Francesca Raimondi

Role of tricuspid regurgitation in fetal echocardiographic diagnosis of pulmonary atresia with intact ventricular septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare cardiac malformation with a broad spectrum of anatomical manifestations, varying from types suitable for biventricular repair to those with diminutive right ventricle and primitive ventriculocoronary connections (VCC), more suitable for single‐ventricle palliation or heart transplantation. We sought to test whether tricuspid regurgitation is an easily detectable prenatal criterion with which to identify PAIVS patients at lower risk of needing postnatal single‐ventricle palliation.

Discordances Between Pre-Natal and Post-Natal Diagnoses of Congenital Heart Diseases and Impact on Care Strategies

BACKGROUND Pre-natal diagnosis of congenital heart disease (CHD) allows anticipation of urgent neonatal treatment and provides adequate information to the parents on cardiac outcomes. OBJECTIVES This study sought to analyze the discordances between expert fetal cardiac diagnosis and final diagnosis of CHD and their impact on neonatal and long-term care strategies. METHODS We included 1,258 neonates with a pre-natally diagnosed CHD and 189 fetopsies following termination of pregnancy at our tertiary center over a 10-year period. Pre-natal echocardiographic and final diagnoses were compared. RESULTS For live births, we identified 368 (29.3%) discordances between pre- and post-natal diagnoses. The pre-natal diagnosis was different from the post-natal diagnosis in 36 cases (2.9%) and partially different with a major impact on neonatal treatment of the CHD in 97 cases (7.7%). In 235 cases (18.7%), the diagnosis was partially different with no impact on neonatal planned treatment. The discordances had a negative impact on late care strategy in 62 cases (4.9%): more complex CHD that was unsuitable for biventricular repair, leading to unplanned compassionate care, additional surgery or increase of the complexity level of the Aristotle score. A positive impact was found in 31 cases (2.5%): less complex CHD that allowed biventricular repair, fewer surgical procedures, or decrease of the complexity of the Aristotle score. For 275 patients (21.9%), there was no impact on late care strategy. Of the 872 terminations of pregnancy and intrauterine fetal deaths, 189 fetopsies were available: 16 (8.5%) different diagnoses, 27 (14.3%) major differences, and 60 (31.7%) minor differences. CONCLUSIONS Correcting fetal cardiac diagnosis after birth can lead to significant changes in neonatal (10.6%) and late (7.4%) care strategies. Tools should be developed to try to improve the accuracy of pre-natal diagnosis of CHD. Clinicians should be cautious when predicting required treatment and outcomes during pre-natal counseling.

Vascular anatomy in children with pulmonary hypertension regarding the transcatheter Potts shunt

Objective To morphometrically characterise the region of adjacent descending aorta (DAo) and left pulmonary artery (LPA) regarding the transcatheter creation of the reverse Potts shunt. Methods and results Retrospective review of the invasive haemodynamic data and measurements of the vessel diameters, distances and angles based on the thoracic CT of children with idiopathic pulmonary arterial hypertension (PAH) with pulmonary-to-systemic systolic pressure ratio ≥0.5. Forty-eight CT scans from 47 patients were analysed. Independent of the PAH severity, the diameters of DAo and LPA, and the area of tightest contact between these vessels were very similar in patients with either infrasystemic or isosystemic/suprasystemic PAH. For total population, the tightest contact area (mean±SD, 51.8±31.9 mm2, range 12.5–177.7 mm2) had an elliptic shape stretched along the DAo length and LPA width. The shortest mean DAo-LPA distance was 1.7±0.8 mm (range 1–5 mm). Only one patient, from the suprasystemic PAH group, had the DAo-LPA distance >4 mm. None had lung tissue identified between these two vessels, while in four patients (8.3%) the prominent bronchial artery was seen coursing exactly between the LPA and DAo. The difference of prevalence of the bronchial arteries between two vessels in patients with either infrasystemic PAH or isosystemic/suprasystemic PAH did not reach statistical significance. Conclusions Children with idiopathic PAH showed no complicating anatomic or morphometric parameters of the region with adjacent DAo and LPA, which potentially determine the planning of the transcatheter creation of Potts shunt. It holds promises for standardisation of the procedure in the future.

Computed tomography imaging in children with congenital heart disease: Indications and radiation dose optimization

Computed tomography (CT) technology is acquiring a key role in the diagnostic process of complex cardiac congenital anomalies. Recent advances and improvements in spatial and temporal resolution and radiation dose are encouraging the use of CT scanning in children. Paediatric cardiologists should have a good knowledge of the potential of CT techniques and their limitations to plan and properly perform CT examinations without forgetting radiation concerns. In this paper, we will discuss the principal indications for CT scans in newborns and children in our clinical practice. We will also outline the most-used strategies for dose reduction. Basic knowledge about the various CT techniques is crucial, not only to perform, but also to interpret CT results, thus helping the medical and surgical management of patients.

Neonatal management and outcomes of prenatally diagnosed CHDs

OBJECTIVES The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD. METHODS A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention. RESULTS The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital. CONCLUSION Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

Specifics of cardiac magnetic resonance imaging in children.

This review points out three specific features of cardiac magnetic resonance imaging (MRI) in children: the small size of the heart modifies the usual balance between signal-to-noise ratio and spatial resolution; the higher and more variable heart rate limits tissue characterization and temporal resolution; and motion artefacts (notably respiratory motions) must be dealt with. In the second part of this review, we present the current and future practices of cardiac magnetic resonance (CMR) in children, based on the experience of all French paediatric cardiac MRI centres.

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome.

BACKGROUND Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach. OBJECTIVE To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up. METHODS A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections. RESULTS Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors. Discussion Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

Myocardial inflammation detected by cardiac MRI in Arrhythmogenic right ventricular cardiomyopathy: A paediatric case series

INTRODUCTION Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease with an elusive association with myocardial inflammation. A myocarditis phenotype has been well established without systematic evidence of a viral trigger. We intend to study the relationship between myocardial inflammation detected by Cardiac magnetic resonance (CMR) and ARVC in a paediatric population. METHODS Retrospective case series of all patients <18 years admitted to two CMR units for clinical suspicion of myocarditis from March 2012 to June 2017 who had genetic testing for inherited cardiomyopathies including analysis for known ARVC genes. RESULTS Six patients were identified experiencing myocarditis-like episodes with chest pain and troponin elevation. All had CMR evidence of active myocardial inflammation often affecting the left ventricle without identification of an infectious trigger. These episodes were likely exercise-induced in 50% of our patients and were multiple in all but one. CONCLUSION We provide evidence that ARVC can present as recurrent myocarditis-like episodes with CMR evidence of myocardial inflammation despite absent infectious trigger in children. We believe they represent an active hot phase of the disease and may lead to disease progression.

Low-dose paediatric cardiac and thoracic computed tomography with prospective triggering: Is it possible at any heart rate?

OBJECTIVE To demonstrate that the use of step-and-shoot (SAS) mode in paediatric cardiac CT angiography (CCTA) is possible at heart rates (HR) greater than 65 bpm, allowing low-dose acquisition with single-source 64-slices CT. METHODS We retrospectively included 125 paediatric patients (0-6 years). CCTA was performed with SAS at diastolic phase in 31 patients (group D, HR < 65 bpm), at systolic phase in 45 patients (group S, HR ≥ 65 bpm) and with non-gated mode in 49 patients (group NG). Effective dose (ED) and image quality using a 3-grade scoring scale (1, excellent; 2, moderate; 3, insufficient) of group S were compared with group D for coronary examinations and group NG for entire thorax vascular anatomy. RESULTS For coronary indications, median ED was 0.6 mSv in group D versus 0.9 mSv in group S (p < 0.01). For whole thorax indications, median ED was 2.7 mSv in group NG versus 1.1 mSv in group S (p < 0.001). The mean image quality score was (1.4 ± 0.6) points in group D, (1.4 ± 0.7) in group S for coronary indications (p = 0.9), (1.3 ± 0.6) in group S for whole thorax indications and (2.0 ± 0.0) in group NG (p < 0.001). CONCLUSION SAS mode is feasible in children with HR greater than 65 bpm allowing low-dose CCTA. It provided comparable image quality in systole, compared to diastole. SAS at the systolic phase provided better image quality with less radiation dose compared to non-gated scans for whole thorax examinations.

Cardiac Magnetic Resonance Myocardial Perfusion After Arterial Switch for Transposition of Great Arteries

Coronary artery (CA) obstruction is the most worrying post-operative complication after the arterial switch operation (ASO) for transposition of the great arteries. Although extremely rare, late mortality is predominantly a result of sudden death and myocardial infarction [(1–3)][1]. We