Cardiology Flashcards
1
Q
Cardiac Anomalies seen in Marfan syndrome
A
Mitral valve prolapse
Aortic regurgitation
Dilated aorta
Aortic aneurysm
2
Q
Murmur heard with ASD
A
Low pitched systolic ejection murmur at base with fixed split S2
3
Q
Murmur heard with Aortic Stenosis
A
Systolic ejection murmur radiating to neck
4
Q
Murmur of pulmonary stenosis, PDA, coarctation
A
Systolic ejection murmur radiating to back
5
Q
Murmur of VSD and mitral regurgitation
A
High pitched short systolic regurgitant murmur at the apex or LLSB
6
Q
What are the symptoms of Neurocardiogenic Syncope (benign)?
A
Prodrome – grey-out, nausea
Short lived (usually 1-2min) never > 5 min
Often associated with position change
Not during exercise (though may follow exercise)
May be situational (see blood)
Not associated with palpitations or chest pain
Usually occurs in otherwise healthy children
Often a family history of neurocardiogenic fainters
Normal examination
7
Q
What are the symptoms of cardiac syncope?
A
Little or no prodrome Prolonged LOC (>5 min) Exercise-induced Fright/startle induced Associated chest pain or palpitations History of cardiac disease – AS, Pulmonary hypertension Positive family history for: – Long QT, arrhythmia syndromes, devices – Cardiomyopathy – Sudden death May have abnormal exam
8
Q
What symptoms on history would make you think about long QT?
A
History
– Details of any “events” – palpitations, syncope, SOB
– Sudden onset palpitations or syncope with exercise or startle/fright
– “unusual” Seizures, or seizures not responsive to usual medications
– Palpitations while swimming
– Deafness
– Family history of sudden death, unexplained MVA, drownings, deafness
9
Q
What exam findings are in keeping with long QT syndrome?
A
Normal Examination
10
Q
Equation for Qtc
A
QTc= QT/√RR
11
Q
What are normal ranges for prolonged QT
A
Boys older than 10 years <0.45
Girls <0.47
12
Q
Causes of prolonged QT
A
Long QT interval not the same as long QT syndrome
– Low Ca, Low Mg, Low K, Drugs (eg. tricyclic
antidepressants)
13
Q
Work up of prolonged QT
A
• Suspected long QT syndrome or borderline QT interval:
– Careful history and physical. If still unclear or risk factors:
• Schwartz Score – available online
• Serial ECG’s on child
• Parental ECG’s may diagnose a patient with borderline long QT if parent has long QT on their ECG
• Echo
• Holter
• Exercise test – NB recovery period
14
Q
Treatment for prolonged QT
A
• Beta blockers are first line therapy
15
Q
Bad complications prolonged QT
A
Polymorphic VT: torsades de pointes
16
Q
Causes of torsades de pointes
A
• Occurs in long QT syndrome and hypomagnesemia
17
Q
What is WPW?
A
Accessory atrioventricular pathway allows “early” depolarization of ventricles (pre-excitation)
18
Q
What conditions are associated with WPW?
A
Associated with Ebsteins and CCTGA
19
Q
What are the EKG findings found with WPW?
A
Seen only in sinus rhythm
– Short PR interval
– delta wave
20
Q
What are the side effects/arrhythmias seen with WPW?
A
• Can cause both SVT (retrograde conduction through accessory pathway) and sudden death (rapid antegrade conduction of atrial arrhythmia)
21
Q
Treatment for WPW
A
• Optimal treatment controversial
– No symptoms – No treatment
– SVT – beta blockers or ablation (no digoxin)
– Fainting with palpitations - ablation
22
Q
Most common diagnosis/CHD with trisomy 21
A
AVSD (RAD)
VSD (LAD)
23
Q
EKG with VSD
A
NSR, normal axis, biventricular hypertrophy, RV strain
24
Q
EKG with LAD: what is the differential?
A
– Left axis deviation
• AVSD
• Small RV (eg tricuspid atresia)
• Noonan Syndrome
25
LV hypertrophy EKG findings?
– LV Hypertrophy
• Tall R waves for age V6
• Deep S waves for age V1
26
RV hypertrophy EKG findings?
```
– RV Hypertrophy
• Tall R waves for age V1
• Deep S waves for age V6
• Q waves in V1
• Upright T waves in V1 after day 5 and before “latency age” 9-10 years
```
27
Who gets the RSV vaccine?
– CPS Recommends immunization for children under 1
year old with cyanotic CHD or hemodynamically
significant acyanotic CHD (requiring meds) if you need meds to control, that’ hemodynamically significant
– Given in 5 doses beginning with start of season
– Give anytime after OR
• Flu shot also recommended for those over 6mo
• Regular immunizations given on schedule
28
What is the Norwood procedure and what is it for?
- Used for patients with Hypoplastic Left Heart Syndrome
- Connect PA to aorta, augment the aortic arch, create atrial septal defect and place a shunt from right subclavian to RPA or Sano shunt from RV to PA
- Usual saturations 75-85%
29
When is the Norwood performed?
1-2 weeks
30
What is the modified Blalock-Thomas-Taussig shunt
- Done in patients with inadequate pulmonary blood flow who cannot undergo complete correction as an initial procedure
- Usual saturations 75- 85%
31
What is the operation performed on TGA?
• Arterial switch operation (TGA)
| – Usual saturations 100%
32
Complications of Fontan's surgery?
Long-term Complications
• Arrhythmias
– Sinus node dysfunction
– Atrial flutter/SVT
• Cyanosis
– Collaterals
– Pulmonary AVM’s
* Protein Losing Enteropathy
* Plastic Bronchitis
* Thromboembolism
33
Surgeries for single ventricle?
- Glenn or Bidirectional cavopulmonary connection
(4-6mo)
– SVC to RPA
– Expected sats75-85%
• Fontan or Total cavopulmonary connection (2-4 years)
– IVC to RPA
– Expected sats >90% (usually)
34
Who needs ASA in Kawasaki disease?
Under the new guidelines, unless there was never a problem or if it completely resolves, ASA needs to be considered long-term
35
What are the two conditions that need to be met for needing SBE prophylaxis?
High Risk Lesion
| + High Risk Procedure
36
What are high risk lesions that need SBE prophylaxis?
Patients that have ...
– A prosthetic heart valve or who have had a heart valve
repaired with prosthetic material.
– A history of endocarditis.
– A heart transplant with abnormal heart valve function
– Certain congenital heart defects including:
• Cyanotic congenital heart disease
• A congenital heart defect that's been completely repaired with prosthetic material or a device for the first six months after the repair procedure.
• Repaired congenital heart disease with residual defects, such as persisting leaks or abnormal flow at or adjacent to a prosthetic patch or prosthetic device.
37
What are high risk procedures that need SBE prophylaxis?
- A dental procedure where the gums or lining of the mouth are likely to be injured (eg extraction or surgery)
• Usually not routine cleaning - ? scaling
• Gut or genitourinary surgery through an area
that is infected
38
What are the cyanotic congenital heart defects?
```
6T’s:
– Transposition of the great arteries
– Tetralogy of Fallot
– Tricuspid atresia
– Total anomalous pulmonary venous connections – Truncus arteriosus (common arterial trunk)
– ‘Tingle’ Ventricle (Single ventricle)
• 2A’s
– Pulmonary atresia
– Ebsteins anomaly
```
39
Management of cyanotic newborn?
Warm, sweet and pink (B Sinclair)
• ABC + iv access
• Transillumination and CXR
• Labs: CBC/diff, Ca, Mg, glucose, gas, cultures
• Maintain normothermia
• Correct hypoglycemia and acidosis
• Start antibiotics if sepsis a concern (usual)
• If no response to O2 and no other cause obvious, start prostaglandin (0.05mcg/kg/min)
– Monitor continuously for apnea
– Intubate if necessary
• Urgent consult - cardiology (CALL
40
What findings on history would make you suspicious of pericarditis?
```
History
– More common in older children
– Sharp, stabby or squeezing chest pain
– Better sitting, worse lying down
– Often pleuritic
```
41
What exam findings make you suspicious of pericarditis?
Exam
– Pericardial friction rub
– Narrow pulse pressure
– Pulsus paradoxus >15mmHg
42
What investigations would make you think about pericarditis?
Investigations
ECG: 4 stages. ST elevation/PR depression, T wave flattening, T wave inversion, resolution
CXR-worry if big heart
Echocardiogram
43
Management of pericarditis?
Management – ABC’s
– If stable: NSAIDS (naproxen or ibuprofen) with regular echo f/u
– Steroids if persistent
– Pericardial tap if evidence of tamponade or persistent on meds
– Antibiotics if suppurative pericarditis suspected (uncommon and very sick)
44
What is congestive heart failure?
– Inability of the heart to pump enough blood to meet the body’s needs
– Results in loss of equilibrium between hydrostatic and osmotic pressures in the tissues, thus causing “congestion”
45
What are the main causes of CHF during the first week of life?
```
First Week of life: Obstructions primarily
– Hypoplastic Left Heart Syndrome (d 3-5)
– Severe aortic stenosis
– Coarctation(d7-10)
• Asphyxia
• Severe mitral or tricuspid
regurgitation
• Uncontrolled tachycardias (eg SVT>24h)
```
46
What are the main causes of CHF weeks 2-6
```
Week 2-6: Left to right shunts primarily
• Ventricular Septal Defects (VSD)
• Atrioventricular Septal Defects (AVSD)
• PDA
• NOT ASD
```
47
What are the main causes of CHF in older children?
Older Children - “Pump failure”
• Dilated Cardiomyopathy
• Myocarditis
• Tachycardias
48
Three main symptoms of CHF in children?
* Tachycardia
* Tachypnea
* Hepatomegaly
49
Treatment of CHF?
• Supportive:
– head-up position (decrease respiratory distress)
– tube feeds (decrease work for heart)
– high calorie formula (increase growth, limit free water)
– salt restriction (in older children- avoids excess preload)
– fluid limitation (if severe)
• Medication:
– Improve contractility
• Dopamine, dobutamine, milrinone, epi, norepi
– Decrease preload or filling of the heart
• Diuretics(serialtubular block)
– Decrease afterload
• ACE inhibitors, angiotensin receptor blockers
– Minimize ongoing damage
• Beta blockers
• Surgery
50
What arrhythmia is associated with deafness?
Long QT
51
Are Osler nodes tender or non tender?
tender!
| O=Ow
52
Are Janeway lesions tender or non tender
non tender!
53
What are some term findings of infective endocarditis?
Janeway lesions
Osler nodes
Splinter hemorrhages
thromboembolism
54
Symptoms of infective endocarditis?
Fever
Malaise and endurance fatigue
A new or changing heart murmur
weight loss
coughing
Vascular phenomena: septic embolism, Janeway lesions, bleeding in the brain, conjunctival hemorrhage, splinter hemorrhages, kidney infarcts, and splenic infarcts.
Infective endocarditis can also lead to the formation of mycotic aneurysms.
Immunologic phenomena: glomerulonephritis, Osler's nodes, Roth's spots on the retina, positive serum rheumatoid factor
Other signs may include night sweats, rigors, anemia, spleen enlargement
55
What are Aschoff bodies associated with?
Rheumatic fever
56
How do you make the diagnosis of rheumatic fever?
Need 2 major or 1 major and 2 minor criteria for new Dx
```
Major diagnostic criteria
– Carditis – MR, AI (clinical OR echo-based)
– Polyarthritis
– Chorea
– Subcutaneous nodules
– Erythema marginatum
```
Minordiagnostic criteria
– Fever (>38.5 for low risk)
– Polyarthralgia (low risk)
– Prolonged PR interval on ECG (if carditis not Major)
– Elevated acute-phase reactants (ESR>60, CRP>3mg/dL)
57
What is pulmonary Hypertension?
Pulmonary hypertension = elevated PA pressures above normal (mean PA pressure>25mmHg)
58
What is cor pulmonale
• Cor pulmonale = Right heart dysfunction secondary to pulmonary disease
59
S/Sx Pulm Htn
```
Hx
– SOB on exertion
– Decreased activity tolerance
– Chest pain on exertion
– Feeling faint on exertion
– Syncope on exertion
– Peripheral oedema
– Decreased appetite/energy
– FTT
```
60
Physical exam finding for pulmonary hypertension
```
Phx
– Precordial bulge
– RV heave
– Single S2
– TR, PR murmurs (Graham Steele)
– Pulsatile Liver (Tricuspid regurg)
– Hepatomegaly
– Oedema
```
• Investigations
– ECG – RVH, RV strain
– Echo
61
Cardiomegaly with ++ blood flow on CXR is what?
Hypoplastic L heart
62
Cardiomegaly with decreased pulmonary blood flow is what?
Ebstein
63
Boot shaped heart on CXR?
TOF
64
Egg on a string is what on CXR?
TGA
65
Snowman CXR?
TAPVR
66
Most common cardiac lesion in T21?
AVSD
67
Most common cardiac lesion in DiGeorge?
tetralogy of fallot
and interrupted aortic arch
conotruncal defects (tetralogy of Fallot, truncus arteriosus, double-outlet right ventricle, subarterial VSD)
branchial arch defects (coarctation of the aorta, interrupted aortic arch, right aortic arch).
68
Cardiac lesions associated with turner?
Coarctation
Bicuspid Aortic Valve
Aortic Stenosis
69
Cardiac lesions associated with Williams Syndrome?
Supravalvular aortic stenosis
70
S/Sx pericarditis?
Chest pain: sharp/stabbing, positional, radiating, worse with inspiration, and relieved by sitting upright or prone.
```
Cough
Fever
Dyspnea
Abdominal pain
Vomiting
Muffled or distant heart sounds, tachycardia, narrow pulse pressure, jugular venous distention, and a pericardial friction rub
+ pulses paradoxes if tamponade present
```
71
CXR findings in pericarditis?
Although the chest x-ray findings in a patient with pericarditis without effusion are usually normal
In the presence of a significant effusion, cardiac enlargement will be seen and cardiac contour may be unusual (Erlenmeyer flask or water bottle appearance)
72
Cardiac Findings in pompe disease?
```
cardiomegaly
increased wall thickness
SVT
short PR interval
extremely tall QRS complexes
```
73
Presentation of infantile pompe disease?
Infantile Pompe disease (IPD) is uniformly lethal without enzyme replacement therapy (ERT) with alglucosidase alfa.
Affected infants present in the 1st day to weeks of life with hypotonia, generalized muscle weakness with a floppy infant appearance, neuropathic bulbar weakness, feeding difficulties, macroglossia, hepatomegaly, and hypertrophic cardiomyopathy, which if untreated leads to death from cardiorespiratory failure or respiratory infection, usually by 1 yr of age.
74
EKG findings with hyperkalemia
Repolarization abnormalities:
Peaked T waves
Progressive paralysis of the atria:
P wave widens and flattens, eventually disappears
PR segment lengthens
Conduction abnormalities and bradycardia:
Prolonged QRS interval with abnormal morphology
High-grade AV block with slow junctional and ventricular escape rhythms
Any kind of conduction block (bundle branch blocks, fascicular blocks)
Sinus bradycardia
Development of a sine wave appearance (a pre-terminal rhythm)
Cardiac arrest
Asystole
Ventricular fibrillation
PEA with bizarre, wide complex rhythm
75
Treatment for hyperkalemia
Potential treatment
I ) protecting the heart (eg. calcium ion); IVinfusion of calcium ion acts rapidly but its effects last only approximately I hour.
2) shifting K intravascular space to the intracellular space (ventolin, insubin/glucose) - . effects last for a few hours
3) eliminating K from the body (dialysis, ion exchange resins). Dialysis cannot be instituted immediately. Ion exchange resins, such as sodium polystyrene sulfonate, work slowly.
76
What is a first degree heart block?
In first-degree AV block , the PR interval is prolonged, but all the atrial impulses are conducted to the ventricle.
If the R is far from P, then you have a first degree
77
What is a second degree AV block?
In second-degree AV block , not every atrial impulse is conducted to the ventricle.
78
What is Mobitz 1/Wenckebach heart block?
In the variant of second-degree block known as the Wenckebach type (also called Mobitz type I ), the PR interval increases progressively until a P wave is not conducted. In the cycle following the dropped beat, the PR interval normalizes.
longer longer longer drop! Then you have a wenkebach!
79
What is Mobitz II?
In Mobitz type II there is no progressive conduction delay or subsequent shortening of the PR interval after a blocked beat. This conduction defect is less common but has more potential to cause syncope and may be progressive.
If some Ps just don't get through, then you have a Mobitz two!
80
What is a 2:1 AV block?
A related condition is high-grade second-degree AV block , in which 2 or more P waves in a row fail to conduct. This is even more dangerous.
81
What is 3rd degree heart block?
In third-degree AV block (complete heart block) , no impulses from the atria reach the ventricles. An independent escape rhythm is usually present but may not be reliable, leading to symptoms such as syncope.
If Ps and Qs don't agree, then you have a third degree!
82
Causes of complete heart block?
- Mother who has systemic lupus erythematosus (SLE) or Sjögren syndrome.
- Patients with complex CHD and abnormal embryonic development of the conduction system.
- Myocardial tumors and myocarditis.
- Myocardial abscess secondary to endocarditis.
- Genetic abnormalities, including LQTS and Kearns-Sayre syndrome.
- Postoperative AV block can be a complication of CHD repair; in particular repairs involving ventricular septal defect closure.
83
Describe a Still's Murmur
```
Short ejection systolic murmur
musical or vibratory quality
Heard best between Apex and LSB
The murmur increases in intensity with high output states, such as fever, anxiety, and exercise.
Most common in kids 2-7
```
84
Describe a physiologic pulmonary flow murmur
```
LUSB
Heard in 3 onwards
Soft systolic ejection murmur
Grade 1-2
Louder when patient is supine
```
85
Peripheral Pulmonic Stenosis Murmur
Newborn, up to 3 months
| low intensity systolic ejection murmur heard best over lung fields and also over back
86
Describe Sinus Arrhythmia
Sinus arrhythmia: The heart rate increases with inspiration and decreases with expiration in a reproducible pattern.
87
what is a PAC?
Extrasystoles
Premature atrial contractions (PACs) and premature ventricular contractions (PVCs) – rare symptoms of single skipped beats or single hard beats.
PAC - atrial beat that occurs before the next expected sinus beat and originates from atrial tissue that is not the sinus node = different looking p wave. Will either a) be conducted normally = normal QRS b) be blocked or c) be slowly conducted = wide QRS. PACs alone do not need ot be investigated further.
88
what is a PVC?
PVC is an early beat arising from the ventricular muscle resulting in a QRS complex that is different from sinus and is not preceded by a P wave.
Occurring in up to 40% of pediatric patients.
89
What conditions are PVCs associated with?!
Occasionally PVCs can be associated with myocarditis, cardiomyopathy, or channelopathies.
90
when are PVCs a concern??
Ususlly no problem, unless occuring >3 in a row or during exercise. If frequent, should be followed by cardiologist.
91
Which PVCs are treated and what is used?
Treatment of PVCs is reserved for those rare patients who are symptomatic from the PVCs or who develop ventricular dysfunction.
First-line therapy can be antiarrhythmic agents, but some patients may elect to go straight for an ablation procedure to avoid medication use.
92
What are the lengths of pathologic QTC (prolonged)?
QTc interval > 440 ms in prepubertal boys
> 450 ms in prepubertal girls
QTc > 470 ms should prompt an evaluation for long QT syndrome
93
Causes of prolonged QTC
QTc interval can happen for a variety of reasons - medications, electrolyte abnormalities, neurologic abnormalities.
94
presentation of. prolonged QTC
Most patients who have long QT syndrome are asymptomatic, presenting symptoms typically related to the occurrence of torsades de pointes, sudden onset palpitations, syncope, cardiac arrest, sudden death.
95
Treatment of prolonged QT
β-blockers are almost always first-line therapy in all patients with diagnosed long QT syndrome. An implantable cardioverter-defibrillator may be indicated in high-risk long QT syndrome
96
AVR Ventricular Tachycardia...how does it present?
AVR – rates 15% to 20% faster than the expected or a ventricular rate <120 beats/min in teenager.
benign, rarely requires treatment, same prognosis as PVCs. at any age and may present first hours after birth. longitudinal follow-up to assess for resolution and monitor for the development of ventricular dysfunction.
In older children, AVR can be followed much like that of isolated PVCs.
97
Monomorphic VT....bad or benign?
Monomorphic VT in the presence of a normal heart - benign
98
Polymorphic VT...bad or benign?
Polymorphic VT - beat-to-beat variations in the QRS morphology/axis during VT
Rare in childhood almost always associated with an underlying cardiac abnormality.
thorough investigation into underlying structural heart disease, cardiomyopathy, myocarditis, and channelopathies. metabolic/electrolyte abnormalities and toxicity from medications or recreational drugs should be investigated
longitudinal follow-up, may require antiarrhythmic agents or even implantation of an internal cardioverter-defibrillator
99
Which CHD accounts for 30-50% of all congenital cardiac defects?
Ventricular septal defect
100
what is the clinical presentation of a VSD??
Small ventricular septal defects are typically asymptomatic and usually discovered on routine clinical examination
Moderate to large ventricular septal defects typically manifest during infancy with symptoms and signs of congestive heart failure and holosystolic murmur
101
Management of a VSD?
Conservatively manage patients who have small asymptomatic ventricular septal defects, as most will close spontaneously
Medically treat patients who have moderate to large ventricular septal defects and symptoms of congestive heart failure to alleviate symptoms before surgical closure
102
When should VSDs e repaired by?
Moderate to large ventricular septal defects should undergo surgical repair before age 1 year; without intervention, these defects may cause left to right shunting, which leads to pulmonary hypertension, heart failure, and Eisenmenger syndrome
103
What is Eisenmenger Syndrome?
Eisenmenger syndrome, a complication of unrepaired ventricular septal defect, results in shunt reversal.
Consequently, physical signs (eg, murmurs, thrills) can disappear; this should not be mistaken for evidence that ventricular septal defect is closing spontaneously
104
What are the 4 abnormalities with tetralogy of fallout?
Most common cyanotic CHD
Anterior malalignment of the interventricular septum, which leads to a
1. VSD
2. overriding aorta.
3. RV outflow tract obstruction (Narrowing of the pulmonary outflow tract due to the septal deviation)
4. RV hypertrophy
105
Murmur associated with TOF?
Harsh SEM LUSB = pulmonic stenosis. may be a single S2.
106
What is the most common cyanotic CHD?
TOF
107
What is the pathophysiology of a tet spell?
Tet spells: change in balance between PVR and SVR (decreased SVR because of crying valsalva → decreased pulmonary blood flow = cyanosis)
108
Treatment of a tet spell
Keep calm, give O2, knee to chest, maybe fluid, morphine, phenylephrine or propranolol
109
Repair of TOF
Treatment
Palliation: Blalock-Taussig shunt, dilation
Complete repair: patch VSD, resection
110
Most common cyanotic heart disease presenting in the first week of life?
Transposition of the Great Arteries
111
What is TGA?
Aorta arising from the RV and the pulmonary artery arising from the LV. Systemic venous (deoxygenated) blood returning to the right atrium, RV, and going out the aorta again. There is mixing of blood at the atrial level through foramen ovale or an ASD or at the ventricular level through a VSD.
112
Clinical presentation of a TGA
Cyanosis within the first 12 ho after birth and not responsive to oxygen or mechanical ventilation.
CXR - Narrow mediastinal silhouette (egg on a string)
EKG normal or show RV hypertrophy.
No murmur; however, may be a single S2
113
Management of TGA
Reparative surgery to switch the great vessels to the appropriate ventricles, known as the arterial switch procedure.
Most patients undergo a catheter-based procedure called balloon atrial septostomy to help create or enlarge the ASD to allow more mixing while awaiting surgery.
114
What is trunks arteriosus?
Truncus Arteriosus—Common Arterial Trunk
Common truncal outflow does not divide into an aorta and a main pulmonary artery.
There is always an associated VSD
There is a common truncal valve.
115
What genetic syndrome is associated with truncus arteriosus?
⅓ have a 22q11.2 deletion.
116
How does a truncus arteriosus present?
Present within first 48 h after birth with symptoms of profound pulmonary overcirculation.
Systolic murmur LLSB as well as a loud S2.
May also demonstrate features of 22q11.2 deletion.
117
Management of Truncus Arteriosus?
Symptoms of pulmonary overcirculation may be managed with diuresis and fluid restriction.
These children undergo surgical repair in the first 2 weeks after birth.
They need close cardiology follow-up as they outgrow their RV-to–pulmonary artery conduit and their truncal valve may become regurgitant.
Serum electrolyte testing for calcium levels should also be performed in all cases while awaiting chromosomal microarray analysis.
118
What is TAPVR?
Total Anomalous Pulmonary Venous Drainage
Abnormal return of the pulmonary veins to the systemic veins or the right atrium
Total : none of the pulmonary veins return to the left atrium.
RA → RV → lungs → PA → RA → PFO/ASD → LA → systemic requires PFO/ASD
The number of veins returning anomalously and degree of obstruction determine rate and severity.
119
How does TAPVR present?
TAPVR presents with respiratory distress/cyanosis within 12-24 h after birth
CXR (a) whiteout lung fields due to backing up of blood flow prior to the site of the obstruction of pulmonary vein drainage (b) small heart.
Respiratory distress does not respond to oxygen and potentially worsened by starting prostaglandin as more blood starts shunting to the pulmonary circulation at the ductal level, to the point of pulmonary edema or lung bleeding.
120
Treatment of TAPVR
Respiratory distress does not respond to oxygen and potentially worsened by starting prostaglandin as more blood starts shunting to the pulmonary circulation at the ductal level, to the point of pulmonary edema or lung bleeding.
Obstructed TAPVD is a surgical emergency.
After repair of TAPVD, patients continue to be closely monitored pulmonary venous obstruction can recur, with reported rates around 15%.
121
What is the Norwood Procedure?
HLHS, Tricuspid Atresia, and Single-Ventricle Palliation
Norwood procedure: “neoaorta” for systemic blood flow and RV-to–pulmonary artery conduit (to supply blood flow to the pulmonary arteries), and creating an ASD.
within the first 2 weeks after birth. Saturations after the first-stage palliation can vary from 80% up to 95%
Should always have a shunt murmur
122
Babies of mother who have rubella (or other viral illnesses) during pregnancy are at risk of what CHD?
TOF
123
What is a Glenn Procedure
Glenn procedure, involves anastamosing the superior vena cava to the pulmonary arterial system = passive venous return to the lungs. Take down of the RV to PA conduit.
4-6 months of age. Sats 80-90%
124
What is a Fontan Procedure
Fontan procedure: connect the IVC to the pulmonary arterial system as well = complete venous return to lungs
2-4 years of age. Sats >90%
125
Presentation of HLHS
In patients with HLHS, when the duct starts to close: the infant begins to have signs of poor systemic perfusion with feeble pulses, poor urine output, shock, and mottling, pulmonary overcirculation, seen as tachypnea and respiratory distress.
Chest radiographs will show pulmonary vascular congestion, in addition to possible cardiomegaly due to right ventriculomegaly.
126
What are some maternal risk factors for TOF
Other pregnancy-related risks include poor nutrition, alcohol abuse, poorly controlled diabetes, and the mother's age (over 40).
Certain environmental factors, such as air pollution, also may increase a mother's chances of having a baby with TOF.
127
Are boys or girls more likely to have coarc?
Boys> girls 2:1
128
What percent of ASDs are secundum, primum and sinus venosus?
```
Atrial Septal Defects - 7% to 10% of CHD.
Secundum ASDs (75% of ASD), Primum ASDs (15% of ASD), sinus venosus ASDs (10%)
```
Secundum ASD occurs in the middle part of the atrial septum.
Primum ASD occurs in the lower part of the atrial septum close to the tricuspid and mitral valves.
Sinus venosis occurs in the upper part of the atrial septum near the veins that drain into the right and left atrium.
129
Consequences of ASD?
Shunting of blood from left to right at the atrial level → increased diastolic blood volume in the RV, which causes right-sided chamber dilation.
130
What is heard on exam for an ASD?
Murmur loudest over the LUSB (from increased flow across the pulm valve) with a fixed splitting of the S2 and a loud S1.
An additional diastolic murmur LLSB from excess flow across the tricuspid valve.
May be left precordial bulging due to the enlarged RV being present during cartilaginous rib development.
Repair
131
EKG findings for ASD?
Secundum ASD have RAD on EKG, and primum have LAD on EKG
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Symptoms of ASD
Usually asymptomatic but may have some fatigue. Infants with an ASD manifesting with features of CHF must be investigated for an additional lesion such as a PDA, VSD with pulmonary arterial stenosis, or a left-sided obstructive lesion (cor triatriatum, coarctation, aortic or mitral stenosis).
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Repair of ASD?
Secundum: percutaneous patch closure
Primum: surgical (has to do with needing a lip for the patch to hang on to)
Usually age 3-5
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What is the most common CHD?
Ventricular Septal Defects
| Most common CHD lesion, 50% - 60% (also most common lesion in T21)
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What is the most common type of VSD?
Perimembranous VSDs (deficiency in a fibrous part of the septum at the base of the heart) are the most common type (80%).
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Symptoms and signs of VSD?
A newborn with a VSD may not initially have a murmur
As the pulmonary resistance decreases with age, an S1-coincident pansystolic murmur can be heard the loudest LLSB
A diastolic rumble at the apex may be heard from excess flow across the mitral valve.
Hemodynamically significant VSD presents with features of pulmonary overcirculation and CHF.
As the child grows, perimembranous VSDs can get occluded by aneurysmal tissue, and muscular VSDs can become smaller in size with muscular growth.
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Management of a VSD?
Management
Children with CHF - diuretics—usually furosemide, chlorothiazide, spironolactone— may require frequent evaluation of electrolyte levels.
Patients with failure to thrive may need up to 125 to 150 kcal/kg per day
Surgical patch repair or percutaneous closure of the VSD is performed in a symptomatic child
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What percent of kids with AVSDs have T21?
Atrioventricular Septal Defects
| About 50% of patients with AVSDs have Down syndrome.
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Murmur with AVSD?
Systolic ejection or holosystolic murmur from the VSD component.
If the VSD is large and there is equalization of pressures in the ventricles (or if pulmonary hypertension is present), a murmur may not be heard.
Murmurs of atrioventricular valve regurgitation, S1-coincident systolic murmur, heard the loudest over the apex in the case of left-sided regurgitation and the loudest over the right lower sternal border in the case of right-sided regurgitation.
A diastolic rumble may be heard, similar to that heard in patients with VSDs.
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Presentation of AVSD?
May also present with features of CHF and failure to thrive, with chest radiographic findings
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Management of AVSD?
Typically require surgical intervention around 4 to 6 months of age or earlier, if they have severe CHF or failure to thrive
diuretic and nutritional management and close monitoring for CHF
