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Molecular Pathways and Animal Models of d-Transposition of the Great Arteries
During normal cardiovascular development, the outflow tract becomes septated and rotates so that the separate aorta and pulmonary trunk are correctly...
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Human Genetics of Tricuspid Atresia and Univentricular Heart
Tricuspid atresia (TA) is a rare congenital heart condition that presents with a complete absence of the right atrioventricular valve. Because of the...
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Human Genetics of Tetralogy of Fallot and Double-Outlet Right Ventricle
Tetralogy of Fallot (TOF) and double-outlet right ventricle (DORV) are conotruncal defects resulting from disturbances of the second heart field and...
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Human Genetics of Ebstein Anomaly
Ebstein anomaly (EA) is a rare, congenital cardiac defect of the tricuspid valve with a birth prevalence between 0.5 and 1 in 20,000 [1]. It is...
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Molecular Pathways and Animal Models of Ebstein’s Anomaly
Ebstein’s anomaly is a congenital malformation of the tricuspid valve characterized by abnormal attachment of the valve leaflets, resulting in...
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Neural Crest
This chapter discusses the role of cardiac neural crest cells in the formation of the septum that divides the cardiac arterial pole into separate...
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Molecular Pathways and Animal Models of Tricuspid Atresia and Univentricular Heart
The process of valve formation is a complex process that involves intricate interplay between various pathways at precise times. Although we have not...
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Cardiac Metabolism
The heart is composed of a heterogeneous mixture of cellular components perfectly intermingled and able to integrate common environmental signals to...
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Human Genetics of d-Transposition of Great Arteries
Although several genes underlying occurrence of transposition of the great arteries have been found in the mouse, human genetics of the most frequent...
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Congenital Coronary Blood Vessel Anomalies: Animal Models and the Integration of Developmental Mechanisms
Coronary blood vessels are in charge of sustaining cardiac homeostasis. It is thus logical that coronary congenital anomalies (CCA) directly or...
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Clinical Presentation and Therapy of Total Anomalous Pulmonary Venous Return
Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with...
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Molecular Pathways and Animal Models of Arrhythmias
Arrhythmias account for over 300,000 annual deaths in the United States, and approximately half of all deaths are associated with heart disease....
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Human Genetics of Truncus Arteriosus
Integrated human genetics and molecular/developmental biology studies have revealed that truncus arteriosus is highly associated with 22q11.2...
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Molecular Pathways and Animal Models of Cardiomyopathies
Cardiomyopathies are a heterogeneous group of disorders of the heart muscle that ultimately result in congestive heart failure. Rapid progress in...
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Human Genetics of Cardiomyopathies
The identification of a disease-causing variant in a patient diagnosed with cardiomyopathy allows for presymptomatic testing in at risk relatives....
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Clinical Presentation and Therapy of Atrioventricular Septal Defect
Atrioventricular septal defects (AVSDs) consist of a number of cardiac malformations that result from abnormal development of the endocardial...
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Molecular Pathways and Animal Models of Semilunar Valve and Aortic Arch Anomalies
The great arteries of the vertebrate carry blood from the heart to the systemic circulation and are derived from the pharyngeal arch arteries. In...
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Inflow Tract Development
The development of the inflow tract is undoubtedly one of the most complex remodeling events in the formation of the four-chambered heart. It...
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Hemodynamics During Development and Postnatal Life
A well-developed heart is essential for embryonic survival. There are constant interactions between cardiac tissue motion and blood flow, which...
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Molecular Pathways and Animal Models of Tetralogy of Fallot and Double Outlet Right Ventricle
Tetralogy of Fallot and double-outlet right ventricle are outflow tract (OFT) alignment defects situated on a continuous disease spectrum. A myriad...