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CONGENITAL HEART DISEASE

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AORTIC STENOSIS

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A form of acyanotic congenital heart disease (CHD) in which obstruction of the left ventricular outflow tract leads to a systolic pressure gradient between the left ventricle (LV) and the aorta.

  • Aortic valve stenosis: Most common form of aortic stenosis; frequently due to bicuspid aortic valve; identified in up to 2% of adults

  • Subvalvular stenosis: Due to fibromuscular ring or shelf below the aortic valve; may be associated with malalignment ventricular septal defect (VSD) or aortic coarctation

  • Supravalvular stenosis: Least common form of aortic stenosis; may be localized or diffuse; may be associated with Williams syndrome

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EPIDEMIOLOGY
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  • 3–6% of congenital heart defects; male:female = 4:1

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PATHOPHYSIOLOGY
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  • Critical aortic stenosis: High pressure gradients across the aortic valve may result in LV failure, low cardiac output, and pulmonary edema

  • Pressure gradient may increase as cardiac output increases with growth during childhood

  • Abnormal diastolic filling is due to LV hypertrophy

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CLINICAL MANIFESTATIONS
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  • Often asymptomatic in infancy, but can present with irritability, paleness, tachycardia, tachypnea, retractions, and rales; symptoms depend on severity and location of obstruction

  • Heart failure is most common in neonates with critical disease or in adults with untreated disease

  • Ventricular arrhythmias and sudden death may occur

  • Physical examination: Early systolic ejection click at the apex; harsh ejection systolic murmur at the base radiates to the neck; palpable left ventricular lift; precordial systolic thrill at base

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DIAGNOSTICS
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  • Chest x-ray (CXR): May show cardiomegaly (evidence of LV hypertrophy) and pulmonary edema with critical obstruction

  • ECG: LV hypertrophy or LV strain in severe disease

  • Echocardiography: Defines anatomy and hemodynamic severity of the lesion

  • Cardiac catheterization: Required to establish severity and measure pressure gradient across aortic valve, and for treatment of aortic stenosis

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MANAGEMENT
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Medical
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  • Prostaglandin E1 (PGE1) dilates the ductus arteriosus to augment systemic output in severely ill neonates with critical obstruction

  • Inotropic support with dopamine or epinephrine may also be required to maintain adequate cardiac output both before and after the intervention

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Surgical or Catheter-based Interventions
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  • Aortic valve stenosis: Percutaneous balloon aortic valvuloplasty is the preferred approach for aortic stenosis; surgical valvotomy; aortic valve replacement with pulmonary autograft or Ross procedure (preferred in infants and young children) prosthetic valve or aortic homograft

  • Subaortic stenosis: Surgical removal of fibromuscular shelf or membrane, myomectomy may be required for muscular tunnel-like obstruction (Konno procedure)

  • Supravalvular stenosis: Surgery to widen or repair stenotic segment

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ATRIAL SEPTAL DEFECT

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A form of acyanotic CHD characterized by openings in the atrial septum at one of the following four locations:

  • Ostium secundum: Located at site of foramen ovale; accounts for 50–70% of all atrial septal defects (ASDs)

  • Ostium primum:...

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