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Gen Med Unit 7a: Cardio 2 > Congential Heart Diseases: Cyanotic > Flashcards

Congential Heart Diseases: Cyanotic Flashcards

1
Q

What are the characteristics of cyanotic congenital heart defects?

A
  1. Around the mouth & lips, fingertips, toes
  2. All have right to left shunts
  3. Many CHD are ductus dependent
    A. Neonate may not have sx’s until DA closes in the first few days of life
    B. May not be diagnosed until after discharge
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2
Q

What are the cyanotic heart diseases?

A
  1. Tetralogy of Fallot: 7-10% of cases of CHD
  2. Pulmonary Atresia/IVS: 1-3% of all CHD
  3. Hypoplastic Left Heart Syndrome: 2-3% of all CHD
  4. Transposition of Great Vessels: about 3% of CHD
    A. Dextro-TGA most common type
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3
Q

What are the 4 anomalies in the tetralogy of Fallot?

A
  1. Subaortic septal defect (VSD)
  2. Pulmonary stenosis
    (RV outflow obstruction)
  3. Overriding Aorta (dilated)
  4. Right ventricular hypertrophy (RVH)
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4
Q

What may be asst. with tetralogy of Fallot?

A 
1. Down Syndrome
A. Trisomy 21
B. 50% have a type of CHD
2. Alagille Syndrome
A. Mutation in JAG1
3. DiGeorge & Velocardiofacial Syndromes
A. Microdeletion syndrome (chromosome 22q11)
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5
Q

What ths pathophys of ToF?

A
  1. Largely dependent upon the degree of right ventricular outflow obstruction (Pulm Stenosis)
  2. Direction of blood flow across the VSD determined by the path of least resistance for blood flow
    A. Predominate a left-to-right shunt
    B. Patient acyanotic
    C. Right-to-left shunt across the VSD
    D. Large volume of desaturated blood entering the systemic circulation
    E. Cyanosis and polycythemia
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6
Q

Define Tet spell or hypercyanotic spell

A

In the most dramatic situation, there can be near occlusion of the right ventricular outflow tract with profound cyanosis

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7
Q

What are the sxs of tof dependent on?

A

Clinical presentation depends upon the degree of pulmonary stenosis
More severe stenosis leads to greater reduction of pulmonary blood flow and increased cyanosis

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8
Q

What are the sxs of severe obstruction?

A

profound cyanosis

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9
Q

What are the sxs of moderate obstruction?

A

Heart murmur

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10
Q

What are the sxs of minimal obstruction?

A

Heart failure

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11
Q

What are the common sxs of ToF?

A
  1. Most children are symptomatic and cyanotic
  2. Right ventricular thrill
  3. Harsh systolic ejection murmur (SEM) along left mid to upper sternal border with radiation posteriorly
  4. Murmur may disappear during “tet” spells due to shunting of blood away from obstruction
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12
Q

What are the dx studies for ToF?

A
  1. ECHO
  2. CXR
  3. EKG
  4. Cardiac Cath
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13
Q

What are the results of ECHO in ToF?

A

Confirms Dx of four cardiac anomalies

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14
Q

What are the results of EKG in ToF?

A

RAE, RVH, R Axis Deviation

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15
Q

What are the results of CXR in ToF?

A
  1. Boot shaped heart
  2. Upturned apex
  3. Right aortic arch in 25% cases
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16
Q

What are the results of Cardiac cath in ToF?

A

Delineate degree of outflow obstruction

17
Q

What is the tx for ToF?

A
  1. Intracardiac repair

2. Patch closure VSD and repair of pulmonary stenosis

18
Q

How can tet spells be managed?

A
  1. Knee-chest position to ↑ systemic vascular resistance
    A. Promotes movement of blood from right ventricle to pulmonary circulation rather than LV
  2. Pharmacologic measures
    A. Morphine, fluids, beta blockers
19
Q

Describe pulmonary valve atresia

A
  1. Anatomic abnormalities:
    A. Pulmonary valve is closed
    -Complete obstruction to . RV outflow
    B. Varying degrees of RV hypoplasia and tricuspid valve hypoplasia
  2. Intact ventricular septum (PA/IVS)
20
Q

What are the physiologica consequences of pulmonary atresia?

A
  1. Pulmonary valve does not open
  2. Pulmonary blood flow is dependent on PDA
    A. Blood flow from aorta to pulmonary artery
    B. PDA is crucial for survival in neonate
    -Sole source of pulm blood supply
21
Q

What are the sxs of pulmonary atresia?

A 
1. Cyanotic
A. newborn can usually be diagnosed w/IN hours or minutes after birth
2. Hypoxic on room air & on 100% oxygen
3. Mild tachypnea
4. Single 2nd heart sound (S2 not split)
22
Q

What are teh CXR results in pulmonary atresia?

A

CXR

Cardiomegaly

23
Q

Wha tsi the treatment for pulmonary atresia?

A
  1. Only source of pulmonary blood flow is apatent ductus arteriosus
  2. Stabilize neonate w/ prostaglandin E1 infusion to keep DA patent, then surgical repair
24
Q

What is the prognosis if pulmonary atresia is unrepaired?

A

almost uniformly fatal disease

W/in 2 wks, approx 50% die & by 6 mos 85% will die

25
Q

Describe hypoplastic left heart syndrome

A
  1. Underdeveloped left heart w/normally placed great vessels , AND hypoplasia of the ascending aorta and arch
    A. Hypoplasia of LV including atresia, stenosis or hypoplasia of the aortic or mitral valve, or both valves
26
Q

What can HLHS lead to?

A

LV unable to support systemic circulation

Can lead to cardiogenic shock, ventilatory insufficiency, and death

27
Q

What is the initial tx for HLHS?

A

Initial treatment - stabilize neonate
Maintain patency of DA via prostaglandin E1 infusion
Need PDA and ASD initially to survive
Inter-atrial mixing of oxygenated and deoxygenated blood necessary to survive

28
Q

What is the definitive tx for HLHS?

A

Long Term treatment is surgery

Staged repair or heart transplant

29
Q

Describe transposition of the great arteries

A

Complete transposition of Aorta and PA

30
Q

What is the pathophys of d-TGA?

A
  1. Deoxygenated blood from the right heart is pumped thru the aorta bypassing the lungs altogether
  2. Left heart pumps oxygenated blood continuously back into the lungs through the PA
  3. 2 separate “circular” (parallel) circulatory systems are created
31
Q

What is simple d-TGA?

A

NO other heart defects

32
Q

What is complex d-TGA?

A 
Co-existing cardiac anomalies (Complex d-TGA)
VSD
50% cases
L ventricular outflow tract obstruction
25% cases
33
Q

What are the sxs of d-TGA?

A
  1. Cyanosis
  2. Clubbing of the fingers or toes
  3. Poor feeding
  4. Shortness of breath
34
Q

How is d-TGA diagnosed?

A
  1. Difficult to diagnose on fetal ultrasound
  2. Most cases diagnosed by clinical suspicion of cyanotic congenital heart Dz
  3. Dx confirmed by echocardiography
35
Q

How is d-TGA treated?

A 
1. Prostaglandin infusion
A. Maintain open Ductus Arteriosus 
2. Balloon Atrial Septostomy 
A. Cath lab or bedside
3. Anatomical surgical correction
A. Arterial switch operation

Gen Med Unit 7a: Cardio 2 (8 decks)

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