Klein - Noncyanotic Congenital Heart Disease AI

Vocabulary flashcards covering principal anatomic lesions, echocardiographic terms, physiologic concepts, and syndromic associations discussed in the lecture on noncyanotic congenital heart disease.

Secundum Atrial Septal Defect (Secundum ASD)

The most common type of atrial septal defect, located in the fossa ovalis region of the atrial septum.

Subcostal 4-chamber view

Echocardiographic imaging plane obtained from the subxiphoid window that is perpendicular to the atrial septum; optimal for detecting ASDs.

Sinus Venosus Atrial Septal Defect

An ASD located near the entry of the superior or inferior vena cava, frequently associated with anomalous right pulmonary venous drainage.

Anomalous Right Pulmonary Venous Connection

Partial anomalous pulmonary venous return in which one or more right pulmonary veins drain to the SVC or right atrium; commonly paired with sinus venosus ASD.

Atrioventricular Septal Defect (AVSD)

Spectrum of defects involving deficiency of the atrioventricular septum and a common AV valve; includes primum ASD, inlet VSD, or both (complete AVSD).

Primum Atrial Septal Defect (Primum ASD)

ASD forming the atrial component of AVSDs; located near the AV valves with their leaflets inserting at the same level.

Complete AVSD

Combination of a primum ASD, an inlet VSD, and a common atrioventricular valve orifice.

Cleft Mitral Valve

Linear division in the anterior leaflet of the left AV valve seen in AVSDs, leading to mitral regurgitation.

Left Ventricular Outflow Tract (LVOT) "Goose-neck"

Elongated LVOT seen in AVSDs due to absence of the AV septum, giving a characteristic angular appearance.

Supracristal (Subpulmonary) Ventricular Septal Defect

VSD located just beneath the pulmonary valve; also called doubly committed or conal septal defect.

Aortic Cusp Prolapse

Downward displacement of an aortic valve cusp into a supracristal VSD, often producing progressive aortic regurgitation.

Aortic Insufficiency (AI)

Regurgitation of blood through an incompetent aortic valve, frequently acquired in patients with supracristal VSDs.

RV Volume Overload

Hemodynamic state of increased right-ventricular preload, typical of large ASDs but not of restrictive VSDs.

Equal RV and LV Pressure

Physiologic hallmark of a large, nonrestrictive VSD causing pressure equalization between the ventricles.

Discrete Subaortic Membrane

Circumferential fibrous ridge located proximal to the aortic valve within the LVOT, producing subaortic stenosis.

Tunnel-type Subaortic Stenosis

Less common form of subaortic obstruction characterized by a long, narrow fibromuscular channel.

Bernoulli Equation (simplified)

Doppler formula ∆P = 4 v² used to convert velocity (m/s) into pressure gradient (mm Hg) across stenotic lesions.

Peak Instantaneous Gradient

Maximum pressure difference across a valve or defect at any point in the cardiac cycle, derived from Doppler velocity.

Perimembranous VSD

Most frequent type of VSD, situated beneath the aortic valve in the membranous septum; often associated with coarctation.

Muscular (Trabecular) VSD

Defect entirely surrounded by muscle within the trabecular septum; high likelihood (80–90 %) of spontaneous closure.

Swiss-cheese Septum

Multiple small muscular VSDs scattered through the trabecular septum.

Coarctation of the Aorta

Discrete narrowing of the descending thoracic aorta, usually juxtaductal, causing upper-body hypertension and weak lower pulses.

Juxtaductal Coarctation

Coarctation segment located opposite the insertion of the ductus arteriosus.

Aberrant Right Subclavian Artery

Right subclavian artery arising distal to the left subclavian; may mask arm–leg pressure gradient in coarctation.

Bicuspid Aortic Valve

Aortic valve with two cusps; present in ~80 % of patients with coarctation.

Interruption of the Aortic Arch – Type A

Complete discontinuity of the aortic arch distal to the left subclavian artery.

DiGeorge Syndrome (22q11 deletion)

Genetic syndrome frequently associated with conotruncal defects such as interrupted aortic arch and truncus arteriosus.

Coanda Effect (in Supravalvar AS)

Tendency of a high-velocity jet to adhere to the aortic wall and channel flow into the right innominate artery, causing arm pressure discrepancy.

Supravalvar Aortic Stenosis

Obstruction at or above the sinotubular junction; hallmark lesion of Williams syndrome.

Patent Ductus Arteriosus (PDA)

Persistent fetal connection between the descending aorta and pulmonary artery; Doppler shows continuous flow systole and diastole.

Bidirectional Shunt (PDA)

Flow across the ductus that reverses direction between systole and diastole, reflecting near-equal PA and aortic pressures.

Discrete Valvar Pulmonary Stenosis

Thickened pulmonic leaflets causing systolic doming and systolic pressure gradient across the pulmonary valve.

Dynamic RV Infundibular Obstruction

Muscular narrowing of the RV outflow tract that intensifies in late systole, producing a dagger-shaped Doppler profile.

Double-chambered Right Ventricle (DCRV)

Subdivision of the RV by anomalous muscle bundles, generating mid-cavity obstruction often associated with membranous VSD.

Noonan Syndrome

Autosomal-dominant disorder featuring short stature, webbed neck, chest deformity, and high incidence of pulmonary valve stenosis and hypertrophic cardiomyopathy.

Hypertrophic Cardiomyopathy (HCM)

Genetic myocardial disease with unexplained LV hypertrophy; may coexist with pulmonary stenosis in Noonan syndrome.

Prostaglandin E₁ (PGE₁) Infusion

Pharmacologic agent used to maintain ductal patency in duct-dependent cardiac lesions such as critical coarctation.

Subcostal Sagittal View

Echocardiographic scan plane from the subxiphoid window directed cephalad, ideal for imaging systemic venous connections and locating sinus venosus ASDs.

Pulmonary Vascular Resistance (PVR)

Resistance to blood flow through the pulmonary circulation; influences magnitude of left-to-right shunts.

Ventricular Compliance

Distensibility of the ventricle; primary determinant of shunt direction across ASDs.

Transcatheter ASD Device Closure

Percutaneous placement of an occluder device to close secundum ASDs with adequate rims, avoiding surgical repair.

Discrete Membranous Subaortic Stenosis

Fibrous shelf in LVOT producing obstruction; most common subtype of subaortic stenosis.

Systolic Anterior Motion (SAM) of the Mitral Valve

Anterior displacement of the mitral valve into the LVOT during systole, characteristic of hypertrophic cardiomyopathy.