Cyanotic Congenital Heart Disease
Occurs when blood is shunted from the venous to the arterial system as a result of abnormal communication bet. the two systems (deoxy to oxy. R to L shunts) |
Decreased pulmonary blood flow (Right to left shunt) |
Tetralogy of Fallot (Cyanotic)
Localized narrowing of the aorta, the most common cardiac malformation responsible for cyanosis in a child over 1 year. 4 major defects: |
Pulmonary stenosis |
Ventricular septal defect |
Aorta overriding VSD |
Right ventricular hypertrophy |
Pathophysiology |
•PS impedes the flow of blood to lungs |
•increases pressure in the right ventricle (hypertrophy) |
•Forces deoxy blood through the septal defect to the left ventricle |
•Overriding aorta receives blood from both right and left ventricles. |
Signs & Symptoms |
•Clubbing of the fingers, murmur |
•Severe dyspnea, hypercyanotic spells & acidosis |
•Polycythemia & clot formation |
•Failure to thrive and growth retardation. |
Treatments |
•If O2 level ex low, prostaglandins (IV) to keep PDA open. |
•Complete repair when 6 mos. of age. |
•Closure of the VSD with dacron patch |
•The narrowed pulmonary valve is enlarged |
•Coronary arteries will be repaired |
•Hypertrophy of right heart should remodel when pressure in right side is reduced. |
Acute Rheumatic Fever (RF)
Streptococcal infections causes damage to the heart muscle and valves. All heart layers are affected. |
•Pancarditis (all layers) |
•Pericarditis |
•Myocarditis |
•Endocarditis |
Pathogenesis |
•An acute attack of streptococcal pharyngitis by group A beta-hemolytic streptococci |
•Within 2-4 weeks after this attack anti-streptococcal antibodies are formed and attack the heart and the extra cardiac sites. |
Diagnostic test |
•Throat swab, blood test, ECG heart test |
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Tricuspid Atresia (Cyanotic)
Tricuspid valve fails to develop causing complete closure of the tricuspid valve that results in mixed blood flow. |
Signs & Symptoms |
•Cyanosis in NB, Chronic hypoxia, clubbing |
•No blood flow form the right A to the right V |
•Heart failure, chronic hypoxemia |
•Failure to thrive and growth retardation. |
Diagnosis |
•Listening with a steth for changes in heart sounds |
•O2 sat to see how much O2 is getting into blood |
•Chest x-ray to see the size & position of the heart |
•ECG to check the electrical activity |
•Ultrasound scan (echocardiography) |
Treatment |
•A glenn | Cavopulmonary | HemiFontan shunt |
making a passage between the superior vena cava to the right lung (pulmonary) artery |
•A fontan | Total Cavo-Pulmonary Connection |
This operation joins the inferior vena cava to the pulmonary arteries. |
Erythema Marginatum
Nonpruritic, painless erythematous rash on trunk and/or proximal extremities. |
•Macular lesions with raised margins & central clearing |
•May last from weeks to months |
Jones Criteria for Diagnosis of RF
Minor Manifestations |
Clinical: Fever, arthralgia, history of RF |
Lab test: Acute phase reactants, ECG changes |
Major Manifestations |
• Carditis: friction rub, murmur, cardiomegaly, CHF |
•Arthritis: migratory polyarthritis, swollen, red, tender |
•Chorea, subcutaneous nodules, Erythema marginatum |
Sydenham's Chorea
A neurologic disorder with muscular weakness, emotional lability and involuntary, uncoordinated, purposeless movement |
•Disappear during sleep, lasts 2-4 months |
•Mainly occurs in hands, feet and face, sensation intact |
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Acquired Heart Defects
• An autoimmune disease affecting the heart and extra cardiac sites ( joints, brain, skin) |
•Occurs 10 days to 6 weeks after pharyngitis |
•The disease affects children and young adults (5-15yrs) |
•The disease follows upper respi infection (tonsillitis) |
Cardiac Manifestations |
•Pericardial friction rubs, weak heart sounds |
•Tachycardia, arrythmias |
•Extracardiac: fever, polyarthritis, arthralgia, skin lesions, chorea |
•Pharyngeal culture may be negative |
• Anti streptolysin O (ASO) titer will be high |
Arthralgias and Arthritis
•May be migratory. last for 2 - 3 weeks |
•Warm, swollen, tender joints, inflamed membrane |
•Usually involves the knees, ankles, elbows, and wrists |
•Eroded cartilage and narrowed joint space |
Subcutaneous Nodules
Usually associated with severe carditis and occur several weeks after |
•Firm, painless nodules (up to 2cm) found over bony surfaces and tendons. persist for 1-2 weeks. |
•Occur near elbows, knees, wrists, achilles tendon, vertebral joints |
Kawasaki Disease
Idiopathic multisystem disease. vasculitis of small & medium blood vessels & arteries. Usually <5 yrs old. |
Mucocutaneous Ocular Syndrome (MCOS) the original name of kawasaki disease. The cause is unknown. |
Diagnostic Criteria |
•Changes in extremities: edema, erythema, desquam. |
•Polymorphous exanthem, usually truncal |
•Conjunctival injection, fissuring of lips & oral cavity |
•Cervical lymphadenopathy |
Symptoms |
•Red eyes, changes in lips, tongue & mouth |
•Redness, cracking on lips, red strawberry tongue |
•Hands & feet: red, swollen, hands & soles. |
•Body rash : 1st appears w/fever. bumpy to touch |
•Swelling of glands in the neck, soft, painless nodes |
Phases |
Acute: 1-2 weeks from onset, febrile, irritable, oral changes, rash, edema/erythema of feet |
•Subacute: 2-8 weeks, desquamation, persistent arthritis or arthralgias, gradual improvement. |
• Convalescent (months to years later) |
Treatment |
•No single medicine can treatment or cure |
• Meds : Aspirin and gamma globulin |
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