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1. Pulmonary Stenosis
2. RV Hypertrophy
3. Overriding Aorta
4. VSD

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Abnormal opening between RA/LA that is not the normal Foramen Ovale.

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Sinus Venosus: Least common
Ostium Secundum: Next to Foramen Ovale, difficult to see in utero, most common!
Ostium Primum: Associated with trisomy 21

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Interruption of growth of tricuspid leaflets, causing valve to be hypoplastic or atretic.

Atresia- opening between RA/RV completely occluded

Hypoplastic Right Ventricle

Echogenic, non-opening valve

Right Ventricular Outflow Tract (RVOT) & Pulmonary Artery (PA) generally are diminished in size

Cyanotic- no access for blood to travel to lungs unless ASD or VSD is present. ONLY WAY TO SURVIVE

Stenosis- valvular narrowing (varies in degree)

Valve may be echogenic or dysmorphic

If VSD is present, blood flows from high-pressure LV across VSD into hypertrophied RV and out pulmonary outflow tract.

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Normal aortic valve comprises three semilunar cusps that open in systole and close completely in diastole.

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More common in males

Associated with: increased risk of hydrops, mitral stenosis, pulmonic stenosis, coarctation of the aorta, VSD, Williams-Bourne Syndrome

Findings: Post-stenosis dilation of the ascending aorta, peak velocities >200 cm/sec, LT to RT shunt across Foramen Ovale

3 types: Valvular (most common), Supravalvular, Subvalvular (subaortic)

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Valvular Stenosis: Bicuspid Aortic Valve

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Valvular Stenosis: Bicuspid Aortic Valve

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Happens during development issues during first trimester: 3 aortic cusps may not fully separate.

Most common type diagnosed prenatally. Should be well visualized at 27 weeks, late 2nd trimester.

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Membrane covers the LVOT

Doppler view shows increased velocity across obstructive membrane.

Color flow imaging shows increased turbulence at area of narrowing.

Described in patients with Turner’s syndrome, Noonan’s syndrome, and congenital rubella.

Aortic stenosis and pulmonary stenosis associated with maternal rubella.

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Narrowing of the ascending aortic root; may be related to Williams’ syndrome

50-75% will have hour glass deformity of aorta

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Signifies end-stage left ventricular dysfunction

In 2nd or 3rd trimester, infection or other viral process caused aortic leaflet to thicken and close prematurely

Easier to detect due to abnormal 4-chamber view

LV enlarges from increased pressure in ventricle because LVOT is blocked

Ventricular walls are thin and bulge into RV cavity

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EFE results from blood entering a chamber being unable to exit efficiently, so fibrin develops and is deposited along the walls

Classified according to LV size; can be dilated (most common) or contracted. Regardless of size, EFE results in stiff, poorly contracting chamber

Ultrasound findings; dilated LV with poor contractibility and a hypoechoic thickening of the endocardia surface.

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Aortic stenosis, atresia, or absence can lead to HLHS

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Aortic stenosis, atresia, or absence can lead to HLHS

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Small, hypertrophied LV w/ aortic and/or mitral atresia & small LA

Associated with Turner syndrome, IUGR, Trisomy 13 & 18, Noonan, and Holt-Oram, premature closure of Foramen Ovale

MOST COMMON CHD CAUSE AND DEATH OF NEONATE

Most common cause is Mitral Atresia; higher incidence in males. Blood cannot fill LV to provide volume. Aortic valve becomes atretic, Hypoplastiv LV

If cause is Aortci Atresia (w/ patent Mitral Valve); LV will be enlarged, echogenic. Myocardium shows extreme hypertrophy and increased pressure overload.

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Echogenic AV (stenotic or atretic)

Hypoplastic arch

Small LV and LA

Echogenic endocardium (EFE)

Mitral stenosis and atresia

HEMODYNAMICS
ALWAYS retrograde flow in aortic arch (when present), and LT-RT atrial flow across Foramen Ovale.

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Prognosis for fetus has improved with cardiac transplantation

NORWOOD developed series of surgical repairs that are performed in the newborn

Followed by the GLENN procedure at 4-6 months

Followed by a FONTAN operation between 18 mo-3 yrs

WITHOUT SURGICAL INTERVENTION HLHS IS ALWAYS LETHAL!

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Abnormal condition that exists when aorta connected to RV and PA connected to LV (opposite connections): VENTRICULOARTERIAL DISCORDANCE

When ventricles are opposite from atriums but great arteries are still connected to right ventricles: ATRIOVENTRICULAR CONCORDANCE

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Transposition occurs when the conotruncal septum doesn’t spiral but grows in a straight line causing a parallel relationship between the aorta and pulmonary artery.

Usually aorta is anterior and to the right of pulmonary artery in this pathology.

Associated cardia anomalies: VSD (40%) & Plumonary stenosis + VSD (30%)

Extracardiac and chromosomal abnormalities are very rare

Surgical Correction involves MUSTARD/SENNING REPAIR

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Septal Defects

Conotruncal abnormalities

Hypertrophic CMO

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CHD

Tetralogy of Fallot

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Complete heart block

CMO

ASD

Pulmonary Stenosis

L-TGA

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Fetal goiter- fetal tachycardia & hydrops

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Aortic and pulmonic stenosis

Septal defects

PDA

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Non-immune hydrops

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Non-immune hydrops

Decreased motion or occlusion of the Foramen Ovale

Right heart overload

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IUGR

Fetal demise

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IUGR

Non-specific heart defects

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VSD

Coarctation of the aorta

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VSD, ASD

Tetralogy of Fallot

Coarctation of the aorta

Pulmonary Stenosis

PDA

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Epstein Anomaly

Tricuspid Atresia

ASD

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IUGR

Dextroposition

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IUGR

Placental insufficiency

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MOST COMMON (60%)

Usually multiple (90%) & in ventricles or IVS

Echogenic, non-shadowing

Usually detected between 20-30 weeks and may regress after birth (<80%)

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Heart lesion in which ONE GREAT ARTERY arises from the base of the heart, from which arise the pulmonary trunk, the systemic arteries, and the coronary arteries.

Develops in the early embryologic period when the conotruncus fails to separate into 2 great arteries.

Associated anomalies: mitral atresia, atrial septal defect, univentricular heart, and aortic arch abnormalities.

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A discrete shelflike lesion present in the isthmus of the arch, or more commonly, at the site of the ductal insertion near the left subclavian artery.
May be discrete, long segment, or tubular.

90% of cases include intracardiac malformations: aortic stenosis, aortic insufficiency, septal defects, transposition of the great arteries, Truncus arteriosus, & double-outlet right ventricle.

Connected to TURNERS SYNDROME: coarctation of the aorta and ventricular septal defects

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The normal pulmonic valve comprises three semilunar cusps that open in systole and close completely in diastole.

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The right side of the heart is underdeveloped because of obstruction of the right ventricular outflow tract secondary to pulmonary stenosis.

Several forms: pulmonary atresia with an intact interventricular septum, pulmonary valve fusion with an intact interventricular septum and atrial septal defect, & pulmonary atresia with a normal aortic root diameter.

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Most common congenital lesion of the heart, accounting for 30% of all the structural heart defects.

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25% of congenital cardiac tumors

Benign germ cell tumor

Usually single, encapsulated attached to base, impinges on right atrium

Almost always accompanied by pericardial effusion

Mixed echogenicity with cystic areas

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12% of all fetal cardiac tumors

Single, non-encapsulated, echogenic

Usually in Left Ventricle free wall or IVS

Do not regress postnatal, may require resection

Associated with pericardial effusion

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3% of all fetal heart tumors

Can involve any chamber

Benign

Highly Vascular with feeding vessel

Hypoechoic, polypoid

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Benign and very rare

Mixture of cells arranged in bizarre fashion

Mixed echogenicity

May be associated with pericardial effusion

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Mostly due to TV dysplasia

Associated with poor outcome

Associate with pulmonary hypoplasia

Enlarged Right Atrium

Associated with: Dilated cardiomyopathy, Trisomy 21, Puolmonary stenosis, PA-IVS, Ebstein Anomaly

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Atrialization of the Right Ventricle

Most commonly displacement of SEPTAL tricuspid valve leaflet

Septal and posterior leaflets may be dsyplastic or absent

Anterior leaflet of tricuspid valve least affected of the three leaflets

Associated with: ASD present in 60%, RVOT obstruction, Arrhythmias, cardiomegaly, severe levocardia may be associated

Extracardiac Association: Patau syndrome, Down syndrome, Edwards syndrome period, Lithium carbonate during maternal use

45% die in utero

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When endocardial cushion development is interrupted (day 33), mitral valve may not fully develop.

Usually associated with other forms of left heart obstruction- AO coarctation & VSD

Parachute Mitral Valve:

Mitral Atresia: may appear thickened and dysplastic with shortened chordate and closely spaced

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Associated with hypoplastic left heart syndrome (HLHS)

Whole left side of heart may no develop due to mitral atresia

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Fetal mitral regurgitation probably from cleft mitral valve or congenital mitral stenosis

Cleft Mitral Valve:

Left Atrial cavity would become enlarged because of leakage of blood from defective mitral valve

Color Doppler flow pattern of disturbed flow in left atrial cavity is seen on A4CV and PLA

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MOST COMMON FORM OF RVOT OBSTRUCTION; 2ND MOST COMMON CHD

3 forms: Valvular, Subvalvular, Supravalvular

Valvular: Abnormal cusps become thickened or fused. Visible through cardiac cycle

Subvalvular: muscular thickening or fibrosis of infundibulum, just inferior to cusp opening (recipient of TTTS)

Supravalvular: narrowing of pulmonary artery distal to valve (Williams Syndrome)

COMMONLY ASSOCIATED WITH: Noonan syndrome, maternal rubella, the most common cardiac finding in fetuses affected with twin-twin transfusion

Can progress to Pulmonic Atresia

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Pulmonary Atresia (HYPOPLASTIC RIGHT HEART)

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Pulmonary Atresia (HYPOPLASTIC RIGHT HEART)

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Pulmonary atresia with intact interventricular septum (IVS) - most common form: Hypoplastic RV- ALWAYS CYANOTIC FOR NEONATE AFTER BIRTH

Hypoplastic right ventricle has been associated with maternal condition of rubella

NOT CYANOTIC FOR FETUS: Foramen Ovale provides pathway for oxygenated blood from the placenta to

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VSD changes how blood flows through the heart and lungs. Oxygen-rich blood gets pumped back to the lungs instead of out to the body. The oxygen-rich blood mixes with oxygen-poor blood. These changes may increase blood pressure in the lungs and require the heart to work harder to pump blood.