Term
| Why are congenital heart diseases important in pediatrics? |
|
Definition
Common
Serious
Often treatable |
|
|
Term
| What is the incidence of congenital heart disease? |
|
Definition
One of the most common causes of morbidity & mortality in the NICU
Occurs in ~ 1% of all live births
Nearly half of the cases are diagnosed during first week of life |
|
|
Term
| What are the characteristics of congenital heart disease? |
|
Definition
Failure of the heart to follow the correct sequence of embryologic events
Occurs during the first trimester of fetal development
Resultant abnormal or incomplete structural contributions may lead to minor or major deformities |
|
|
Term
| What is congenital heart disease? |
|
Definition
| Congenital heart disease is the failure of the heart to follow the correct sequence of emryologic events during the first trimester of fetal development. Resultant abnormal or incomplete structural contributions may lead to minor congenital cardiac anomalies that can be managed conservatively, or to major deformities that may require immediate medical and/or surgical intervention |
|
|
Term
| What circulatory changes occur at birth? |
|
Definition
Colossal decrease in pulmonary vascular resistance
Pronounced rise in systemic vascular resistance
Decrease in blood flow to the right atrium
Abrupt rise in pulmonary arterial blood flow
Functional closure of foramen ovale & ductus arteriosus |
|
|
Term
| What are the immediate alterations that occur at birth? |
|
Definition
1. A colossal decrease in pulmonary vascular resistance associated with the expansion of the lungs.
2. A pronounced rise in systemic vascular resistance associated with the elimination of the low-resistance placental circulation.
3. A decrease in blood flow to the right atrium because of the abolition of umbilical venous return.
4. An abrupt rise- as much as tenfold- in pulmonary arterial blood flow, which is promptly translated into an increase in left arterial volume and pressure.
5. Functional closure of the valve of the foramen ovale because of the rise in left atrial pressure and the fall in right atrial pressure.
6. Constriction of the ductus arteriosus in response to rises in systemic arterial oxygen tension and declining pulmonary vascular resistance. |
|
|
Term
| What is the gross anatomy of the heart? |
|
Definition
4 chambers, left separated from right
Valves
Outflow tracts |
|
|
Term
| What are the basic principles of heart physiology? |
|
Definition
A chamber that has to pump against higher than normal pressure can fail with time.
If a chamber does not pump efficiently, may cause “backing up” of blood (congestion) in the circulation that feeds that chamber. This also causes strain on chamber that is pumping against the congestion. |
|
|
Term
| What is an example of primary disturbance/blood flow? |
|
Definition
If lesion, for instance, VSD, blood will flow from area with higher pressure (LV) to lower pressure (RV)
Short term, this disturbance causes increased volume delivered to RV (dilation), and increased volume delivered to pulmonary circulation.
Long term, this disturbance causes changes in pulmonary vasculature, increased pulm resistance, RV failure |
|
|
Term
| How does the infant and Child present with CHD? |
|
Definition
Critically ill Neonate with inadequate pulmonary blood flow (PBF) or systemic blood flow (SBF)
Heart Murmur
Arrythmia
PRE NATAL DIAGNOSIS |
|
|
Term
| What are the clinical findings of childhood CHD? |
|
Definition
Poor feeding
Growth failure
Sweating
Tachycardia
Cardiomegaly
Gallop rhythm
Pulsus paradoxus
Pulsus alternans |
|
|
Term
| What are the manifestations of pulmonary edema/congestion? |
|
Definition
Tachypnea
Wheezing
Rales
Dyspnea
Cough
Cyanosis |
|
|
Term
| What are the manifestations of Systemic venous congestion? |
|
Definition
Hepatomegaly
Neck vein distension
Peripheral edema |
|
|
Term
| What is the Evaluation of CHD patients? |
|
Definition
History
Physical examination
ECG
CXR
Blood gases
Hyperoxia test
Echocardiogram
Cardiac catheterization
CT angiogram
MRI |
|
|
Term
| What is a hyperoxia test? |
|
Definition
Done when patient is cyanotic
RA Arterial PaO2 by ABG while patient is breathing room air
RA Arterial PaO2 by ABG after inhaling 100% O2 for 10 minutes
A “failed” hyperoxia test is a neonatal emergency - urgent intervention. |
|
|
Term
| What are the Po2 levels of a hyperoxia test? |
|
Definition
pO2 < 100; cyanotic CHD likely
pO2 100-250; cyanotic CHD possible
pO2 > 250; cyanotic CHD unlikely |
|
|
Term
| What is the Evaluation of CHD patients History? |
|
Definition
Color (ever turned blue)
How does baby feed (tiring, sweating, breathing heavy/fast)
Cough
Weight gain
Exercise tolerance (older children)
Syncope/dizziness |
|
|
Term
| What vitals are taken with Evaluation of CHD patients Physical Exam? |
|
Definition
| heart rate, blood pressure, respiratory rate, oxygen saturations, growth curves |
|
|
Term
| What cardiovascular assessment is done with Evaluation of CHD patients Physical Exam? |
|
Definition
color, precordial activity, jugular venous distension, pulses in all extremities, thrill, S1, S2 (split?), S3, S4, capillary refill, edema, murmur (turbulence - systolic/diastolic, intensity, character, location, radiation).
know location of where to best hear valves (aortic, pulmonary, tricuspid, mitral) |
|
|
Term
| What chest assessment is done for eval of CHD? |
|
Definition
| effort, retractions, wheezes, rhales, crackles |
|
|
Term
| What abdominal assessment is done with evaluation of CHD? |
|
Definition
|
|
Term
| What are the 2 classifications of CHD lesions? |
|
Definition
Acyanotic lesions
Cyanotic lesions |
|
|
Term
| What are the characteristics of Acyanotic lesions? |
|
Definition
Ventricular Septal Defect (VSD)
Atrial Septal Defect (ASD)
Patent Ductus Arteriosus (PDA)
Atrioventricular Septal Defect (AVSD)
Pulmonary Stenosis (PS)
Aortic Stenosis (AS)
Coarctation of the Aorta |
|
|
Term
| What are the characteristics of Cyanotic lesions? |
|
Definition
Truncus Arteriosus
Transposition of the Great Arteries (TGA)
Tricuspid Atresia
Tetralogy of Fallot
Total Anomalous Pulmonary Venous Return (TAPVR) |
|
|
Term
| Which type of CHD lesion is more common? |
|
Definition
Uncommon, each <1% of CHD, pulmonary atresia, Ebstein’s anomaly, truncus arteriosus, single ventricle, double outlet right ventricle
Acyanotic more common than cyanotic |
|
|
Term
| What are the classifications of excessive pulmonary blood flow? |
|
Definition
| VSD, ASD, PDA -> ALL HOLES! Letting more blood through because of the pressure |
|
|
Term
| What is Inadequate Pulmonary Blood Flow? |
|
Definition
|
|
Term
| What is Inadequate or obstruction to systemic Blood flow? |
|
Definition
| HLH, Coarctation of Aorta -> Coarctation = STENOSIS! Narrowing of the aorta |
|
|
Term
|
Definition
|
|
Term
| What are the characteristics of Ventricular septal defect? |
|
Definition
Membranous
Muscular
15-50% close spontaneously
Murmur
Congestive failure
SBE prophylaxis |
|
|
Term
| What kind of lesion of VSD? |
|
Definition
Acyanotic Lesion with increased pulm blood flow
Most common form of CHD,
20-25% of all CHD
(VSD) |
|
|
Term
| What are the ECG readings of VSD? |
|
Definition
ECG: small VSD nrml,
med VSD LVH +/- LAE
large VSD BVH +/- LAE |
|
|
Term
| What are the CXR readings of VSD? |
|
Definition
| CXR: increased pulm vasculature (depends on LR shunt), possible cardiomegally |
|
|
Term
| What are the exam findings of VSD? |
|
Definition
2-5 / 6 holosystolic murmur loudest at LLSB,
possible thrill at LLSB, P2 may be increased
(large VSD and pulm HTN), hyperdynamic precordium |
|
|
Term
|
Definition
| Acyanotic Lesion with increased pulm blood flow (ASD) |
|
|
Term
| What are the exam findings of ASD? |
|
Definition
wide, fixed split S2
2-3 / 6 SEM at LUSB (from increased blood flow across pulm flow tract) |
|
|
Term
| What are the ECG findings of ASD? |
|
Definition
ECG:
small ASD nrml
hemodynamically signif ASD RAD
and mild RVH or RBBB |
|
|
Term
| What are the CXR findings of ASD? |
|
Definition
CXR:
possible cardiomegally and increased pulm vascular markings |
|
|
Term
|
Definition
| Acyanotic Lesion with increased pulm blood flow (PDA) |
|
|
Term
| What are exam findings of PDA? |
|
Definition
| 1-4 continuous “machinery-like” murmur loudest at LUSB |
|
|
Term
| What are the ECG findings of PDA? |
|
Definition
Small to moderate PDA: Normal or LVH
Large PDA: BVH
CXR: |
|
|
Term
| What are the CXR findings with PDA? |
|
Definition
| Possible cardiomegaly with increased pulmonary vascular markings |
|
|
Term
|
Definition
| More common in premature infants |
|
|
Term
|
Definition
| Acyanotic Lesion with increased pulm blood flow (AVSD) |
|
|
Term
| What are exam findings of AVSD? |
|
Definition
Hyperactive precordium with systolic thrill at LLSB and loud S2
Possible holosystolic regurgitant murmur (MR)
Possible middiastolic rumble |
|
|
Term
| What are ECG findings with AVSD? |
|
Definition
Superior QRS axis
possible RVH and LVH |
|
|
Term
| What are the CXR findings with AVSD? |
|
Definition
| Cardiomegaly with increased pulmonary vascular markings |
|
|
Term
| Who does AVSD occur often in? |
|
Definition
| 30-60% occur in Down Syndrome |
|
|
Term
| What is Pulmonary Stenosis ? |
|
Definition
|
|
Term
| What are the exam findings of Pulmonary Stenosis? |
|
Definition
Ejection click at LUSB with valvular PS
Click intensity decreases with inspiration, increases with expiration
Widely split S2, diminished P2 intensity
Systolic ejection murmur with radiation to back and sides |
|
|
Term
| What are the ECG findings of Pulmonary Stenosis? |
|
Definition
Mild PS: normal
Moderate to severe: RAD and RVH |
|
|
Term
| What are the CXR findings of Pulmonary Stenosis? |
|
Definition
| Normal heart size with normal to decreased pulmonary vascular markings |
|
|
Term
| What is Aortic Stenosis ? |
|
Definition
|
|
Term
| What are the exam findings of Aortic Stenosis? |
|
Definition
Systolic thrill at RUSB
Ejection click, not varying with respiration
Harsh SEM (2-4 / 6) RUSB with radiation to neck
Possible early diastolic murmur (AR)
Narrow pulse pressure if severe AS |
|
|
Term
| What are the ECG findings of Aortic Stenosis? |
|
Definition
| Mild AS: normal, moderate to severe: LVH |
|
|
Term
| What are the CXR findings of Aortic Stenosis? |
|
Definition
|
|
Term
| What are coarctation of the aorta? |
|
Definition
Turners
Isolated
With intracardiac defects
Surgical repair
Balloon angioplasty |
|
|
Term
| What are the exam findings of coarctation of aorta? |
|
Definition
2-3 / 6 SEM at LUSB
If Bicuspid valve, ejection click at RUSB
BP in upper extr > BP in lower extr
Pulse ox difference > 5% between upper extr and lower extr suggests coarc |
|
|
Term
| What is coarctation of the aorta? |
|
Definition
| Acyanotic Lesion with Inadequate systemic Blood flow or obstruction |
|
|
Term
| What are the ECG findings of coarctation of the aorta? |
|
Definition
Infancy: RVH or RBBB
Children: LVH |
|
|
Term
| What are the CXR findings of coarctation of the aorta? |
|
Definition
Cariomegaly
Pulmonary venous congestion
Rib notching after age 5 from collateral circulation development |
|
|
Term
| What is the infant presentation of coarctation of the aorta? |
|
Definition
|
|
Term
| What is the childhood presentation of coarctation of the aorta? |
|
Definition
|
|
Term
| What are the cyanotic lesions? |
|
Definition
Truncus Arteriosus
Transposition of the Great Arteries (TGA)
Tricuspid Atresia
Tetralogy of Fallot
Total Anomalous Pulmonary Venous Return (TAPVR) |
|
|
Term
| What is transposition of the great arteries? |
|
Definition
Reversal of anatomic relation of great arteries
Ventriculo-arterial discordance
Balloon atrial septostomy
Arterial switch (Jatene)
Mustard/Senning |
|
|
Term
|
Definition
| Cyanotic Lesion with abnromal mixing |
|
|
Term
| What are exam findings of TGA? |
|
Definition
Can have extreme cyanosis
Single S2, loud
If associated VSD or PS, murmur |
|
|
Term
| What are ECG findings of TGA? |
|
Definition
RVH because RV acting as systemic ventricle
upright T wave in V1 after 3 days old may be only abnormality |
|
|
Term
| What are CXR findings of TGA? |
|
Definition
Classic finding: “Egg on a string” with cardiomegaly
May have increased pulmonary vascular markings |
|
|
Term
| What is balloon arterial septostomy? |
|
Definition
| Creates connection between systemic and pulmonary circulations– TGA, pulm atresia with intact atrial septum |
|
|
Term
| What is Tricuspid Atresia? |
|
Definition
Cyanotic lesion
Lesion: absent tricuspid valve and hypoplastic RV and PA. |
|
|
Term
| What are the exam findings of Tricuspid Atresia? |
|
Definition
Single S2
If VSD, 2-3 /6 systolic regurgitation murmur at LLSB |
|
|
Term
| What must tricuspid atresia have to survive? |
|
Definition
| ASD, PDA, or VSD for survival. |
|
|
Term
| What are the ECG findings of Tri. Atresia? |
|
Definition
| Right atrial enlargement or common atrial enlargement and LVH |
|
|
Term
| What are the CXR findings of Tri. Atresia? |
|
Definition
Normal or slightly enlarged heart
May have “boot shaped” heart |
|
|
Term
| What is Tetralogy of Fallot ? |
|
Definition
Ventricular septal defect
Overriding aorta
Subpulmonary stenosis
Right ventricular hypertrophy
Cyanotic Lesion with inadequate pulmonary blood flow |
|
|
Term
| How is Tetralogy of Fallot corrected? |
|
Definition
|
|
Term
| What are common findings with Tetralogy of Fallot? |
|
Definition
Large VSD
RVOT obstruction
RVH
Overriding aorta |
|
|
Term
| What does the degree of RVOT obstruction determine? |
|
Definition
| amount of shunting across VSD, pulm flow, and cyanosis. |
|
|
Term
| What are the manifestations of MILD ps? |
|
Definition
| L to R shunting, no cyanosis |
|
|
Term
| What are the manifestations of SEVERE ps? |
|
Definition
| R to L shunting, cyanosis |
|
|
Term
| What are common exam findings of Tetralogy of Fallot ? |
|
Definition
| loud systolic murmur along LSB, loud single S2, possible thrill. |
|
|
Term
| What are common ECG findings of Tetralogy of Fallot ? |
|
Definition
|
|
Term
| What are the CXR findings of Tetralogy of Fallot? |
|
Definition
boot shaped heart”
normal size
+/- increased pulmonary vascular markings depending on pulm blood flow (degree of RVOT) |
|
|
Term
| What are TET spells that are associated with Tetralogy of Fallot? |
|
Definition
Occur in young infants
Increased RVOT obstruction or decreased systemic resistance leads to R L shunting across VSD, resulting in decreased pulm blood flow |
|
|
Term
| What are the manifestations of TET spells? |
|
Definition
| Cyanosis, decreasing murmur |
|
|
Term
| How are TET spells treated? |
|
Definition
| calming child, oxygen, knee chest position, morphine. All aimed at increasing systemic resistanc, decreasing pulmonary resistance to promote pulmonary bood flow |
|
|
Term
| What is Total Anomalous Pulmonary Venous Return (TAPVR) ? |
|
Definition
Cyanotic Lesion
Pulmonary veins drain into RA or other location instead of LA. Must have ASD or PFO for survival. |
|
|
Term
| What is Supracardiac Total Anomalous Pulmonary Venous Return (TAPVR)? |
|
Definition
|
|
Term
| What is Cardiac Total Anomalous Pulmonary Venous Return (TAPVR)? |
|
Definition
| : PV into coronary sinus or RA |
|
|
Term
| What is sub diaphragmatic Total Anomalous Pulmonary Venous Return (TAPVR)? |
|
Definition
| PV into IVC, portal vein, ductus venosus or hepatic vein |
|
|
Term
| What are exam findings of Total Anomalous Pulmonary Venous Return (TAPVR) ? |
|
Definition
Hyperdyanmic RV impulse
S2 fixed and widely split
2-3 / 6 SEM at LUSB and middiastolic rumble at LLSB |
|
|
Term
| What are ECG findings of Total Anomalous Pulmonary Venous Return (TAPVR) ? |
|
Definition
|
|
Term
| What are CXR findings of Total Anomalous Pulmonary Venous Return (TAPVR) ? |
|
Definition
Cardiomegaly
Increased pulmonary vascular markings |
|
|
Term
| What is the most common chromosomal defect? |
|
Definition
Trisomy 21
Most frequent chromosomal defect affecting live-born infants (1 in 660 live births) |
|
|
Term
| What are the most common congenital Heart Malformations associated with Trisomy 21? |
|
Definition
Atrio-ventricular septal defect
Ventricular septal defect
Atrial septal defect
Patent ductus arteriosus |
|
|
Term
| What are the cardiac effects of FAS? |
|
Definition
| Half of patients with FAS have congenital heart disease: VSD, ASD, TOF. |
|
|
Term
| What are the cardiac effects of Turner Syndrome? |
|
Definition
| Coarctation of aorta, bicuspid aortic valve, aortic stenosis |
|
|
Term
| What is the dominant cardiac defect in downs? |
|
Definition
|
|
Term
| What are the dominant defects in noonan syndrome? |
|
Definition
|
|
Term
| What is the dominant cardiac defect of Turner Syndrome? |
|
Definition
|
|
Term
| What is the dominant cardiac defect of Marfan syndrome? |
|
Definition
|
|
Term
| What is the dominant cardiac defect of William's syndrome? |
|
Definition
|
|
Term
| What is the dominant cardiac defect of FAS? |
|
Definition
|
|
Term
| What is the dominant cardiac defect of DiGeorge syndrome? |
|
Definition
| Interrupted aortic arch, TOF |
|
|
Term
| What is the dominant cardiac defect of CHARGE syndrome? |
|
Definition
|
|
Term
| If you are severely compromised with sickle cell trait, what can happen? |
|
Definition
| Your trait can turn into sickle cell anemia! |
|
|
Term
| Name five Cyanotic Congenital Heart Malformations that start with the letter “T” ? |
|
Definition
Tetralogy of Fallot
Transposition of the Great arteries
Tricuspid atresia
TAPVR
Truncus arteriosus |
|
|
Term
| Name two congenital Heart malformations in the newborn that are dependent on their patent ductus for survival? |
|
Definition
Tetraolgy of the Heart
Transposition of the Great Arteries |
|
|
Term
| How is patency of the PDA maintained? |
|
Definition
| Prostaglandin Prostaglandins are utilized to maintain the patency of the ductus arteriosus until surgical ligation is performed. When surgical ligation is not indicated, prostaglandin inhibitors (eg, nonsteroid antiinflammatory drugs [NSAIDs]) are used to close the ductus arteriosus. |
|
|
Term
| What was the first interventional Catheterization technique done in infants and children? |
|
Definition
| Balloon atrial septostomy |
|
|
Term
| Which side during utero does NOT receive a lot of blood? |
|
Definition
RIGHT because it's pulmonary!
They are not using their lungs! |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Aortic Stenosis
Acyanotic Lesion |
|
|
Term
|
Definition
|
|
Term
|
Definition
Tricuspid Atresia
Cyanotic Lesion |
|
|
Term
|
Definition
Tetralogy of Fallot
Ventricular septal defect
Overriding aorta
Subpulmonary stenosis
Right ventricular hypertrophy |
|
|
Term
|
Definition
| Total Anomalous Pulmonary Venous Return (TAPVR) Cyanotic Lesion |
|
|
Term
C. A. is a 10 day old male who presents to the emergency department with a 2 day history of poor feeding and lethargy. The child is pale, heart rate is 102 beats per minute and his blood pressure in the left upper extremity is 100/48. His cry is weak
How do you evaluate this patient? |
|
Definition
|
|
Term
| What do you think his cardiac condition is? |
|
Definition
Cyanosis?
HPT?
Murmur?
Tachycardia?
Ductus closed after 10 days |
|
|
Term
| What is your differential diagnosis? |
|
Definition
|
|
Term
| How do you initiate therapy? |
|
Definition
|
|
Term
| What is surgical/ interventional option? |
|
Definition
| Balloon Arterial Sepsostomy |
|
|
Term
| What is the long term prognosis? |
|
Definition
|
|
