CHD pt. 4

“Crab Claw” view

Showcases normal SVC presentation

Persistent Left Superior Vena Cava

• most common congenital anomaly that affects the systemic veins

• The LSVC drains into the Coronary Sinus for blood to return to the RA

• No tx is necessary

• No complications

Where should contrast be injected in order to diagnose a persistent lt SVC?

Left arm

Persistent Left SVC:

• dilated Coronary Sinus

• May follow vessel

• May mimic:

  • Pericardial effusion

  • Anomalous pulmonary venous return

  • Descending aorta

Cor Triatriatum

• fibromuscular membranous partition

  • LA divided into 2 parts

    • Called “Sinister” or “Sinistrum”

    • Pulm venous return must pass through a false “chamber” / compartment posterior to the LA

    • membrane may have fenestrations

    • Diagnose obstruction of flow in the LV

      • mimics MS

• RA division

  • rare

  • thought to be remnant of fetal circulation

  • called “Dextrum” or “Dexter”

Cor Triatriatum Sinister

• associated w/:

  • ASD

  • PHTN

• complications:

  • CHF

    • d/t increased pressure in LA and PA w/ MS

Cor Triatriatum Sinister 2D findings:

• best views:

  • PLAX / A4C

• see membrane within LA cavity

Kawasaki’s DIsease

• acute febrile vasculitis

  • thought to be an autoimmune disorder

  • 1800 cases/yr

    • 50% of pt’s are <2 y/o

    • affects ~6 months - 5 yrs

  • only 20% have CA involvement

Kawasaki’s Syndrome findings:

• Aneurysmal Coronary Arteries

  • may be lined w/ thrombus

  • >3mm <5 y/o

  • >4mm >5 y/o

• may affect myocardial perfusion

  • causes WMA’s

• MR + AI

Kawasaki’s Disease symptoms:

• FUO lasting > 5 days

  • unresponsive to antibiotics or acetaminophen

• swollen cervical lymph nodes

• body rash

• erythema of hands and feet

  • skin peeling occurs within 2-4 weeks

• swollen, cracked, reddened lips

• strawberry tongue**

Kawasaki’s Disease clinical signs:

• elevated C-reactive protein, alpha-1 antitrypsin protein, and WBC

• proteinuria

Conotruncal Defects

• failure of conotruncal septation may result in following CHD’s:

  • TOGV

  • ToF

  • Pulmonary Atresia w/ VSD

  • DORV w/ VSD

  • truncus arteriosus

• More common in infants born to DM mothers

Cyanotic Heart Disease

• cyanosis may be present in many CHD’s

  • blue discoloration of mucus membranes, skin, lips, nailbeds, etc

  • cyanosis that is cardiac in origin will:

    • increase w/ crying

    • not be affected by O₂ administration

• these lesions allow systemic venous blood to mix w/ the systemic arterial circulation

Congenital defects that most commonly cause cyanosis:

• TOGV

• ToF

• Pulmonary Atresia

• Tricuspid Atresia

• TAPVR

• Truncus Arteriosus

• Single Ventricle / Atrium

• Hypoplastic Lt Heart

• DORV

• Ebstein’s Anomaly

  • d/t R → L ASD

What is one method to treat cyanotic heart disease?

• give Prostaglandin to improve systemic O₂ saturation

  • dilates the:

    • PDA

      • maintains a shunting source

    • Pulm arterioles

    • systemic vessels

Transposition of Great Vessels (TGV)(TGA)(TOGV)

• Dx when the Ao is anterior to the Pulm A

  • PSAX

2 types of TOGV:

1. D-Transposition

   1. D = dextro

   2. latin for “right”

2. L-Transposition

   1. L = levo

   2. latin for “left”

D-Transposition of the Great Vessels (DTGV)

• there is one wrong connection / mismatch

  • correct atrium is connected to correct ventricle

  • the wrong great vessel is attached to each ventricle

    • aka complete transposition

    • unsaturated bl→RA→RV→ Ao

    • saturated bl→LA→LV→PA

• associated w/ cyanosis

• must have large ASD, VSD, or PDA to survive

What type of open defect is DTGV most associated with?

membranous VSD

DTOGV surgical tx:

• atrial switch

• mustard or senning repair

  • re-direct atrial blood to exit correct great vessel

• jatene repair

  • switches the great vessels

Helpful hints to remember D-transpos:

• D = death

• must have surgery to switch/redirect to survive

L-Transposition of the Great Vessels

• 2 wrong connections

  • atria are connected to the wrong ventricles

  • ventricles are connected to the wrong great vessels

  • overall connection is “correct”

    • “Congenitally Corrected”

    • “Double Discordance”

    • unsaturated bl→RA→LV→PA

    • saturated bl→LA→RV→Ao

LTOGV findings:

• Lt A-V valve is the morphologic TV

  • Ebsteinization often present

• not associated w/ cyanosis

• less common than D-transposition

LTGV is associated w/:

• Ao Coarctation

• EKG abnormalities

  • conduction system in abnormal location

Tetralogy of Fallot (TOF)

• Combination of 4 congenital lesions:

1. membranous VSD

   1. anterior + rt displacement

2. overriding Ao (over the IVS)

   1. may be infundibular / subinfundibular stenosis

      1. usually d/t posterior malalignment of IVS from overriding Ao

   2. PV or PA may be atretic

3. RVOTO / PS

4. RVH

   1. d/t obstruction

Tetralogy of Fallot key findings:

1. membranous VSD

2. overriding Ao (over the IVS)

3. RVOTO / PS

4. RVH

TOF blood flow:

• since there is RVOTO + large membranous VSD, flow from either ventricle may exit via the Ao

  • this results in:

    • cyanosis

    • R → L shunt

• also depends on the amount of resistance met in each great vessel

TOF 2D findings:

• PLAX

  • overriding Ao

  • VSD

  • RVH

• PSAX

  • VSD (mid)

  • PS/RVOTO (base)

TOF Dp findings:

• of a subvalvular obstruction

  • dynamic obstruction → peaks in late systole

    • dagger shaped spectral trace

• PW to localize region of stenosis

• CW for max pressure gradient across the stenosis

TOF chest x-ray findings:

• “boot shaped” heart

• rt sided Ao arch

What is TOF associated with?

• CA anomalies**

  • LAD arising from the RCA and crossing over the RVOT

  • single CA where a major branch crosses the RVOT

• digit clubbing

• cyanotic nail beds

Pulmonary Atresia

• atresia of the PV / PA

• no pulmonary blood flow exists

  • except through:

    • PDA

    • systemic to pulm A collaterals

      • “bronchiolar collaterals”

        • arise from the Desc Ao

  • poor prognosis

What is Pulmonary Atresia associated with?

• Tetralogy of Fallot

• Turner’s Syndrome

• Noonan’s syndrome

What are the 2 types of Pulmonary Atresia?

1. VSD

2. intact IVS

VSD Pulm Atresia

• similar to severe TOF

  • all ventricular blood leaves via the Ao

    • causes cyanosis

  • pulm flow is provided through PDA

Intact IVS (IIVS) Pulm Atresia

• associated w/:

  • TV atresia

    • creates rt → lt ASD

  • RV hypoplasia

RV Hypoplasia

• RV underdevelopment

• due to compromised RV flow in fetal life

  • caused by:

    • TV atresia / stenosis

    • PV/PA atresia w/ IIVS

• results in a small noncompliant RV that contracts minimally

The 2 types of Anomalous Pulmonary Venous Return include:

1. Partial (PAPVR)

2. Total (TAPVR)

Partial Anomalous Pulm Venous Return (PAPVR)

• some Pulm V’s drain into venous structures and return blood to the RA

• frequency of occurence in order:

  • RPV → SVC

  • RVP → RA

  • RPV →. IVC

  • LPV → Coronary Sinus

• associated w/ Sinus Venosus ASD (15%)

Total Anomalous Pulm Venous Return (TAPVR)

• all 4 Pulm V’s drain into the RA or to one of the systemic veins

  • venous connection to RA

  • venous connection “above heart”

  • venous connection “below diaphragm”

• critical need for a large ASD

  • so blood can reach the left heart

TAPVR venous connection “above heart” connects to:

• SVC

• azygous V

• Lt innom V

• Coronary Sinus

TAPVR venous connection “below diaphragm” connects to:

• Portal V

• Ductus venosus (PV to IVC to bypass liver)

Persistent Trucus Arteriosus

• one large great vessel

• carries both RVOT and LVOT

• serves as both Ao and MPA

  • Rt + Lt PA’s usually arise from truncus

• has a single common S-L Valve

  • abn w/ >3 cusps

  • usually incompetent

    • loud diastolic murmur

• associated w/ VSD

  • both ventricles eject bl to truncus via large VSD

Depending on PA’s size, PTA will demonstrate:

• diminished pulm blood flow

  • causes cyanosis

• increased pum bl flow

  • causes CHF

Truncus Arteriosus anatomy:

• 1 - PA arises from truncus + shares common valve annulus

• 2 + 3 - PA branches arise separately

Truncus Arteriosus 2D findings:

• PLAX

  • large overriding truncal root

  • VSD

• PSAX

  • # of leaflets present

  • absence of MPA is its usual location

  • examine truncal root for PA origin

Truncus Arteriosus surgical tx:

• Rastelli Procesure

  • dacron conduit w/ a prosthetic valve b/w the RA and PA

Single Ventricle aka:

• double inlet LV

• common ventricle

• univenticular heart

Univentriular heart

• both A-V valves connect to a single ventricular chamber which then directs blood to both great vessels

  • volume of blood flow to each GV is determined by the reisstane in each arterial circuit

2D findings of a single ventricle:

• absence of IVS

  • best view → A4C

Double Outlet RV (DORV)

• both great vessels arise from the morphologic RV

  • determine great vessel relation to each other

    • normal

      • PA ant + left of the Ao

    • side-by-side

      • in save TRV plane

    • dextromalposition

      • Ao ant + to the rt

    • levomalposition

      • Ao ant + to the lt

What is DORV always associated w/?

• VSD

  • sole outlet for the LV

    • usually large

  • most often membranous

DORV is often associated w/:

• ASD

• PDA

• MV abnormalities

• LVOTO

• pulm stenosis and/or RVOTO (50%)

  • causes diminished pulm blood flow

    • causes cyanosis

  • those w/o RVOTO will have increased pulm blood flow

    • causes CHF

What is the most severe form of LVOTO?

Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome

• small underdeveloped LV w/ poor contractility

• may be caused by:

  • Ao atresia

  • Ao hypoplasia

  • severe AS

Hypoplastic Left Heart Syndrome includes:

• MV + AoV atresia

• endocardial thickening

• small LA

What does HLHS have to be associated with?

• PDA

  • pt given prostaglandins to maintain patency

  • blood shunts from MPA directly to desc Ao to maintain systemic flow

    • rt → lt shunt

  • will observe retrograde flow in asc ao

    • must feed CA’s

Hypoplastic Left Heart Syndrome is also associated w/:

• RVE

• small Ao root (usually <5mm)

• dilated PA

HLHS prognosis:

poor → d/t organ hypoperfusion

HLHS Tx : Norwood Procedure

• concerts RV into main pumping chamber

1. Blalock-Taussig shunt

   1. shunt inserted btwn reconstructed Ao and MPA

      1. supplies blood t lungs

2. Glenn operation

   1. SVC anastomosed w/ MPA (bi-directional Glenn)

   2. BT shunt removal

      1. initial separatio of pulm _ systemic circulations

3. Fontan shunts

   1. IVS anatomoses w/ MPA

• Pulm A recives all systemic venous return

• all systemic blood flow originates from RV