Congenital Anomalies of the heart DR RANIA GABR
objectives • Discuss the congenital anomalies related to the heart development.
Congenital Heart Defect • AcyanoticCyanotic volume load pressure load ↑ pulmonary flow ↓ pulmonary flow left-to-right shunts obstr. ventric. outflow • atrial septal defect • pulmonary valve stenosis transpos. of gr. Vessels tetralogy of Fallot • ventricular septaldefect aortic valve stenosis single ventricle pulmonary atresia • AV canal coarctation of aortatruncusarteriosustricuspid atresia • patent ductusarteriosustotal anomalous pulm. total anomalous pulm. return w/o obstructionreturn w/ obstruction
Atrial Septal defects: • Septum PrimumDefect: Patent foramen ovale Defect in the interatrial septum Due to absence of the septum Primum (Patent foramen ovale)
Septum Secondum Defect: Defect in the interatrial septum Due to absence of the septum Secondum(Patent foramen Secondum)
Complete absence of the interatrial septum (Core TriloculareBiventriculare): There is complete absence of both septum primum and septum secondum. i.e. there is a common atrium.
Patent osteumprimum: • The foramen primum may fail to close inspite of formation of the foramen secondum . • This will cause disturbance in the valvular mechanism of the interatrial septum.
Premature closure of foramen ovale: Rarely , it is closed BEFORE birth. It results in marked enlargement of the Right atrium and ventricle.
Persistent atrioventricular canal: • The A-V cushions and the septum intermedium fail to develop. • The A-V canal remains divided into rt and lt parts. • There is usually: 1- Patent foramen primum 2- IV septaldefect 3- Abnormalities in the leaflets of the valves guarding the A-V canal.
Ventricular Septaldefects: Usually in the membranous part
Tetralogy of Fallot: Four co-occurring heart defects: • Pulmonary stenosis • Ventricular septal defect • Overriding aorta (dextroposition) • Right ventricular hypertrophy
Eisenmenger'ssyndrome: Characteristics of Eisenmenger's syndrome summarized as: • persistent truncusarteriosus • ventricular septal defect • left-right ventricular shunt • right ventricle hypertrophy
Congenital Aortic valve stenosis: • Occurs due to fusion of the cusps of the aortic valve . • Very narrow aortic orifice will cause Lt ventricular hypertrophy. • Regurge of blood from the aorta to the left ventricle will increase the ltventr. Hypertrophy.
Congenital Aortic valve atresia: • Under developed lt ventricle • Narrow ascending aorta • Patent ductusarteriosus to carry the blood into the aorta
Pulmonary atresia • The pulmonary artery is underdeveloped, the right ventricle very small, and also sometimes the tricuspid valve. The condition is also sometimes referred to as hypoplastic right heart.
Transposition of the great arteries: • Most common cyanotic neonatal heart defect • Failure of aorticopulmonary septum to take a spiraling course • Fatal without PDA, ASD, & VSD
A univentricular heart may be best described as a defect that encompasses two complete atria that provide venous inflow into a dominant ventricle (either right or left) via a malformed AV connection. • Single ventricle:
EctopiaCordis: • The heart is bulging outside the chest due to wide separation in the 2 parts of the sternum
Dextrocardia: • The heart and great vessels are reversed as in mirror image • It might be a separate condition or part of Situsinversus