Which congential heart defect is described as the incomplete fusion of the endocardial cushions?
Last updated: May 4, 2022 Show
SummaryAcyanotic heart defects are congenital cardiac malformations that affect the atrial or ventricular walls, heart valves, or large blood vessels. Common causes include genetic defects (e.g., trisomies), maternal infections (e.g., rubella), or maternal use of drugs or alcohol during pregnancy. Acyanotic heart defects are pathophysiologically characterized by a left-to-right shunt, which causes pulmonary hypertension and right heart hypertrophy. The symptoms depend on the extent of the malformation and the resulting impairment of cardiac function. Infants may be asymptomatic or present with exercise intolerance, failure to thrive, and symptoms of heart failure. Characteristic heart murmurs are important clues for establishing the diagnosis, which is typically confirmed by visualizing the defect on echocardiography. Chest x-ray, MRI, or cardiac catheterization may also be required to determine indications for surgery and plan the procedure. Acyanotic heart defects requiring treatment are repaired via catheter procedures or surgery. Supportive medical therapy is required in cases of heart failure (e.g., diuretics, inotropic agents) or if surgery cannot be performed (e.g., prostaglandin). Common complications include arrhythmias, embolisms, and infective endocarditis, especially if treatment is delayed. OverviewCommonly associated conditions and risk factorsGeneral pathophysiological processes [1]
Pulmonary arterial hypertension (Eisenmenger syndrome) may cause untreated left-to-right shunts (acyanotic heart defects) to progress to right-to-left shunts (cyanotic defects) if right ventricular pressure exceeds left ventricular pressure. Left-to-Right shunts = LateR cyanosis. Right-to-Left shunts = eaRLy cyanosis. General clinical featuresFor specific features, see “Clinical features” in the subsections below.
General treatment considerations
The “3 Ds” of acyanotic CHDs (in order of frequency): VSD, ASD, PDA Overview of ASD and VSD
Ventricular septal defect (VSD)Epidemiology
Etiology
Pathophysiology
Clinical features [7]
Symptoms of heart failure in children with VSD only develop when PVR decreases to adult levels and thus allows left-to-right shunting to occur. Treatment [5][7]
Complications
Atrial septal defect (ASD)Etiology
Pathophysiology
Clinical features
Complications [11]
Patent foramen ovale (PFO)Patent ductus arteriosus (PDA)Etiology
Pathophysiology
Clinical features
PDA comes with Prolonged Deafening Auscultation findings. Treatment [15]
Complications [14]
Coarctation of the aortaEtiology [17]
Pathophysiology [17]
Clinical features [17]
Diagnostics [17]
Complications
Endocardial cushion defectEtiology
Pulmonary valve stenosisReferences
Which congential heart defect is described as the incomplete fusion of the endocardial cushions?This is called an ostium primum defect. The failure of the endocardial cushions to fuse results in an abnormally low position of the AV valves and an abnormally high position of the aortic valve.
What is endocardial cushion defect called?Endocardial Cushion Defect (also called atrioventricular ( AV ) canal or septal defects)
What forms the endocardial cushion?The endocardial cushions are thought to arise from a subset of endothelial cells that undergo epithelial-mesenchymal transition, a process whereby these cells break cell-to-cell contacts and migrate into the cardiac jelly (towards the interior of the heart tube).
What is Tetralogy of Fallots?Tetralogy of Fallot (pronounced te-tral-uh-jee of Fal-oh) is a birth defect that affects normal blood flow through the heart. It happens when a baby's heart does not form correctly as the baby grows and develops in the mother's womb during pregnancy.
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