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Congenital Heart Defects Flashcards

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1
Q

What are the congenital heart defects?

A
  • Ventricular septal defect
  • Pulmonary stenosis
  • Atrial septal defect
  • Persistent ductus arteriosus
  • Aortic valve stenosis
  • Coarctation of the aorta
  • Transposition of the great arteries
  • Tetralogy of Fallot
  • Hypoplastic left heart syndrome
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2
Q

What congenital heart diseases are acyanotic?

A
  • ASD
  • VSD
  • PDA
  • PV stenosis
  • Coarctation of the aorta
  • Aortic stenosis
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3
Q

What congenital heart diseases are cyanotic?

A
  • Tetralogy of Fallot
  • Pulmonary atresia
  • Tricuspid atresia
  • Hypoplastic left heart syndrome
  • Transposition of the great arteries
  • Total anomalous pulmonary venous return
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4
Q

What is acyanotic heart disease?

A

Heart defects presenting without cyanosis caused by fetal heart malformation. Can lead to heart failure

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5
Q

What can ASD, PDA, and VSD cause?

all acyanotic heart disease

A
  • Left to right shunt
  • Oxygenated blood flows redundantly through pulmonry circulation and becomes Eisenmenger syndrome over time
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6
Q

Signs and symptoms of acyanotic heart disease

A
  • Sometimes asymptomatic, can lead to heart failure, Eisenmenger syndrome
  • Poor feeding/failure to thrive
  • Fluid retention
  • Pulmonary congestion
  • Hepatomegaly
  • Respiratory distress
  • Elevated JVP
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7
Q

What is Eisenmenger syndrome?

A

Cyanosis, palpitations, dyspnea, chest pain, and syncope with exertion

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8
Q

What are 2 categories of acyanotic congenital heart disease?

A

Left to right shunts and outflow obstruction

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9
Q

What causes outflow obstruction leading to acyanotic heart disease?

A
  • Pulmonary stenosis
  • Aortic stenosis
  • Coarctation of aorta
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10
Q

Left to right shunt lesions leading to acyanotic heart disease

A
  • Atrial septal defect
  • Ventricular septal defect
  • Atrioventricular septal defect
  • Patent ductus arteriosus
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11
Q

Hole in heart wall dividing left and right atria

A

Atrial septal defect

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12
Q

Signs and symptoms of atrial septal defect

A
  • Fixed, split S2 and pulmonic ejection murmur (louder with age)
  • Infants and children: respiratory infections, failure to thrive
  • Adults (before 40): palpitations, exercise intolerance, dyspnea, fatigue
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13
Q

Development of the atrial septum

A
  • Septum primum develops leaving ostium primum “first opening”
  • Septum primum closes
  • Ostium secundum “second opening” develops in septum primum
  • Septum secundum has opening for foramen ovale
  • Complete closure at birth
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14
Q

What are types of ASD?

A
  • Ostium secundum - at site of foramen ovale and ostium secundum, most common, associated with fetal alcohol syndrome
  • Ostium primum - at level of TV and MV endocardial cushion defect found in 25% of Down’s syndrome
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15
Q

Diagnosis of ASD

A
  • Chest x ray: right heart dilation and prominent pulmonary vascularity
  • Transesophageal echocardiography: visualize size and location accurately
  • Right heart catheterization shows increased oxygen saturation in right atrium, right ventricle, pulmonary artery
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16
Q

Treatment of ASD

A
  • Surgery
  • Percutaneous surgical closure
  • In asymptomatic child with hemodynamically significant defect, closure performed electively at 1-3 years before late complications of RV dysfunction and dysrhythmias occur
  • Closure of moderate to large defect when child between 4 and 6 (defects >8 mm unlikely to close on own)
  • Adults: surgery in cases of R ventricular enlargement, paradoxical embolism, right to left shunt
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17
Q

Hole in septum dividing lower two chambers of heart leading to more blood pumped into lung and pulmonary artery

A

Ventricular septal defect

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18
Q

What does increased blood pumped into lung and pulmonary artery in VSD cause?

A
  • Heart failure
  • Pulmonary HTN
  • Arrhythmias
  • Stroke
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19
Q

Development of ventricles

A

Membranous region grows downward and muscular ridge grows upward, they don’t match up in VSDs (majority in membranous region)

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20
Q

3 types of VSD

A
  • Membranous: upper septum (MC)
  • Muscular: lower septum
  • Intlet: posterior portion of the V septum beneath the TV
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21
Q

What is the most common congenital heart disease?

A

VSD

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22
Q

What does VSD cause?

A

Acyanotic left to right shunt between ventricles

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23
Q

How is size related to treatment of VSD

A
  • Small- moderate: 3-6 mm usually asymptomatic and 50% close spontaneously by 2
  • Moderate- large: almost always have symptoms and will require surgical repair
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24
Q

What does increased blood volume in the right ventricle due to VSD lead to?

A

Pulmonary hypertension and Eisenmenger syndrome

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25
Signs and symptoms of VSD
* Asymptomatic in utero * At birth: holosystolic murmur (loud, high-pitched) located at lower left sternal border * Small --> asymptomatic, murmur * Large --> sweating, poor feeding/failure to thrive, respiratory infections, murmur plus thrill and diastolic rumble in mitral * Signs of congestive heart failure * Eisenmenger's syndrome
26
What are signs of congestive heart failure in VSD?
* Dyspnea * Persistent cough * Pulmonary vascular resistance
27
VSD diagnostic imaging
* Chest X ray: unreliable but may indicate left atrial enlargement, right ventricular hypertrophy, left ventricular hypertrophy, or pulmonary artery enlargement * Echocardiogram determines location and size * MRI used if echo does not diagnose
28
If echo and MRI do not diagnose and individual still has pulmonary hypertension in suspected VSD, what diagnostic tool should be used?
* Cardiac catheterization
29
What would be seen on ECG in VSD?
Left ventricular hypertrophy, may also see right ventricular hypertrophy and left and right atrial enlargement
30
Treatment of VSDs
* Most small close on own * Medical management with diuretics and higher calorie feeds for symptomatic patients * Surgery for larger shunts by age 2 to prevent pulmonary hypertension with patch (preferred) or transcatheter closure
31
Indications for surgical closure of VSD
* Large VSD with medically uncontrolled symptomatology and continued FTT * Pulmonary HTN * Aortic insufficiency * LA/LV dilation
32
Persistence of normal fetal vessel that joins the PA to the aorta, normally closes in 1st weeks of life
Patent ductus arteriosus
33
Risk factors for patent ductus arteriosus
Preterm infants <1500 grams and infants born at higher altitudes >10,000 ft Females > Males Can be associated with other defects, ie coarctation or VSD
34
What does ductus arteriosus become after birth?
Ligamentum arteriosum
35
Signs and symptoms of small PDA
* Usually asymptomatic * Neonates: holosystolic "machine-line" murmur on auscultation * infants, children, adults: continuous murmur
36
Signs and symptoms of moderate PDA
* Exercise intolerance * Continuous murmur * Wide systemic pulse pressure * Displaced ventricular apex
37
Signs and symptoms of larger PDA
* Infants: leads to heart failure * Children: shortness of breath, fatigability, Eisenmenger syndrome
38
Diagnoses of PDA
* Echocardiogram: 2D suprasternal * CXR: normal/cardiomegaly * ECG: left ventricular hypertrophy, left atrial enlargement
39
Treatment of PDA
* Small asymptomatic PDA: monitor * Neonates (10-14 days): close PDA using prostaglandin inhibitor * Symptomatic/large PDA during heart failure: digoxin, furosemide * Surgery for symptomatic moderate/large PDA
40
Symptomatic moderate/large PDA surgery recommendations
* Closure for symptoms of left to right shunting, left sided volume overload, reversible pulmonary arteries hypertension * Children < 5 kg/11 lbs: surgical ligation * >5 kg/11 lbs: percutaneous occlusion, surgical ligation for large PDA
41
What are 3 possible scenarios arising from pulmonary stenosis
* Stenosis of valve itself: 3 leaflets either thickened or fused * Thickened muscle below valve makes tight * Stenosis of pulmonary artery below valve
42
What does pulmonary stenosis lead to?
* Right-sided heart failure * Microangiopathic hemolytic anemia --> schistocytes (d/t going through tight valve)
43
Pathophysiology of pulmonary stenosis
* Obstruction of blood flow across pulmonary valve * Increased work by R ventricle --> hypertrophy * If obstruction severe, R-L shunt (Eisenmenger syndrome) at atrial level through PFO * If critical pulmonary stenosis in neonates, only way to get blood to lungs is through PDA ---> prostaglandin given at time of birth to keep PDA open
44
Signs and symptoms of pulmonary stenosis
* Asymptomatic with normal health if mild to moderate PS * Normal pulses * May show symptoms later in adolescence or adulthood * Systolic ejection murmur at the LUSB that increases with inspiration * S2 followed by opening click that becomes louder with expiration * RV life on palpation of precordium
45
Diagnosis of pulmonary stenosis
* Diagnostic imaging: CXR: normal heart size * Post stenotic dilation of main pulmonary artery and left pulmonary artery * EKG: usually normal with mild obstruction, RVH with moderate to severe PS * Echo: confirms diagnosis, defines anatomy, identifies lesions * Cardiac cath/reserved for therapeutic balloon valvuloplasty
46
Treatment of pulmonary stenosis
* Mild-moderate: no intervention * Moderate-severe symptoms: undergo intervention * Percutaneous balloon valvuloplasty = procedure of choice * Surgery when balloon valvuloplasty unsuccessful
47
What is the procedure of choice for pulmonary stenosis?
* Percutaneous balloon valvuloplasty: as effective as surgery in relieving obstruction and causes less valve insufficiency
48
Part of aortic arch is narrower than usual causing blockage of normal blood flow to the body, backing blood flow into the left ventricle causing muscles to work harder to get the blood out of the heart
coarctation of the aorta
49
Most females with coarctation of the aorta have what?
Turner syndrome
50
What is the leading cause of heart failure in the first month of life?
Coarctation of the aorta alone or in combination with VSD, ASD, or other congenital cardiac anomalies
51
What issues does coarctation of the aorta
* Upstream issues: blood flow increases into aortic branches before coarctation --> blood flow, pressure increases in upper extremities, head * Downstream issues: decreased blood flow, decreased pressure in lower in lower extremities, kidneys receive less blood --> activate RAAS --> secondary hypertension
52
Types of coarctation of the aorta
* Preductal coarctation: associated with Turner syndrome, PDA. May go unnoticed unless severe. Presents as postductal coarctation * Postductal coarctation: distal to ligamentum arteriosum, presents in adulthood, blood pressure higher upstream and lower downstream
53
Signs and symptoms of PDA
* Depends on presence and severity of PDA * Systolic murmur: diamond-shaped murmur * Diastolic murmur: high-pitched decrescendo murmur Infants * Lower extremity cyanosis * Absent or delayed femoral pulse * Failure to thrive/poor feeding * Blood pressure higher in upper extremities compared to lower extremities Secondary hypertension: severe heart failure, shock if/when PDA closes
54
What symptoms of coarctation of the aorta may be more apparent with age?
* Chest pain, cold extremities, claudication on exertion * Left ventricular impulse palpable, sustained * Pulsations felt in intercostal spaces
55
What are signs and symptoms of coarctation of the aorta in adults?
* Hypertension * Hypotension in lower extremities * Bilateral lower extremity claudication * Dyspnea on exertion * Delayed/weak femoral pulses
56
Diagnostics of coarctation of the aorta
* Angiogram: visualize narrowing in aorta, anatomy, & severity * CXR: rib notching: 3 sign (narrowed aorta resembles notch of number 3 due to prestenotic of aortic arch and postenotic of descending aorta dilatation) * Echocardiogram: visualize location, size, blood turbulence * ECG: left ventricular hypertrophy, left atrial enlargement
57
Treatment of coarctation of the aorta
Medications * Prostaglandin E to increase flow to lower extremities and keep PDA open Surgery * Resection with end-to-end anastomosis * If unfeasible, bypass graft across area of coarctation * Long-segment coarctation: subclavian aortoplasty * Prosthetic patch aortoplasty * Balloon angioplasty with possible stent
58
Prognosis of coarctation of the aorta
* Survival through neonatal period without developing heart failure tend to do well throughout childhood and adolescence * Infective enocarditis rare before adolescence, but can occur in both repaired and unrepaired coarctation * Correction after age 5 are at increased risk for HTN and myocardial dysfunction * Exercise testing is mandatory for these children prior to their participation in athletic activities
59
Obstruction to the outflow from the left ventricle at or near the aortic valve
Aortic stenosis
60
Are males or females more likely to get aortic stenosis?
Males
61
What are the 3 types of aortic stenosis?
* Valvular (75%) * Subvalvular (23%) * Supravalvular (2%) Occasional severe heart failure when critical obstruction at birth
62
What are signs of aortic stenosis?
* Harsh, systolic ejection murmur at upper right sternal border with radiation to the neck * Systolic ejection click at the apex
63
Diagnostics for congenital aortic stenosis
* CXR: dilation of ascending aorta * Echo: standard for diagnosis * Catheterization for patients whose resting gradient reached 60-80 mmHg and intervention planned
64
Treatment of aortic stenosis
* Percutaneous balloon valvuloplasty: standard initial treatment with valvular AS * In subvalvular or supravalvular AS, interventional cath not effective: surgery is required * Consider surgery in symptomatic patients with a high resting gradient * Ross procedure = alternative for mechanical valve placement in infants and children
65
What is the Ross procedure?
* Patient's pulm valve is moved to the aortic position, and an RV to pulmonary artery conduit is used to replace the pulm valve
66
Prognosis of aortic stenosis
* All tend to be progressive * Mild-moderate obstruction: normal oxygen consumption and maximum exercise capacity. Children with normal resting and exercise EKGs may safety participate in vigorous physical activity, including non-isometric competitive sports * Severe obstruction: predisposed to ventricular dysrhythmias and refrain from vigorous activity; avoid all isometric exercise
67
Bluish discoloration of skin and mucous membranes Presence of >3 g/dL deoxygenation HgB correlates with 80-85% SpO2
Cyanosis
68
What can cyanosis be misdiagnosed or missed in?
* Dark races and anemia * Can be mistaken as CCHD if acrocyanosis, pulmonary causes
69
What is hyperoxia test?
* Tool in absence of echo for CHD * Differentiates cardiac and no cardiac causes of cyanosis * Prostin may be initiated based on results of test * PaO2>250 = no structural cyanotic heart disease * <100 mmHg intracardiac shunting, initiate prostin * 100-250 intracardiac mixing lesions
70
A pAO2 >100 mmHg suggests cyanotic ----- disease. Little or no change in PAO2 suggests cyanotic ---- disease
Lung, heart
71
In order to screen a newborn for cyanotic congenital heart disease, how old must they be?
>24 hours of age and more accurate if older
72
What are the 5 T's of cyanotic heart disease?
* Truncus arteriosus * TGA (d-transposition of the great arteries) * Tricuspid atresia * Tetralogy of fallot * total anomalous pulmonary venous return * Pulmonary atresia * Ebstein's anomaly
73
What are the 4 components of tetralogy of fallot?
* Pulmonary stenosis * Large VSD * Overriding aorta * Right ventricular hypertrophy
74
This is the most common cause of cyanosis in infancy/childhood
tetralogy of fallot
75
What is severity of cyanosis in tetralogy of fallot proportional to?
Severity of RVOT
76
What does pulmonary stenosis cause in tetralogy of fallot?
* Harder for deoxygenated blood to get to pulmonary circulation causing right ventricular hypertrophy * Blood cannot get to pulmonary artery so gap between ventricles allows blood shunting (ventricular septal defect)
77
What is seen on xray in right ventricle hypertrophy?
Boot sign
78
How does blood move through the ventricular septal defect in tetralogy of fallot?
Right to left due to increased pressure in R ventricle
79
The overriding aorta in tetralogy of fallot receives blood from where?
Point at which there is VSD so mixed oxygenated and deoxygenated blood
80
Tet spell clinical presentation
* Hyper cyanotic episode * Decreased SVR causing increased R --> L shunt, increasing cyanosis * Lifethreatening if untreated
81
What are triggers for tet spell?
* Agitation (worsens sub pulmonary stenosis obstruction) * Decreased SVR (hot bath, fever, exercise)
82
How are tet spells managed?
* Squatting when cyanosis begins --> kinks femoral arteries --> Increases vascular resistance in peripheral arteries --> increase pressure in systemic circulation --> increase pressure in L >R side --> shunt temporarily reverses * Keep calm * Give oxygen * IV fluids * Meds that improve pulmonary blood flow * Morphine IV (decrease agitation, decrease dynamic RVOT obstruction) * Bicarbonate to correct metabolic acidosis (decrease PVR) * Phenylephrine (to increase SVR) * Beta-blocker (decrease dynamic RVOT obstruction) * Surgical repair
83
When should tet spell be surgically repaired and how?
most recommend complete repair during neonatal or infant period regardless of patient size * Balloon dilation, stent placement, all eventually require open heart surgery. Complete repair before age 2 usually produces good result and patients live well into adulthood
84
What is transposition of the great arteries?
* Pulmonary artery and aorta are switched in position (transposed) and aorta comes off RV and PA comes off LV * Considered critical heart defect
85
What are the levels of transposition of great arteries
* d-TGA: (dextro) aorta on right, complete * l-TGA: (levo) aorta on left, congenitally corrected --> ventricles, valves switched. Great vessels in normal orientation, but connected to wrong ventricle. Normal blood flow circuits preserved
86
What happens in d-transposition of the great arteries?
* blood from body never gets oxygenated * blood to lungs never gets deoxygenated
87
What happens in l-TGA?
* Adults at risk of heart failure * Acyanotic * Tricuspid valve and right ventricle carry blood to the aorta (designed for lower pressure) * Mitral valve and left ventricle on R carry blood to pulmonary artery (built for high pressure)
88
What are outcomes of d-TGA?
Death unless pulmonary and systemic mix via foramen ovale, VSD, or ductus arteriosus
89
What is the second most common cyanotic congenital heart defect?
Transposition of the great arteries
90
What sex has a greater risk of TGA?
Males
91
If left untreated, transposition of the great arteries can lead to what?
Severe, life threatening hypoxemia
92
What is the pathophysiology of transposition of the great arteries?
* Survival impossible unless way to mix pulmonary and systemic circuits * Majority of mixing at atrial level so interatrial communication (PFO or ASD) critically important, without this patient will be severely cyanotic at birth * Left unreapired transposition is associated with high incidence of early pulmonary obstructive disease
93
Risk factors for TGA
* Diabetes * Rubella * Poor nutrition * Consumption of alcohol * >40 years old
94
Signs and symptoms of d-TGA?
In utero asymptomatic * Cyanosis, unchanged with supplemental oxygen * Tachypnea * Acidosis
95
Signs and symptoms of l-TGA
Asymptomatic
96
Diagnostic imaging for TGA
* Echocardiogram: evaluate heart function, structure * CXR: classic triad of egg on a string/egg on its side appearance, lung congestion, cardiomegaly * Angiogram: pre-surgery
97
Treatment of TGA
* Medication: prostaglandin E: short term to keep ductus arteriosus open * Surgical: balloon atrial septostomy as short term solution(hole created in atrial septum), surgically switch great vessels
98
What is hypoplastic left heart syndrome?
* Underdeveloped left ventricle, ascending aorta * Aortic/mitral valves may be affected, narrow, or absent (atresia) * If untreated: left sided heart failure --> cardiogenic shock --> death
99
What does patient with hypoplastic left heart syndrome need to survive outside of the womb?
ASD or PDA * Right heart functions normally --> oxygenated blood enters left atrium --> blood backs up due to small mitral valve, small left ventricle --> high pressure in left atrium, blood cirulated ineffectively by left ventricle
100
What happens in hypoplastic left heart syndrome if ASD, PDA is present?
* Right heart function present but impaired; sometimes asymptomatic at birth * Oxygenated blood in left atrium flows into right atrium through ASH --> pulmonary artery --> PDA --> aorta --> body * Within 1 day ductus arteriosus begins closing --> cyanosis
101
Signs and symptoms of hypoplastic left heart syndrome
* Cyanosis * Respiratory distress * Poor feeding/failure to thrive * Left-sided heart failure * Cardiogenic shock * Death if not treated
102
Diagnosis of hypoplastic left heart syndrome
* Prenatal ultrasound * Chest x-ray: cardiomegaly * ECG: Right ventricular hypertrophy after birth
103
Treatment of hypoplastic left heart syndrome
Medication: prostaglandin E: short-term solution to keep ductus arteriosus open Surgical: 3 step surgery * 1-2 weeks old: Norwood (new aorta to RV and tube to PA) * 4-6 months: bidirectional glenn (PA &SVC connection; upper body blood to lungs) * 2 years old: Fontan (PA &IVC; lower body blood to lungs)
104
What are activity restrictions on patients with hypoplastic left heart syndrome
With surgical repair can participate in recreational activities but restricted from competitive and vigorous activities
105
6 common innocent murmurs of childhood
* Newborn murmur * Peripheral pulmonary artery stenosis * Still's murmur * Pulmonary ejection murmur * Venous hum * Neck/supraclavicular "carotid bruit"
106
What are characteristics of newborn murmur
* First few days of life * LLSB without radiation * Soft SEM * Resolves by 1 mo
107
What are characteristics of peripheral pulmonary artery stenosis
* Caused by normal branching of PA * ULSB, back, axillae * SOft SEM * Disappears by 2 * Echo needed to differentiate from harmful
108
Characteristics of Still's murmur
* Most common innocent murmur of childhood * 2-7 yo * Apex and LLSB * Vibratory, soft systolic * Loud when supine; disappears when inspiration or sitting * Loud with anemia and fever
109
Characteristics of pulmonary ejection murmur
* Age 2 and older * Soft, grade I-II at ULSB
110
Characteristics of venous hum
* After age 2 * Right infraclavicular area * Continuous, musical, grade I-III
111
Characteristics of neck/supraclavicular carotid bruit
* Supraclavicular area aortic stneosis below clavicle * Brief, physiologic * SEM, harsh, grade II-III

Pediatrics (12 decks)

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