Evaluation of cardiac fibrosis and subclinical cardiac changes in children with sickle cell disease using magnetic resonance imaging, echocardiography, and serum galectin-3.

BACKGROUND: Myocardial fibrosis has recently been proposed as one of the contributing factors to the diverse pathogenicity of cardiomyopathy in sickle cell disease. OBJECTIVE: In this study, cardiac fibrosis and subclinical cardiac changes in children with sickle cell disease were evaluated using cardiac magnetic resonance imaging (MRI), tissue Doppler echocardiography and serum galectin-3. MATERIALS AND METHODS: The study included 34 children with sickle cell disease who were compared with a similar number of healthy controls. Cardiac MRI was used to evaluate late gadolinium enhancement, native T1 mapping, extracellular volume, and T2* for estimation of iron load. Cardiac function and myocardial performance index (MPI, evaluated by tissue Doppler echocardiography) and serum galectin-3 were compared to controls. RESULTS: The mean age of the included patients was 13.3 ± 3.2 years. Myocardial iron load by T2* was normal. The mean level of extracellular volume (35.41 ± 5.02%) was significantly associated with the frequency of vaso-occlusive crises (P = 0.017) and negatively correlated with hemoglobin levels (P = 0.005). Galectin-3 levels were significantly higher among cases than controls (P = 0.00), at a cutoff value on the receiver operating characteristic curve of 6.5 ng/ml, sensitivity of 82.5% and specificity of 72.8%. The extracellular volume was significantly higher in cases, with a MPI > 0.4. CONCLUSION: Diffuse interstitial myocardial fibrosis can be detected early in children with sickle cell disease using T1 mapping and is associated with a high frequency of vaso-occlusive crisis. MPI of the left ventricle and serum galectin-3 are recommended screening tools for subclinical cardiac abnormalities.

Multiparametric Rest and Dobutamine Stress Magnetic Resonance in Assessment of Myocardial Viability.

BACKGROUND: MR feature-tracking (FT) is a novel technique that quantitatively calculates myocardial strain and can assess myocardial viability. PURPOSE: To evaluate the feasibility of FT at rest and with low-dose dobutamine (LDD), visual assessment of contractility with LDD and left ventricle (LV) end-diastolic wall thickness (EDWT) in the assessment of viability in ischemic cardiomyopathy (ICM) patients compared to delayed gadolinium enhancement (DGE). STUDY TYPE: Prospective. SUBJECTS: Thirty ICM patients and 30 healthy volunteers. FIELD STRENGTH/SEQUENCES: A 1.5 T with balanced steady-state free precession (bSSFP) cine and phase-sensitive inversion prepared segmented gradient echo sequences. ASSESSMENT: LDD (5 µg/kg/min and 10 µg/kg/min) was administered in the patient group. LV was divided into 16 segments and MR-FT was derived from bSSFP cine images using dedicated software. Viable segments were defined as those with a dobutamine-induced increase in resting MR-FT values >20%, a dobutamine-induced increase in systolic wall thickening ≥2 mm by visual assessment, ≤50% fibrosis on DGE, and resting EDWT ≥5.5 mm. STATISTICAL TESTS: One-way analysis of variance (ANOVA), two-sampled t-test, paired samples t-test, and receiver operating characteristic (ROC) curve analysis. A P value < 0.05 was considered statistically significant. RESULTS: Resting peak global circumferential (Ecc) and radial (Err) strains were significantly impaired in patients compared to controls (-11.7 ± 7.9 vs. -20.1 ± 5.7 and 19.7 ± 13.9 vs. 32.7 ± 15.4, respectively). Segments with no DGE (n = 354) and ≤ 50% (n = 38) DGE showed significant improvement of both Ecc and Err with LDD while segments with >50% DGE (n = 88) showed no improvement. In comparison to viable and nonviable segments identified by reference-standard DGE, the sensitivity, specificity, and diagnostic accuracy of the four methods were: 74%, 92%, and 89%, respectively, for Ecc; 70%, 89%, and 86%, respectively, for Err; 67%, 88%, and 84% for visual assessment; and 39%, 90%, and 80% for EDWT. DATA CONCLUSION: Quantitative assessment of MR-FT, along with EDWT and qualitative visual assessment of myocardial contractility with LDD, are feasible alternative methods for the assessment of myocardial viability with moderate sensitivity and high specificity. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage: 2.

Pulmonary capillary hemangiomatosis: a case series and review of literature.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) of unknown etiology resulting from pulmonary capillary proliferation. Clinically, PCH is seen in young adults with equal sex predilection and rarely reported familial predisposition. PCH's main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations, and later irreversible pulmonary hypertension and right-sided heart failure. Hereby, we report three PCH cases, each case presented with a peculiar presentation with a comprehensive literature review highlighting etiology, clinical presentations, diagnostic modalities and pathology in establishing a diagnosis, current treatment options, and prognosis of PCH. In conclusion, defining PCH as the underlying cause of PH is of utmost importance as most medications used for PH are ineffective in PCH. Vasodilators should be avoided due to the increased risk of pulmonary oedema. Pathological examination of the lung is still considered the most definitive diagnostic tool, yet it is associated with complications risk. High-Resolution Computed Tomography (HRCT) chest is currently considered the cornerstone non-invasive modality for the diagnosis of PH. So far, no definitive treatment of PCH excluding lung transplantation with preliminary promising results with angiogenesis Inhibitors. PCH carries a very poor prognosis with a median survival of 3 years from the time of diagnosis.

Multi-detector computed tomography in the assessment of tetralogy of Fallot patients: is it a must?

BACKGROUND: Tetralogy of Fallot (TOF) accounts for 10% of all CHD. It classically consists of ventricular septal defect (VSD), aortic overriding, right ventricular outflow tract (RVOT) obstruction, and RV hypertrophy. There are many anatomic variants, associated intracardiac and extracardiac anomalies that must be taken into consideration when imaging and planning the surgical procedure needed. Multi-detector computed tomography (MDCT), with its high spatial and temporal resolution, has a pivotal role in the evaluation of complex anatomical findings in both unrepaired and repaired TOF patients. MAIN BODY: Though MDCT has a limited role in the initial diagnosis of TOF, it is particularly important when there is a question about anatomy of pulmonary arteries (PAs) (whether sizable, hypoplastic, or atretic), presence of major aorto-pulmonary collaterals (MAPCAs) and presence of additional VSDs. Additionally, MDCT is crucial in the diagnosis of different anatomical variants of TOF. TOF patients with absent pulmonary valve classically have hugely dilated PAs which raise an important question about the degree and severity of airways compression. This question can be accurately answered by MDCT. TOF with double-outlet RV (DORV) has variable degrees of aortic override which can be assessed by MDCT. An atrio-ventricular septal defect (AVSD) is seen in about 13% of TOF cases and typically occurs in patients with Down syndrome. MDCT can assess the size and extent of inlet VSD and size of both ventricles (balanced or unbalanced AVSD). Coronary artery anomalies are common and important association. MDCT can identify the presence of a major coronary artery crossing the RVOT, a left anterior descending (LAD) from RCA, or a dual LAD. The clinical importance of these anomalies is its susceptibility to injury during ventriculotomy incision required for TOF repair necessitating changing the usual approach of surgery. Patients with reduced pulmonary blood flow undergo a systemic to pulmonary shunt. MDCT can assess the patency of the shunt, stenotic, or occluded segments. In surgically repaired TOF patients, MDCT can identify the sequalae and long-term complications including residual RVOT obstruction, conduit stenosis, RVOT patch aneurysm, RVH, and aortic root dilatation. CONCLUSION: MDCT is a safe and reliable imaging modality that provides accurate assessment of anatomical variants and associated anomalies of TOF.

Three-Dimensional Constructive Interference in Steady State Sequences and Phase-Contrast Magnetic Resonance Imaging of Arrested Hydrocephalus.

OBJECTIVE: To evaluate the role of three-dimensional constructive interference in steady state (3D-CISS) sequences and phase-contrast magnetic resonance imaging (PC-MRI) in patients with arrested hydrocephalus. METHODS: A prospective study of 20 patients with arrested hydrocephalus was carried out. All patients underwent PC-MRI and 3D-CISS for assessment of the aqueduct. Axial (through-plane), sagittal (in-plane) PC-MRI, and sagittal 3D-CISS were applied to assess the cerebral aqueduct and the spontaneous third ventriculostomy if present. Aqueductal patency was graded using 3D-CISS and PC-MRI. Quantitative analysis of flow through the aqueduct was performed using PC-MRI. RESULTS: The causes of obstruction were aqueductal obstruction in 75% (n = 15), third ventricular obstruction in 5% (n = 1), and fourth ventricular obstruction in 20% (n = 4). The cause of arrest of hydrocephalus was spontaneous third ventriculostomy in 65% (n = 13), endoscopic third ventriculostomy in 10% (n = 2), and ventriculoperitoneal shunt in 5% (n = 1), and no cause could be detected in 20% of patients (n = 4). There is a positive correlation (r = 0.80) and moderate agreement (κ = 0.509) of grading with PC-MRI and 3D-CISS sequences. The mean peak systolic velocity of cerebrospinal fluid was 1.86 ± 2.48 cm/second, the stroke volume was 6.43 ± 13.81 µL/cycle, and the mean flow was 0.21 ± 0.32 mL/minute. CONCLUSIONS: We concluded that 3D-CISS and PC-MRI are noninvasive sequences for diagnosis of the level and cause of arrested hydrocephalus.