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Anatomic stenosis – causes obstruction to blood flow•Pressure load on the ventricle•Decreased cardiac outputClinical manifestations – associated with low cardiac outputTX:Aortic stenosis and coarcation – treated the same as defects that increase pulmonary blood flowHypoplastic left heart syndrome – treated the same as defects that decrease pulmonary blood flow and mixed defectsDefects that ↓ Pulmonary Blood Flow (Pulmonary Stenosis, Tetralogy of Fallot, Pulmonary Atresia)Common causes•Obstruction of pulmonary blood flow to lungs•No connection of the right-sided blood flow to lungsCommon manifestations•Cyanosis•Hypercyanotic spells•Poor weight gain•PolycythemiaTreatment - depends upon defectCyanosis:Hgb reduced by 5 g/100ml in veinsAnemia: O2 sat very low before cyanosis is observed Polycythemia: cyanosis is obvious at a higher O2 satCrying: cyanosis worse. Prolongedcrying should be avoidedCyanotic spells require immediate treatment.
Managing Hypercyanotic Episodes:Goal -to decrease pulmonary resistance•Calm the child•Oxygen•Morphine and propranolol intravenously•PRBC •Goal – to increase systemic vascular resistance•knee-chest position•IV fluids to exand circulatory volume•Dopamine (sympathomimetic agent)•Immediate palliative or corrective surgeryMixed Defects:Complex defects •Transposition of the great arteries •Truncus arteriosusCyanosis and congestive heart failure•If the pulmonary blood flow is increased –– then what symptoms????•If pulmonary blood flow is decreased – then what symptoms??TX:Early intervention to prevent secondary damageSurgical repair - See Table 26-7Palliative surgery initiallyAntibiotic prophylaxis for infective endocarditisProstoglandin E1 (PGE1) to reopen the ductus arteriosus Aggressive treatment of hypercyanotic episodesCardiomyopathy:Etiology – primary or no known causeSymptoms: CHFApprox. 40% die within 2 years or receive a heart transplantThese diseases enlarge your heart muscle or make it thicker and more rigid than normal. In rare cases, scar tissue replaces the muscle tissue. Some people live long, healthy lives with cardiomyopathy. Some people don't even realize they have it. In others, however, it can make the heart less able to pump blood through the body. This can cause serious complications, including Heart failure Abnormal heart rhythms Fluid buildupin your lungs or legs Endocarditis, an inflammation of the heart lining Heart attacks, high blood pressureor infections can all cause cardiomyopathy.Etiology – primary or no known cause, Viruses-myocarditis
Dilated (and hypertrophic) cardiomyopathy presents with signs and symptoms of congestive heart failure (CHF). Infants with this disease may demonstrate fast and heavy breathing with feedings, sweating with feedings, lethargy or inactivity, or poor weight gain. Older children may have difficulty