Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients.

Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8.4 ± 4.3 and 5.4 ± 4.1 years post-Fontan) and 8 adults (average age 31.5 ± 8.9 and 21.1 ± 4 years post-Fontan) using standard serum laboratory investigations assessing hepatic integrity and function, the FibroTest, liver ultrasound, and transient elastography (FibroScan). In adult Fontan patients, hemoglobin, C-reactive protein, and gamma-glutamyl transpeptidase were significantly increased, and white blood cell and platelet counts were significantly decreased in comparison to the pediatric cohort. International normalized ratio was mildly elevated in both children and adults. FibroTest results were suggestive of fibrosis regardless of time post-Fontan. FibroScan measurements were significantly correlated with time post-Fontan, but the incidence of ultrasound-detected liver abnormalities was variable. No cases of hepatocellular carcinoma were identified. Abnormalities suggestive of FALD occur in both children and adults post-Fontan. Select laboratory tests, and possibly ultrasound and FibroScan in some patients, appear to have the most promise for the non-invasive detection of FALD.

Evaluation of single right atrial volume and function with magnetic resonance imaging in children with hypoplastic left heart.

BACKGROUND: Standardized methods to evaluate atrial properties in single ventricles are lacking. OBJECTIVE: To determine the feasibility of quantifying right atrial volumes and function in hypoplastic left heart using MRI. MATERIALS AND METHODS: We studied 15 infants with hypoplastic left heart prior to Glenn surgery (mean age 4.2 months [standard deviation 0.3]) who underwent cardiac MRI with evaluation of atrial volumes and emptying fraction using monoplane two-chamber, monoplane four-chamber, and biplane methods, all of which were compared to the atrial short-axial oblique stack method. We compared atrial end-diastolic volume, end-systolic volume and emptying fraction among these methods. We analyzed reproducibility of the methods using Bland‒Altman plots. RESULTS: Both four-chamber and biplane methods showed high correlations for atrial end-diastolic volume (r = 0.7 and r = 0.8, respectively; P < 0.01) and end-systolic volume (r = 0.8 and r = 0.9, respectively; P < 0.01) with small mean differences (-0.2 ± 2.9 standard deviation [SD] ml and -0.8 ± 1.6 ml, respectively, for atrial end-diastolic volume and -0.8 ± 1.5 ml and -0.9 ± 0.9 ml, respectively, for atrial end-systolic volume). The short-axial oblique method was the most reproducible, followed by the four-chamber method. CONCLUSION: MRI assessment of atrial volume and function is feasible in hypoplastic left heart and might provide further insight into single-ventricle mechanics.

Which cardiovascular magnetic resonance planes and sequences provide accurate measurements of branch pulmonary artery size in children with right ventricular outflow tract obstruction?

Children with right ventricular outflow tract obstructive (RVOTO) lesions require precise quantification of pulmonary artery (PA) size for proper management of branch PA stenosis. We aimed to determine which cardiovascular magnetic resonance (CMR) sequences and planes correlated best with cardiac catheterization and surgical measurements of branch PA size. Fifty-five children with RVOTO lesions and biventricular circulation underwent CMR prior to; either cardiac catheterization (n = 30) or surgery (n = 25) within a 6 month time frame. CMR sequences included axial black blood, axial, coronal oblique and sagittal oblique cine balanced steady-state free precession (bSSFP), and contrast-enhanced magnetic resonance angiography (MRA) with multiplanar reformatting in axial, coronal oblique, sagittal oblique, and cross-sectional planes. Maximal branch PA and stenosis (if present) diameter were measured. Comparisons of PA size on CMR were made to reference methods: (1) catheterization measurements performed in the anteroposterior plane at maximal expansion, and (2) surgical measurement obtained from a maximal diameter sound which could pass through the lumen. The mean differences (Δ) and intra class correlation (ICC) were used to determine agreement between different modalities. CMR branch PA measurements were compared to the corresponding cardiac catheterization measurements in 30 children (7.6 ± 5.6 years). Reformatted MRA showed better agreement for branch PA measurement (ICC > 0.8) than black blood (ICC 0.4-0.6) and cine sequences (ICC 0.6-0.8). Coronal oblique MRA and maximal cross sectional MRA provided the best correlation of right PA (RPA) size with ICC of 0.9 (Δ -0.1 ± 2.1 mm and Δ 0.5 ± 2.1 mm). Maximal cross sectional MRA and sagittal oblique MRA provided the best correlate of left PA (LPA) size (Δ 0.1 ± 2.4 and Δ -0.7 ± 2.4 mm). For stenoses, the best correlations were from coronal oblique MRA of right pulmonary artery (RPA) (Δ -0.2 ± 0.8 mm, ICC 0.9) and sagittal oblique MRA of left pulmonary artery (LPA) (Δ 0.2 ± 1.1 mm, ICC 0.9). CMR PA measurements were compared to surgical measurements in 25 children (5.4 ± 4.8 years). All MRI sequences demonstrated good agreement (ICC > 0.8) with the best (ICC 0.9) from axial cine bSSFP for both RPA and LPA. Maximal cross sectional and angulated oblique reformatted MRA provide the best correlation to catheterization for measurement of branch PA's and stenosis diameter. This is likely due to similar angiographic methods based on reformatting techniques that transect the central axis of the arteries. Axial cine bSSFP CMR was the best surgically measured correlate of PA branch size due to this being a measure of stretched diameter. Knowledge of these differences provides more precise PA measurements and may aid catheter or surgical interventions for RVOTO lesions.

Magnetic resonance imaging of a restrictive atrial septal defect in a univentricular connection.

Magnetic resonance imaging (MRI) provides accurate and valuable information regarding cardiac shunts including their location, size, and flow. We describe the MRI findings of a restrictive atrial septal defect within a complex case of congenital heart disease: univentricular atrioventricular connection of left ventricular type with right-sided discordant atrioventricular connection (single), absent left atrioventricular connection, and ventriculoarterial discordance. Few similar cases are reported in the literature. Magnetic resonance imaging may add valuable information regarding restrictive atrial shunts in univentricular hearts.

Mitral valve surgery for marantic endocarditis and multiple cerebral embolisation.

Valvular involvement is common in antiphospholipid syndrome (APS) with increased risk of thrombo-embolic events. We report a patient with APS and multiple cerebral infarcts. Echocardiography demonstrated verrucous vegetations of the mitral valve in keeping with marantic endocarditis. The patient underwent successful mitral valve replacement. Post-operative clinical and echocardiographic follow-up showed excellent short term results.

Subscapular tumoral calcinosis in a toddler: case report.

Tumoral calcinosis is uncommon in toddlers, and rare within the subscapular area. Although typically benign, tumoral calcinosis is often incorrectly diagnosed prior to biopsy. We present a case of subscapular tumoral calcinosis in a 16-month old girl and discuss the radiological findings on X-ray, ultrasound, computed tomography and magnetic resonance imaging, including the first description of T1-weighted post contrast imaging, which demonstrate the fibrotic components of tumoral calcinosis.

Giant left atrial appendage aneurysm in a neonate.

Giant aneurysm of the left atrial appendage (LAA) is a rare condition typically presenting in adulthood. This case report describes the investigations and surgical management of a giant LAA aneurysm in a neonate, emphasizing the role of echocardiography and cardiovascular magnetic resonance imaging in preoperative evaluation as well as challenges in surgical repair of this defect.

The evolving approach to paediatric myocarditis: a review of the current literature.

Paediatric myocarditis remains challenging from the perspectives of diagnosis and management. Multiple aetiologies exist and the majority of cases appear to be related to viral illnesses. Enteroviruses are believed to be the most common cause, although cases related to adenovirus may be more frequent than suspected. The clinical presentation is extremely varied, ranging from asymptomatic to sudden unexpected death. A high index of suspicion is crucial. There is emerging evidence to support investigations such as serum N-terminal B-type natriuretic peptide levels, as well as cardiac magnetic resonance imaging as adjuncts to the clinical diagnosis. In the future, these may reduce the necessity for invasive methods, such as endomyocardial biopsy, which remain the gold standard. Management generally includes supportive care, consisting of cardiac failure medical management, with the potential for mechanical support and cardiac transplantation. Treatments aimed at immunosuppression remain controversial. The paediatric literature is extremely limited with no conclusive evidence to support or refute these strategies. This article summarises the current literature regarding aetiology, clinical presentation, diagnosis, and management of myocarditis in paediatric patients.