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The article presents a case study of a 4-year-old girl diagnosed with persistent truncus arteriosus (PTA), highlighting her imaging manifestations as observed through echocardiography (ECHO) and cardiac computed tomography angiography (CTA). Topics discussed include the clinical presentation of PTA, the embryonic development failure leading to this condition, and the importance of early diagnosis and surgical intervention to improve patient outcomes.

The ventricular papillary muscles (VPMs) can be a source of premature ventricular contractions (PVCs). Catheter ablation of VPM PVCs is challenging because of the anatomical complexity, such as the apical structures in proximity to the ventricular walls. The QDOT MICRO catheter (Biosense Webster, Diamond Bar, CA, USA) has microelectrodes embedded along the circumference of its distal tip and can provide information on which side of its tip myocardial activation is earlier. This repaired truncus arteriosus case demonstrates the usefulness of the microelectrode recording in identifying a PVC origin in a right VPM apex close to the right ventricular anterior wall. [ABSTRACT FROM AUTHOR]

Background: Proper development of the great vessels of the heart and septation of the cardiac outflow tract requires cardiac neural crest cells. These cells give rise to the parasympathetic cardiac ganglia, the smooth muscle layer of the great vessels, some cardiomyocytes, and the conotruncal cushions and aorticopulmonary septum of the outflow tract. Ablation of cardiac neural crest cells results in defective patterning of each of these structures. Previous studies have shown that targeted deletion of the forkhead transcription factor C2 (Foxc2), results in cardiac phenotypes similar to that derived from cardiac neural crest cell ablation. Results: We report that Foxc2‐/‐ embryos on the 129s6/SvEv inbred genetic background display persistent truncus arteriosus and hypoplastic ventricles before embryonic lethality. Foxc2 loss‐of‐function resulted in perturbed cardiac neural crest cell migration and their reduced contribution to the outflow tract as evidenced by lineage tracing analyses together with perturbed expression of the neural crest cell markers Sox10 and Crabp1. Foxc2 loss‐of‐function also resulted in alterations in PlexinD1, Twist1, PECAM1, and Hand1/2 expression in association with vascular and ventricular defects. Conclusions: Our data indicate Foxc2 is required for proper migration of cardiac neural crest cells, septation of the outflow tract, and development of the ventricles. Developmental Dynamics 247:1286–1296, 2018. © 2018 Wiley Periodicals, Inc. Key Findings: Foxc2 is expressed in migrating neural crest cells and cardiac mesenchyme.On the 129s6/SvEv genetic background, Foxc2 loss‐of‐function embryos exhibit persistent truncus arteriosus and ventricle hypoplasia, leading to embryonic lethality at E13.5.Aberrant cardiac neural crest migration in association with disruption in guidance cues precedes reduced contribution to the outflow tract.Ventricle patterning and development is impaired in the absence of Foxc2. [ABSTRACT FROM AUTHOR]

Complete atrioventricular septal defect is generally diagnosed on the four-chamber view of the fetal heart. This defect can be accompanied by additional outflow tract lesions, including truncus arteriosus. Although truncus arteriosus in isolation can be associated with interrupted aortic arch, we describe a fetal case with the rare association of all three diagnoses: complete atrioventricular septal defect with truncus arteriosus and interrupted aortic arch. This case points to the importance of evaluating the complete fetal heart even after one congenital cardiac abnormality is noted. The complexity of heart disease has implications for prenatal counseling and postnatal management. [ABSTRACT FROM AUTHOR]

Background: Retinoic acid (RA), the bioactive derivative of vitamin A, is essential for vertebrate heart development. Both excess and reduced RA signaling lead to cardiovascular malformations affecting the outflow tract (OFT). To address the cellular mechanisms underlying the effects of RA signaling during OFT morphogenesis, we used transient maternal RA supplementation to rescue the early lethality resulting from inactivation of the murine retinaldehyde dehydrogenase 2 ( Raldh2) gene. Results: By embryonic day 13.5, all rescued Raldh2−/− hearts exhibit severe, reproducible OFT septation defects, although wild-type and Raldh2+/− littermates have normal hearts. Cardiac neural crest cells (cNCC) were present in OFT cushions of Raldh2−/− mutant embryos but ectopically located in the periphery of the endocardial cushions, rather than immediately underlying the endocardium. Excess mesenchyme was generated by Raldh2−/− mutant endocardium, which displaced cNCC derivatives from their subendocardial, medial position. Conclusions: RA signaling affects not only cNCC numbers but also their position relative to endocardial mesenchyme during the septation process. Our study shows that inappropriate coordination between the different cell types of the OFT perturbs its morphogenesis and leads to a severe congenital heart defect, persistent truncus arteriosus. Developmental Dynamics 245:388-401, 2016. © 2015 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Objective: To describe antenatal sonographic signs that help in the differentiation of truncus arteriosus Types II and III (TA-II/III) from pulmonary atresia with ventricular septal defect (PA-VSD).Methods: From a database of fetal echocardiographic examinations, we identified fetuses with sonographic features of a single great artery with VSD and relatively normal four-chamber view. Records were reviewed, comparing fetuses with TA-II/III and those with PA-VSD, with particular focus on: 1) characteristics of the overriding vessel, 2) appearance of the semilunar valves, 3) competence of the semilunar valves, 4) presence of major aortopulmonary collateral arteries (MAPCA), 5) main pulmonary artery being without antegrade flow, 6) site of arterial branching from the great artery and 7) other minor features, such as cardiac axis or associated anomalies.Results: Seventeen fetuses were identified, eight with TA-II/III and nine with PA-VSD. Among the eight fetuses with TA-II/III, seven had abnormal valves and six had valve regurgitation, compared with none of the nine PA-VSD fetuses. Five TA-II/III fetuses had early branching to supply the lungs, whereas most fetuses with PA-VSD had more distal branching. Notably, in six of the TA-II/III fetuses, the root of the single great artery originated predominantly from the right ventricle, while all but one of the PA-VSD fetuses had typical equal overriding of the VSD. The main pulmonary artery was without antegrade flow in two cases with PA-VSD. Finally, four cases with PA-VSD had MAPCA, in two of which this was identified prenatally.Conclusion: Identification of abnormal arterial valves or valve regurgitation, site of origin of branching, presence of overriding of the great artery, a main pulmonary artery without antegrade flow and MAPCA are helpful in differentiating between TA-II/III and PA-VSD. [ABSTRACT FROM AUTHOR]

The coincident occurrence of tricuspid atresia and aortopulmonary window (APW) is exceedingly rare, with one previous case reported in the literature. We present a patient with tricuspid atresia, pulmonary atresia, and APW. Postnatal echocardiograms demonstrated no visible pulmonary valve, and additional defects including a bicuspid aortic valve, right aortic arch and anomalous coronary arteries raised suspicion for tricuspid atresia with persistent truncus arteriosus. However, fetal echocardiography and direct visualization of the anatomy confirmed the alternate diagnosis. The patient underwent successful palliation consisting of APW repair, atrial septectomy and a 3.5 mm modified Blalock-Taussig shunt, followed by a bidirectional cavopulmonary connection. [ABSTRACT FROM AUTHOR]

Truncus arteriosus (TA) was diagnosed in a 6-year-old neutered female domestic short-haired cat by two-dimensional and M-mode echocardiography, colour flow imaging and spectral Doppler examinations. The lesion was characterized by a single large artery originating from the right ventricle. A single ascending aorta and a single pulmonary trunk arose from the common arterial trunk. The residual pulmonary trunk immediately split into left and right branches. The lesion was identified as a type I (TA). This case is of interest because it is the first reported echo-Doppler description of such a malformation in felines, and because of the age of the cat at the time of diagnosis. [ABSTRACT FROM AUTHOR]

A Simmentaler × Braunvieh calf had been anorexic during its first 36 hours of life, had a loud heart murmur and was suspected to have pneumonia. In the light of the results of radiographic and echocardiographic examinations, right heart catheterization and angiography, a diagnosis of persistent truncus arteriosus and patent foramen ovale was made. The diagnosis was confirmed on postmortem examination. [ABSTRACT FROM AUTHOR]

Truncus arteriosus with anterior origin of a main pulmonary artery is a very rare condition. In this report we present a newborn who has a truncus arteriosus with anterior origin of a main pulmonary artery. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 27-year-old man who underwent catheter ablation (CA) due to ventricular tachycardia (VT). His medical history includes truncus arteriosus with a surgical correction, aortic valve replacement with a mechanical valve and a new conduit. Among other things, the patient has a congenital heart disease and it outlines the challenges of treating the VT due to varied arrhythmogenic substrates and anatomies.

The article presents a case study of a pediatric phantom tumor in a 10-month-old female infant caused by invasive cardiac catheterization. The patient experienced pleural effusion in an interlobar fissure diagnosed by echocardiography. It mentions that phantom tumors or vanishing tumors have observed in the right side of her chest.

Background: The heart develops under reduced and varying oxygen concentrations, yet there is little understanding of oxygen metabolism in the normal and mal-development of the heart. Here we used a novel reagent, the ODD-Luc hypoxia reporter mouse (oxygen degradation domain, ODD) of Hif-1α fused to Luciferase (Luc), to assay the activity of the oxygen sensor, prolyl hydroxylase, and oxygen reserve, in the developing heart. We tested the role of hypoxia-dependent responses in heart development by targeted inactivation of Hif-1α., Methods and Results: ODD-Luciferase activity was 14-fold higher in mouse embryonic day 10.5 (E10.5) versus adult heart and liver tissue lysates. ODD-Luc activity decreased in 2 stages, the first corresponding with the formation of a functional cardiovascular system for oxygen delivery at E15.5, and the second after birth consistent with complete oxygenation of the blood and tissues. Reduction of maternal inspired oxygen to 8% for 4 hours caused minimal induction of luciferase activity in the maternal tissues but robust induction in the embryonic tissues in proportion to the basal activity, indicating a lack of oxygen reserve, and corresponding induction of a hypoxia-dependent gene program. Bioluminescent imaging of intact embryos demonstrated highest activity in the outflow portion of the E13.5 heart. Hif-1α inactivation or prolonged hypoxia caused outflow and septation defects only when targeted to this specific developmental window., Conclusions: Low oxygen concentrations and lack of oxygen reserve during a critical phase of heart organogenesis may provide a basis for vulnerability to the development of common septation and conotruncal heart defects., (© 2014 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)