Embryology heart cardiac

This report presents the case of an 82 year old male with known dextrocardia and situs inversus totalis who developed increasing dyspnea on exertion and was diagnosed with severe aortic stenosis. Transcatheter aortic valve replacement was decided upon requiring deviation from standard techniques for patients with normal anatomy and left-sided aortic arch. We describe two technical differences required for patients with dextrocardia and right-sided aortic arch which facilitate transcatheter aortic valve replacement in this patient group...

Myocardial bridging is a congenital coronary artery anomaly in which the coronary artery has a partly "tunnelled" intramyocardial course. This tunnelling leads to compression of the affected vessel segment during ventricular systole. It is considered to be a benign variation of the norm in about 25% of the population caused by an aberrancy of embryologic coronary development. The bridging is also thought to cause severe cardiac conditions in a few of those affected. The series of six young patients presented here is the largest series so far to report on symptomatic myocardial bridging in children with different underlying heart diseases...

BACKGROUND: Coronary vasculature irrigates the myocardium and is crucial to late embryonic and adult heart function. Despite the developmental significance and clinical relevance of these blood vessels, the embryonic origin and the cellular and molecular mechanisms that regulate coronary arterio-venous patterning are not known in detail. In this study, we have used the avian embryo to dissect the ontogenetic origin and morphogenesis of coronary vasculature. RESULTS: We show that sinus venosus endocardial sprouts and proepicardial angioblasts pioneer coronary vascular formation, invading the developing heart simultaneously...

Direct insertion of the anterior papillary muscle (DPM) into the mitral valve is uncommon. During the routine dissection of an adult female, a DPM into the mitral valve with abnormal chordae tendinae was observed. This abnormal papillary muscle was measured as 28.0 mm in length from myocardial to insertion, 14.8 mm in width, and 7.0 mm in depth. The embryology, symptoms, associated cardiac diseases, and surgical precautions of this congenital malformation in the heart are reviewed.

The pulmonary veins carry oxygenated blood from the lungs to the heart, but their importance to the radiologist extends far beyond this seemingly straightforward function. The anatomy of the pulmonary veins is variable among patients, with several noteworthy variant and anomalous patterns, including supernumerary pulmonary veins, a common ostium, anomalous pulmonary venous return, and levoatriocardinal veins. Differences in pulmonary vein anatomy and the presence of variant or anomalous anatomy can be of critical importance, especially for preoperative planning of pulmonary and cardiac surgery...

Right ventricular failure predicts adverse outcome in patients with pulmonary hypertension (PH), and in subjects with left ventricular heart failure and is associated with interstitial fibrosis. This review manuscript discusses the cellular effectors and molecular mechanisms implicated in right ventricular fibrosis. The right ventricular interstitium contains vascular cells, fibroblasts, and immune cells, enmeshed in a collagen-based matrix. Right ventricular pressure overload in PH is associated with the expansion of the fibroblast population, myofibroblast activation, and secretion of extracellular matrix proteins...

The ratites are a distinctive clade of flightless birds, typified by the emu and ostrich that have acquired a range of unique anatomical characteristics since diverging from basal Aves at least 100 million years ago. The emu possesses a vestigial wing with a single digit and greatly reduced forelimb musculature. However, the embryological basis of wing reduction and other anatomical changes associated with loss of flight are unclear. Here we report a previously unknown co-option of the cardiac transcription factor Nkx2...

This chapter will discuss the role of cardiac fibroblasts as a target of various immunological inputs as well as an immunomodulatory hub of the heart through interaction with immune cell types and chemokine or cytokine signaling. While the purpose of this chapter is to explore the immunomodulatory properties of cardiac fibroblasts, it is important to note that cardiac fibroblasts are not a homogeneous cell type, but have a unique embryological origin and molecular identity. Specific properties of cardiac fibroblasts may influence the way they interact with the heart microenvironment to promote healthy homeostatic function or respond to pathological insults...

Atrioventricular septal defect results from a failure of normal endocardial cushion fusion during embryologic cardiac development. This developmental aberration results in defects in the atrial and/or ventricular septum and malformation of the atrioventricular valves. The pathophysiology of atrioventricular septal defect is variable, and ranges from mild left to right shunting similar to a simple atrial septal defect to complex single-ventricle heart disease. This review focuses on the spectrum of atrioventricular septal defect from partial to complete, without associated cardiac defects...

Clinical data and basic research indicate that the structural and functional alterations that characterize the evolution of cardiac disease towards heart failure may be, at least in part, reversed. This paradigm shift is due to the accumulation of evidence indicating that, in peculiar settings, cardiomyocytes may be replenished. Moving from the consideration that cardiomyocytes are rapidly withdrawn from the cell cycle after birth, independent laboratories have tested the hypothesis that cardiac resident stem/progenitor cells resided in mammalian hearts and were important for myocardial repair...

Ebstein anomaly is a rare form of congenital heart disease with a uniquely high prevalence of arrhythmias. The most prevalent arrhythmia mechanisms are intrinsic to the underlying embryologic defects and may manifest at any stage. Current electrophysiological and surgical strategies are well equipped to address these arrhythmia mechanisms, yet despite available technology and a robust understanding of the mechanisms, these cases remain challenging. Surgical techniques that render arrhythmia substrates unreachable mandate comprehensive presurgical electrophysiological assessment and potential ablation...

Despite advances in cardiovascular biology and medical therapy, heart disorders are the leading cause of death worldwide. Cell-based regenerative therapies become a promising treatment for patients affected by heart failure, but also underline the need for reproducible results in preclinical and clinical studies for safety and efficacy. Enthusiasm has been tempered by poor engraftment, survival and differentiation of the injected adult stem cells. The crucial challenge is identification and selection of the most suitable stem cell type for cardiac regenerative medicine...

Cardiac lipomas are rare and usually present as benign, encapsulated masses outside the heart; however, they can also be found within the atria. No single theory-including molecular genetic mutation-adequately explains why this occurs. Extensive career experience and broadened knowledge in embryology and cardiac physiology have helped us to develop a hypothesis based on invagination of extracardiac tumors. This report describes a vexing case of a giant right atrial lipoma, from 1985, in which the diagnosis was made incidentally during management of a patient's acute limb ischemia...

The life-long story of the heart starts concomitantly with primary differentiation events occurring in multipotent progenitors located in the so-called heart tube. This initially tubular structure starts a looping process, which leads to formation of the final four-chambered heart with a primary contribution of geometric and position-associated cell sensing. While this establishes the correct patterning of the final cardiac structure, it also provides feedbacks to fundamental cellular machineries controlling proliferation and differentiation, thus ensuring a coordinated restriction of cell growth and a myocyte terminal differentiation...

It is customary, at the current time, to consider many, if not most, of the lesions involving the ventricular outflow tract in terms of conotruncal malformations. This reflects the introduction, in the early 1940s, of the terms conus and truncus to describe the components of the developing outflow tract. The definitive outflow tracts in the postnatal heart, however, possess three, rather than two, components. These are the intrapericardial arterial trunks, the arterial roots, and the subvalvar ventricular outflow tracts...

The Chiari network is an embryological remnant found in the right atrium, mostly without any significant pathophysiological consequences. However, several cardiac associations are reported in the literature including supraventricular tachyarrhythmias. We present a case of a 96-year-old body donor with a stroke episode and intermittent atrial fibrillations. The dissection of the heart revealed the presence of an immense Chiari network with a large central thrombus. The role of a Chiari network in the pathogenesis of stroke and pulmonary embolism is discussed...

Congenital heart disease is diagnosed in 0.4% to 5% of live births and presents unique challenges to the pediatric anesthesiologist. Furthermore, advances in surgical management have led to improved survival of those patients, and many adult anesthesiologists now frequently take care of adolescents and adults who have previously undergone surgery to correct or palliate congenital heart lesions. Knowledge of abnormal heart development on the molecular and genetic level extends and improves the anesthesiologist's understanding of congenital heart disease...

A wide range of ion channels, transporters, signaling pathways and tissue structure at a microscopic and macroscopic scale regulate the electrophysiological activity of the heart. Each region of the heart has optimised these properties based on its specific role during the cardiac cycle, leading to well-established differences in electrophysiology, Ca(2+) handling and tissue structure between atria and ventricles and between different layers of the ventricular wall. Similarly, the right ventricle (RV) and left ventricle (LV) have different embryological, structural, metabolic and electrophysiological features, but whether interventricular differences promote differential remodeling leading to arrhythmias is not well understood...

Congenital cardiovascular malformations are the most common birth defects, with a complex multifactorial etiology. Genetic factors play an important role, illuminated by numerous cytogenetically visible abnormalities, as well as submicroscopic genomic imbalances affecting critical genomic regions in the affected individuals. Study of rare families with Mendelian forms, as well as emerging next-generation sequencing technologies have uncovered a multitude of genes relevant for human congenital cardiac diseases...

Hypertrophic cardiomyopathy (HCM) is caused by mutations in sarcomeric proteins, the commonest being MYBPC3 encoding myosin-binding protein C. It is characterised by left ventricular hypertrophy but there is an important pre-hypertrophic phenotype with features including crypts, abnormal mitral leaflets and trabeculae. We investigated these during mouse cardiac development using high-resolution episcopic microscopy. In embryonic hearts from wildtype, homozygous (HO) and heterozygous (HET) Mybpc3-targeted knock-out (KO) mice we show that crypts (one or two) are a normal part of wildtype development but they almost all resolve by birth...