Clinical Assessment of Congenital Heart Disease
Terms
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- Life-threatening cardiac disease in newborn infants occur in about ___ in 1,000 live births.
- 3
-
Risk and Risk Factors
Maternal Diabetes ______________ - HCM, VSD, TGA, COA
- SLE could mean _________
- congenital heart block
- Congenital Rubella could mean _________-
- PDA & PPS
- ___ risk in the general population
- 1%
- ____ risk for second pregnancy if first resulted in CHD
- 2-6%
- ______ for 3rd child if 1 & 2 = CHD
- 20-30%
- Stenotic Lesions could be ________ or _______
-
Aortic stenosis
Pulmonary stenosis - Shunting Lesions could be ______ or _______
-
Right to Left
Left to Right - Mixing lesions could be ______,______, or_______
-
Truncus
TAPVR
HLHS - Acyanotic Congenital Heart lesions can be _________ or ________
- volume lesions or obstructive lesions
- Volume lesions involve left to right shunts or shunting of _________ blood back to the lungs. Increasing shunting leads to increased pulmonary blood flow, decreased pulmonary compliance, increase work of breathing and finally increase left ventricular ou
- fully oxygenated
- Volume lesions include ______, ______,______, & ______
- VSD, ASD, AV canal, and PDA
- __________ hinder cardiac/pulmonary output leading to increased ventricular muscle mass (hypertrophy) to pump against the obstruction to maintain cardiac/pulmonary output
- Obstructive lesions
- Obstructive lesions include ________, __________, & _______
- Valvular pulmonic stenosis, aortic stenosis, and coarctation of the aorta
- ______ is the most common cardiac anomaly with an incidence of 1.5 to 2.5 per 1,000 live births
- VSD
- VSD Seldom cause significant problems and are detected because of associated ________ usually not present at birth
- heart murmur
- 4 types of VSD are
-
Perimembranous
Muscular
Subarterial defects
Malalignment defects - 30-40% of VSD close spontaneously by age ____ months
- 6
-
______ has a murmur that is:
loud
harsh
blowing
pansystolic
LLSB +/- thrill - VSD
- _____ of all Congenital Heart Disease is an Atrial Septal Defect
- 5-10%
-
______ has a murmur that is:
Ejection
Medium pitched
LMSB
2nd heart sound widely split
secondary to increase RV diastolic volume and prolonged ejection time - Atrial Septal Defect
- Symptoms of ASD develop in the ____ decade of life
- 3rd
-
Late manifestations of ASD include:
________ Hypertension
Tricuspid and Mitral ________
______ arrhythmias
__________, paradoxical embolization through an ASD -
Pulmonary
regurgitation
Atrial
Cerebrovascular accident - _____________ involves a normal part of fetal anatomy which allows 80-92% of right ventricular outflow to bypass the lungs and enter the descending aorta.
- Patent Ductus Arteriosus PDA
- PDA remains open in fetal life because of low ____.
- PaO2
- PDA usually closes in a term infant within __ day
- 1
- Persistence of the PDA may lead to ______ and _________
-
pulmonary vascular disease
infective endocarditis - The __________ in PDA is a continuous musical/machinery like murmur heard best at the ULSB and subclavicular areas
- Murmur
-
PDA also involves:
- ______ pulses
- Hyperdynamic precordium
- ______ EKG or mild LVH
- CXR
- _____ LA and LV -
Bounding
Normal
Large - Coarctation of the Aorta (COA) is _____ of all CHD
- 8-10%
- 98% of COA occur _____the origin of the left subclavian artery
- below
- COA is _____ more likely in males
- 2X
- 30 % of those with ________ have COA
- Turners Syndrome
- those with COA often have ________ aortic valve in (70%)
- Bicuspid
- There is _______ BP proximal to COA & ________ BP distal to COA
-
Increased
Decreased -
In COA, infants often present with signs and symptoms of _________ including:
-Weak peripheral pulses with poor perfusion
-Poor urine output
-Lethargy
-Poor feeding
-Metabolic acidosis
-No history of illness symptoms - cardiovascular collapse
-
What is this murmur?
S2 single and loud
No murmur in 50 %
Systolic ejection murmur
Gallop rhythm
Blood pressure differential upper vs lower extremity - Coarctation in Infant
-
What is this murmur?
Short systolic
LSB 3-4th space
Transmitted to back and neck
Interscapular murmur - Coarctation - Older Child
- ___% of patients w/ coarction have upper extremity hypertension
- 90
- ___% of patients w/ coarction have weak/absent femoral/pedal pulse
- 40
-
Some people with coarctation die between 20-40 yrs. of age secondary to ________
-Premature coronary artery disease
-Congestive heart failure
- Hypertensive encephalopathy
-Intracranial hemorrhage
Infective Endocarditis - hypertension
- Cyanotic - 5 T’s and 1H
-
Tetralogy of Fallot
Transposition of the Great Arteries
Total Anomalous Pulmonary Venous Return
Tricuspid Atresia
Truncus Arteriosus
Hypoplastic Left Heart Syndrome - TOF occurs in _____% of CDH. Pulmonary Atresia in __%
-
10%
1% - Transposition of the Great Arteries occurs in _____ of those w/ CHD
- 5%
- Total Anomalous Pulmonary Venous Return occurs in ____ of those w/ CHD
- 1%
- Tricuspid Atresia occurs in _____% of those w/ CHD
- 1-3%
- Truncus Arteriosus occurs in _____% of those w/ CHD
- <1%
- Hypoplastic Left Heart Syndrome occurs in _____% of those w/ CHD
- 1%
- _______ is the most common cyanotic heart defect in children beyond infancy
- Tetralogy of Fallot--TOF
- Tetralogy of Fallot includes ____________, _________, _____________, ___________, _________
-
Pulmonary stenosis
Ventricular septal defect
Right ventricular hypertrophy
Overriding Aorta
25 % have a right sided aortic arch - With TOF _____ develops at birth or later depending on size of VSD (pink Tets)
- Cyanosis
-
Your listening to a pts heart and you hear:
-S2 single in aortic component only
-A heart murmur with:
-Systolic ejection murmur
- long murmur
-Grade 3-5/6
-LUSB - LMSB - TOF
-
Occurring in am following crying, feeding, defecation, a pt w/ a CHD experiences:
-Severe hypoxia
-Hypernea
-Irritability/crying
** DECREASED HEART MURMUR** - TOF - Tet Spell
-
A severe spell can lead to:
-Limpness
-Seizure
-Cerebrovascular accident
-_________ - Death
-
Tet Spell - Treatment
-Knee chest position
-Morphine
-Treat acidosis
-Vasoconstrictors (i.e., phenylephrine, Ketamine)
-Propranolol
note: ________ - doesn’t help - Oxygen
-
A 2 day old infant is noted to be feeding poorly with labored breathing. You note that the tongue appears blue tinged. Pulse Ox shows sats at 85 percent. 100% oxygen hood is given over 20 minutes. Blood gas reveals a PaO2 of only 78%.
CXR shows the - Transposition of the Great Arteries
- Transposition of the Great Arteries is ____% of all Congenital Heart Disease
- 5%
-
In TOTGV the _____arises from the right ventricle and carries desaturated blood to the body.
The __________ arises from the left ventricle and carries saturated blood to the lungs. -
aorta
pulmonary artery - Transposition of the Great Arteries ______ involve atrial or septal defects
- may
- Transposition of the Great Arteries involves Male: female ratio of _____
- 3:1
-
A child has cyanosis at birth
CHF with tachypnea, no respiratory distress.
S2 is single and loud with
amurmur (diagnosis does not depend on murmer, may or may not have murmur). There is a VSD. What is the likely diagnosis? - TGA-Transposition of the great arteries
- A child with TGA that has a murmur has a _____________ as well.
- Ventricular septal defect
- TGA will show _______-hyperoxic challenge 100% FiO2 without increase of peripheral saturations or PaO2.
- hypoxia
- TGA will show Metabolic _______
- acidosis
-
TGA CXR will show:
-________ with increased PVM
-Egg on a string = __________ -
cardiomegaly
narrow mediastinum - ECG will show ______ & _____
- RAD, RVH/CVH
- A newborn is noted to have profound cyanosis at birth. No murmur is heard EKG shows marked RVH. CXR demonstrates a large amount of interstitial fluid and pulmonary congestion. There is also an abnormal cardiac silhouette.
- Total Anomalous Pulmonary Venous Connection
- Total Anomalous Pulmonary Venous Connection is ___ of all Congenital Heart Disease
- 1%
- Total Anomalous Pulmonary Venous Connection has a male: female ratio of _______.
- Male > Female (4:1)
- in _______________there is not a direct communication of the pulmonary veins to the left atrium
- Total Anomalous Pulmonary Venous Connection
-
There are three types of Total Anomalous Pulmonary Venous Connection
1._____________
2._____________
3._____________ -
Supracardiac 50%
Intracardiac 20%
Infracardiac (subdiaphragmatic) 20% - present in first 3 days of life. - A baby is born that is cyanotic with congestive heart failure - tachypnea, dyspnea, hepatomegaly, tachycardia. S2 is widely split, increased P2. She has a gallop rhythm w/ no murmur. What is the likely diagnosis?
- TAPVC
-
A 2 month old male infant presents for his well child check. He does not appear to be growing well (FTT). You note a large +4/6 SEM with a thrill that is harsh and located in the LLSB. You note mild cyanosis of the lips.
CXR shows a normal size hear - Tricuspid Atresia
- Tricuspid Atresia is ____ of all Congenital Heart Disease
- 1-3%
- In ___________ Tricuspid valve is absent. No outflow for venous blood from the heart. Blood shunted from a patent foramen ovale or large VSD. Hypoplastic right ventricle
- Tricuspid Atresia
-
Severe cyanosis at birth or maybe normal depending on the size of VSD and PFO
Tachypnea and poor feeding.
Single S2
Murmur when VSD present
This presentation describes __________. - Tricuspid Atresia
-
A 3 week old infant is seen for turning blue with crying. Mom states no problems with the baby at delivery or in the newborn period. At the 2 week well visit a physiologic murmur is noted in the chart.
On examination the infant is tachypneic. A +2/6 - Truncus Arteriosus
- Truncus Arteriosus is ____ of all Congenital Heart Disease
- <1%
- Truncus Arteriosus is associated with ________ and _____________
- Associated with DiGeorge syndrome and absent thymus
- In ___________ there is a single arterial trunk to the arterial and pulmonary system, almost always associated with a large VSD ventricular septal defect. There is a single valve bicuspid, tricuspid, quadracuspid.
- Truncus Arteriosus
- The four types of truncus arteriosus are differentiated by the position of the peripheral __________ on the arterial trunk.
- pulmonary arteries
- Those with TA may not have _______ at birth, depends on degree of PVR
- cyanosis
-
Those with truncus Arteriosus develop ________
including:
-Tachypnea
-Poor feeding
-Failure to thrive - congestive heart failure
- Those with TA have _______ pulses
- Bounding
- Those with TA have single S2 - with _______
- click
-
Murmur for TA:
-Harsh systolic 2-4/6 LSB - VSD
-Diastolic decrescendo murmur at apex
-Truncal valve ________ - regurgitation
-
A 2 day old female infant has been doing well in the newborn nursery. Suddenly she becomes lethargic and develops circulatory collapse.
Baby appears grayish and mottled. All peripheral pulses are absent. No murmur is heard.
CXR shows severe car - Hypoplastic Left Heart Syndrome
- Hypoplastic Left Heart Syndrome is ___ of Congenital Heart Disease
- 1%
- Those with hypoplastic Left Heart Syndrome may do well initially until the ____ closes then shock, acidosis and hypotension with cardiac failure occurs
- PDA
- In those with Hypoplastic Left Heart Syndrome EKG reveals severe ____
- RVH
- HLHS involves mitral valve ______ vs atresia
- stenosis
- HLHS involves ________ left ventricle
- Hypoplastic
- HLHS involves Aortic valve ________ vs atresia
- stenosis
- HLHS involves, ______ ascending aorta and arch
- Hypoplastic
- Cardiac anomalies associated with HLHS are _______, _______, & ________.
-
Atrial septal defect
Ventricular septal defect
Coarctation of the aorta - Those with HLHS are Critically ill in the first few hours of life - dependent on timing of __________
- ductal closure
-
The circulatory shock of HLHS results in ____ peripheral perfusion & ___
pulses -
Poor
weak -
HLHS results in severe cyanosis, Tachypnea, dyspnea
Single loud ___,No murmur, &
Gallop - S2