Left and Right Ventricular Strain Mechanics in Fetal Tachyarrhythmia.

Introduction: Assessment of myocardial function in fetuses with supraventricular tachyarrhythmia is challenging. Speckle-tracking echocardiography (STE) is a newer sensitive method to assess ventricular systolic function. We sought to assess left (LV) and right (RV) ventricular myocardial strain mechanics in fetuses with tachyarrhythmia and hypothesized that strain mechanics are impaired in this patient population even after conversion to sinus rhythm.

Outcomes of Hypoattenuating Leaflet Thickening Post-Transcatheter Pulmonary Valve Replacement.

Background: Transcatheter pulmonary valve replacement (TPVR) is a mainstay of therapy for right ventricular outflow tract dysfunction, especially in adult congenital heart disease.

Objectives: This study aimed to assess the prevalence, characteristics, and clinical implications of hypoattenuating leaflet thickening (HALT) and hypoattenuation affecting motion (HAM) after TPVR.

Methods: This was a retrospective cohort study of TPVR performed from 2018 to 2024.

Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome.

We present a unique case of progressive neoaortic root and neoascending aortic dilatation, with poly-valvar regurgitation in a patient with Marfan syndrome and hypoplastic left heart syndrome palliated to Fontan procedure. This progressive dilation and valvar dysfunction necessitated a modified Bentall procedure and tricuspid valve repair. Our case highlights the impact of both a primary and secondary aortopathy complicating the Fontan procedure and the need for mechanical aortic valve replacement.

Retrospective study of left and right ventricular strain mechanics among neonates undergoing therapeutic hypothermia for neonatal encephalopathy.

Purpose: Early diagnosis of impaired myocardial function and timely therapeutic hypothermia is vital among patients with Neonatal Encephalopathy (NE). Traditional markers of myocardial function (Left Ventricular Ejection Fraction (LV EF) & LV Fractional Shortening (LV FS) can be variably reduced. Speckle tracking echocardiography (STE) is a more sensitive marker for impairment but remains inadequately studied in this patient population.

Fetal Left Ventricular Apical Aneurysm Progressing to Dilated Cardiomyopathy Due to Glycogen Storage Disease.

Fetal dilated cardiomyopathy is a rare anomaly characterized by ventricular dilation and dysfunction. Its causes are diverse, and its outcomes are generally dismal. We describe a rare case of prenatally diagnosed left ventricular apical aneurysm that progressed rapidly to dilated cardiomyopathy.

Optimizing evaluation in pediatric and young adult patients with Marfan syndrome: Novel longitudinal metrics to track growth of aortic structures.

Objective: Surveillance metrics in pediatric and young adult Marfan syndrome (pMFS) are challenging. We evaluated the utility of aortic root cross-sectional area/height index (CSA/Ht) on echocardiogram among pMFS patients as a risk stratification and surgical triage metric.

Methods: Genotype or phenotype positive pMFS patients aged 25 years or younger seen at our center from 2001 to 2020 were identified.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Prenatal Delineation of Coronary Anatomy in Dextro-Transposition of Great Arteries.

Background: Dextro-transposition of the great arteries (D-TGA) is the second-most common cyanotic congenital heart disease with variable coronary artery anatomy. The arterial switch procedure has revolutionized outcomes for this defect, with coronary anatomy being a key determinant of both short- and long-term outcomes following surgical repair. The assessment of coronary anatomy is usually undertaken in the postnatal period by transthoracic echocardiography, with assessment prenatally not being well studied.

Life saved and a lesson learned-anomalous origin of the left coronary artery from pulmonary artery in pregnancy.

Anomalous origin of the left coronary artery from pulmonary artery constitutes 0.5% of all CHD (Boutsikou M, Shore D, Li W, et al, Int J Cardiol 261: 49-53, 2018). Fifteen percent survive into adulthood undiagnosed and 90% present with sudden death (Yau JM, Singh R, Halpern EJ, Fischman D, Clin Cardiol 34: 204-210, 2011; Alexi-Meskishvili V, Berger F, Weng Y, Lange PE, Hetzer R, J Card Surg 10: 309-315, 1995).

Left Atrial Strain and Function in Pediatric Hypertrophic Cardiomyopathy.

Background: Left atrial (LA) strain and dysfunction are early markers of diastolic dysfunction, associated with poor exercise capacity in adults with hypertrophic cardiomyopathy (HCM). Literature on assessment of LA mechanics in pediatric HCM is lacking. The aim of this study was to assess LA strain and LA function in pediatric patients who have HCM with (phenotype positive [P+]) and without (genotype positive, phenotype negative [G+P-]) ventricular hypertrophy and evaluate their correlation with exercise stress test parameters.

Unusual coronary artery arrangement in D-transposition of the great arteries with ventricular septal defect.

A term female with prenatally diagnosed D-Transposition of the great arteries, large membranous ventricular septal defect with inlet extension, moderate secundum atrial septal defect, and large patent ductus arteriosus (Fig 1) was born by scheduled caesarean section. Transthoracic echocardiogram confirmed the anatomy with both coronary arteries arising from a single sinus with separate ostia. The right coronary artery arose from right posterior facing sinus (Fig 2).

Selective Coronary Artery Angiography in Hypoplastic Left Heart Syndrome.

Coronary artery disease in palliated hypoplastic left heart syndrome is uncommon. Myocardial infarction from a coronary thrombus, serving as a substrate for ventricular arrhythmia in Fontan physiology, is under-reported despite known hypercoagulopathic state. Traditional risk factors for coronary artery occlusion include intracardiac thrombi, hyperlipidemia, and hypertension.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia.

Outcomes of truncus arteriosus repair and predictors of mortality.

Objective: The objective of this study was to identify patient and hospitalization characteristics associated with in-hospital mortality in infants with truncus arteriosus.

Methods: We conducted a retrospective analysis of a large administrative database, the National Inpatient Sample data set of the Healthcare Cost and Utilization Project for the years 2002 to 2017. We also sought to evaluate the resource utilization in the subgroup of subjects with truncus arteriosus and 22q11.

Isolated Endocarditis of Native Pulmonary Valve in a Pediatric Patient: The Unusual within the Unusual.

• Native PV IE is extremely rare. • Recurrent septic emboli from PV IE may masquerade as severe pneumonia. • Failure of medical management may require surgical removal of the focus of infection.

Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm.

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site.

Short-term results in infants with multiple left heart obstructive lesions.

Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.

Incidental finding of type A aortic dissection in a paediatric heart transplant recipient.

Aortic dissection causes significant morbidity and mortality in adults and treatment guidelines are based on well-documented risk factors. Conversely, dissection after orthotopic heart transplantation is very rare, especially in the absence of infection, hypertension, or donor-recipient aortic size mismatch. Several forms of CHD are associated with aortic dilatation, but the incidence of aortic dissection and aneurysm in children is also low, which makes use of adult guidelines in children challenging.

Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making.

Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right-sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis.

Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia.

In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy.

Pentalogy of Cantrell: A View From Fetus to Operation With Multimodality Imaging.

Pentalogy of Cantrell and its surgical management have been previously described in the literature. To our knowledge, our case report is the first that is able to demonstrate the full use of multimodality imaging to describe it. Viewing this rare defect chronologically from fetal echocardiography, postnatal ultrasonography, and magnetic resonance imaging, as well as intraoperatively aided in the management of this patient.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.

Background: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.