Z-score evaluation and inferences for management of aortopathy patients.

Echocardiographic Z-score models play a crucial role in defining cardiac pathology in paediatric patients. There are multiple models that practitioners utilize in the United States without guiding principles to standardize their use. Discrepant interpretations can occur depending on the model chosen, even if standardized Z-score cutoffs are applied.

The Ongoing Relationship Between Offspring Congenital Heart Disease and Preeclampsia Across Pregnancies.

Background: Prior literature has described an association between preeclampsia and offspring congenital heart disease (CHD), while suggesting there may be a stronger relationship in individuals with early preeclampsia.

Objectives: The authors sought to explore the relationship between offspring CHD and preeclampsia among pregnancies in a population-based study.

Methods: Retrospective cohort study all singleton pregnancies delivered in the state of California 2000 to 2012.

The Evolving Role of Genetic Evaluation in the Prenatal Diagnosis and Management of Congenital Heart Disease.

Congenital heart disease (CHD) is increasingly diagnosed prenatally and the ability to screen and diagnose the genetic factors involved in CHD have greatly improved. The presence of a genetic abnormality in the setting of prenatally diagnosed CHD impacts prenatal counseling and ensures that families and providers have as much information as possible surrounding perinatal management and what to expect in the future. This review will discuss the genetic evaluation that can occur prior to birth, what different genetic testing methods are available, and what to think about in the setting of various CHD diagnoses.

Perinatal Stressors and Consequences for Neonates with Critical Congenital Heart Disease.

Introduction: The prenatal diagnosis of congenital heart disease (CHD) is a traumatic event that can cause expectant parents to experience anxiety, depression, and toxic stress. Prenatal exposure to stress may impact neonatal postoperative outcomes. In addition, expectant parents may have other psychosocial stressors that may compound maternal stress.

Structured Framework for Multidisciplinary Parent Counseling and Medical Interventions for Fetuses and Infants with Trisomy 13 or Trisomy 18.

Objective: Trisomy 13 (T13) and 18 (T18) are aneuploidies associated with multiple structural congenital anomalies and high rates of fetal demise and neonatal mortality. Historically, patients with either one of these diagnoses have been treated similarly with exclusive comfort care rather than invasive interventions or intensive care, despite a wide phenotypic variation and substantial variations in survival length. However, surgical interventions have been on the rise in this population in recent years without clearly elucidated selection criterion.

Homogenous access to fetal cardiac care in a heterogeneous state.

Background: Timely prenatal diagnosis of CHD allows families to participate in complex decisions and plan for the care of their child. This study sought to investigate whether timing of initial fetal echocardiogram and the characteristics of fetal counselling were impacted by parental socio-economic factors.

Methods: Retrospective chart review of fetal cardiac patients from 1 January, 2017 to 31 December, 2018.

Assessment of the mechanism of mitral valve prolapse in children: An echocardiography study.

Background: The high complexity of mitral valve anatomy and function in mitral valve prolapse (MVP) is not yet fully understood.

Objective: The purpose of this study was to analyze each part of the mitral valve apparatus in children to determine its impact on the presence of MVP and to assess the interaction between the coaptation length (CL) and mitral regurgitation severity.

Methods: We prospectively analyzed transthoracic echocardiograms of 60 patients with MVP (mean age 9.

Prenatal Congenital Heart Disease-It Takes a Multidisciplinary Village.

Prenatal diagnosis of congenital heart disease (CHD) allows for thoughtful multidisciplinary planning about location, timing, and need for medical interventions at birth. We sought to assess the accuracy of our prenatal cardiac diagnosis, and postnatal needs for patients with CHD utilizing a multidisciplinary approach. We performed a retrospective chart review of fetal CHD patients between 1/1/18 and 4/30/19.

Heart Rate Induced Dynamic Coronary Ischemia Secondary to Aortic Valve Insufficiency from a Diminutive Aortic Valve Leaflet: Diagnosis and Repair.

Hemodynamically significant congenital obstruction of the coronary arteries is a rare phenomenon. The potential etiology of these obstructions is variable including coronary os stenosis, occlusive fibrous webs, or a dysplastic aortic valve leaflet resulting in obstruction. In infants, diagnosis of these coronary lesions is often difficult due to the small anatomic size and limitations of imaging modalities in the setting of the underlying rapid heart rates.

Myocardial Perfusion in Hypoplastic Left Heart Syndrome.

Background: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle's coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS.

Fetal counseling for congenital heart disease: is communication effective?

Purpose: To assess the accuracy of maternal understanding of fetal cardiac defects following initial fetal counseling.

Methods: Pregnant women with a fetal diagnosis of congenital heart disease (CHD) were surveyed regarding understanding of their fetus's heart defect. The survey asked: (1) for a description of the heart condition; (2) how confident they were in the diagnosis; (3) whether their fetus would require heart surgery.

Pulse Oximetry Screening and Critical Congenital Heart Disease in the State of Oregon.

Late diagnosis of critical congenital heart disease (CCHD) is associated with higher levels of morbidity and mortality in neonates. Nearly all states have passed laws mandating universal pulse oximetry screening (POxS) of newborns to improve early detection rates of CCHD. We performed a retrospective chart review of all transthoracic echocardiograms (TTEs) interpreted by our institution on patients between 0 and 30 days of life in the years 2010 (prior to POxS) and 2015 (after POxS).

Relationship between patient understanding and timeliness of penicillin prophylaxis in rheumatic heart disease prevention programmes in American Samoa.

Aim: While mostly eradicated in developed nations, rheumatic heart disease (RHD) is still the leading cause of preventable cardiovascular disease in children. RHD and its antecedent acute rheumatic fever remain endemic in many low to middle income countries, as well as in vulnerable communities in wealthy ones. Evidence-based interventions are particularly important in resource-poor settings.

The Utility of Echocardiography in Pediatric Patients with Structurally Normal Hearts and Suspected Endocarditis.

The objective of this study was to evaluate the utility of transthoracic echocardiography (TTE) in children with structurally normal hearts suspected of having infective endocarditis (IE). We hypothesized that the diagnostic yield of TTE is minimal in low-risk patients with normal hearts. We performed a retrospective chart review of TTEs performed for concern for endocarditis at a pediatric tertiary care referral center in Portland, Oregon.

Cerebrovascular blood flow in patients with tetralogy of fallot: prediction for early surgical intervention.

Objectives: Children with tetralogy of Fallot (TOF) require initial intervention at varying times reflecting a spectrum of obstruction to pulmonary blood flow. In utero, fetal patients with right sided obstructive lesions demonstrate increased middle cerebral artery (MCA) pulsatility index (PI). We sought to determine whether MCA flow patterns are associated with the degree of pulmonary obstruction and the consequential need for early surgical intervention (ESI).

Determination of right ventricular function with adjusted systolic to diastolic duration ratio after transannular patch repair of tetralogy of Fallot.

Background: Early detection of right ventricular dysfunction after transannular patch for tetralogy of Fallot (TOF-TAP) is essential for management.

Objectives: To evaluate echocardiographic metrics of ventricular function correlate with functional MRI measurements, in patients with TOF-TAP.

Methods: A retrospective review of patients with TOF-TAP between 2007 and 2017 who had an echocardiogram and MRI within six months were analyzed.

Echocardiographic Screening of Rheumatic Heart Disease in American Samoa.

While rheumatic heart disease (RHD) is a treatable disease nearly eradicated in the United States, it remains the most common form of acquired heart disease in the developing world. This study used echocardiographic screening to determine the prevalence of RHD in children in American Samoa. Screening took place at a subset of local schools.

Routine intra-operative trans-oesophageal echocardiography yields better outcomes in surgical repair of CHD.

Objectives: Trans-oesophageal echocardiographic imaging is valuable in the pre- and post-operative evaluation of children and adults with CHD; however, the frequency by which trans-oesophageal echocardiography guides the intra-operative course of patients is unknown.

Methods: We retrospectively reviewed 1748 intra-operative trans-oesophageal echocardiograms performed between 1 October, 2005 and 31 December, 2010, and found 99 cases (5.7%) that required return to bypass, based in part upon the intra-operative echocardiographic findings.

Abnormal myocardial blood flow in children with mild/moderate aortic stenosis.

Objective: To quantify myocardial blood flow in infants and children with mild or moderate aortic stenosis using adenosine-infusion cardiac magnetic resonance.

Background: It is unclear whether asymptomatic children with mild/moderate aortic stenosis have myocardial abnormalities. In addition, cardiac magnetic resonance-determined normative myocardial blood flow data in children have not been reported.

High flow velocity through congenital cardiac lesions predicts preoperative platelet dysfunction.

Background: Platelet dysfunction resulting from abnormal fluid shear stress has been reported in adults with aortic stenosis. Blood flowing through a congenital heart defect at greater than normal velocity is subjected to increased shear stress. The primary aim was to determine whether peak flow velocity through congenital cardiac lesions predicts preoperative platelet dysfunction.

Frequency and outcomes of cardiac operations and catheter interventions in Turner syndrome.

Cardiac malformations occur commonly in Turner syndrome (TS), but the outcomes of cardiac operations and catheter-based procedures are unknown. The Pediatric Cardiac Care Consortium database was queried for individuals with TS and other female subjects without genetic abnormalities or syndromes (non-TS [NTS]). Procedures for left-sided heart lesions represented most TS procedures (95.