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Cardiology > Cyanotic CHD > Flashcards

Cyanotic CHD Flashcards

1
Q

Which conditions present in the first 24 hours of life with cyanosis?

A

TGA
Single Ventricle
Mixing Lesions
Outflow tract obstruction

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2
Q

Which group of conditions present on Day 2+ with cyanosis?

Give examples of these conditions.

A

Lesions that are dependent on the PDA for pulmonary blood flow or mixing.

Pulmonary blood flow = Critical PS, Pulmonary atresia, single ventricle with either of those
Mixing = TGA, TAPVD, PS, pulmonary atresia, Ebstein’s anomaly

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3
Q

What are the physiological and pathological consequences of Cyanosis?

A 
Polycythaemia
Clubbing
Vascular stroke
Bleeding disorders/thrombocytopenia
Scoliosis
Hyperuricaemia and Gout
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4
Q

Which is the most common congenital heart defect in developed countries?

Is it more common in males or females?
In what ratio?

A

TGA

Male 3:1

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5
Q

What makes a TGA compatible with life?

What is the most desirable associated defect and why?

A

If there is an associated defect allowing communication between the two circuits eg PFO, ASD, VSD, PDA

Large ASD allows good mixing with less pressure overload than a VSD

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6
Q

What does the ECG show in TGA?

What does the CXR show?

A

ECG: RVH (upright T wave in V1) or BVH (if large VSD or PDA)
May be RAH

CXR: Cardiomegaly, increased pulmonary vascularity, egg shaped cardiac silhouette

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7
Q

What is the survival procedure to correct TGA?

When is it done?

A

Arterial switch operation.

Performed in the first few days of life.

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8
Q

What does the Echo show in TGA?

A

Double circles instead of circle and sausage.

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9
Q

What other lesion is always associated with truncus arteriosius?

Which other lesions are commonly associated?

Which syndrome is commonly associated?
What fraction of patients with Truncus have this syndrome?

A

Large VSD

Interrupted aortic arch and right sided aortic arch

DiGeorge syndrome - 1/3

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10
Q

How does Truncus Arteriosus usually present?

What is the clinical course if untreated?

A

Cyanosis at birth or in failure within the first few weeks.

CHF will progress and lead to death, or patients can develop Pulmonary Vascular Obstructive Disease.

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11
Q

How is Truncus Arteriosus surgically corrected?

Describe the procedure.

When is the procedure normally done?

A

Various versions of the Rastelli procedure.

  1. Seperate pulmonary arteries from truncus
  2. Connect RV to pulmonary arteries using a conduit
  3. Repair VSD

Performed within the first week of birth/presentation

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12
Q

Which conditions are included in the Single Ventricle spectrum?

A

Hypoplastic Left Heart Syndrome
Double-Outlet Right Ventricle
Doublet-Inlet Left Ventricle
Tricuspid Atresia (causes RV hypoplasia)

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13
Q

How many cases of single ventricle involve the Left heart?
Where does the second great artery arise from?

What is the most commonly associated defect with a single ventricle?
How often does this occur?

What other associated defects are common?

A

80%
A rudimentary chamber off the ventricle

Transposition of the Great Arteries, 85% of cases

Interrupted aortic arch, COA, anomalies of the AV valve

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14
Q

What determines saturations in single ventricle?

What are the consequences of a normal pulmonary valve?

A

Pulmonary blood flow, ie presence or absence of pulmonary stenosis, pulmonary atresia or pulmonary artery stenosis

Excess pulmonary blood flow with lead to congestive heart failure

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15
Q

What does the ECG show in single ventricle?

A

Abnormal Q waves
Ventricular hypertrophy pattern in all precordial leads
First or second degree AV block

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16
Q

What is the first stage of single ventricle repair?
When is it typically performed?

What is the second stage?
When is it typically performed?

A

If there is significant PBF - PA banding
If PBF is low, either Damus-Kaye-Stansel (patch between PA and Aorta) or BT shunt (between right subclavian and Right PA)
Performed in first few days of life

Second stage is Bidirectional Glenn, where SVC is plumbed directly into the pulmonary artery.
Typically done at 3-6 months

17
Q

What is the third stage of repair for single ventricle?
When is it typically performed?
What complications can arise prior to stage 3?

A

Fontan procedure - connects IVC directly to PA
Typically done at 18-24 months
Complications: Thrombosis and shunt obstruction, arrhythmias, Chlyothorax, protein losing enteropathy

18
Q

What at the complications of arterial switch surgery?

A

Coronary artery obstruction
Pulmonary stenosis
Neonaortic stenosis or regurgitation

19
Q

What are the four classical defects seen in Tetralogy of Fallot?

What percentage of congenital heart disease does TOF make up?

In which direction does blood shunt through the VSD?

A

RV outflow obstruction (infundibular pulmonary stenosis), VSD with an overriding aorta and right ventricular hypertrophy

5-10%

Shunt depends on degree or RVOT obstruction:
If mild obstruction, shunt is L>R, patient is acyanotic
If severe obstruction, shunt if R>L, patient is cyanotic

20
Q

Describe what you would hear when auscultating the heart in Tetralogy.

What would a long and loud murmur signify?

What would you see on an ECG of a patient with Tetralogy?

A

Heart Sounds: Ejection systolic murmur at ULSE, grade 3-5/6
Single S2, Ejection click in aortic area

Loud murmur means mild pulmonary stenosis

ECG: Right axis deviation, right ventricular hypertrophy

21
Q

What causes a “Tet” spell?

What happens to the cardiac shunt? And the heart murmur?

What does that mean for pulmonary blood flow?

What are the possible consequences of a “Tet” spell?

A

Drop in systemic vascular resistance or increase in heart rate eg crying, activity, defaecation.

Cardiac shunt increases from R>L.
Murmur disappears or gets quieter because there is minimal blood flow through the pulmonary valve.

Pulmonary blood flow decreases hence the hypoxia.

Can cause stroke, seizures, death.

22
Q

How do you treat a “Tet” spell?

How does each treatment help?

A

Squatting/Knee-chest position - Reduces systemic vascular return
Oxygen - Improve saturations
Morphine - Respiratory depression
Sodium bicarbonate- Corrects acidosis caused by poor perfusion
Vasoconstrictors eh phenylephrine - increases SVR
Ketamine - Sedation and increase SVR

23
Q

How is Tetralogy treated medically?

How is it corrected surgically?
When is the procedure normally done?
Why might it be done earlier?

A

Propranolol can prevent “Tet” spells.
Important to treat iron deficiency.

May require a BT shunt initially if pulmonary atresia or hypoplastic pulmonary artery.
Full repair involves widening of RVOT and closure of VSD, usually done at 1-2y of age. Performed earlier if sats <75% or frequent spells.

24
Q

What percentage of patients with TOF have Pulmonary Atresia?

Where does pulmonary blood supply come from if this is the case?

How will these patients present?

A

15-20%

Either PDA or collaterals

Present with cyanosis at birth, no murmur but loud single S2

25
Q

What is the path of deoxygenated blood in Tricuspid Atresia?

How does blood become oxygenated?

What defect is usually also present?

A

Deoxygenated blood enters the right atrium and then passes into the left sided circulation via a PFO/ASD.

Mixed blood from the left ventricle will either:

  • Pass through a VSD into the hypoplastic right ventricle and then into the pulmonary arteries
  • Enter the pulmonary arteries via a PDA

Pulmonary stenosis is usually present (often also associated with TGA)

26
Q

What does the ECG show in Tricuspid Atresia? And why?

What does the CXR show?

A

Right atrial enlargement (P pulmonale/peaked P waves) from R>L shunt cause right atrial hypertrophy
Left ventricular hypertrophy occurs because LV is receiving both systemic and pulmonary circulation
LVH = High voltage R waves in V5/6, high voltage S waves in V1/2, tall T waves in V5/6

CXR: Normal heart size or slight cardiomegaly (RA/LV), decreased pulmonary vascular markings

27
Q

Is TAPVD more common in males or females?

What is the defect present?
How are these lesions classified?

Which group causes the most trouble? Why?

A

Males (ratio 4:1)

Pulmonary veins drain into the right atrium via an anomalous/abnormal connection
Classification: Supracardiac, cardiac or infracardiac

Infracardiac lesions because they are most likely to be obstructive

28
Q

What associated lesion is required for survival in TAPVD? Why?

Describe the haemodynamics of obstructive TAPVD.

A

ASD, otherwise oxygenated blood would not enter systemic circulation

Haemodynamics:

  • R>L shunting occurs through ASD
  • There is complete mixing in the right atrium
  • Obstruction of pulmonary venous return causes pulmonary venous hypertension
29
Q

How does obstructive TAPVD typically present?

What does the CXR show?

What are the differential diagnoses?

A

Cyanotic neonate with respiratory distress

CXR shows a small heart or right atrial prominence, with pulmonary oedema

May be mistaken for meconium aspiration, pneumothorax, congenital lung lesion

30
Q

What is the classical CXR sign of a supracardiac TAPVD?
Why does it occur?

What are the classical exam findings of a non-obstructive TAPVD?

A

Snowman sign - dilated brachiocephalic vein/SVC on the top and dilated right atrium on the bottom

Mild cyanosis, respiratory distress, ejection systolic murmur (PS) and diastolic murmur (TS), widely split S2 and S3/S4 present

31
Q

How is TAPVD corrected?

What is the mortality rate of surgery?

What are the complications of surgery?

A

Redirection of pulmonary veins to the LA

5% mortality

Complications: pulmonary hypertension, pulmonary vein obstruction at anastomosis, atrial arrhythmias

32
Q

Which genetic conditions are associated with hypoplastic left heart syndrome?

What is the most common associated lesion?
What percentage of cases have this?

30% of patients have abnormalities in which non-cardiac system?

A

Turners syndrome, trisomy 18 (Edwards), Jacobsen syndrome

Coarctation of the aorta, 75%

Brain abnormalities - microcephaly, holoprosencephaly, agenesis of the corpus callosum

33
Q

What are the typical examination findings for a neonate presenting with HLHS?

What would an ECG show?

What would a CXR show?

A

Tachycardia, cyanosis, weak pulses, dyspnoea, non-specific systolic murmur, pulmonary crackles, gallop rhythm, loud single S2, hepatomegaly

ECG: RVH +/- LVH (leads picking up right heart too)

CXR: Significant cardiomegaly, pulmonary oedema

34
Q

What is Ebstein’s Anomaly?

What happens to the right ventricular outflow tract?

What happens to the right ventricle?

A

An anomaly of the tricuspid valve which involves downward displacement of the septal and posterior leaflets into the RV cavity

RVOT can be obstructed by the redundant valvular tissue

Some of it is incorporated into the right atrium, and the remainder is hypoplastic

35
Q

Where and in which direction is the shunt in Ebstein’s Anomaly?

How does this condition usually present?

What murmur will you hear and where?

A

Right to left shunt through a PFO or ASD

Cyanosis and failure, may be in the first few days of life

Soft holosystolic murmur at the left lower sternal edge (tricuspid regurgitation)

36
Q

How is Ebstein’s anomaly corrected?

A

Depends on how functional the RV is.

Biventricular (Danielson or Carpenter) or univentricular repair (Fontan like procedure)

Cardiology (6 decks)

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