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LMCC Study - Peds > Cyanotic Congenital Heart Disease > Flashcards

Cyanotic Congenital Heart Disease Flashcards

1
Q

What causes cyanosis in cyanotic heart disease?

A
  • Right to left shunt
  • Some of the systemic venous return crosses from the right side of the heart to the left and returns to the body without going first to the lungs
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2
Q

What are the 5 T’s of cyanotic heart disease?

A

1) Tetralogy of fallot
2) Transposition of the great arteries
3) Tricuspid atresia
4) Truncus arteriosus
5) Total anomalous pulmonary venous return

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3
Q

What is the most common cyanotic congenital heart defect?

A
  • Tetralogy of Fallot

- Occurs in 10% of all congenital heart defects

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4
Q

What are the four structural defects in tetralogy of fallot?

A

1) Ventricular septal defect (VSD)
2) Pulmonary stenosis
3) Overriding aorta
4) Right ventricular hypertrophy

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5
Q

What causes the cyanosis in tetralogy of fallot?

A
  • Right to left shunt!
  • Pulmonary stenosis in combination with VSD
  • Increasing severity of stenosis results in more right pressure. This results in increased Right to Left shunting
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6
Q

What are “tet” spells

A
  • Hypoxic spells during which cyanosis occurs during crying or feeding
  • Potentially lethal
  • Unpredictable, thought to be due to spasm of infundibular septum, which acutely worsens right ventricular outflow tract obstruction (Makes pulmonary stenosis worse)
  • Older kids will squat which increases Peripheral vascular resistance and thus lowers R-L shunt (increases L pressure)
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7
Q

What clinical findings are associated with tetralogy of fallot?

A
  • Cyanosis
  • Pulmonary stenosis murmur (harsh systolic ejection murmur)
  • Tet spells (hypoxic)
  • Dyspnea
  • Poor feeding/weight gain
  • Squatting
  • Syncope, convulsions, hemiparesis, death
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8
Q

What is the classic X-ray finding associated with tetralogy of fallot?

A
  • Boot shaped heart
  • Due to small (concave) main pulmonary artery and right ventricle hypertrophy
  • Also:
  • Diminished pulmonary vascular markings
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9
Q

How do you manage a “tet” hypoxic spell?

A
  • Oxygen administration
  • Placing child in the knee to chest position
  • Morphine sulfate to relax pulmonary infundibulum and for sedation
  • If necessary, systemic vascular resistance can be increased using an alpha-adrenergic agonist such as phenylephrine
  • Surgical repair ultimately needed
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10
Q

Describe the murmur associated with tetrology of fallot

A
  • Harsh systolic ejection murmur

- Upper left sternal border (Pulmonic)

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11
Q

What is transposition of the great vessels?

A
  • The aorta arises from the right ventricle and the pulmonary artery from the left ventricle
  • Resulting in desaturated blood returning to the right side of the heart and being pumped out into the body, and oxygenated blood being pumped back to the lungs
  • Without mixing of blood it is fatal
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12
Q

Where are the three areas there can be mixing of oxygenated and deoxygenated blood in a newborns heart?

A

1) Patent foramen ovale or an ASD
2) Ventricular septal defect
3) Patent ductus arteriosus

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13
Q

What are the clinical features of transposition of the great vessels?

A
  • Severe cyanosi hours after birth
  • tachypnea
  • tachycardia
    • Metabolic acidosis often in clinical vignettes ** from tissue hypoxia
  • Single S2 sound (also common for vignettes) * Reason: Aorta is more anterior, pulmonary artery posterior. Making the pulmonic sound too quiet to be auscultated
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14
Q

What is the egg on a string sign? What is it associated with?

A
  • Cardiomediastinal silhouette which appears like an egg on its side
  • Due to abnormal convexity of the right atrial border and left atrial enlargement
  • Seen in transposition of the great vessels
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15
Q

Definitive diagnosis of transposition of the great vessels is achieved by which means?

A
  • Echocardiogram showing transposition of vessels
  • Shows the sites and amounts of blood mixing
  • Also shows any associated lesions
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16
Q

How would you mange a new patient with transposition of the great vessels?

A
  • Prostaglandin E1 (keep ductus arteriosus patent)
  • If hypoxia persists with PgE1 balloon atrial septostomy (further opens foramen ovale)
  • Ultimately Aterial switch operation is needed
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17
Q

What is tricuspid atresia?

A
  • Atresia: Abscence or abnormal narrowing of an opening or passage in the body
  • Absence of tricuspic valve (closed)
  • So all systemic venous return must cross atrial septum
  • A PDA or VSD is needed to allow blood to reach the lungs
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18
Q

What are the clinical manifestations of tricuspid atresia?

A
  • Severe cyanosis
  • Single S2 on auscultation (no blood enters RA so no/low flow across pulmonic valve)
  • Murmur is a VSD is present
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19
Q

What might be seen on ECG in a patient with tricuspid atresia?

A
  • Left ventricular hypertrophy

all blood going to left side of the heart

20
Q

What might be seen on CXR in a patient with tricuspid atresia?

A
  • Decreased pulmonary vascular marking

any pulmonary flow must come through PDA or VSD so less flow

21
Q

What can be seen on Echo in a patient with tricuspid atresia?

A
  • Anatomy including valves
  • Associated lesions (ex. VSD, PDA)
  • Source of pulmonary blood flow (PDA or VSD)
22
Q

How is tricuspid atresia managed?

A
  • Initally dependent on presence of absence of VSD and amount of antegrade pulmonary flow
    a) If no VSD or poor pulmonary flow prostaglandin E1 to maintain ductal patency
    b) Is large VSD PGE1 not needed
  • Surgical correction ultimately needed (result in direct systemic venous return to pulmonary arteries)
23
Q

What is the truncus arteriosus?

A
  • Embryonic structure

- Arterial trunk that originates from both ventricles of the heart and later divides into the aorta and pulmonary trunk

24
Q

What is persistent truncus arteriosus?

A
  • Results from failure of septation of the truncus arteriosus during the first 3-4 weeks of gestation
  • Single arterial trunk arises from the heart with a large VSD immediately below the truncal valve
  • The TA divides to pulmonary artery and aorta further upstream
  • After birth pulmonary pressure decreases so larger amount of blood flow to lungs with less to body
25
Q

What are the clinical manifestations of persistent truncus arteriosus?

A
  • Cyanosis (varying degree depending on amount of pulmonary blood flow)
  • If not diagnosed at birth signs of CHF due to decreased pulmonary resistance (more blood to lungs causing congestion)
  • Tachypnea
  • Cough
  • Bounding peripheral pulses due to diastolic run off into pulmonary arteries
  • SINGLE S2 (only one valve)
26
Q

What creates S1 and S2 sounds?

A

S1: Closure of the mitral and tricuspid valves
S2: Closure of the pulmonic and aortic valves

27
Q

What causes the single S2 in persistent truncus arteriosus?

A
  • There is only one combined valve for aorta/pulmonic artery
28
Q

What causes the single S2 in transposition of the great vessels?

A
  • Pulmonary artery behind aorta, making the sound faint and difficult to auscultate
29
Q

What cause the single S2 in tricuspid atresia?

A
  • Since no blood enters the right atrium there is no blood flow across the pulmonic valve (blood can enter artery through PDA)
  • I imagine there would be second sound with VSD?
30
Q

What can be seen in ECG and CXR in persistent truncus arteriosus?

A
  • Combined ventricular hypertrophy
  • Cardiomegaly
  • CXR will also show increased pulmonary blood flow (less resistance = greater share of blood flow)
31
Q

In a patient with persistent truncus arteriosus what will be seen on echo?

A

Anatomy including:

  • VSD
  • Truncal valve function
  • Origin of pulmonary arteries
32
Q

What is the management for a patient with persistent truncus arteriosus?

A
  • Anticongestive medications

- Surgical repair (close the VSD and create connection between right ventricle and pulmonary arteries

33
Q

What is total anomalous pulmonary venous return?

A

Occurs when the pulmonary veins fail to connect to the left atrium and return abnormally via the right atrium
- An atrial septal defect is required for systemic cardiac output and survival (creating R-L shunt)

34
Q

What are the clinical manifestations of total anomalous pulmonary venous return?

A
  • Mild cyanosis
  • Widely split S2 due to ++ right ventricular volume
  • Systolic ejection murmur (ASD) left sternal border
  • Mid-diastolic murmur low sternal border (Increased flow to tricuspid)
  • Poor growth
  • Tachypnea, dyspnea
  • Right sided heart failure
  • hepatomegaly
  • Pulmonary edema,
  • Crackles
35
Q

What is seen on ECG in patient with TAPVR?

A
  • Right atrial enlargement (larger p waves)
  • Right ventricular hypertrophy
  • Right axis deviation
36
Q

What can be seen on CXR in a patient with TAPVR?

A
  • Cardiomegaly or Normal
  • Increased pulmonary blood flow
  • pulmonary edema
37
Q

What can be seen on Echo in a patient with TAPVR?

A
  • Volume overloaded right side and enlargement
  • ASD with R-L shunting
  • Anatomy (pulmonary vein site, any obstruction there)
38
Q

How is total anomalous pulmonary venous return managed?

A
  • Surgically

- Pulmonary vein opened into L atrium

39
Q

What is hypoplastic left heart syndrome?

A
  • Underdeveloped left side of the heart
  • Occurs when there is failure of development of the mitral valve or aortic valve or the aortic arch
  • Associated with ASD which is needed for life along with PDA
  • A small left ventricle is unable to support
  • MOST COMMON CAUSE OF DEATH from cardiac defects in first month of life
40
Q

What are the clinical manifestations of hypoplastic left heart syndrome?

A
  • Infant becomes critically ill with closure of ductus arteriosus
  • weak pulses
  • Respiratory distress
  • Poor feeding
  • cyanosis
  • grayish colour
  • Mottled skin
  • Cool skin
41
Q

There are two areas where blood is shunted in hypoplastic left heart syndrome explain them?

A

1) Left to right shunt in the atria
- Due to hypoplastic left ventricle and narrowed valves there is high resistance in the left heart which does not allow adequate amounts of blood to pass. An ASD is needed for this blood to pass
2) Right - Left shunt at PDA
- Mixed oxygenated (from atrial shunt) and deoxygenated from systemic venous return pass through PDA to perfuse the body

42
Q

What can be seen on echo in a patient with hypoplastic left heart syndrome?

A
  • Small L heart
  • Degree of stenosis in mirtal and/or aortic valves
  • assess the left to right atrial shunting and right to left ductal shunting
43
Q

How is hypoplastic left heart syndrome managed?

A
  • Prostaglandin E1 (ductal patency)
  • Correction of acidosi, ventillatory and blood pressure support if needed
  • Surgical repair
44
Q

What is an ebstein anomaly?

A

Congenital malformation that is characterized by:

  • Abnormality of tricuspid valve
  • Abnormality of right ventricle
45
Q

What positional change will help a child in a tet spell?

A

Knees to chest

LMCC Study - Peds (12 decks)

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