Feasibility of gray-blood late gadolinium enhancement evaluation in young patients with congenital and acquired heart disease.

Background: Late gadolinium enhancement (LGE) sequences have become common in pediatric cardiovascular magnetic resonance (CMR) to assess for myocardial fibrosis. Bright-blood late gadolinium enhancement (BB-LGE) by conventional phase-sensitive inversion recovery (PSIR) is commonly utilized, but similar inversion time (TI) value of fibrosis and left ventricular (LV) blood pool can make subendocardial areas difficult to assess. A gray-blood LGE (GB-LGE) technique has been described, targeting nulling of the LV blood pool and demonstrating improvement in ischemic scar detection over BB-LGE in adult patients. We sought to evaluate the feasibility of the GB-LGE technique in a young population with congenital and acquired heart disease and compare its ability to detect subendocardial scar to conventional BB-LGE. Methods: Seventy-six consecutive patients referred for clinical CMR underwent both BB-LGE and GB-LGE on 1.5 T and 3 T scanners. Conventional PSIR sequences were obtained with TI to null the myocardium (BB-LGE) in short-axis and horizontal long-axis stacks. Same PSIR stacks were immediately repeated with TI to null the blood pool (GB-LGE). Both sequences were reviewed separately a week apart by two readers, blinded to the initial clinical interpretation. Studies were analyzed for overall image quality, confidence in scar detection, confidence in detection of LGE, LGE class, inter- and intra-observer agreement for the presence of scar, and intraclass correlation coefficient (ICC) for total scar burden. Results: Overall confidence in myocardial scar detection by BB-LGE or GB-LGE as well as grading of image quality were not statistically different [(p = 1 and p = 1) and (p = 0.53, p = 0.18), respectively]. There was very good inter-observer agreement for the presence of scar on BB-LGE (K = 0.88, 95% CI 0.77-0.99) and GB-LGE (K = 0.84, 95% CI 0.7-0.96), as well as excellent intra-observer agreement for both readers (K = 0.93, 95% CI 0.87-0.99; and K = 0.81, 95% CI 0.69-0.95). Interclass correlation coefficient for total scar burden was excellent for BB-LGE (ICC = 0.98, 95% CI 0.96-0.99) and GB-LGE (ICC = 0.94, 95% CI 0.91-0.97). Conclusions: The GB-LGE technique is feasible in the pediatric population with congenital and acquired heart disease. It can detect subendocardial/ischemic scar similar to conventional bright-blood PSIR sequences in the pediatric population.

Reference Values for Fetal Cardiac Dimensions, Volumes, Ventricular Function and Left Ventricular Longitudinal Strain Using Doppler Ultrasound Gated Cardiac Magnetic Resonance Imaging in Healthy Third Trimester Fetuses.

BACKGROUND: Recent advances in hardware and software permit the use of cardiac MRI of late gestation fetuses, however there is a paucity of MRI-based reference values. PURPOSE: To provide initial data on fetal cardiac MRI-derived cardiac dimensions, volumes, ventricular function, and left ventricular longitudinal strain in healthy developing fetuses >30 weeks gestational age. STUDY TYPE: Prospective. POPULATION: Twenty-five third trimester (34 ± 1 weeks, range of 32-37 weeks gestation) women with healthy developing fetuses. FIELD STRENGTH/SEQUENCE: Studies were performed at 1.5 T and 3 T. Cardiac synchronization was achieved with a Doppler ultrasound device. The protocol included T2 single shot turbo spin echo stacks for fetal weight and ultrasound probe positioning, and multiplanar multi-slice cine balanced steady state free precession gradient echo sequences. ASSESSMENT: Primary analyses were performed by a single observer. Weight indexed right ventricular (RV) and left ventricular (LV) volumes and function were calculated from short axis (SAX) stacks. Cardiac dimensions were calculated from the four-chamber and SAX stacks. Single plane LV longitudinal strain was calculated from the four-chamber stack. Interobserver variability was assessed in 10 participants. Cardiac MRI values were compared against available published normative fetal echocardiogram data using z-scores. STATISTICAL TESTS: Mean and SDs were calculated for baseline maternal/fetal demographics, cardiac dimensions, volumes, ventricular function, and left ventricular longitudinal strain. Bland-Altman and intraclass correlation coefficient analysis was performed to test interobserver variability. RESULTS: The mean gestational age was 34 ± 1.4 weeks. The mean RV and LV end diastolic volumes were 3.1 ± 0.6 mL/kg and 2.4 ± 0.5 mL/kg respectively. The mean RV cardiac output was 198 ± 49 mL/min/kg while the mean LV cardiac output was 173 ± 43 mL/min/kg. DATA CONCLUSION: This paper reports initial reference values obtained by cardiac MRI in healthy developing third trimester fetuses. MRI generally resulted in slightly larger indexed values (by z-score) compared to reports in literature using fetal echocardiography. EVIDENCE LEVEL: 1 TECHNICAL EFFICACY: Stage 2.

Myocardial strain-curve deformation patterns after Fontan operation.

Myocardial deformation analysis by cardiac MRI (CMR) yielding global circumferential and longitudinal strain (GCS and GLS) is an increasingly utilized method to accurately quantify systolic function and predict clinical events in patients with Fontan circulation. The purpose of this study was to use principal component analysis (PCA) to investigate myocardial temporal deformation patterns derived from strain-time curves to learn about latent strain features beyond peak values. We conducted the study with specific attention to dominant single left or right ventricle (SLV and SRV) morphologies. Methods and Results: Patients remote from Fontan operation who underwent follow-up CMR were analyzed for standard volumetric and function hemodynamics including myocardial deformation parameters including GCS and GLS. We applied PCA to investigate in an unbiased fashion the strain-time curve morphology and to calculate patient specific shape scores. All variables were subjected to single variable Cox regression analysis to detect composite clinical outcome including death, heart transplant, protein losing enteropathy and plastic bronchitis. A total of 122 patients, (SLV = 67, SRV = 55) with a mean age of 12.7 years underwent comprehensive CMR analysis. The PCA revealed 3 primary modes of strain-curve variation regardless of single ventricle morphology and type of strain investigated. Principle components (PCs) described changes in (1) strain-time curve amplitude, (2) time-to-peak strain, and (3) post-systolic slope of the strain-time curve. Considering only SLV patients, GCS was only CMR variable predictive of clinical events (HR 1.46, p = 0.020). In the SRV group, significant CMR predictors of clinical events were derived indexed end-diastolic (HR 1.02, p = 0.023) and end-systolic (HR 1.03, p = 0.022) volumes, GCS (HR 1.91, p = 0.003) and its related first component score (HR 1.20, p = 0.005), GLS (HR 1.32, p = 0.029) and its third component score (HR 1.58, p = 0.017). CMR derived global strain measures are sensitive markers of clinical outcomes in patients with Fontan circulation, particularly in patients with the SRV morphology. Myocardial strain-time curve morphology specific to SLV and SRV patients inspired by unbiased PCA technique can further aid with predicting clinical outcomes.

Labetalol Infusion Attenuates Paradoxical Hypertension and Decreases Plasma Renin Activity After Repair of Coarctation of the Aorta in Children.

OBJECTIVE: Paradoxical hypertension after repair of coarctation of the aorta in children is associated with the release of catecholamines and activation of the renin-angiotensin system. The objective of the present study was to describe the effects of labetalol infusion on blood pressure, plasma catecholamine levels, and plasma renin activity in a series of children undergoing repair of coarctation of the aorta. DESIGN: Prospective, observational cohort study. SETTING: Tertiary children's hospital with university affiliation. PARTICIPANTS: The study was comprised of 15 consecutive children older than 1 year undergoing repair of coarctation of the aorta. INTERVENTIONS: Intravenous infusion of labetalol, up to 20 µg/kg/min, was administered when patients became hypertensive after release of the aortic cross-clamp. Supplementation with nitroprusside was allowed as needed. MEASUREMENTS AND MAIN RESULTS: Blood pressure was maintained below baseline values throughout the labetalol infusion. Plasma norepinephrine increased from 160 ± 81 pg/mL (preoperative) to 657 ± 268 pg/mL (6 h after release of aortic cross-clamp). Plasma renin activity decreased from 16.6 ± 9.7 ng/kg/h (at cross-clamp release) to 2.2 ± 2.2 ng/kg/h (6 h after cross-clamp release). Nitroprusside was added for 12 patients, at a highest mean dose of 2.4 ± 2.5 µg/kg/min. CONCLUSIONS: Labetalol inhibited activation of the renin-angiotensin system and helped control paradoxical hypertension after coarctation repair in children.

Proximal pulmonary vascular stiffness as a prognostic factor in children with pulmonary arterial hypertension.

Aims: Main pulmonary artery (MPA) stiffness and abnormal flow haemodynamics in pulmonary arterial hypertension (PAH) are strongly associated with elevated right ventricular (RV) afterload and associated with disease severity and poor clinical outcomes in adults with PAH. However, the long-term effects of MPA stiffness on RV function in children with PAH remain poorly understood. This study is the first comprehensive evaluation of MPA stiffness in children with PAH, delineating the mechanistic relationship between flow haemodynamics and MPA stiffness as well as the prognostic ability of these measures regarding clinical outcomes. Methods and results: Fifty-six children diagnosed with PAH underwent baseline cardiac magnetic resonance (CMR) acquisition and were compared with 23 control subjects. MPA stiffness and wall shear stress (WSS) were evaluated using phase contrast CMR and were evaluated for prognostic potential along with standard RV volumetric and functional indices. Pulse wave velocity (PWV) was significantly increased (2.8 m/s vs. 1.4 m/s, P < 0.0001) and relative area change (RAC) was decreased (25% vs. 37%, P < 0.0001) in the PAH group, correlating with metrics of RV performance. Decreased WSS was associated with a decrease in RAC over time (r = 0.679, P < 0.001). For each unit increase in PWV, there was approximately a 3.2-fold increase in having a moderate clinical event. Conclusion: MPA stiffness assessed by non-invasive CMR was increased in children with PAH and correlated with RV performance, suggesting that MPA stiffness is a major contribution to RV dysfunction. PWV is predictive of moderate clinical outcomes, and may be a useful prognostic marker of disease activity in children with PAH.

Myocardial Perfusion Reserve Index in Children With Kawasaki Disease.

BACKGROUND: Coronary artery lesions in patients with Kawasaki disease (KD) can impair myocardial perfusion, yet evaluation of perfusion defects by cardiac magnetic resonance (MR) in children is often qualitative. PURPOSE: In this study we aimed to use a quantitative method of myocardial perfusion using stress cardiac MR-derived myocardial perfusion reserve index (MPRI) in children with KD and compare MPRI with ventricular mechanical performance evaluated by cardiac MR strain analysis. STUDY TYPE: This study was a retrospective review. SUBJECTS: Twenty-one children with a diagnosis of KD who underwent stress perfusion cardiac MR were compared with nine controls. FIELD STRENGTH/SEQUENCE: First-pass perfusion imaging using a T1 -weighted gradient echo sequence was performed at rest and stress after administration of adenosine with 1.5T or 3T magnets. ASSESSMENT: The MPRI was calculated as the ratio of maximum slope of myocardial enhancement during stress compared to rest and was evaluated with the American Heart Association 17 segment model. STATISTICAL TESTS: Demographic and clinical characteristics among KD patients and controls were compared using Student's t-test for normally distributed continuous variables, Wilcoxon-rank sum test for nonnormally distributed variables, and χ2 for categorical variables. RESULTS: There was a significant decrease in MPRI in Segment 7 (1.53 vs. 2.23, P = 0.0058) in KD patients compared with controls. The reduction in MPRI in Segment 12 approached statistical significance (1.58 vs. 2.31, P = 0.0636). Three patients who underwent serial studies had decreased MPRI longitudinally. No differences were seen in circumferential or radial strain. DATA CONCLUSION: MPRI shows impaired myocardial perfusion in patients with KD. MPRI can change over time, suggestive of progressive coronary artery changes, which may precede fibrosis and mechanical decline. MPRI can assess segmental and global perfusion defects in patients with KD and should be a part of routine cardiac MR evaluation in KD. LEVEL OF EVIDENCE: 3 Technical Efficacy Stage 3 J. Magn. Reson. Imaging 2017.

Right Ventricular Tissue Doppler Myocardial Performance Index in Children with Pulmonary Hypertension: Relation to Invasive Hemodynamics.

Right ventricular (RV) failure is a significant cause of morbidity and mortality in patients with pulmonary hypertension (PH). Myocardial performance index measured by tissue Doppler imaging (TDI-MPI) has been useful in assessing RV dysfunction in adults with PH. However, TDI-MPI as a marker for RV dysfunction or disease severity has not been evaluated in pediatric PH. The aim of this study was to investigate TDI-MPI and correlate with invasive hemodynamics in pediatric PH patients. Eighty pediatric PH patients undergoing cardiac catheterization and simultaneous transthoracic echocardiography were analyzed. RV TDI-MPI was averaged over three cardiac cycles and measured under each condition of vasodilatory testing during the catheterization. TDI-MPI was compared between PH patients and age-matched controls and correlated to invasive hemodynamics. RV TDI-MPI was increased in PH patients compared to controls (0.49 vs. 0.35, p < 0.0001). Significant associations (beta ± SE) are seen between RV TDI-MPI and baseline mean pulmonary arterial pressures (0.0002 ± 0.001, p < 0.05), indexed pulmonary vascular resistance (0.007 ± 0.002, p < 0.002), and pulmonary-to-systemic arterial pressure ratio (0.146 ± 0.063, p < 0.05). No statistically significant associations were seen with vasodilatory testing. RV TDI-MPI is elevated in children with PH, suggestive of RV dysfunction. RV TDI-MPI shows correlation with severity of PH at baseline but lacks sensitivity to evaluate the RV response to acute changes in afterload in children with PH. Therefore, while RV TDI-MPI can help identify RV dysfunction in children with PH, its utility as a non-invasive surrogate marker for acute changes in hemodynamics is limited.

Effectiveness of an anesthetic continuous-infusion device in children with cerebral palsy undergoing orthopaedic surgery.

BACKGROUND: Effective postoperative pain management is a vital component of orthopaedic surgical care in the pediatric population. In children with cognitive impairments pain management can be difficult, making these children vulnerable to ineffectively managed postoperative pain. This prospective, randomised study evaluated the use of a local anesthetic continuous infusion device (pain pump) to manage postoperative pain in children with cerebral palsy (CP) undergoing lower extremity orthopaedic surgical procedures. METHODS: Children with a diagnosis of CP who were undergoing select orthopaedic outpatient procedures were enrolled in this study. Postoperatively, patients were randomised to receive either a pain pump in addition to oral analgesics or oral analgesics only. Patient's parents were asked to record the amount of medication administered and assess pain intensity with the use of a modified visual analogue scale for 3 days postoperatively. Parents also completed an overall pain management satisfaction questionnaire at the end of the study participation period. RESULTS: Fifty-four patients were enrolled in this study and data were analyzed on 37 patients. The mean daily pain intensity in the pain pump group was significantly lower for the day of surgery and for 2 days postoperatively, and there was an overall significant difference between the groups (P<0.0001). The amount of analgesic medication administered was significantly lower for the first 2 postoperative days, but there was not a significant difference between the 2 groups overall (P=0.29). Parent satisfaction with both pain management techniques was high and responses were similar between the 2 groups. CONCLUSIONS: Children with CP present unique postoperative pain management concerns that can be effectively addressed through the use of multimodal analgesic techniques. This study found that the pain pump is an effective pain management technique that significantly reduces pain intensity in children with CP after lower extremity orthopaedic procedures. LEVEL OF EVIDENCE: Therapeutic Level II.

Femoral head resection as a salvage procedure for the severely dysplastic hip in nonambulatory children with cerebral palsy.

BACKGROUND: Proximal femoral head resection (FHR) has been used as a treatment option in patients with cerebral palsy (CP) who suffer from pain, contractures, and impaired personal hygiene. We analyzed the overall functional outcome following this surgery and associated patient satisfaction. METHODS: This study was a retrospective case series with a follow-on questionnaire. A consecutive sample of 27 patients with CP who underwent proximal FHR between 1988 and 2004 were analyzed. Medical and radiographic records were used alongside a patient satisfaction questionnaire. Average follow-up time was 7.8 years (range, 2.3-16.5 years). RESULTS: We found an improvement in hip pain, range of motion, activities of daily living, and quality of life after surgery. Age at surgery, type of immobilization, and presence of heterotopic ossification or femoral migration did not affect the long-term surgical outcome. CONCLUSION: This study confirms the efficacy of proximal FHR for the treatment of chronic severe neuromuscular hip dysplasia in children with CP. LEVEL OF EVIDENCE: Level IV.

Rectus femoris transfer in children with cerebral palsy: evaluation of transfer site and preoperative indicators.

BACKGROUND: Rectus femoris transfer (RFT) is a treatment option for children with cerebral palsy (CP) who exhibit a stiff-knee gait pattern. The rectus femoris muscle is transferred to different sites to overcome problems such as tripping and falling. Indications for RFT often include preoperative decreased knee range of motion (KROM) during swing phase, reduced peak knee flexion in swing (PKFSW), abnormal rectus femoris firing patterns on electromyography recordings, and a positive Duncan-Ely test. This study investigated the effect of different RFT sites on kinematic outcome variables and evaluated the relationship between commonly used preoperative surgical indicators and postoperative changes in kinematics. METHODS: Thirty-eight patients with CP, who underwent RFT, with preoperative and postoperative instrumented gait analyses, were evaluated and divided by transfer site: semitendinosus, sartorius, and gracilis. Preoperative and postoperative comparisons were made for 5 knee kinematic variables: range of motion (KROM), peak flexion at loading response, peak extension at terminal stance, PKFSW, and peak extension at terminal swing. Analysis was performed in accordance to different transfer sites and overall postoperative change. RESULTS: When all limbs were analyzed together for the 5 outcome variables, it was found that patients experienced significant (P < 0.05) improvements in sagittal-plane kinematics in 3 of the outcome variables: KROM, PKFSW, and peak extension at terminal swing. Patients had a significant improvement in postoperative KROM when the preoperative KROM was less than 80% of normal. Electromyography pattern and Duncan-Ely test were not found to be useful indicators of surgical success. CONCLUSION: The authors recommend RFT in children with CP who exhibit a stiff-knee gait, regardless of transfer site. LEVEL OF EVIDENCE: Level 3.

A trial of fresh autologous whole blood to treat dilutional coagulopathy following cardiopulmonary bypass in infants.

BACKGROUND: Transfusion of fresh whole blood is superior to blood component therapy in correcting coagulopathies in children following cardiopulmonary bypass (CPB); however, a supply of fresh homologous whole blood is difficult to maintain. We hypothesized that transfusion of fresh autologous whole blood obtained prior to heparinization for CPB and infused following CPB would be associated with improved coagulation function when compared with standard therapy. METHODS: A total of 32 infants 5-12 kg undergoing noncomplex open cardiac surgery were randomly assigned to either the treatment or control group. In the treatment group, 15 ml x kg(-1) of autologous whole blood was collected into a CPDA bag prior to heparinization while 15 ml x kg(-1) of 5% albumin was infused intravenously. After reversal of heparin, coagulation tests were drawn in both groups, and the autologous whole blood was infused over 20 min in the treatment group. RESULTS: The treatment group had greater (P < 0.05) improvement in platelet count, prothrombin time, and fibrinogen than the control group. CONCLUSIONS: We conclude that collection of fresh autologous whole blood prior to heparinization and reinfusion following CPB is associated with greater improvement of coagulation status after CPB in infants.

Duration of preoperative fast correlates with arterial blood pressure response to halothane in infants.

UNLABELLED: In this study, we sought to determine whether the duration of preoperative fasting affects the decrease in blood pressure observed in infants and children during halothane anesthesia. Two-hundred-fifty pediatric patients were divided into 5 age groups: term neonates (n = 50), 1-6 mo (n = 50), 6-24 mo (n = 50), 2-6 yr (n = 50), and 6-12 yr (n = 50). After anesthetic induction with halothane, end-tidal halothane was maintained at 2 minimum alveolar anesthetic concentration (MAC) for 10 min to allow myocardial uptake. Patients were grouped by duration of preoperative fast (0-4 h, 4-8 h, 8-12 h, and >12 h). Changes in heart rate and systolic (SAP) and mean (MAP) arterial blood pressure from preinduction to 2 MAC were compared among fasting groups within each age group. In the 1- to 6-mo age group, the changes in SAP and MAP were significantly greater in infants fasting 8-12 h than in those fasting 0-4 h (SAP, -51 mm Hg versus -31 mm Hg, respectively; MAP, -48 mm Hg versus -32 mm Hg; P < 0.05). No statistically significant differences were noted in the older age groups. The results of this study demonstrate that prolonged preoperative fasting is associated with a greater decrease in blood pressure in infants. This exacerbation of the already significant hemodynamic depression observed in infants during halothane anesthesia underscores the importance of adherence to published fasting guidelines. IMPLICATIONS: We studied changes in blood pressure during halothane anesthesia in infants and children and found that blood pressure decreased to a greater extent in infants who fasted for longer than 8 h before surgery. This exacerbation of the already significant hemodynamic depression observed in infants during halothane anesthesia underscores the importance of adherence to published fasting guidelines.