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4000500-1905000019050036195000Congenital Heart Disease 258127515875000Acyanotic Congenital Heart DiseasesTypesAtrial Septal DefectVentricular Septal DefectPatent Ductus ArteriosusDescriptions Ostium Primum (15%)Due to the failure of fusion between Endocardial cushion Septum primum Often associated with other anomalies in structures derived from endocardial cushionTricuspid valves Bicuspid/mitral valves Ostium Seccundum (75%)Failure of Septum Seccundum to grow sufficiently to cover the Ostium Seccundum Sinus Venosus (10%)Frameshift mutations in the NKX2.5 transcription factor Failure of closure of sinus venosus into sinus venorum The most common anomaly of the heart present at birth Due to the incomplete closure of ventricular septum leading to Left-Right shunt Often involved the membranous region This is the last part where endocardial cushion and interventricular muscle ridge fuse together to form the septum Persistent opening of the ductus arteriosus after 3 months of life Ductus arteriosus should be obliterated to form ligamentum arteriosum within 3 months of life due to ↑arterial oxygenation ↓pulmonary vascular resistance ↓local production of PGE2 Ductus arteriosus connects between the arch or aorta (distal origin of left subclavian artery branch) with the pulmonary trunks Morphological Abnormalities Located at the lowest part of atrial septum may extend to endocardial cushion derivatives Tricuspid valves Bicuspid/mitral valves AV valves abnormalities always present Bicuspid/mitral valves Cleft in the anterior leaflet Tricuspid valves Cleft in the septal leaflet Severe from of Ostium Primum ASD When accompanied with Ventricular wall defect Bicuspid and tricuspid valves deformities Resulting in common AV canal Located at the middle part of atrial septum Smooth walled defect of the foramen of ovale Due to left to right shunt, it presents as Right ventricular Dialtation Hypertrophy Right atrial dilatation Pulmonary artery dilatation Indicates massive blood overload in the right side chambersAccompanied by anomalous drainage pulmonary veins into either Right atrium Superior Vena Cava Size and location of VSD varies Size Minute lesion Large lesion virually involved the whole septum Location Membranous portion of septum Muscular portion of septum Right ventricular HypertrophyDilated Dilated pulmonary artery due to volume overload Vascular damages due to pulmonary hypertension Connects between arch of aorta (distal origin of left subclavian artery) to the pulmonary trunk Due to the PDA, oxygenated blood from aorta shunted into the pulmonary trunk and back to the lung sRight heart hypertrophy and dilatation may occur due to ↑pulmonary hypertension Arterosclerosis of the pulmonary trunk Proliferative changes of the distal portion of pulmonary turnkClinical Manifestations The most commonly diagnosed heart defect later in life (adulthood)Generally it is well tolerated Assymtomatic when lesion <1cm Gradually develops symptoms when lesion is large Overtime ↑pulmonary vascular resistance ↑pulmonary hypertension Complications if it is not treated Ostium Primum ASD Valves stenosis Congestive heart failure due to Eisenmenger syndrome Ostium Seccundum ASDPulmonary Hypertension Massive increased in the pressure inside right chambers lead to Eisenmenger syndrome Cynosis is a late complication General complicationsHeart failure Paradoxical embolism Irreversible pulmonary vascular diseases Small lesion often assymptomatic Spontaneous closure may happen in muscular portion of septum during Infanthood Childhood Larger defect often leads to Progressive pulmonary hypertension Eisenmenger syndrome due to massive increase in right side chambers Endocarditis secondary to the damage of endocardium Congestive heart failure Machinery cardiac murmurs Due to the high pressure of left to right shunt Massive overloading blood in the right chambers will lead to right to left shunt (Eisenmenger syndrome)Cyanosis is a late manifestation Infective endocarditis can occur secondary to destruction of endocardium *PDA’s can be stimulated to close by administering Indomethacin (Selective COXII inhibitor)Cyanotic Congenital Heart DiseasesTypesTetralogy of FallotTransposition of the Great ArteriesDescriptionsThe most common cause of cyanotic congenital heart disease Four fundamental feature of Tetralogy of Fallot Overriding of aorta Right ventricular hypertrophy Pulmonary valve stenosis Ventricular septal defect Discordant (abnormal) connection between ventricle and its vascular outflow Aorta arised from right ventricle Pulmonary trunk arised from left ventricle Concordant (normal) connection between atrium and ventricle Right atrium empties into right ventricle Left atrium empties into left ventricle Leads to separation between systemic and pulmonary circulations which are incompatible with life, unless presents with alternative pathway TGA with VSD Shunting is more stable TGA with PDA or ASD Shunting is less stable Needs immediate surgical intervention during the first few days of life Morphological AbnormalitiesRight ventricular hypertrophy, heart appeared Large Boot shapedThe right ventricle is marked thickened even greater than that of left ventricle Left sided heart chambers are normal Location VSD located at the membranous portion of vetricular septum Aortic valve lies just above the VSD Pulmonary valve is stenotic In some cases, beneficial defects come along, permitting pulmonary blood flow ASD PDA Discordant (abnormal) connection between ventricle and its vascular outflow Aorta arised from right ventricle Pulmonary trunk arised from left ventricle TGA can be associated with VSDPDA ASD Right ventricle becomes hypertrophic as it deals higher pressure Left ventricle becomes somewhat atrophic as it deals lower pressure Clinical ManifestationsRight to left shunting Right ventricular hypertrophy Pulmonary valve stenosis ↓pulmonary blood flow The extent of stenosis determines the severity of the shuntingIf mild stenosis Resembles isolated VSD; left to right shunt instead of right to left shunt Acynotic in the early stage of life If severe stenosis Rigth to left shunting Cyanotic is the early life manifestation As tetralogic patient grows Defective orifices remain patent (not growing)Heart enlarges dispropotinately with the defective orifices Degree ot stenosis becomes severe and cyanosis becomes even more prominent Lung is protected from any heamodynamic changes as pulmonary valves are stenotic Complications Secondary to cyanotic condition, erythrocytosis developes ↑viscosity of the blood Hyperthropic osteoarthropathyInfective endocarditis Systemic embolism Brain abscesses Early cyanosis Clinical manifestation depends on Degree of shunting Magnitude of tissue hypoxia Ability of right ventricle to cope with higher pressure Clinical intervention to increase oxygen saturation includes Admin of PGE2 to maintain PDA Septostomy to form ASD Obstructive Congenital Heart DiseaseTypesCoarctation of AortaValves Stenosis and AtresiaAortic ValvesPulmonary ValvesDescriptionsObstruction and narrowing of the aorta distal to the origin of Subclavian artery The most important form of obstructive congenital heart disease Epidemiology Male twice more susceptible than female Female with Turner syndrome often presented with CoA Categorized into Infantile/Preductile CoA with PDA Adult/Postductile CoA whitout PDACan be peresented either Solitary CoA CoA with Bicuspid/aortic valves stenosis ASDVSD Bicuspid regurgitation Berry aneurysm in the circle of Willis Narrowing of the aortic valves Happens since birth Obstruction of the pulmonary valves Relatively frequent Morphological AbnormalitiesInfantile/Preductile CoA (less common)Tubular narrowing of aorta segment between PDA Subclavian artery PDA is the main source of blood supply to the distal portion of arch of aorta Right ventricular hypertrophy and dilated As the rightventricle needs to supply thhe blood to the systemic circulation Pulmonary trunk dilatationAdult/Postductile CoA (more common)Aorta is sharply constricted Distal to that of ligamentum arteriosum Constricted area composed of Tunica media composed ofSmooth muscle cells Elastic fibers Tunica intima is thickened Proximal to the constricted part, arteries are markedly dilated Left ventricle is hypertrophy Left ventricular hypertrophy If coexisted with PDA, perfusion is possible and patient might survive If ductus arteriosus is closed, it is not compatible with life Right ventricular hypertrophy Severity depends with the degree of stenosis Severe – cyanosis Mild – asymptomatic Clinical ManifestationsClincial manifestation solely depends on Degree of constriction Patency of ductus arteriosus Infantile/Preductile CoAEarly manifestations Cyanosis of the lower half of the body Femoral pulse always weaker than that of upper extremities Adult/Postductile CoAAsymptomatic during the early years of life Upper extremities hypertension Due to poor renal perfusion Ischemia of the lower extremities Coldness and claudication of lower extremities Hypotension Weak pulse Enlargement of intercostal and mammary arteries Presented as NOTCHING of the ribs upon X-ray Complications Thromboembolism Bacterial endocarditis Heart failure ................
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