Adult Congenital Heart Disease

title: Adult Congenital Heart Disease tags: #FFICM #cardiac notebook: ð-FFICM type: anki

[[Acute Coronary Care]]

chapter: [[Valvular and congenital heart disease and infective endocarditis]]

category:

Congenital heart disease is common, up to 1% of live births. Most will survive to adulthood (more than 85%, lets say 1 in 10 die before adulthood). ¹

Why do adults with congenital heart disease die? Most die of HF. ¹

Flashcard	type:basic
How common is congenital heart disease in live births?	1%
How many children born with congenital heart disease will survive to adulthood?	85%
What do most adults with congenital heart disease die of?	Heart Failure
What is an aortic coarctation?	A narrowing of the aorta
For both the left and right side of the heart, congenital obstructive lesions can be either:	A valve problem or an artery problem
Pulmonary valve stenosis can be categorised by location as:	Valvular Subvalvular Supravalvular
In congenital heart disease, the right sided equivalent of aortic coarctation is:	Pulmonary Artery Stenosis
In congenital heart disease, the left sided equivalent of pulmonary artery stenosis is:	Aortic Coarctation

What kinds of anatomical problems are there?

Circulation

flowchart LR
subgraph body
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> ra[Right Atrium]
svc --> ra

Shunts

L to R
ASD
VSD
PDA

Obstructive Lesions

Left

Bicuspid Aortic Valve
Aortic Coarctation

Right

Pulmonary Stenosis
Valvular
Subvalvular
Supravalvular/PA Stenosis

Tetralogy of Fallot

Pulmonary Outflow Obstruction (RVOT) (through Pulmonary Stenosis)
Overriding Aorta
VSD
RV Hypertrophy

Flashcard	type:cloze
The four abnormalities in Tetralogy of Fallot are: {{c1::Pulmonary Valve Stenosis, Causing RVOT}} {{c2::VSD}} {{c3::Overriding Aorta}} {{c4::Right Ventricular Hypertrophy}}

flowchart LR
subgraph tetralogy-fallot
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
        rv<==>lv
        rv -.->aorta
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa -.-> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] -.-> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> ra[Right Atrium]
svc --> ra

TGA (Transposition of Great Arteries)

d-TGA (Dextrotransposition)

The two circuits (pulm and systemic) are totally separate loops. Left Ventricle goes to pulmonary and back to left. RV goes to systemic and back.

flowchart LR
subgraph dextrotransposition-great-arteries
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> aorta
lv[Left Ventricle] --> pa[Pulmonary Artery]
ivc --> ra[Right Atrium]
svc --> ra

l-TGA (levotransposition) / Congenitally Corrected DGA

Like d-TGA, left goes to pulmonary and right goes to systemic. But this time the loops are connected. So blood goes from left ventricle, to pulmonary, back to right ventricle, then off to circulation, then back to left. So the right is doing the work designed for the left.

flowchart LR
subgraph levotransposition-great-arteries
direction BT

    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
pulmveins[Pulmonary Veins] --> ra[Right Atrium]
rv[Right Ventricle] --> aorta
lv[Left Ventricle] --> pa[Pulmonary Artery]
ivc --> la[Left Atrium]
svc --> la
end

Flashcard	type:basic
An overriding aorta (seen in ToF) is where:	Because of a VSD, the aorta is taking in oxygenated and deoxygenated blood from the LV and the RV
The four abnormalities in Tetralogy of Fallot are:	Pulmonary Valve Stenosis VSD Overriding Aorta RV Hypertrophy
There are two types of Transposition of Great Arteries	D and L Dextrotransposition + Levotransposition
Dextrotransposition of Great Arteries (D-TGA) is where	Two Separate Loops of blood Pulm artery and Aorta have switched ventricles Venous Drainage is as usual D for Distinct Loops D for DOOM
Levotransposition of Great Arteries (L-TGA) is where	Blood flow is one Circuit still Pulm artery and Aorta have switched ventricles PLUS Vena Cava and Pulmonary Veins have switched atria L for Long Circuit L for Less Bad
The TGA where the pulm artery and aorta have switched ventricles is called	Dextrotransposition D-TGA D for distinct loops
The TGA where the pulm artery and aorta PLUS the pulm veins and IVC have switched places is called	Levotransposition L-TGA L for Long Circuit
In congenital heart disease, what is Ebstein's anomaly?	A displaced tricuspid valve, slid down towards apex. So the RV becomes tiny and "atrialised".
The congenital heart disease problem where you have a displaced tricuspid valve, lower down than it should be, towards the apex of the RV is called	Ebstein's Anomaly

Ebstein's

This is where there is a displaced TV (down towards apex), meaning the RV becomes "atrialised". Usually there's also an ASD.

What types of specific procedures are there?

PA Banding

In situations where there's too much blood flow to pulmonary instead of systemic circulation. You put a band round the PA. This increases pressure here, meaning blood will flow along systemic circulation instead (following path of less resistance). This will improve systemic perfusion/decrease pulm blood flow.

Situations like:

Single Ventricle
(A)VSD
Double Outlet Ventricle

Flashcard	type:basic
For congenital heart disease, what is done in a PA Banding procedure?	You put a band around the pulmonary artery to narrow it.
What is the physiological result and rationale behind a PA Banding procedure?	The band increases pulmonary artery pressures. In patients with blood coming from RV and LV into the pulmonary artery, increased pressures in the PA means that more blood will go to the systemic circulation instead.
In congenital heart disease, which patients may receive a PA Banding procedure?	Any patients with mixing of RV and LV blood in the heart. So Single Ventricle patients, (A)VSD patients, and Double Outlet Ventricle Patients.

Blalock-Taussig-Thomas Shunt (BT)

This is for in situations where there isn't enough blood flow to pulmonary circulation. You create a shunt from subclavian artery to same side PA.

flowchart LR
subgraph Blalock-Taussig-Shunt
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta --> syscirculation
        aorta[Aorta] --> subclavian --> syscirculation[Systemic Vasculature]
        subclavian ==> pa
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa ==> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation ==> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> ra[Right Atrium]
svc --> ra

Flashcard	type:basic
In congenital heart disease, what is a Blalock-Taussig-Thomas (BT) Shunt?	A shunt from the subclavian artery to the pulmonary artery on the same side.
In congenital heart disease, what is it called when you surgically put a shunt from the subclavian artery to the pulmonary artery?	A Blalock-Taussig (BT) Shunt
In congenital heart disease, physiologically why would you want to create a BT shunt?	To increase blood flow to pulmonary circulation.
In congenital heart disease, what is the other name for a hemi-fontan procedure?	A Glenn-bidirectional shunt
In congenital heart disease, what is the other name for a Glenn-bidirectional shunt?	A hemi-fontan procedure
What is the physiological aim of a hemi-fontan procedure?	To increase blood flow to the pulmonary circulation
In congenital heart disease, what is the anatomical change created by a hemi-fontan procedure	An anastomosis between the SVC to the Right Pulmonary Artery. So deoxygenated blood from the SVC goes straight into the pulmonary artery, skipping the heart.
In congenital heart disease, what is the name of the surgical procedure where you make an anastamosis between the SVC and the Right Pulmonary Artery?	A hemi-fontan procedure
How old are patients when they receive a hemi-fontan procedure?	2 months
In congenital heart disease, what is the other name for a Fontan Procedure?	Total Cavopulmonary Connection
In congenital heart disease, what is the other name for a Total Cavopulmonary Connection	Fontan Circulation
In congenital heart disease, what is the anatomical change carried out surgically in a Fontan Procedure	You divert all blood from the IVC to the Pulmonary Artery
In congenital heart disease, what has changed anatomically when a patient has a Fontan Circulation	They have a single ventricle circulation, blood is pumped by LV round the body, and rather than the RV pumping it into the Lungs, the lungs are fed by venous drainage.
In congenital heart disease, what is it called when a patients blood is pumped round body by the left ventricle, then that venous drainage goes straight into pulmonary circulation (skipping the RV)	Fontan Circulation / Total Cavopulmonary Connection
In congenital heart disease, what is the overall aim of a Norwood procedure?	To convert a bad two ventricle circulation to a single ventricle Fontan circulation
In congenital heart disease, what is it called when you convert a bad two ventricle circulation to a single ventricle Fontan circulation?	A Norwood procedure
In congenital heart disease, what five steps is a Norwood Procedure a combination of?	Turn Pulmonary Artery into a New Aorta Cut out the interatrial septum (Atrial Septectomy) Put in a BT Shunt (To Improve Pulmonary Circulation) Put in a BDG (To Improve Pulmonary Circulation) Complete the Fontan Circulation

Glenn-bidirectional(BDG)/hemi-Fontan

This is the first procedure done for folk with a single ventricle. Done to improve blood flow to pulm circulation. Here you create a shunt (anastamosis) from SVC to right PA. This is done ~ 2 months.

flowchart LR
subgraph Glenn-Bidirectional-HemiFontan
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> ra[Right Atrium]
svc -.-> ra
svc ==> pa

Fontan/Total Cavopulmonary Connection

This is the second procedure, after a BDG. Here you're diverting all blood from IVC to PA. So this is for patients who you can't create a 2 ventricle circulation. This is done around 2 years to 4 years.

flowchart LR
subgraph Fontan
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> pa
ivc-.- ra
svc -.-> ra[Right Atrium]
svc ==> pa

Norwood

This is where you deliberately make a single ventricle circulation like you would see after a Fontan. You've started with a 2 ventricle circulation, but a shite one, so a hypoplastic left heart. It's a combo of:

Create New Aorta from PA
Atrial Septectomy
BT shunt
BDG
Fontan

flowchart LR
subgraph Norwood
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
        ra===la
    end
        subgraph syscirc
        direction BT
        aorta --> syscirculation
        aorta[Aorta] --> subclavian --> syscirculation[Systemic Vasculature]
        subclavian ==> pa
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa ==> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation ==> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> pa
ivc-.-ra
svc -.-> ra[Right Atrium]
svc ==> pa

Pott's Shunt

This isn't really done any more. It's a palliative procedure seen in TOF and severe PAH. You connect the left PA and the descending aorta. Either to offload the RV in severe PAH, or to improve pulm blood flow when severe RVOT obstruction.

flowchart LR
subgraph Pott-Shunt
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> pa[Pulmonary Artery]
lv[Left Ventricle] --> aorta
ivc --> ra[Right Atrium]
svc --> ra
pa ==> aorta

Switches

Atrial Switch (Mustard, Senning)

This is in cases of the d-TGA, where there are two separate loops that don't overlap. What you want is for them to overlap. So you place a "baffle" (diverting tunnels) within the atria to redirect systemic blood to the LV, and redirect pulmonary blood to the RV. That looks like you've got the wrong blood going to the wrong ventricle, but that is right. You end up with the RV doing the work the LV was supposed to do.

flowchart LR
subgraph Atrial-Switch
    direction BT
    subgraph heart
        direction LR
        subgraph right
            direction TB
            ra-.-rv
        end
        subgraph left
            direction TB
            la-.-lv
        end
        la ==>rv
        ra ==> lv
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] --> aorta
lv[Left Ventricle] --> pa[Pulmonary Artery]
ivc --> ra[Right Atrium]
svc --> ra

Arterial Switch (Rastelli, Jatene)

This is also for d-TGA. In an atrial switch you are making the overlap in the two circulations at the point of venous return. In an arterial switch you are making it at the point of arterial exit. You swap over the aorta and the PA. This is getting you basically back to a normal circulation.

flowchart LR
subgraph Arterial-Switch
    direction BT
    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
end
pulmveins[Pulmonary Veins] --> la[Left Atrium]
rv[Right Ventricle] ==> pa[Pulmonary Artery] 
lv[Left Ventricle] ==> aorta
rv -.-aorta
lv-.-rv
ivc --> ra[Right Atrium]
svc --> ra

Flashcard	type:basic
In congenital heart disease, the two types of "switches" for D-TGA are	Atrial Arterial
In what congenital heart disease would an Atrial Switch or Arterial Switch be performed	DEXTROtransposition of Great Arteries
In congenital heart disease, what is done surgically in an atrial switch	Put in diverting tunnels, so blood from LA goes to RV, and blood from RA goes to LV. You've converted a D-TGA into an L-TGA!
In congenital heart disease, what type of circulation does a patient have following an atrial switch?	Basically youve got the RV doing the work of the LV, and the LV doing the work of the RV. You've converted a D-TGA into an L-TGA!
In D-TGA, what is it called when you surgically put in tunnels in the atrium, to divert blood from the LA to the RV, and from the RA to the LV?	An atrial switch
In congenital heart disease, what is done surgically in an Arterial Switch?	You chop off the misplaced pulmonary artery and aorta from the wrong ventricles. You then reattach the pulmonary artery to the Right Ventricle, and the aorta to the left ventricle
In D-TGA, what is it called when you chop off the misplaced pulmonary artery and aorta, and reattach them to the correct ventricles?	An arterial switch
In congenital heart disease, what type of circulation does a patient have following an arterial switch?	A normal one!

Double-Switch

This is for l-TGA. Here in l-TGA, circulation has got the right heart and the left heart the "wrong way around". So what you need to do is snip off the arterial and venous connections, and flip L to R and R to L.

flowchart LR
subgraph Double-Switch
direction BT

    subgraph heart
        direction TB
        subgraph right
            direction TB
            ra --> rv
        end
        subgraph left
            direction TB
            la --> lv
        end
    end
        subgraph syscirc
        direction BT
        aorta[Aorta] --> syscirculation[Systemic Vasculature]
        syscirculation --> ivc[IVC]
        syscirculation --> svc[SVC]
    end
    subgraph pulmcirc
        direction BT
        pa --> pulmcirculation[Pulmonary Vasculature]
        pulmcirculation --> pulmveins
    end
pulmveins[Pulmonary Veins] ==> la[Left Atrium]
rv[Right Ventricle] ==> pa[Pulmonary Artery]
rv-.-aorta
lv-.-pa
lv[Left Ventricle] ==> aorta
ivc ==> ra[Right Atrium]
svc ==> ra
ivc -.- la
svc -.- la
end

Flashcard	type:basic
In congenital heart disease, what is it called when for L-TGA, you chop off the misplaced venous drainage and arteries from both sides of the heart, and reattach them to the correct side?	A double switch
In congenital heart disease, what disease is a double switch procedure carried out for?	LEVOtransposition of the Great Arteries (L-TGA)
In congenital heart disease, what is done surgically in a double switch procedure	You chop off the misplaced venous drainage and arterial exits from both sides of heart, and reattach them to the correct sides.

What do I need to know with an aCHD patient?

What diagnosis?
What surgery?
Things that might cause problems:
Shunt?
- Risk of Paradoxical Emboli
- Effect on oxygenation
- Effect on cardiac output
Left or Right ventricle for systemic?
Increased Pulm Vascular Resistance?
How will PEEP affect CV?
How will changing preload affect CV?
How will vasodilators and negative inotropes affect?
Does the circulation make sense for normal central venous access?

Sources

[Acute Coronary Care]: "Acute Coronary Care"

Adult Congenital Heart Disease ↩↩