic function indices or cumulative anthracycline dose. CONCLUSIONS: There is evidence for mild diffuse extracellular volume expansion in some asymptomatic CCS; myocyte loss could be part of the mechanism, accompanied by subtle changes in systolic and diastolic function. These findings suggest mild myocardial damage and remodelling after anthracycline treatment in some CCS which requires continued monitoring.

Pediatric Fontan patients are at risk for myocardial fibrotic remodeling and dysfunction.

BACKGROUND: Patients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation. METHODS: Consecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study. Native T1 times (T1) and extracellular volume fractions (ECV) in the free wall of the dominant ventricle (left, SLV; right, SRV) were compared with controls and correlated with hemodynamic and clinical parameters. RESULTS: Twenty-one SV patients (9.7±4.6years; 13 SLV; 8 SRV) and 24 healthy control children (13.9±2.6years, p=0.002) were included. T1 and ECV were higher in SRV patients than in controls (1036±46ms vs 974±27ms, p<0.001; 28±4% vs 22±3%, p=0.002) and SLV patients (978±39ms, p=0.002; 23±5%, p=0.012) while there was no difference between SLV patients and controls. Age at bidirectional cavopulmonary connection was correlated with T1 (R=0.55, p=0.015), while systolic blood pressure (R=-0.68, p<0.001) and body weight (R=-0.54, p=0.012) inversely correlated with ECV. T1 negatively correlated with radial and circumferential strain by CMR feature tracking. CONCLUSIONS: Fontan patients with a SRV show increased CMR markers of diffuse myocardial fibrosis, which are associated with decreased myocardial contractility. Whether their increased fibrosis burden conveys a greater risk for long-term complications in this population remains to be investigated.

Assessment of Diffuse Ventricular Myocardial Fibrosis Using Native T1 in Children With Repaired Tetralogy of Fallot.

BACKGROUND: Myocardial fibrosis is linked with adverse clinical outcomes in adults after tetralogy of Fallot repair (rTOF). Native T1 times (T1) by cardiac magnetic resonance have been shown to be a surrogate marker of diffuse myocardial fibrosis. The objective was to quantify native T1 in children post-rTOF and to evaluate their relationship with surgical, imaging, and clinical factors. METHODS AND RESULTS: A retrospective cross-sectional study was performed. Midventricular native T1 were obtained in 100 children post-rTOF using a modified look-locker inversion recovery cardiac magnetic resonance sequence and compared with 35 pediatric controls. rTOF patients, aged 13.0±2.9 years, had higher indexed right ventricular (RV) end-diastolic (range 85-326 mL/m2, mean 148 mL/m2) volumes, and lower RV and left ventricular (LV) ejection fractions compared with controls. RV, but not LV, T1 were higher in patients than in controls (1031±74 versus 954±32 ms, P<0.001) and female patients had higher RV T1 compared with males (1051±79 versus 1017±68 ms, P=0.02). LV T1 correlated with RV T1 (r=0.45, P<0.001), cardiopulmonary bypass (r=0.30, P=0.007), and aortic cross-clamp times (r=0.32, P=0.004). RV T1 correlated inversely with RV outflow tract gradient (r=-0.28, P=0.02). Longer aortic cross-clamp times were independently associated with LV and RV T1 on multivariable analysis. There was no association between exercise intolerance, arrhythmia, and native T1 or LV extracellular volume. CONCLUSIONS: Children after rTOF do not have elevated LV native T1 or LV extracellular volume, but show evidence of increased RV native T1 suggestive of diffuse RV fibrosis, for which volume loading seems to be a risk factor. Surgical bypass and cross-clamp times are associated with fibrotic remodeling over a decade later.

Histological validation of cardiovascular magnetic resonance T1 mapping markers of myocardial fibrosis in paediatric heart transplant recipients.

BACKGROUND: Adverse fibrotic remodeling is detrimental to myocardial health and a reliable method for monitoring the development of fibrotic remodeling may be desirable during the follow-up of patients after heart transplantation (HTx). Quantification of diffuse myocardial fibrosis with cardiovascular magnetic resonance (CMR) has been increasingly applied and validated histologically in adult patients with heart disease. However, comparisons of CMR findings with histological fibrosis burden in children are lacking. This study aimed to compare native T1 times and extracellular volume fraction (ECV) derived from CMR with the degree of collagen on endomyocardial biopsy (EmBx), and to investigate the association between myocardial fibrosis and clinical as well as functional markers in children after HTx. METHODS: EmBx and CMR were performed on the same day. All specimens were stained with picrosirius red. The collagen volume fraction (CVF) was calculated as ratio of stained collagen area to total myocardial area on EmBx. Native T1 values and ECV were measured by CMR on a mid-ventricular short axis slice, using a modified look-locker inversion recovery approach. RESULTS: Twenty patients (9.9 ± 6.2 years of age; 9 girls) after HTx were prospectively enrolled, at a median of 1.3 years (0.02-12.6 years) post HTx, and compared to 24 controls (13.9 ± 2.6 years of age; 12 girls). The mean histological CVF was 10.0 ± 3.4%. Septal native T1 times and ECV were higher in HTx patients compared to controls (1008 ± 32 ms vs 979 ± 24 ms, p < 0.005 and 0.30 ± 0.03 vs 0.22 ± 0.03, p < 0.0001, respectively). CVF showed a moderate correlation with native T1 (r = 0.53, p < 0.05) as well as ECV (r = 0.46, p < 0.05). Native T1 time, but not ECV and CVF, correlated with ischemia time (r = 0.46, p < 0.05). CONCLUSIONS: CMR-derived fibrosis markers correlate with histological degree of fibrosis on EmBx in children after HTx. Further, native T1 times are associated with longer ischemia times.

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot.

BACKGROUND: Unfavorable left ventricular (LV) remodelling may be associated with adverse outcomes after Tetralogy of Fallot (TOF) repair. We sought to assess T1 cardiovascular magnetic resonance (CMR) markers of diffuse LV myocardial fibrosis in children after TOF repair, and associated factors. METHODS: In this prospective, cross-sectional study, native (=non-contrast) T1 times and extracellular volume fraction (ECV) were quantified in the LV myocardium using CMR. Results were related to ventricular volumes and function, degree of pulmonary regurgitation, as well as surgical characteristics, and exercise capacity. RESULTS: There was no difference in native T1 times or ECV between 31 TOF patients (age at CMR 13.9 ± 2.4 years, 19 male) and 15 controls (age at CMR 13.4 ± 2.6 years, 7 male). Female TOF patients had higher ECVs than males (25.2 ± 2.9 % versus 22.7 ± 3.3 %, p < 0.05). In the patient group, higher native T1 and ECV correlated with higher Z-Scores of right and left ventricular end-diastolic volumes, but not with reduced left and right ventricular ejection fraction or higher pulmonary regurgitation fraction. Longer cardiopulmonary bypass and aortic cross clamp times at surgery correlated with increased native T1 times and ECVs (r = 0.48, p < 0.05 and r = 0.65, p < 0.01, respectively). Maximum workload (percent of predicted for normal) correlated inversely with ECV (r = -0.62, p < 0.05). Higher native T1 times correlated with worse LV longitudinal (r = 0.50, p < 0.05) and mid short axis circumferential strain (r = 0.38, p < 0.05). CONCLUSIONS: As compared to controls, TOF patients did not express higher markers of diffuse fibrosis. Longer cardiopulmonary bypass and aortic cross clamp times at surgery as well as biventricular enlargement and reduced exercise tolerance are associated with markers of diffuse myocardial fibrosis after TOF repair. Female patients have higher markers of diffuse myocardial fibrosis than males.

Magnetic resonance imaging of the transplanted pediatric heart as a potential predictor of rejection.

AIM: To evaluate cardiac magnetic resonance imaging (CMR) as a non-invasive tool to detect acute cellular rejection (ACR) in children after heart transplant (HT). METHODS: Thirty pediatric HT recipients underwent CMR at the time of surveillance endomyocardial biopsy (EMB) and results were compared to 14 non-transplant controls. Biventricular volumes, ejection fractions (EFs), T2-weighted signal intensities, native T1 times, extracellular volumes (ECVs) and presence of late gadolinium enhancement (LGE) were compared between patients and controls and between patients with International Society of Heart and Lung Transplantation (ISHLT) grade ≥ 2R rejection and those with grade 0/1R. Heart rate (HR) and brain natriuretic peptide (BNP) were assessed as potential biomarkers. RESULTS: Significant ACR (ISHLT grade ≥ 2R) was an infrequent event in our population (5/30, 17%). Ventricular volumes, EFs, LGE prevalence, ECVs, native T1 times, T2 signal intensity ratios, HR and BNP were not associated with the presence of ≥ 2R ACR. CONCLUSION: In this pilot study CMR did not reliably identify ACR-related changes in pediatric HT patients.

Cardiac MRI in patients with complex CHD following primary or secondary implantation of MRI-conditional pacemaker system.

OBJECTIVES: In patients with CHD, cardiac MRI is often indicated for functional and anatomical assessment. With the recent introduction of MRI-conditional pacemaker systems, cardiac MRI has become accessible for patients with pacemakers. The present clinical study aims to evaluate safety, susceptibility artefacts, and image reading of cardiac MRI in patients with CHD and MRI-conditional pacemaker systems. Material and methods CHD patients with MRI-conditional pacemaker systems and a clinical need for cardiac MRI were examined with a 1.5-T MRI system. Lead function was tested before and after MRI. Artefacts and image readings were evaluated using a four-point grading scale. RESULTS: A total of nine patients with CHD (mean age 34.0 years, range 19.5-53.6 years) received a total of 11 cardiac MRI examinations. Owing to clinical indications, seven patients had previously been converted from conventional to MRI-conditional pacemaker systems. All MRI examinations were completed without adverse effects. Device testing immediately after MRI and at follow-up showed no alteration of pacemaker device and lead function. Clinical questions could be addressed and answered in all patients. CONCLUSION: Cardiac MRI can be performed safely with high certainty of diagnosis in CHD patients with MRI-conditional pacemaker systems. In case of clinically indicated lead and box changing, CHD patients with non-MRI-conditional pacemaker systems should be considered for complete conversion to MRI-conditional systems.

Diffuse Myocardial Fibrosis in Children After Heart Transplantations: A Magnetic Resonance T1 Mapping Study.

BACKGROUND: It is unclear whether the myocardium undergoes accelerated fibrotic remodeling in children after heart transplantation (HTx). METHODS: In this prospective study, cardiac magnetic resonance (CMR) studies in 17 patients 1.3 years (median, range 0.03-12.6 years) after HTx (mean age, 9.8 ± 6.2 years; 8 girls) were compared to CMR studies in 9 healthy controls (mean age, 12.4 ± 2.4 years; 4 girls). T1 measurements were performed at a midventricular short axis slice before (ie, native T1 times) and after the application of 0.2 mmol/kg gadopentetate dimeglumine in the interventricular septum, left ventricular (LV) free wall and encompassing the entire LV myocardium. The tissue-blood partition coefficient (TBPC), reflecting the degree of diffuse myocardial fibrosis, was calculated as a function of the ratio of T1 change of myocardium compared to blood. Native T1 times and TBPC were correlated with echocardiographic parameters of diastolic function. RESULTS: Native T1 times were significantly higher in HTx patients compared to controls in all regions assessed (LV free wall 973 ± 42 vs 923 ± 12 ms; P < 0.005; interventricular septum 1003 ± 31 vs 974 ± 21 ms, P < 0.05; entire LV myocardium 987 ± 33 vs 951 ± 16 ms; P < 0.005) and correlated with LV E/e' as an echocardiographic marker of diastolic dysfunction (r = 0.54, P < 0.05). The TBPC was elevated in the LV free wall (0.45 ± 0.06 vs 0.40 ± 0.03, P < 0.005) and the entire LV myocardium (0.47 ± 0.06 vs 0.43 ± 0.03, P < 0.05). CONCLUSIONS: Evidence of diffuse myocardial fibrosis and is already present in children after HTx. It appears to be associated with diastolic dysfunction.

MRI-based computational fluid dynamics for diagnosis and treatment prediction: clinical validation study in patients with coarctation of aorta.

PURPOSE: To reduce the need for diagnostic catheterization and optimize treatment in a variety of congenital heart diseases, magnetic resonance imaging (MRI)-based computational fluid dynamics (CFD) is proposed. However, data about the accuracy of CFD in a clinical context are still sparse. To fill this gap, this study compares MRI-based CFD to catheterization in the coarctation of aorta (CoA) setting. MATERIALS AND METHODS: Thirteen patients with CoA were investigated by routine MRI prior to catheterization. 3D whole-heart MRI was used to reconstruct geometries and 4D flow-sensitive phase-contrast MRI was used to acquire flows. Peak systolic flows were simulated using the program FLUENT. RESULTS: Peak systolic pressure drops in CoA measured by catheterization and CFD correlated significantly for both pre- and posttreatment measurements (pre: r = 0.98, p = 0.00; post: r = 0.87, p = 0.00). The pretreatment bias was -0.5 ± 3.33 mmHg (95% confidence interval -2.55 to 1.47 mmHg). CFD predicted a reduction of the peak systolic pressure drop after treatment that ranged from 17.6 ± 5.56 mmHg to 6.7 ± 5.58 mmHg. The posttreatment bias was 3.0 ± 2.91 mmHg (95% CI -1.74 to 5.43 mmHg). CONCLUSION: Peak systolic pressure drops can be reliably calculated using MRI-based CFD in a clinical setting. Therefore, CFD might be an attractive noninvasive alternative to diagnostic catheterization.

Pressure fields by flow-sensitive, 4D, velocity-encoded CMR in patients with aortic coarctation.

This study compared pressure fields by 4-dimensional (4D), velocity-encoded cine (VEC) cardiac magnetic resonance imaging (CMR) with pressures measured by the clinical gold standard catheterization. Thirteen patients (n = 7 male, n = 6 female) with coarctation were studied. The 4D-VEC-CMR pressure fields were computed by solving the Pressure-Poisson equation. The agreement between catheterization and CMR-based methods was determined at 5 different measurement sites along the aorta. For all sites, the correlation coefficients between measures varied between 0.86 and 0.97 (p < 0.001). The Bland-Altman test showed good agreement between peak systolic pressure gradients across the coarctation. The nonsignificant (p > 0.2) bias was +2.3 mm Hg (± 6.4 mm Hg, 2 SDs) for calibration with dynamic pressures and +1.5 mm Hg (± 4.6 mm Hg, 2 SDs) for calibration with static pressure. In a clinical setting of coarctation, pressure fields can be accurately computed from 4D-VEC-CMR-derived flows. In patients with coarctation, this noninvasive technique might evolve to an alternative to invasive catheterization.

Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging reveals abnormal blood flow patterns in the aorta and pulmonary trunk of patients with transposition.

BACKGROUND AND OBJECTIVES: Flow profiles are important determinants of fluid-vessel wall interactions. The aim of this study was to assess blood flow profiles in the aorta and pulmonary trunk in patients with transposition and different ventriculoarterial connection, and hence different mechanics of the coherent pump. METHODS: In all, 29 patients with operated transposition--concordant atrioventricular and discordant ventriculoarterial connection, and no other cardiac malformation--and eight healthy volunteers were assessed with cardiac magnetic resonance imaging: n = 17 patients after atrial redirection, with a morphologic right ventricle acting as systemic pump and a morphologic left ventricle connected to the pulmonary trunk, and n = 12 patients after the arterial switch procedure, with physiologic ventriculoarterial connections. Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging was used to analyse systolic flow patterns in the aorta and pulmonary trunk, relating to helical flow and vortex formation. RESULTS: In the aorta, overall helicity was present in healthy volunteers, but it was absent in all patients independent on the operation technique. Partial helices were observed in the ascending aorta of 58% of patients after arterial switch. In the pulmonary trunk, mostly parallel flow was seen in healthy volunteers and in patients after arterial switch, whereas vortex formation was present in 88% of patients after atrial redirection. CONCLUSION: Blood flow patterns differ substantially between the groups. In addition to varying mechanics of the coherent pumping ventricles, the absent overall helicity in all patients might be explained by the missing looping of the aorta in transposition.

Surgery impacts right atrial function in tetralogy of Fallot.

OBJECTIVE: To analyze the impact of surgery and pericardial integrity on right atrial function and total heart volume variation in the setting of pulmonary valve insufficiency. METHODS: Right atrial function and total heart volume variation were analyzed in 2 subgroups of patients with pulmonary valve insufficiency compared with healthy controls: group 1 with surgically repaired tetralogy of Fallot (n = 20 patients) and group 2 after balloon angioplasty of pulmonary valve stenosis in patients with isolated valve disease without surgery (n = 7 patients). Volumetric analysis of magnetic resonance imaging data revealed parameters of atrial function (reservoir, conduit, and pump functions and cyclic volume change) and of total heart volume (end-diastolic and end-systolic total heart volume and the variation). Statistical analysis included uncorrected and corrected pairwise comparisons and the calculation of groupwise Pearson correlation coefficients. RESULTS: In group 1 with a pulmonary regurgitation fraction of 31.0% ± 14.9%, right atrial function was clearly impaired, with reduced reservoir and elevated conduit function, and total heart volume variation was elevated to 13.9% ± 3.4%. In group 2 with a pulmonary regurgitation fraction of 22.8% ± 6.9%, the values were close to normal, with unaffected atrial function and a total heart volume variation of 9.9% ± 3.3%. CONCLUSIONS: The hydrodynamic effect of pulmonary valve insufficiency alone is likely not the only reason for impaired right atrial function and elevated total heart volume variation in patients with tetralogy of Fallot; it is rather the scar in the right atrium, the injured pericardium, and the disease itself that are responsible for the energetically unfavorable alterations.

Four-dimensional velocity-encoded magnetic resonance imaging improves blood flow quantification in patients with complex accelerated flow.

PURPOSE: To evaluate the use of four-dimensional (4D) velocity-encoded magnetic resonance imaging (VEC MRI) for blood flow quantification in patients with semilunar valve stenosis and complex accelerated flow. MATERIALS AND METHODS: Peak velocities (Vmax) and stroke volumes (SV) were quantified by 2D and 4D VEC MRI in volunteers (n = 7) and patients with semilunar valve stenosis (n = 18). Measurements were performed above the aortic and pulmonary valve with both techniques and, additionally, at multiple predefined planes in the ascending aorta and in the pulmonary trunk within the 4D dataset. In patients, 4D VEC MRI streamline analysis identified flow patterns and regions of highest flow velocity (4D(max-targeted)) for further measurements and Vmax was also measured by Doppler-echocardiography. RESULTS: In patients, 4D VEC MRI showed higher Vmax than 2D VEC MRI (2.7 ± 0.6 m/s vs. 2.4 ± 0.5 m/s, P < 0.03) and was more comparable to Doppler-echocardiography (2.8 ± 0.7 m/s). 4D(max-targeted) revealed highest Vmax values (3.1 ± 0.6 m/s). SV measurements showed significant differences between different anatomical levels in the ascending aorta in patients with complex accelerated flow, whereas differences in volunteers with laminar flow patterns were negligible (P = 0.004). CONCLUSION: 4D VEC MRI improves MRI-derived blood flow quantification in patients with semilunar valve stenosis and complex accelerated flow.

Flow-sensitive four-dimensional magnetic resonance imaging facilitates and improves the accurate diagnosis of partial anomalous pulmonary venous drainage.

OBJECTIVES: To assess if flow-sensitive four-dimensional velocity-encoded cine magnetic resonance imaging adds value in diagnosing patients with suspected partial anomalous pulmonary venous drainage. METHODS: In six patients with echocardiographically suspected partial anomalous pulmonary venous drainage, anatomy was evaluated using standard magnetic resonance imaging including angiography. Functional analysis included shunt calculations from flow measurements. We used four-dimensional velocity-encoded cine magnetic resonance imaging for visualisation of maldraining pulmonary veins and quantification of flow via the maldraining veins and interatrial communications, if present. RESULTS: In all patients, the diagnosis of partial anomalous pulmonary venous drainage was confirmed by standard magnetic resonance imaging. Shunt volumes ranged from 1.4:1 to 4.7:1. Drainage sites were the superior caval vein (n = 5) or the vertical vein (n = 1). Multiple maldraining pulmonary veins were found in three patients. Pulmonary arteries and veins could be clearly distinguished by selective visualisation using four-dimensional velocity-encoded cine magnetic resonance imaging. Flow measured individually in maldraining pulmonary veins in six patients and across the interatrial communication in three patients revealed a percentage of the overall shunt volume of 30-100% and 58-70%, respectively. CONCLUSION: Selective visualisation of individual vessels and their flow characteristics by four-dimensional velocity-encoded cine magnetic resonance imaging facilitates in distinguishing adjacent pulmonary arteries and veins and thus improves the accurate diagnosis of maldraining pulmonary veins. By detailed quantification of shunt volumes, additional information for planning of treatment strategies is provided. This method adds clinical value and might replace contrast-enhanced magnetic resonance angiography in these patients in the future.

Flow-sensitive four-dimensional cine magnetic resonance imaging for offline blood flow quantification in multiple vessels: a validation study.

PURPOSE: To further validate the quantitative use of flow-sensitive four-dimensional velocity encoded cine magnetic resonance imaging (4D VEC MRI) for simultaneously acquired venous and arterial blood flow in healthy volunteers and for abnormal flow in patients with congenital heart disease. MATERIALS AND METHODS: Stroke volumes (SV) obtained in arterial and venous thoracic vessels were compared between standard two-dimensional (2D), 4D VEC MRI with and without respiratory navigator gating (gated/nongated) in volunteers (n = 7). In addition, SV and regurgitation fractions (RF) measured in aorta or pulmonary trunk of patients with malformed and/or insufficient valves (n = 10) were compared between 2D and nongated 4D VEC MRI methods. RESULTS: In volunteers and patients, Bland-Altman tests showed excellent agreement between 2D, gated, and nongated 4D VEC MRI obtained quantitative blood flow measurements. The bias between 2D and gated 4D VEC MRI was <0.5 mL for SV; between 2D and nongated 4D VEC MRI the bias was <0.7 mL for SV and <1% for RF. CONCLUSION: Blood flow can be quantified accurately in arterial, venous, and pathological flow conditions using 4D VEC MRI. Nongated 4D VEC MRI has the potential to be suited for clinical use in patients with congenital heart disease who require flow acquisitions in multiple vessels.

Integrated analysis of atrioventricular interactions in tetralogy of Fallot.

The atria play an important role in cardiac performance. We evaluated their function and the atrioventricular interaction in operated patients with tetralogy of Fallot (TOF). Twenty patients who had undergone surgical repair of TOF and seven controls were investigated. Patients had residual pulmonary but no major tricuspid valve insufficiency. Atrial and ventricular strain rates were obtained by echocardiographic speckle tracking. Cine MRI-derived volumetric analysis provided atrial and ventricular time volume and time volume change curves yielding emptying and filling parameters. In addition, at the atrial level, reservoir, conduit and pump function, and cyclic volume change were calculated. At the atrioventricular valve level, tricuspid and mitral annular plane systolic excursion (TAPSE and MAPSE, respectively) were measured by two-dimensional echocardiography. In the patients compared with controls, right ventricular end-diastolic volumes were increased and biventricular ejection fraction was decreased (all P < 0.05). Biventricular measures of early diastolic ventricular filling were at control levels, but in late diastole, right ventricular filling parameters and strain rates were decreased (P < 0.001). The maximal right atrial size was slightly but not significantly diminished, but cyclic volume change was significantly reduced (P < 0.0001). Pump and reservoir function were decreased (P < 0.05), and conduit function was elevated (P < 0.001). The left atrium showed reduced reservoir function and cyclic volume change (P < 0.05). TAPSE and MAPSE were also decreased (P < 0.05). There were statistically significant interdependencies between RV ejection fraction, TAPSE, and right atrial filling and emptying parameters (all P < 0.05). In TOF patients, moderate systolic and diastolic right ventricular dysfunction is associated with clearly impaired right atrial function. The left atrium is affected to a lesser extent.

The practical clinical value of three-dimensional models of complex congenitally malformed hearts.

OBJECTIVE: Detailed 3-dimensional anatomic information is essential when planning strategies of surgical treatment for patients with complex congenitally malformed hearts. Current imaging techniques, however, do not always provide all the necessary anatomic information in a user-friendly fashion. We sought to assess the practical clinical value of realistic 3-dimensional models of complex congenitally malformed hearts. METHODS: In 11 patients, aged from 0.8 to 27 years, all with complex congenitally malformed hearts, an unequivocal decision regarding the optimum surgical strategy had not been reached when using standard diagnostic tools. Therefore, we constructed 3-dimensional virtual computer and printed cast models of the heart on the basis of high-resolution whole-heart or cine magnetic resonance imaging or computed tomography. Anatomic descriptions were compared with intraoperative findings when surgery was performed. RESULTS: Independently of age-related factors, images acquired in all patients using magnetic resonance imaging and computed tomography proved to be of sufficient quality for producing the models without major differences in the postprocessing and revealing the anatomy in an unequivocal 3-dimensional context. Examination of the models provided invaluable additional information that supported the surgical decision-making. The anatomy as shown in the models was confirmed during surgery. Biventricular corrective surgery was achieved in 5 patients, palliative surgery was achieved in 3 patients, and lack of suitable surgical options was confirmed in the remaining 3 patients. CONCLUSION: Realistic 3-dimensional modeling of the heart provides a new means for the assessment of complex intracardiac anatomy. We expect this method to change current diagnostic approaches and facilitate preoperative planning.

Partial anomalous pulmonary venous drainage in young pediatric patients: the role of magnetic resonance imaging.

Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in infants and young children. Data on 26 children under 10 years old that underwent MRI over the past 2 years for suspected PAPVD were assessed. The MRI protocol included shunt quantification by velocity-encoded cine as well as morphological and functional assessment by multislice multiphase and contrast-enhanced MR techniques. MRI was performed in the compliant patient in breath-hold (n = 8; age range, 4.6-9.5 years) and in the noncompliant patient in conscious-sedation free breathing (n = 18; age range, 0.4 to 7.5 years). In 22 patients, PAPVD was diagnosed with MRI and confirmed during surgery. In four patients with large atrial septal defects not accessible to percutaneous closure, normal pulmonary venous return was demonstrated by MRI and confirmed during surgery. MRI under conscious sedation accurately specifies the anatomy of pulmonary veins in infants and small children. Therefore, we suggest performing MRI in patients with inconclusive transthoracic echocardiographic results in the preoperative assessment of PAPVD.