Magnetic resonance three-dimensional steady-state free precession imaging of the thoracic duct in patients with Fontan circulation and its relationship to outcomes.

BACKGROUND: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes. METHODS: This was a retrospective, single-center study of patients with Fontan circulation who underwent CMR. Frequency matching of age at CMR was used to construct a comparison group of patients with repaired tetralogy of Fallot (rTOF). TD characteristics included maximum diameter and a qualitative assessment of tortuosity. Clinical outcomes included protein-losing enteropathy (PLE), plastic bronchitis, listing for heart transplantation, and death. A composite outcome was defined as presence of any of these events. RESULTS: The study included 189 Fontan patients (median age 16.1 years, IQR 11.0-23.2 years) and 36 rTOF patients (median age 15.7 years, IQR 11.1-23.7 years). The TD diameter was larger (median 2.50 vs. 1.95 mm, p = 0.002) and more often well visualized (65% vs. 22%, p < 0.001) in Fontan patients vs. rTOF patients. TD dimension increased mildly with age in Fontan patients, R = 0.19, p = 0.01. In Fontan patients, the TD diameter was larger in those with PLE vs. without PLE (age-adjusted mean 4.11 vs. 2.72, p = 0.005), and was more tortuous in those with NYHA class ≥ II vs. class I (moderate or greater tortuosity 75% vs. 28.5%, p = 0.02). Larger TD diameter was associated with a lower ventricular ejection fraction that was independent of age (partial correlation = - 0.22, p = 0.02). More tortuous TDs had a higher end-systolic volume (mean 70.0 mL/m2 vs. 57.3 mL/m2, p = 0.03), lower creatinine (mean 0.61 mg/dL vs. 0.70 mg/dL, p = 0.04), and a higher absolute lymphocyte count (mean 1.80 K cells/µL vs. 0.76 K cells/µL, p = 0.003). The composite outcome was present in 6% of Fontan patients and was not associated with TD diameter (p = 0.50) or tortuosity (p = 0.09). CONCLUSIONS: The TD is well visualized in two-thirds of patients with Fontan circulation on 3D-bSSFP images. Larger TD diameter is associated with PLE and increased TD tortuosity is associated with an NYHA class ≥ II.

Three-dimensional heart locator and compressed sensing for whole-heart MR angiography.

PURPOSE: We sought to develop a whole-heart magnetic resonance angiography technique with three-dimensional (3D) respiratory motion compensation and reduced scan time. METHODS: A novel respiratory motion compensation method was implemented that acquires a 1D navigator (NAV) and a low-resolution 3D-image of the heart (3D-LOC) just before the angiography data. The central 10% of SSFP k-space was fully acquired using NAV-gating, and then 10% of peripheral k-space was randomly undersampled to complete the scan. Spatial registration of the 3D-LOC information was used to correct the central and peripheral k-space lines for the bulk respiratory motion in three dimensions, and then the remaining k-space data was estimated using compressed sensing (CS). Ten volunteers each underwent two angiography acquisitions with 1 mm(3) resolution: (i) conventional NAV with CS, and (ii) the new 3D-LOC with CS. RESULTS: Compared with conventional NAV, the new 3D-LOC with CS technique had a shorter scan time (4.8 ± 1.1 versus 6.3 ± 1.7 min; P < 0.001), better objective vessel sharpness for all three coronary arteries (P < 0.05), and no difference in subjective vessel sharpness for all three coronary arteries. CONCLUSION: Compared with conventional NAV with CS, acceleration and respiratory motion correction using 3D-LOC with CS reduces scan time and improves objective vessel sharpness.

Free-breathing steady-state free precession cine cardiac magnetic resonance with respiratory navigator gating.

PURPOSE: To develop and validate a respiratory motion compensation method for free-breathing cardiac cine imaging. METHODS: A free-breathing navigator-gated cine steady-state free precession acquisition (Cine-Nav) was developed which preserves the equilibrium state of the net magnetization vector, maintains the high spatial and temporal resolutions of standard breath-hold (BH) acquisition, and images entire cardiac cycle. Cine image data is accepted only from cardiac cycles occurring entirely during end-expiration. Prospective validation was performed in 10 patients by obtaining in each three complete ventricular image stacks with different respiratory motion compensation approaches: (1) BH, (2) free-breathing with 3 signal averages (3AVG), and (3) free-breathing with Cine-Nav. RESULTS: The subjective image quality score (1 = worst, 4 = best) for Cine-Nav (3.8 ± 0.4) was significantly better than for 3AVG (2.2 ± 0.5, P = 0.002), and similar to BH (4.0 ± 0.0, P = 0.13). The blood-to-myocardium contrast ratio for Cine-Nav (6.3 ± 1.5) was similar to BH (5.9 ± 1.6, P = 0.52) and to 3AVG (5.6 ± 2.5, P = 0.43). There were no significant differences between Cine-Nav and BH for the ventricular volumes and mass. In contrast, there were significant differences between 3AVG and BH in all of these measurements but right ventricular mass. CONCLUSION: Free-breathing cine imaging with Cine-Nav yielded comparable image quality and ventricular measurements to BH, and was superior to 3AVG.

Resting heart rate influences right ventricular volume in repaired tetralogy of Fallot.

The aim of this study is to examine the impact of heart rate (HR) on right ventricular end-diastolic volume indexed to body surface area (RVEDVi) in patients with repaired tetralogy of Fallot (TOF). In this cross-sectional study, an institutional database search identified all patients with repaired TOF who underwent cardiac magnetic resonance (CMR) and had a Holter study within 3 months. The association of HR on Holter, HR at the time of CMR, and other clinical and CMR parameters on RVEDVi was explored with univariate and then multivariable models. In the study group (n = 161, median age 23 years), a lower mean Holter HR was associated with a larger RVEDVi (p = 0.004). In a model that also included pulmonary regurgitation fraction, tricuspid regurgitation grade, RV ejection fraction, age at CMR, and gender, mean Holter HR remained associated with RVEDVi (p < 0.0001); for a decrease of 1 bpm, mean RVEDVi increased by 1.09 ml/m(2). When limiting to those with a Holter within 5 days of CMR (n = 70), the impact of mean Holter HR on RVEDVi was stronger (-1.9 ml/m(2)/bpm). HR at time of CMR had a significant but less pronounced relationship to RVEDVi (-0.58 ml/m(2)/bpm, p = 0.002). In conclusion, in repaired TOF patients, a lower HR was significantly associated with a larger RVEDVi. This relationship was stronger with a shorter time interval between the Holter and CMR, and stronger for the mean HR on Holter than for the HR at CMR. Accounting for HR in the interpretation of RVEDVi may impact decisions regarding pulmonary valve replacement and the interpretation of serial CMR data.

Three-dimensional heart locator for whole-heart coronary magnetic resonance angiography.

PURPOSE: Coronary magnetic resonance angiography (MRA) is commonly performed with diaphragmatic navigator (NAV) gating to compensate for respiratory motion, but this approach is inefficient as data must be reacquired when it is outside the acceptance window. We therefore developed and validated a motion compensation technique based on three-dimensional (3D) spatial registration in which data are accepted throughout the respiratory cycle. METHODS: A novel respiratory motion compensation method was implemented that acquires a low-resolution 3D-image of the heart (3D-LOC) just prior to coronary MRA data acquisition. 3D-LOC volumes were registered to the first 3D-LOC to estimate the respiratory-induced heart motion and to modify the coronary MRA data and reconstruct motion-corrected images. Whole-heart coronary MRA datasets were acquired from nine healthy subjects using a diaphragmatic NAV and using 3D-LOC. RESULTS: There was no significant difference between the subjective image score of NAV and 3D-LOC in three main coronary branches. The vessel sharpness of 3D-LOC was higher than NAV in the right (0.44 ± 0.08 vs. 0.49 ± 0.08; P = 0.055) and left circumflex arteries (0.49 ± 0.05 vs. 0.52 ± 0.04; P = 0.039). Scan time for 3D-LOC was significantly shorter than NAV (4.3 ± 0.6 vs. 8.3 ± 2.3 min; P = 0.004). CONCLUSION: Compared to NAV gating, 3D-LOC for coronary MRA reduces scan time by nearly 50% without compromising image quality.

Pulmonary artery conduit in vivo dimensional requirements in a growing ovine model: comparisons with the ascending aorta.

BACKGROUND AND AIM OF THE STUDY: The pulmonary trunk (PT) structure and function are abnormal in multiple congenital cardiovascular diseases. Existing surgical treatments of congenital malformations of the right ventricular outflow tract and PT do not provide a long-term replacement that can adapt to normal growth. Although there is strong interest in developing tissue-engineered approaches for PT conduit replacement, there remains an absence of any complete investigation of the native geometric growth patterns of the PT to serve as a necessary benchmark. METHODS: Eleven Dorset sheep (aged 4-12 months) underwent a single cardiac magnetic resonance imaging study, from which luminal arterial surface points were obtained using a novel semi-automated segmentation technique. The three-dimensional shapes of the PT and ascending aorta (AA) were measured over the same time period to gain insight into differences in the geometric changes between these two great vessels. RESULTS: The volumetric growth of the PT appeared to be a linear function of age, whereas its surface geometry demonstrated non-uniform growth patterns. While tortuosity was maintained with age, the cross-sectional shape of the main pulmonary artery (MPA) evolved from circular in young animals to elliptical at 12 months. In addition, the distal MPA near the pulmonary artery bifurcation tapered with age. CONCLUSION: It can be concluded that postnatal growth of the PT is not a simple proportionate (i.e. isotropic) size increase, but rather exhibits complex three-dimensional geometric features during somatic growth.

Brain volume and metabolism in fetuses with congenital heart disease: evaluation with quantitative magnetic resonance imaging and spectroscopy.

BACKGROUND: Adverse neurodevelopmental outcome is an important source of morbidity in children with congenital heart disease (CHD). A significant proportion of newborns with complex CHD have abnormalities of brain size, structure, or function, which suggests that antenatal factors may contribute to childhood neurodevelopmental morbidity. METHODS AND RESULTS: Brain volume and metabolism were compared prospectively between 55 fetuses with CHD and 50 normal fetuses with the use of 3-dimensinal volumetric magnetic resonance imaging and proton magnetic resonance spectroscopy. Fetal intracranial cavity volume, cerebrospinal fluid volume, and total brain volume were measured by manual segmentation. Proton magnetic resonance spectroscopy was used to measure the cerebral N-acetyl aspartate: choline ratio (NAA:choline) and identify cerebral lactate. Complete fetal echocardiograms were performed. Gestational age at magnetic resonance imaging ranged from 25 1/7 to 37 1/7 weeks (median, 30 weeks). During the third trimester, there were progressive and significant declines in gestational age-adjusted total brain volume and intracranial cavity volume in CHD fetuses relative to controls. NAA:choline increased progressively over the third trimester in normal fetuses, but the rate of rise was significantly slower (P<0.001) in CHD fetuses. On multivariable analysis adjusted for gestational age and weight percentile, cardiac diagnosis and percentage of combined ventricular output through the aortic valve were independently associated with total brain volume. Independent predictors of lower NAA:choline included diagnosis, absence of antegrade aortic arch flow, and evidence of cerebral lactate (P<0.001). CONCLUSIONS: Third-trimester fetuses with some forms of CHD have smaller gestational age- and weight-adjusted total brain volumes than normal fetuses and evidence of impaired neuroaxonal development and metabolism. Hemodynamic factors may play an important role in this abnormal development.

Feasibility of dobutamine stress cardiovascular magnetic resonance imaging in children.

PURPOSE: To evaluate the feasibility of dobutamine stress magnetic resonance (DSMR) in pediatric patients. MATERIALS AND METHODS: The medical records of all DSMR studies performed on patients < or =22 years old at a single institution were retrospectively reviewed. The DSMR protocol included dobutamine doses up to 40 microg/kg/minute and atropine to attain the target heart rate [0.85 . (220 - age)]. RESULTS: Thirty-two DSMR studies were performed in 28 patients (median age = 7.3 years; range = 0.8-22 years). Twenty of the studies were performed under general anesthesia. The protocol was completed in 26 studies, technical problems and interruptions were few, and image quality scores (1-5) for all ventricular wall segments were high (mean = 4.2). A heart rate > or =160 bpm was attained in 84% of the studies, a rate pressure product > or =20,000 beats . mm Hg in 87%, and a heart rate greater than or equal to the target heart rate in 19%. No serious adverse events occurred. One patient had an inducible wall motion abnormality. Interobserver agreement was 100% (kappa = 1.0) for test positivity and 92% (kappa = 0.72) for wall motion scores. CONCLUSION: DSMR in pediatric patients is feasible and provides high-quality imaging of all ventricular wall segments with low interobserver variability. Further exploration of DSMR in pediatric patients is warranted, particularly for those children who are unable to cooperate sufficiently for exercise stress or have poor acoustic windows.

Prenatal diagnosis of thoracic ectopia cordis by real-time fetal cardiac magnetic resonance imaging and by echocardiography.

Ectopia cordis is a rare congenital defect commonly associated with intra- and extra-cardiac anomalies. This report highlights the complimentary use of echocardiography and cardiac magnetic resonance imaging for detailed prenatal characterization of the anomaly at 23-week gestation.