Risk Factors
• Cardiac development occurs very early in fetal life |
• maternal factors (rubella, alcohol, diabetes mellitus) |
• Genetic factors (history of congenital heart disease) |
• Trisomy 21 (Down syndrome) |
• Presence of other congenital anomalies or syndrome. |
Atrial septal defect (Acyanotic)
Opening in the atrial septum permitting free communication of blood between the 2 atria |
Ostium primum (ASD1) open at lower end of septum |
Ostium secundum (ASD2) open near the center septum |
Pathophysiology |
• Blood flows from left to right (oxy to deoxy) because of the stronger contraction of the left side of the heart, causing an increase volume in the right. |
• Right atrium enlarged, ventricular hypertrophy and increased pulmonary artery blood flow. |
Signs and symptoms |
•Dyspnea on exertion, fatigability, mild growth failure |
•Cyanosis does not occur unless CHF is present. |
Diagnosis |
•ECG with color flow doppler reveal enlarged right side of the heart |
• Cardiac catherization reveal separation in atrial septum |
Treatments |
•Surgical or catherization laboratory for ASD2 |
•Surgery : sutured, completed with catheterization |
•Cardiopulmonary bypass : open heart surgery |
•Silastic or Dacron patch : sutured in place |
Complications |
• Infectious endocarditis and eventual heart failure |
• can cause emboli during pregnancy if not treated |
Aortic Stenosis (Acyanotic)
Narrowing at above or below the aortic valve. |
Supravalvular |
ascending aorta. least common |
Valvular |
most common |
Subvalvular |
subaortic. left outflow tract. |
Signs & Symptoms |
•Mild: exercise intolerance, easy fatigability, asymp. |
•Moderate: Chest pain, dyspnea, diziness & syncope |
•Severe: weak pulses, left failure, hypotension, tachy and activity intolerance chest pain and sudden death. |
Treatment |
•Balloon valvuloplasty as the standard treatment |
•Mild: activity should not be restricted |
•Mod-severe: no competitive sports |
•Cardiac catheterization: Balloon dilation |
•Surgical valvotomy: if closed procedure doesn't work. done to older pts. when severe calcium deposits further obstruct the valve. |
•Beta blocker or calcium channel blocker |
•Antibiotic prophylaxis against endocarditis |
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Acyanotic (Congenital heart defect)
Increased pulmonary blood flow |
• Atrial septal defect |
• Ventricular septal defect |
• Patent ductus arteriosus |
• Atrioventricular canal |
Obstruction to blood flow from ventricles |
• Coarction of the aorta |
• Aortic stenosis |
• Pulmonic stenosis |
Diagnostic Tests
Echocardiogram |
Cardiac Catheterization |
Electrocardiogram |
Chest X-ray |
Echocardiography |
MRI |
Ventricular Septal Defect (Acyanotic)
Abnormal opening in ventricular septum, allows free communication between R & L ventricles |
Small to moderate VSD: 3-6 mm, asymptomatic |
Moderate to large VSD: symptomatic, require repair |
Signs & Symptoms |
4-8 weeks |
•Easy fatigue, failure to thrive, dyspnea |
• A loud, harsh murmur on left sternal border (3rd/4th ) |
• Thrill may be palpable, respiratory infections |
Treatment |
•Small VSD: no surgical intervention, just reassurance |
•Symptomatic VSD: meds, afterload reducers, diuretics |
•Moderate: cardiac catheterization |
•Larger: 3mm open heart surgery |
•Exceptionally large: Silastic or Dacron patch |
Complications |
• Cardiac or Heart failure |
• Endocarditis due to recirculating blood flow |
Indication for surgery: Large vsd with uncontrolled symptomatology, Ages 6-12 mo. with large vsd & pulmonary HTN
Pulmonary Stenosis (Acyanotic)
Narrowing of the pulmonary valve or PA that results in the obstruction of blood flow from the ventricles. |
Signs & Symptoms |
•Mild : Asymptomatic, split 2nd heart sound w/ delay |
•Heart failure (right) & cyanosis with severe |
• Systolic ejection murmur, Right ventricular enlargement |
• Exercise intolerance |
Treatment |
•Mild: No intervention needed, close follow up |
•Mid-severe: requires relieve of stenosis |
•Balloon valvuplasty, treatment of choice |
•Surgical valvotomy is also a consideration. |
•Open-heart needed only for more complex valve anomaly. |
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Cyanotic (Congenital heart defect)
Decreased pulmonary blood flow |
• Tetralogy of Fallot |
• Tricuspid atresia |
Mixed blood flow |
• Transposition of great vessels |
• Truncus arteriosus |
• Total anomalous pulmonary venous return |
• Hypoplastic left heart syndrome |
Atrioventricular Septal Defects (Acyanotic)
•Also called an endocardial cushion defect, results from incomplete fusion of the endocardial cushion. At the septum of the heart |
•Consists of a low atrial septal defect continuous with ventricular septal defect & clefts of mitral & tricuspid, creating a large central AV valve |
• Allows blood to flow between all heart chambers. |
Signs and Symptoms |
•CHF in infancy, Failure to thrive, recurrent infections |
•Exercise intolerance, easy fatigability |
•Cardiac enlargement on CX-ray |
•Late cyanosis from pulmonary vascular w/ R to L shunt |
Treatment |
• Surgery is always required |
• Treatment of congestive symptoms. |
•Pulmonary banding maybe req. in premature or <5kg |
Patent Ductus Arteriosus (Acyanotic)
Conduit between pulmonary artery & aorta fails to close and results in increased pul. blood flow (L to R shunt) |
Ductus Arteriousus fetal structure that connects PA to the aorta. Closure at first breath and is incomplete bet. 7 to 14 days. closure not until 3 mos. |
• blood will shunt from aorta (oxy) to the PA (deoxy) because of the increased pressure in the aorta. |
Signs & Symptoms |
• Small PDA: usually asymptomatic |
• Large PDA: symptoms of CHF & growth restriction |
• Bounding arterial pulses, Widened pulse pressure |
• Enlarged heart, prominent apical impulse. |
•Classic continuous machinery systolic murmur |
• Mild-diastolic murmur at the apex. |
Treatment |
• Indomethacin, inhibitor of prostaglandin synthesis |
• Surgical or catheter closure. |
Coarction of the Aorta (Acyanotic)
Narrowing of aorta due to a constricting band |
Signs & Symptoms |
•Diminution or absence of femoral pulses |
•Higher BP in upper extremities, headache, vertigo |
•Weak lower extremity pulses. decrease cardiac output |
• Epistaxis & CVA not in children due to elevated BP |
•Leg pain due to diminished blood supply |
Treatments |
•Surgical intervention, prevent left vent dysfunction |
•Angoplasty is used by some centers |
•Balloon angioplasty is the procedure of choice |
•Antihypertensive - High BP post-op |
•Antibiotic as prophylactic |
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