Incremental Detection of Severe Congenital Heart Disease by Fetal Echocardiography Following a Normal Second Trimester Ultrasound Scan in Québec, Canada.

BACKGROUND: The benefit of fetal echocardiograms (FE) to detect severe congenital heart diseases (SCHD) in the setting of a normal second-trimester ultrasound is unclear. We aimed to assess whether the increase in SCHD detection rates when FE are performed for risk factors in the setting of a normal ultrasound was clinically significant to justify the resources needed. METHODS: This is a multicenter, population-based, retrospective cohort study, including all singleton pregnancies and offspring in Quebec (Canada) between 2007 and 2015. Administrative health care data were linked with FE clinical data to gather information on prenatal diagnosis of CHD, indications for FE, outcomes of pregnancy and offspring, postnatal diagnosis of CHD, cardiac interventions, and causes of death. The difference between the sensitivity to detect SCHD with and without FE for risk factors was calculated using generalized estimating equations with a noninferiority margin of 5 percentage points. RESULTS: A total of 688 247 singleton pregnancies were included, of which 30 263 had at least one FE. There were 1564 SCHD, including 1071 that were detected prenatally (68.5%). There were 12 210 FE performed for risk factors in the setting of a normal second-trimester ultrasound, which led to the detection of 49 additional cases of SCHD over 8 years. FE referrals for risk factors increased sensitivity by 3.1 percentage points (95% CI, 2.3-4.0; P<0.0001 for noninferiority). CONCLUSIONS: In the setting of a normal second-trimester ultrasound, adding a FE for risk factors offered low incremental value to the detection rate of SCHD in singleton pregnancies. The current ratio of clinical gains versus the FE resources needed to screen for SCHD in singleton pregnancies with isolated risk factors does not seem favorable. Further studies should evaluate whether these resources could be better allocated to increase SCHD sensitivity at the ultrasound level, and to help decrease heterogeneity between regions, institutions and operators.

First-Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Cardiology Daily Practice.

Bicuspid aortic valve (BAV) is the most common congenital heart disease. Since heritability is suspected, actual guidelines recommend to perform an echocardiographic assessment for first-degree relatives (FDR) of patient with BAV. This study aimed to assess the effectiveness and the feasibility of the current guidelines for the screening of FDR of patient with BAV in a pediatric cardiology daily practice. Consecutive patients with BAV and their FDR were prospectively included from January 2015 to March 2018 at Centre Hospitalier Universitaire de Laval, Quebec City (Canada). Data were retrospectively collected and analyzed. A total of 713 FDR of 213 consecutives index cases [median age: 11 (6-20) years] were studied. Up to 32 (6.6%) FDR had a BAV and 26 (5.4%) had an aortic valve dysfunction. A total of 14 (2.9%) FDR had an ascending aorta dilatation according to Z-score including 6 (1.2%) patients with an ascending aorta ≥ 45 mm. No statistically significant differences regarding BAV, aortic valve dysfunction and ascending aorta dilatation prevalence were identified between generations. Screening was done in 482 (67.6%), prescribed but not done in 134 (19%), not prescribed in 92 (13%) and declined in 5 (1%) FDR. The prevalence of BAV in FDR was similar to prospective adult studies and supports actual guidelines in pediatric cardiology practice. Ascending aorta dilatation was rare in our young population. Exhaustiveness and additional burden to implement current guidelines remain a challenge in daily practice.

Right atrial catheter "ghost" removal by cardiac surgery: A pediatric case series report.

Fibrin sheath formation around long-term indwelling central venous catheters is common and usually benign. Fibrin sheath can persist after catheter removal and rarely leads to complications. This is a report of three pediatric oncology patients that required cardiac surgery for cardiac embolization of a "ghost" catheter several years after catheter removal. One case required tricuspid valve replacement for complete tricuspid valve destruction and two had erosion through the atrial wall. The severity of these rare complications mandates follow-up of "ghost" catheters in pediatric oncology patients.

More Than 25 Years of Experience With the Ross Procedure in Children: A Single-Center Experience.

BACKGROUND: Aortic valve replacement in children represents an important challenge. Concerns regarding pulmonary autograft and homograft longevity requiring reoperations are well recognized. Very long-term outcomes after the Ross procedure are still unknown. We reviewed our experience with the Ross procedure, aiming to define very long-term survival rate and freedom from reintervention. METHODS: This was a single-center retrospective cohort including 63 consecutive children who underwent the Ross procedure. Median follow-up duration was 20.5 years. Time-related events were assessed using Kaplan-Meier estimator. RESULTS: There were 51 (81%) boys, mean age 10.1 ± 5.8 years. Isolated aortic stenosis was the most common diagnosis (n = 29, 46%) and 34 (54%) patients previously underwent cardiac surgery. There was 1 (1.6%) in-hospital death. Overall survival at 5, 15, and 25 years was 96.7%, 94.4%, and 94.4%, respectively. Freedom from any autograft-related reintervention was 98.1%, 86.4%, and 61.2% at 5, 15, and 25 years, respectively. Fifteen (24%) patients underwent autograft reoperations. Among them, 10 (67%) patients underwent valve-sparing autograft reoperation. Freedom from any pulmonary conduit reintervention was 93.2%, 58.2%, and 28.3% at 5, 15, and 25 years, respectively. Thirty (46.6%) patients underwent conduit reintervention (8 percutaneous, 22 surgical replacements). CONCLUSIONS: The pediatric Ross procedure is associated with excellent long-term survival. Ross-related reinterventions are more than twice as common on the pulmonary homograft than on the autograft.

Rationale and Design of the FREQUENCY Study: The Fetal Cardiac Registry of Québec to Improve Resource Utilization in Fetal Cardiology.

OBJECTIVE: Prenatal detection of congenital heart diseases (CHD) decreases morbidity and cost. To improve detections rates, most physicians refer pregnant women with high-risk pregnancies to fetal cardiologists even when there is no suspicion of CHD at the second trimester screening. This paper presents the rationale and detailed method of the Fetal Cardiac Registry of Québec to Improve Resource Utilization in Fetal Cardiology (FREQUENCY) study. The overall objective is to assess the impact of second trimester ultrasound screening (U/S) and referral pattern in fetal cardiology on detection rates, health care costs, and resource utilization, as well as perinatal morbidity and mortality. METHODS: This multicentre retrospective population-based cohort study will link fetal echocardiography data from all centres performing fetal echocardiography in Québec with administrative health care data. This data linking will allow the determination of a true denominator (all women in Québec who underwent second trimester U/S) with complete follow-up of up to 2 years for offspring. This protocol meets Canadian Task Force Classification II-2. RESULTS: The study investigators have collected and cleaned fetal echocardiography data for 24 259 eligible pregnancies referred to fetal cardiology. These data will be matched to approximately 860 000 pregnancies between 2007 and 2015. CONCLUSION: The results of the FREQUENCY study will shed light on the impact of the current prenatal CHD screening strategy in Canada.

Relationship between interatrial communication, ductus arteriosus, and pulmonary flow patterns in fetuses with transposition of the great arteries: prediction of neonatal desaturation.

BACKGROUND: The relationship between interatrial communication, ductus arteriosus, and pulmonary flow in transposition of the great arteries and intact ventricular septum may help predict postnatal desaturation. METHODS: Echocardiographic data of 45 fetuses with transposition of the great arteries and intact ventricular septum and 50 age-matched controls were retrospectively reviewed. Interatrial communication, left and right ventricular output, flow in the ductus arteriosus, as well as effective pulmonary flow were measured. Patients were divided into two groups on the basis of postnatal saturations: group 1 had saturations ⩽50% and group 2 >50%. RESULTS: Of 45 fetuses, 13 (26.7%) were classified into group 1. Compared with fetuses in group 2, they had a smaller interatrial communication (2.9 versus 4.0 mm, p=0.004) and more retrograde diastolic flow in the ductus arteriosus (92 versus 23%, p=0.002). Both groups showed a significant decrease in ductal flow compared with controls. Patients in group 2 had a higher effective pulmonary flow compared with controls. There was a mild correlation between left ventricular output and size of the interatrial communication (Spearman's rank correlation 0.44). CONCLUSION: A retrograde diastolic flow is present in most of the fetuses with postnatal desaturation. Fetuses with transposition of the great arteries have a lower flow through the ductus arteriosus compared with controls. Fetuses without restrictive foramen ovale have higher effective pulmonary flow. Peripheral pulmonary vasodilatation due to higher oxygen saturation in pulmonary arteries in the case of transposition of the great arteries could be one possible cause.

Comparing Carotid and Brachial Artery Stiffness: A First Step Toward Mechanical Mapping of the Arterial Tree.

Arterial stiffness is a predictor of cardiovascular mortality. It increases with age and is accelerated by hypertension and other cardiovascular risk factors. In addition to the disease state, arterial stiffness increases from the proximal to the distal arterial compartments. Concurrent assessment of various vessels from the same subjects is unavailable in the literature. The aim of this work was to quantify an intrinsic mechanical feature, namely, wall stiffness, of the common carotid artery (CCA) and brachial artery (BA). CCAs and BAs of healthy adolescents were investigated. Cine loops of CCA and BA B-mode data were digitally recorded at the same clinical examination, and arterial elastic moduli were estimated off-line with our proprietary non-invasive Imaging-based BioMarker (ImBioMark) algorithm. The 11 study subjects were 14.4 ± 1.2 years old, with normal body habitus and blood pressures 112.3 ± 10.6/63.6 ± 5.7 mm Hg. BAs had a higher elastic modulus than CCAs (arterial elastic moduli: 129.73 ± 25.67 kPa vs. 49.55 ± 14.75 kPa, p < 0.001). There was a positive correlation between the BA and CCA (slope = 0.36, intercept = 111.62 kPa, R(2) = 0.045). This article documents, for the first time, a correlation between the CCA and BA of the same subject, under the same conditions. We previously reported preliminary data for the aorta and documented the effect of aging on the CCA; we now intend to study the femoral artery as well and include age stratification to pursue our investigations. The results reported here can be seen as the first step toward mechanical mapping of the arterial tree.

Uhl's anomaly: a difficult prenatal diagnosis.

Uhl's anomaly is an evolutive disease leading to terminal right ventricular failure. The most difficult differential diagnosis at presentation is the Ebstein disease. We describe the evolution of a foetus with Uhl's anomaly from 21 to 30 weeks of gestation, with progressive reduction in the right ventricular anterior myocardium suggestive of apoptosis, leading to foetal demise.

Carotid wall elastography to assess midterm vascular dysfunction secondary to intrauterine growth restriction: feasibility and comparison with standardized intima-media thickness.

Several studies have suggested that intrauterine growth restriction (IUGR) increases the risk of cardiovascular disease and early atherosclerosis. Early detection of arteriopathy is essential to early intervention. Although arterial intima-media thickness (IMT) is considered an index of subclinical atherosclerosis in the adult, its validity in pediatric patients may be limited. We have recently introduced a novel imaging-based biomarker (ImBioMark) to assess intrinsic mechanical features of the arterial wall from B-mode ultrasound data. The aim of the work described here was to evaluate the potential of ImBioMark in investigation of cardiovascular health status at the level of the common carotid artery (CCA) in adolescents born after IUGR. We also compared ImBioMark results with automated IMT measurements, a well-established biomarker used in clinical practice and research. The potential sequelae of IUGR on the CCA were examined in a group of adolescents in comparison with healthy controls. Patients with IUGR (n = 7) were 13.85 ± 0.46 y old; the healthy controls (n = 7) were 14.58 ± 0.80 y old (p = 0.058). Cine loops of the CCA B-mode data were digitally recorded, and the arterial elastic modulus was estimated a posteriori with ImBioMark. IMT of the CCA was automatically calculated using QLAB software (Philips, Andover, MA, USA). All patients had been evaluated in utero in our fetal echocardiographic laboratory. ImBioMark detected a significant increase in CCA stiffness in patients with IUGR as compared with healthy controls: elastic modulus = 90.74 ± 11.86 versus 61.30 ± 15.94 kPa, respectively (p = 0.002). There was, however, no significant difference between patients with IUGR and controls in IMT (0.483 ± 0.067 versus 0.476 ± 0.051 mm, respectively, p = 0.831). The impact of IUGR on CCA wall dynamics was confirmed by ImBioMark. The apparent limitation of IMT measurement in this cohort may be the result of geometric arterial changes, that is, the expected thickening, below the level of detection at this age. As early detection of vascular modulation is essential to early intervention in a population at risk, we now intend to extend ImBioMark to investigate larger pathologic cohorts with various degrees of arteriopathy.

The biophysical properties of the aorta are altered following Kawasaki disease.

BACKGROUND: The long-term sequelae of Kawasaki disease (KD) are based on the coronary complications. Because KD causes generalized vasculitis, with documented aneurysms in the femoral, iliac, renal, axillary, and brachial arteries, the aim of this study was to assess the biophysical properties of the aorta (BPA) after KD. The BPA are biometric measurements representing vascular structural and dynamic changes in response to cardiac work. METHODS: Anthropometric and echocardiographic measurements of the aorta in a series of patients with KD were compared with those of healthy subjects. The BPA were calculated noninvasively by extrapolating previously validated equations that were conceived for invasive measurements. Because BPA vary with body habitus, control subjects were used to normalize BPA parameters for height to compute BPA Z-score equations. RESULTS: Between June 2007 and February 2010, BPA were recorded in 57 patients with KD >1 year after the onset of the disease, 45 without and 12 with coronary artery sequelae. The mean intervals between the acute onset of KD and enrollment were 10.0 ± 5.0 and 5.8 ± 4.5 years for patients with and without coronary artery sequelae, respectively (P = .008). Patients with KD with coronary artery sequelae had significantly altered Z scores of aortic diameter modulation, Peterson's elastic modulus, and ß stiffness index (P = .001-.016). Patients with KD without coronary artery sequelae also exhibited altered elasticity, stiffness, and pulse-wave velocity (P = .001-.026). CONCLUSIONS: Altered BPA after KD are detectible despite apparent resolution of acute vasculitis. Future directions toward determining multilevel and multilayer vascular impact, including vascular autonomous homeostasis, require thorough investigation.