DSON Congenital Heart Disease Post Midterm

What are the types of acyanotic CHD? (7)

1. Coarctation of aorta

2. Cor Triatriatum

3. Ebstein’s Anomaly

4. Congenitally Corrected TGA (cc-TGA)

5. Partial Anomalous Pulmonary Venous Return (PAPVR)

6. Persistent Left Superior Vena Cava (L-SVC)

7. Dextrocardia

What does Q_P:Q_S measure/compare

• compare the flow through the pulmonary valve with the flow through the aorta, should be exact same (equal to 1) except for shunts

  • Q= flow= SV, SV =CSA x VTI

• Q_P : Stroke volume at the pulmonary valve area

  • SV of pulmonary system = CSA of RVOT x VTI of the RVOT

• Q_S : Stroke volume at the aorta valve area

  • SV of systemic circulation = CSA of LVOT x VTI of the LVOT

Describe Q_P/Q_S for normal, insignificant shunts, and hemodynamically significant shunts + Eisenmenger

• normal heart: both sides equal, thus Q_P/Q_S = 1.0

• insignificant shunts: travel left to right, thus lungs get more blood flow, Q_P/Q_S = > 1.0 - 1.5 <

• hemodynamically significant shunt when Q_P/Q_S > 1.5

• Eisenmenger shunt is reversed, thus Q_P/Q_S < 1.0

What are echo measurements for Q_P/Q_S

Describe what happens to Q_P/Q_S for Eisenmenger shunts

• shunt ratio will drop, and eventually reverse direction (L to R becomes R to L)

• Beginning stages → Q_P/Q_S lower than expected bc the shunt direction is starting to reverse

  • measured normal but visual assessment is not normal

• Eisenmenger shunts → Q_P/Q_S <1.0 bc the shunt direction completely reverses (R to L, RAP > LAP)

  • systemic getting more blood than pulmonary, and patient will also be getting deoxygenated blood sent to the body

Describe what happens to Q_P/Q_S for PDA when calculated normal way

• insignificant PDA: LA → LV/LVOT → Aorta → PA → LA → LV/LVOT = shunt missed RVOT and went LVOT twice (more blood)

  • Q_P/Q_S for insignificant PDA → < 1.0 if calculated normal way (looks like it signals for Eisenmenger’s)

• significant PDA with Eisenmenger: RV → PA → Aorta → body = blood skips LVOT (less blood than RVOT)

  • Q_P/Q_S for Eisenmenger PDA → > 1.0 if calculated normal way

Describe the adjusted Q_P/Q_S calculations for PDA

• in general, reverse the direction which you divide the numbers:

  • Q_P is measured at LVOT level

  • Q_S is measured at RVOT level

• as a result:

  • insignificant shunts between 1-1.5

  • Eisenmenger shunts to be <1.0

Describe coarctation of the aorta

narrowing of the descending aorta in the areas of the ductus arteriosus (DA)

What is coarctation of aorta associated with? (2)

• bicuspid aortic valve → most common

• Turner Syndrome

  • Genetic condition in only females, leading to infertility, appears short stature with webbed neck

What are hemodynamic consequences: Coarctation of the Aorta (4)

• Patient presentation depends on severity

• Causes increased afterload → LVH

• May have reduced flow to lower extremities

• Systolic pressure higher in upper extremities (elevated BP)

• Development of collateral arteries to supply lower body

Role of Sonography for: Coarctation of the Aorta

• best seen in SSN, look for shelf-like narrowing or tissue ridge extending to aortic lumen

• CW Doppler for highest velocity→ Measure peak velocity and maximum pressure gradient across narrowing

  • PW walkdown to confirm coarctation, then CW to determine highest velocity (>2.5 m/s)

• for severe: persistent pressure gradient across narrowing in systole and diastole

  • not severe: see step up only in systole

What are treatment options for: Coarctation of the Aorta (2)

• infancy: prostaglandin to maintain ductus arteriosus patency (open, keep flow into PDA)

• percutaneous, surgical repair in hemodynamically significant obstruction

Describe Cor Triatriatum

• a perforate membrane caused by failure of regression of an embryonic membrane

• partitions the left or right atrium into two chambers

• size of orifice that divides the atrium determines the severity of obstruction, symptoms, and age of presentation

  • large = asymptomatic/no obstruction of flow

  • small = have to squeeze harder, thus more severe

Compare Cor Triatriatum Sinister vs Dexter, symptoms similar to?

• Cor Triatriatum Sinister: divides the left atrium

  • symptoms similar to valvular mitral stenosis

• Cor Triatriatum Dexter: divides the right atrium

  • extremely rare, and symptoms similar to tricuspid stenosis

How to differentiate Cor Triatriatum Sinister from supravalvular mitral ring in echo (2)

• by relationship of membrane to LAA

  • Cor triatriatum is superior to LAA, and will be further away from MV annulus

  • Supravalvular is inferior to LAA, and will be near MV

• assess membrane/orifice with colour and spectral Doppler:

  • if you see blood pooling before squeezing out, likely a cor triatriatum

  • Coumadin ridge in LA looks similar but the flow will be free flowing and only seen in PLAX

Describe Ebstein’s Anomaly

abnormality of tricuspid valve where there is apical displacement of the septal/posterior leaflets by AT LEAST 8mm (because its already naturally apical of MV)

What are consequences of Ebstein’s Anomaly (3)

• Distortion and displacement of tricuspid leaflets

• Atria-lization of RV→ some of the RV chamber become like an enlarged RA

• Often severe amount of TR → amount determines hemodynamic consequences

  • severe TR → RA/RV dilation → can lead to RHF

• Note: some people may not need treatment, others may need to surgery to replace the TV

Describe Congenitally Corrected TGA (cc-TGA) or L-TGA

• double discordance:

  • Atrioventricular

    • RA to LV

    • LA to RV

  • Ventriculoarterial

    • LV to PA

    • RV to Aorta

• Great arteries in slightly diff locations than normal (seen in SAX)

• ventricles are in wrong position (RV and LV reverse in echo)

Describe the blood flow in cc-TGA/L-TGA

• blood flow maintained bc connections are switched twice due to double discordance

• circulation physiological normal but anatomy is abnormal, may be completely asymptomatic

  • blood goes into lungs to get oxygenated, while body still gets oxygenated blood

What are the associated lesions with cc-TGA (4)

• VSD

• Tricuspid valve anomalies

• outflow tract obstruction (usually RVOT)

• conduction defects

What are some long-term hemodynamic consequences of cc-TGA (3)

• RV and TV are in systemic location, which is higher pressure than they were designed for thus overtime:

  • tricuspid regurgitation

  • RV failure bc cannot pump as well as LV = congestive heart failure

  • rhythm problems: heart blocks are common

What are some treatment options for cc-TGA

• often only if they have associated defects such as VSD or RVOT obstruction

• If needed:

  • heart failure medication

  • pacemaker (bc of heart blocks)

• in severe cases:

  • “double switch surgery” which is rare

Describe Partial Anomalous Pulmonary Venous Return (PAPVR)

• one or more(< 3), BUT not all (not 4), of the pulmonary veins are not connected to the LA → drains into wrong chamber (often RA)

  • they may be connected to systemic vein (SVC/IVC), RA, coronary sinus, and/or left innominate vein

• Left sided pulmonary veins → may connect to coronary sinus and/or the left innominate vein

• Right sided pulmonary veins → usually connect to RA, SVC/IVC

What are the hemodynamic consequences of PAPVR?

• similar to an ASD or VSD

• R-side volume overload → RA, RV, PA dilation

• RV hypertrophy if longstanding shunt causes pulmonary hypertension

• may have also dilated coronary sinus

Anomalous connection of a single pulmonary vein to the right SVC may be an…

isolated lesion or in combination with a sinus venosus ASD

What are echo features of PAPVR (3)

• Dilated right side

• absence of obvious ASD or VSD

• possibly a dilated coronary sinus

How else to confirm PAPVR?

• echo helps, but cannot see outside of the heart structure so most likely will be confirmed by other forms of testing:

  • MRI

  • catheterization

  • OHS

Describe Persistent Left Superior Vena Cava (LSVC)

• L-SVC is formed by confluence of the left jugular and subclavian veins, and descends inferiorly parallel to the RSVC in most cases

• commonly enters coronary sinus, but may enter LA (rare)

• very rare, but likelihood increased with patients with other CHD

  • most commonly associated with ASD

Describe the consequences of persistent LSVC

• LSVC drains into RA via the coronary sinus, so likely no hemodynamics bc blood still follows normal pathway

  • coronary sinus may be dilated

• instead of one SVC carrying all the blood from the upper body, there are two SVCs, each carrying half of the blood from the upper body

What are some extra steps to confirm persistent LSVC?

• injection of agitated saline or dextrose into peripheral left arm vein → opacification of coronary sinus

• persistent LSVC may pose difficulties during cardiac catheterization and/or cardiac surgery

Describe the Dextrocardia echo scanning protocol (4)

1. Subcostal view, with POM 3’o clock as normal (left) → do not correct images

   • Apex pointing left, but heart is right = dextroposition

   • Apex pointing right, but heart is right = dextrocardia

2. Assess liver and great vessel location to see if they have situs inversus

3. Label images as “Dextro”

4. Perform PLAX, Apical windows with patient turning to right side instead

   1. PLAX: align transducer in plane with heart, POM to base of heart (towards aorta and PA) → heart look same/normal on screen

   2. PSAX: clockwise from PLAX → left and right will not be opposite from normal PSAX

   3. A4C: transducer to apex, maintaining PSAX POM → left and right reversed on screen

   4. A2C, A3C: counter clockwise from A4C as normal

   5. SSN: in situs inversus only, the arch will be directed to the right shoulder instead of left

      • rotate transducer to align with structure, a SAX arch sweep will display this more accurately

Define cyanosis

• blue skin tone caused by low blood oxygen

• deoxygenated blood enters the systemic system (bypassing the lungs)

What are types of Cyanotic CHDs? (8)

1. Interrupted Aortic Arch

2. Total Anomalous Pulmonary Venous Return (TAPVR)

3. Tetralogy of Fallot

4. Double Outlet Right Ventricle (DORV)

5. Complete Transposition of the Great Arteries (d-TGA)

6. Truncus Arteriosus

7. Hypoplastic Right Heart Syndrome

8. Hypoplastic Left Heart Syndrome

Describe Interrupted Aortic Arch

• very rare and low survivability

• represents the most severe form of coarctation, narrowing to the point it completely closed off

  • lack of continuity between ascending and descending aorta

• can occur anywhere within the arch

• if PDA remains open, there will be some blood flow into the aorta via PDA, however it will be deoxygenated

What are other cardiac abnormalities associated with interrupted aortic arch? (5)

• PDA

• VSD

• subaortic stenosis

• bicuspid AoV

• ASD

Describe hemodynamic consequences of interrupted aortic arch with no ASD versus with ASD

No ASD

• upper body receives oxygenated blood via aorta

• lower body received deoxygenated blood via PDA

  • PA → PDA → descending aorta

• incompatible with life

With ASD

• ASD allows some mixed O₂ blood in the RA, which eventually enters lower body, but still overall receive very low O₂ content

• with addition of PDA (still open) → can provide time for baby to live longer prior to surgery

  • PA → PDA → descending aorta

Describe the prognosis of interrupted aortic arch

• failure to treat will result in 90% mortality rate by 4 days old

• Cause of death include:

  • if there is an associated ASD/VSD, excessive blood flow through the defect → severe RHF

  • if no ASD/VSD, death will occur if PDA closes naturally and no blood will enter lower body → organ failure

    • acute kidney failure will cause blood to become too acidic → death

Describe treatment options for interrupted aortic arch

• prostaglandin given intravenously immediately after birth to avoid closure of the ductus arteriosus

• surgical connection of the aorta through thoracotomy incision → preferably within 1st week of life

• Assess descending aorta for any post surgical stenosis, assess with CDI, and PW/CW Doppler

Describe Total Anomalous Pulmonary Venous Return (TAPVR) or TAPVC (Connection)

• all 4 pulmonary veins have no connection to LA → no blood to the left side at all

• drain directly or indirectly into RA

• can occur with or without obstruction of the pulmonary veins

What are the 3 types of TAPVR, and are there different hemodynamics?

• hemodynamics are the same for all 3 types

1. Supracardiac: pulmonary veins drain into the SVC → above heart

2. Infracardiac: pulmonary veins drain into the portal vein, IVC, or ductus venous → below heart

3. Cardiac: pulmonary veins drain into coronary sinus

Describe hemodynamic consequences of TAPVC with no septal defects or PDA versus with ASD or VSD or PDA

No septal defects or PDA

• Blood go to pulmonary veins → IVC → RA → mix blood → PA → oxygenation → pulmonary veins: never pass left side

• no blood in left side AND to body → incompatible with life

With ASD or VSD or PDA

• Shunts will go from right to left because the RAP > LAP

• Mixed blood in the RA and flow to two routes:

  • → RV → PA → lungs

  • → LA → LV → body

• allow mixed blood to enter left side, which gives enough time for baby to go into surgery

  • mixed blood is not sufficient for long term, still not enough oxygenated blood

• RA and RV volume and pressure overloading → pulmonary hypertension

What are some treatment options for TAPVC

• palliative/relief care:

  • atrial septostomy until corrective surgery is performed

• corrective:

  • anasotomosis of common pulmonary vein to left atrium

  • closure of ASD

Role of echo for TAPVR

1. Assess for RV dilation /PHTN

2. Crab view - assess all 4 veins

3. Assess for pulmonary vein stenosis

Describe the Tetralogy of Fallot

• most common cyanotic lesion, comprised of 4 cardiac abnormalities:

1. Overriding aorta (sit directly over both RV and LV):

   • aorta override ventricular system

2. VSD

   • large subaortic defect that extends from right and non-coronary cusps of the aortic valve superiorly to the membranous septum inferiorly

3. RVOT obstruction and/or Pulmonary Stenosis → symptoms determining factor/ hemodynamic severity

   • muscular obstruction usually beings at crista supraventricularis and extends to pulmonary valve annulus, which is often hypoplastic (underdeveloped)

4. RVH

   • caused by RVOT obstruction or pulmonic stenosis

   • RVH may become stiff and eventually fails

When is Tetralogy of Fallot diagnosed

usually during infancy, but if severity of defects is minor, may go unnoticed for years

What are some risk factors for Tetralogy of Fallot (bc unknown cause)

• viral illness during pregnancy, such as rubella

• alcoholism during pregnancy

• poor nutrition during pregnancy

• mother older than age 40

• parent who as Tetralogy of Fallot

• presence of down syndrome or DiGeorge syndrome

What are hemodynamic consequences of Tetralogy of Fallot

• baby will be blue/cyanotic because oxygen going to the body does not carry enough oxygen

• blood from RA → RV cannot go to PA due to RVOT obstruction, and some goes to LV from the VSD → mixed blood, with low oxygenated content will go to the rest of the body

What are some signs and symptoms of Tetralogy of Fallot

• cyanosis

• SOB

• Loss of consciousness

• clubbing of fingers and toes

• poor weight gain

• irritability

What happens to patients with untreated Tetralogy of Fallot

• survival to adulthood is rare → only 2% to age 40, most will die by age 20

• underdeveloped, weak, easily tired due to low oxygen circulation

• decreased oxygenated blood through coronary arteries leads to cardiac dysfunction and fatal ventricular arrhytmias

What are some treatment options for Tetralogy of Fallot, what are the 2 types of surgical options?

• surgical repair is recommended between ages of 3-11 months, options include Rastelli Procedure or Intracardiac Repair:

1. Intracardiac Repair:

   1. Patch the VSD

   2. PV repair or replacement

   3. Surgically widen the pulmonary artery to reduce RVOT obstruction

2. Rastelli

Role of sonography for Tetralogy of Fallot

• may be first person to diagnose this disease, if the severity of pulmonary stenosis is very minor bc most blood can get to PA → asymptomatic

• Assess the following:

  • degree of PS

  • VSD → hold size

  • other complications (RVH, RVF) or congenital defects

  • RV pressure overload → “D” in PSAX

• Post-surgery follow up echos, and assess for the following:

  • residual shunting across VSD patch

  • PV area, and flow through PA

  • any complications

Describe Double Outlet Right Ventricle (DORV)

• both great arteries and arterial trunks arise from morphologic RV

• VSD represents only route to LV

• pulmonary valve or sub-pulmonary stenosis may be present

• majority of cases is associated with not enough oxygen sent to body to sustain life:

  • failure to thrive

  • without treatment → unlikely to survive

What are the 4 types of DORV based on VSD location in relation to great arteries?

1. Subaortic: VSD located anatomically closer to the AoV than to PV (acyanotic)

2. Subpulmonary (Taussig-Bing): VSD located closer to PV (cyanotic)

3. Doubly committed: VSD large and closely related to both semi-lunar valves (mild cyanosis)

4. Non-committed: VSD distant from both semi-lunar valves

Describe Subaortic VSD for DORV

• Main routes:

  • oxygenated blood from LV → VSD → Aorta → body

  • deoxygenated blood from RV → PA → lungs

• However, some of the “other” blood will have access to the other great artery, leading to some mixed blood output

• Acyanotic because mostly oxygenated blood to the body

Describe Sub-pulmonary VSD for DORV

• Main routes:

  • oxygenated blood flows from LV → VSD → PA

  • deoxygenated blood flows from RV → Aorta → body

• cyanotic

• most severe type

Describe Doubly Committed VSD for DORV

• mixed blood enters both PA and Aorta

• mildly cyanotic

Describe Non-Committed VSD for DORV

• VSD located far from PA and AoV

• mixed blood in the RV before traveling to:

  • Aorta → body

  • PA → lungs

What are the associated anomalies with DORV (5)

• Pulmonary valve stenosis

• transposition of the great arteries (TGA)

• pulmonary atresia

• coarctation of the aorta

• mitral valve abnormalities

What are signs and symptoms of DORV

• anything associated with the failure to thrive:

• enlarged heart

• heart murmur

• rapid breathing

• rapid heartbeat

• poor feeding from becoming tired easily

• cyanosis

• clubbing of toes and fingers (late sign)

• failure to gain weight and grow

• pale colouring

• sweating

• swollen legs or abdomen

• trouble breathing

What are hemodynamic consequences of DORV (2)

• deoxygenated blood enters aorta and body → cyanosis, underdevelopment

• excess blood enters pulmonary system → RHF

What is the prognosis of DORV

• surgery almost always needed

  • 1 day post birth - few months post birth

• prognosis for fetuses with DORV is poor, especially those with other associated anomalies

What are treatment options for DORV (3)

• based on the complexity of the intracardiac anatomy:

• Palliative → allow baby to be comfortable for remainder of life

• Surgery → interventricular repair:

  • baffle created through the VSD to join the Aorta to LV + RV w/ PA

• Repair of associated lesions

How are adults who have DORV

• will need lifelong care and regular follow-up evaluations

• more susceptible to valve disease

• monitor PA and Aorta closely

• some adults will have decreased LV and RV function later in life → heart failure

• assess baffle/pathway of blood flow

Describe Complete Transposition of the Great Arteries (d-TGA)

• aorta arises from the morphologic RV, and the PA arises from the morphologic LV

  • caused by failure of the spiral growth pattern of the truncus arteriosus

• left and right sides of the heart are joined in parallel instead of in series

• blood is unable to get from the right heart to the left heart

What are the 2 series of blood flow running in parallel in d-TGA

• Series 1:

  • IVC/SVC → RA → TV → RV → AoV → Aorta → body → IVC/SVC

  • Deoxygenated blood in body

• Series 2:

  • Pulmonary veins → LA → MV → LV → PV → PA → Lungs → pulmonary veins

  • oxygenated blood never communicates with right side

  • body never gets any oxygen → cyanotic

• these 2 series do not communicate

How common is d-TGA?

• relatively common congenital defect accounting for 5-7% of all congenital cardiac malformations

• VSD is most frequent coexisting anomaly (35-40%)

• other associated lesions are: PDA, aortic arch anomalies, ASD/PFO, PS, LVOT obstruction

What happens with the fetus with d-TGA

• prenatally, the fetus has no oxygen symptoms since the lungs are non-functional, and the oxygen is provided via the placenta

• however, upon birth, the baby will become instantly cyanotic after umbilical cord cut

• baby will require immediate intervention

What are the treatment options for d-TGA (2 immediate, 3 surgical options)

• immediate treatment:

  • urgent atrial septostomy

  • maintenance of ductus arteriosus by administration of prostaglandin

• surgical treatment: ideally completed at age 8-14 days (want baby to recover from birth before OHS operations)

  • Jatene repair (current treatment)

  • Mustard or Senning repair (pre 1980)

  • Rastelli

Describe palliative treatment with d-TGA babies

• at birth: this condition is incompatible with life without treatment

  • if no other treatment given in first year → infant will experience failure to thrive, and their health will deteriorate to the point of inoperability

• at birth: the baby will be given:

  • urgent atrial septostomy (hole created in IAS) → oxygenated blood coming into RA → highly deoxy, but mixed blood can now be sent to the body

  • prostaglandin to keep the ductus arteriosus open → allow blood to travel to the lungs to get re-oxygenated

  • these shunts will allow some mixing of blood between the two pathways of circulation → allow baby to stay alive several weeks

Describe Truncus Arteriosus

• failure of division of the fetal truncus arteriosus into two separate vessels

• failure of septation during embryosis → does not divide into 2 parts, so fetus will have single large vessel exiting the heart

• common trunk with a single semi-lunar valve that arises from the heart, overriding an outlet VSD

• coronary, pulmonary, and systemic arteries arise from this single vessel

What is the most common type of truncus arteriosus

• classified based on origin of the pulmonary arteries

• most common is type I: main pulmonary artery arises from truncal root

What are associated anomalies with truncus arteriosus (4)

• coronary artery anomalies

• right aortic arch

• secundum ASD

• interrupted aortic arch

What are the risk factors for truncus arteriosus (causes unknown) (5)

• viral illness during pregnancy, such as rubella

• poorly controlled diabetes during pregnancy

• certain medications taken during pregnancy

• certain chromosomal disorders, such as DiGeorge’s syndrome

• smoking during pregnancy

What are the hemodynamics of the blood flow for truncus arteriosus

• deoxygenated blood enters RA → RV

• oxygenated blood enters LA → LV

• oxygenated + deoxygenated blood mix at the VSD

• mixed blood sent to aorta + PA

• Notably: more blood will be going into lungs because aorta P > PA P → develop pulmonary hypertension

What are complications of truncus arteriosus

• oxygenated and deoxygenated blood mix together, resulting in low oxygen delivery to body

• pulmonary hypertension and trouble breathing

  • abnormal distribution of blood results in too much blood flowing into the lungs

• Cardiomegaly: due to pulmonary hypertension and abnormal blood flow patterns

• Heart failure: due to increased heart mass and lowered oxygen delivery

What is the prognosis for truncus arteriosus

Without Surgery:

• 80% will die before 1 year of age

• very rarely, someone can survive to adulthood, but will have extreme pulmonary hypertension with Eisenmengers

Post surgery survival rates:

• 90% at 5 years, 85% at 10 years, and 83% at 15 years

• poorer prognosis if there was moderate to severe truncal valve insufficiency before repair

• most require follow up surgeries throughout life

  • death post surgery is often due to complications from follow up surgeries, sudden death, or infections

What are the treatment options for truncus arteriosus

• PA banding

• removal of pulmonary arteries from truncal root

• Rastelli procedure/valve conduit to connect the right heart to the branch pulmonary arteries

• patch repair of the VSD

• truncal valve repair or replacement

What are the 2 subtypes of hypoplastic right heart syndrome

1. Tricuspid Atresia

2. Pulmonary Atresia

Describe tricuspid atresia of hypoplastic right heart syndrome

• absence of direct communication between the RA and RV

• in most patients, the floor of the RA is entirely muscular with complete separation from the hypoplastic RV

• form of hypoplasia

What is required for survival for tricuspid atresia

ASD or PFO AND VSD and/or PDA

Describe the result of ASD + PDA for tricuspid atresia

• ASD: allows blood from body to enter left side

• PDA: allows blood to enter lungs

• No ASD: RAP super high = deoxygenated blood return to body → baby dies shortly after birth

• No PDA: blood flow to body but never lungs → no oxygenated blood to body → baby will survive but the blood going to the body still has low oxygenated content

Describe the result of ASD + VSD for tricuspid atresia

• ASD: allows blood from body to enter left side

• VSD: allows blood into RV + lungs

• body will receive blood with low oxygenated content

What are the hemodynamics of tricuspid atresia

• RV does not receive enough blood, and will become hypoplastic (underdeveloped)

• if there is a VSD, excess blood flows through the pulmonary artery and lungs and baby can develop heart failure

• poor O2 content blood sent to body

What is the prognosis of untreated tricuspid artresia

• 10-20% die within the first year of life

• 50% will die by age 5

• between ages 5-15: survival curves stabilize

• however, after age 15, a second fall in survival rates occur due to heart failure

What is the prognosis of treated tricuspid artresia

• some deaths occur during or shortly after surgery, but survival rates are better

• 85% at 1 year

• 82% at 5 years

What are treatment options for tricuspid artersia

• palliative

  • prostaglandin → keep PDA open

  • atrial septostomy

  • Blalock-Taussig shunt: one branch of subclavian artery or carotid artery is separated and connected with the PA

    • pallative surgery while waiting the Fonton Operation

• Surgical

  • Fontan operation

Describe Intact Ventricular Septum Pulmonary Atresia (PA-IVS)

• complete obstruction to RVOT

• an intact IVS

• varying degrees of hypoplasia of the TV and RV

• hallmark of PA-IVS is an atretric pulmonary valve (missing pulmonary valve)

What is necessary for survival for PA-IVS

• an ASD and PDA requires

• ASD: allow blood to travel left side

  • via atrial septostomy

• PDA: allow some blood to get to the lungs

  • prostaglandin to maintain PDA patency

• surgical treatment will depend on suitability for biventricular or univentricular repair

Describe Ventricular Septal Defect Pulmonary Atresia (PA-VSD)

• involves:

  • underdevelopment of the RVOT with atesia of the pulmonary valve

  • a large VSD

  • overriding aorta

• demonstrated wide spectrum of severity, depending on degree of PA development

• frequently considered most severe end of spectrum of TOF

• in 50% of patients, an ASD/PFO is present

• surgical treatment will depend on the anatomy of pulmonary arteries

What are the 2 subtypes of hypoplastic left heart syndrome (HLHS)

1. Mitral Atresia

2. Aortic Atresia

Describe HLHS

• underdevelopment of the left heart-aorta complex

• results in obstruction of the systemic cardiac output and the inability of the left heart to support systemic circulation

What does HLHS may include (4)

• mitral stenosis or atresia → “closed route”

• hypoplasia of the LV

• aortic stenosis or atresia → “closed route”

• hypoplastic aortic arch

What is required for the survivability of HLHS

• PDA and PFO or ASD remaining open

• RV pumps blood both to the lungs and body, allowed bc of the opening connection via PDA

  • because RV is main pump to the body → can lead to RHF

• oxygenated blood returns to LA and then through PFO (bc no where to go)→ back into the RA

What is the untreated prognosis of HLHS

• fatal, usually within the first few weeks of life

• mean life expectancy is 4.5 days

What is the treated prognosis of HLHS

• Before 1980s: 85% mortality within the first month of life

• Current survival rates:

  • 58% at 5 yrs after staged surgical palliation intervention

  • 55-60% at 7 years after cardiac transplantation

What are the complication of HLHS survivors

• tiring easily/ SOB

• arrhythmias

• peripheral edema

• growth restriction

• formation of blood clots that may lead to a pulmonary embolism or stroke

• developmental problems related to the brain and nervous system

• need for additional heart surgery or transplantation

What are the treatment options of HLHS

• newborn infants are treated with prostaglandin to maintain or improve ductal patency

• atrial septostomy is performed if atrial septum intact or restrictive

• these patients are managed (palliated) with a complex three stage procedure called the Norwood procedure

  • because cannot do all at once due to complications

• heart transplant is another option, but waits are often too long

What are the 3 types of simple surgeries

1. atrial septostomy

2. percutaneous closure device

3. coarctation repair

What are the 4 types of palliative treatment shunt surgeries

1. Blalock-Taussig shunt

2. Modified Blalock-Taussig shunt

3. Glenn shunt

4. Pulmonary artery band

Describe atrial septostomy

• performed just after birth/neonate to allow mixing of oxygenated and deoxygenated blood at the ASG

• latrogenic (doctor created) creation of ASD

• most common is balloon atrial septostomy

• Wire goes from IVC → RA → foramen ovale → LA → balloon inflated → balloon pulled back into RA → balloon create an opening/hole in IAS