Lecture 5 (Peds Cardio)-Exam 2 Flashcards
What is congenital heart disease? What is it also known as?
- A malformation of theheart, aorta, or other large blood vessels that change the normal flow of blood through the heart.
- Also known ascongenital heart defect, congenital heart malformation, congenitalcardiovascular disease, congenital cardiovascular defect, & congenital cardiovascular malformation.
Congenital Heart Disease (CHD)
* Most frequent form of what?
* Born with what?
* Affects how many kids?
- CHD is the most frequent form of major birth defect in newborns.
- Born with the defect but can present at all ages
- Affects ~ 8 out of every 1,000 newborns
- Each year more than 35,000 babies in the US are born with CHD
Congenital Heart Disease (CHD)
* What are the sxs?
* What is the spectrum?
- Signs & symptoms – depend on the type and severity of the defect, can include: asymptomatic, cyanosis, CV collapse, CHF
- Benign -> Life threatening
Causes of CHD
* What is the mcc?
* Maternal exposures?
* Family history?
* Prematurity?
- Most congenital heart defects have no known cause
- Maternal Exposures: Medications, Viruses, Diseases
- Family History: 3 x increased risk when a first-degree relative has CHD
- Prematurity: 2 to 3x higher in preterm= gestational age <37 weeks
Causes of CHD
* Genetic? (3)
- Genetic syndromes -Chromosome abnormalities (trisomy)
- Genetic syndromes -Single Gene Mutation (marfan’s)
- Genetic syndromes -Other: CHARGE syndrome, VACTERL association
Three common classifications of congenital heart disease, according to the American Heart Association.
- Septal defects
- Obstructive defects
- Cyanotic defects
What are all the different septal defects?
- Patent Ductus Arteriosus (PDA)
- Atrial Septal Defect (ASD)
- Ventricular Septal Defect (VSD)
- Atrioventricular Canal Defxect (AV canal)
Patent Ductus Arteriosus (PDA)
* Communication between what?
* Shunt?
- Persistent communication between descending thoracic aorta & pulmonary artery
- Left to right shunting (acyanotic aka no blue blood)
* Left to right meaning Aortic blood is being shunted into pulmonary artery which leads to increase blood/dilation of left atrium/ventricule
- Occurs in 3-8 of every 10,000 full-term births,
- 2:1 female-to-male ratio
What are the risks that can cause PDA?(5)
- Risks: prematurity (~8 in 1,000), perinatal distress, hypoxia, Down syndrome, Rubella in first trimester
PDA clinical manifestations:
* May be what into adulthood?
* What are some common signs in babies?(5)
- May be asymptomatic (into adulthood)
- Poor feeding and growth
- Sweating while feeding or crying
- Fast breathing
- Rapid pulse
- Tiring very easily
PDA clinical manifestations:
* What does the exam show?
- Continuous Machinery Murmur (classic sign)
- Bounding peripheral pulses
PDA-Diagnosis
* What imaging can be done? What are the results?
* What is gold standard?
- CXR- normal; may have cardiomegaly (if severe)
- ECG – normal; LVH and left atrial abnormality
- ECHO – Gold Standard test
PDA-Treatment
* What is the treatment?
- Observation (often resolves without intervention)
- IV Indomethacin (preterm infants-28 weeks)-> CLOSE PDA
- Surgical intervention if doesn’t close with Indomethacin
* Surgical ligation <6kg; Percutaneous occlusion >6kg or in adolescents &adults
Atrial Septal Defect (ASD)
* What is it?
* Shunt?
* Difficult to differentiate between what?
- Hole in the septum between the two atria - “hole in the heart”
- One of the most common etiologies of right heart dilation due to L->R Shunting
- Difficult to differentiate a PFO from an ASD
Atrial Septal Defect (ASD)
* How many causes of all congenital heart diease?
* Occurs in how many births in the US?
- 10% - 15% of all congenital heart disease
- Occurs 1-2 in 1,000 live births in the US
What are the risks of ASD?
family history, genetic disorders, drugs/alcohol
ASD
* Most common congenital lesion in adults after what?
Most common congenital lesion in adults after bicuspid aortic valves (Second most common congenital heart defect in adults)
ASD clinical presentation:
* Small ASD=
* Large ASD=
- Small ASD= no symptoms until adulthood, usually >30 years
- Large ASD= infants and children symptomatic
* recurrent respiratory infections, difficulty breathing, tiring when feeding (infants), dyspnea on exertion, fatigue, palpitations, arrhythmias, rarely heart failure
All depends on size of defect
ASD- Clinical Presentation
* Adults manifest what?
* What does the exam show?
Adults manifest same symptoms + increased risk of heart failure
* recurrent respiratory infections, difficulty breathing, tiring when feeding (infants), dyspnea on exertion, fatigue, palpitations, arrhythmias, rarely heart failure
Exam-
* Wide fixed split S2, systolic ejection murmur – left upper sternal border
* Right ventricular heave (at LSB)
ASD patho
* How is the blood moving and why?
* What does the moving of blood cause?
- Difference in compliance between the Right Atrium + Right Ventricle as compared to Left Atrium + Left Ventricle + the size of defect = shunting of oxygenated from left side of heart to right side
- Increased pulmonary blood flow from L->R shunting= increased pulmonary artery pressure = Pulmonary HTN
- Increased blood flow from L->R shunting= increased volume to R Atria = R Ventricle enlargement
What is the MC place for ASD? What are the other places?
ASD diagnosis:
* What are the different tests and results?
* What is the gold standard?
CXR – Normal; may have cardiomegaly with increased pulmonary vascularity (dt increase pHTN)
ECG – Normal; Incomplete RBBB
* Crochetage sign – notching of the peak of the R wave
ECHO- Gold standard test
ASD treatmetn:
* What is the treatment?
- Observation-small ASDs typically resolve by age 5
- Surgical correction for moderate/large ASDs typically > 2 yrs or persistent small ASDs in adults having significant symptoms
What does ASD lead to?
RIGHT Heart Dilatation
* This is because right side is more compliant
What is the EKG sign for ASD?
Patent Foramen Ovale (PFO)
* What is it?
* Not considered an ASD, why?
* Most important potential manifestation is what?
- Open communication between right and left atria
- Not considered an ASD because no septal tissue is missing
- Most important potential manifestation is ischemic stroke due to embolism
Patent Foramen Ovale (PFO)
* Occurs in how many people?
* Often persists into what?
* Most patients are what?
- Occurs in about 25 % of the normal population
- Often persists into adulthood
- Most patients are asymptomatic
What septal defect is not considered CHD and why?
PFO because only occurs after birth when the foramen ovale fails to close (not techially born with it)
What causes Left heart dilation?
VSD and PDA
Ventricular Septal Defect (VSD)
* What is it?
* Most common type of what?
* Occurs in how many people?
- Hole in the septum between the two ventricles
- Most common type of congenital heart disease in childhood
- Occurs 4 in 1,000 live births in the US
ASD=ADULTS
What are the risks of VSD?
family history, genetic disorders (trimosy 21), a drugs/alcohol
VSD-Clinical presentaion
* Small VSD=
* Large VSD=
- Small VSD= asymptomatic, murmur may be detectable at 4 - 10 daysold, systolic thrill present
- Large VSD= present at 3-4 weeks of life with symptoms of tachypnea, sweating, poor feeding, poor weight gain, arrhythmias, absent thrill, possible pulmonary HTN and heart failure
What is shown on exam for VSD?
harsh/blowing holosystolic murmur heard best at lower left sternal border (smaller = louder)
Pathophysio of VSD
* What is happening is small, moderate and large VSD?
- Small: high resistance to flow permits only a small L-> R shunt, RV pressure remains normal or only minimally elevated
- Moderate: RV pressure decreases after birth, the L -> R increases, may lead to volume overload of the left atrium and ventricle
- Large: little resistance to flow, large L-> R shunt = increased pulmonary blood flow ->increased pulmonary venous return-> increased volume load to LV->LV dilation
VSD- Diagnosis
* What are the different tests? Results
* What is goal standard?
- CXR –Normal; increased pulmonary vascular markings, left atrial enlargement, LV & RV hypertrophy
- ECG- Normal; may show LVH in moderate/severe (increased voltage in V5 and V6 or leads II, III)
- ECHO- Gold Standard test
VSD
* What is the treatment?
Observation-small VSDs, 75% close by 2 yrs old
* Surgical- moderate/large VSD with heart failure symptoms-patch closures, transcatheter closures
Eisenmenger’s Syndrome
* What is it?
* Caused by what?
- Left to right shunt reverses to become RL due to prolonged increased pressure
- Caused by: VSD, ASD, PDA, AV Canal defect
Eisenmenger’s Syndrome
* What are the sxs?
* What are the classic exam findings?
* What is the mean age death?
- Symptoms: cyanosis, pulmonary HTN, syncope, atrial and ventricular arrhythmias, heart failure
- Classic exam findings: Cyanotic lower extremity, clubbing of nails, SOB at rest, palpitations
- Mean age death: 37 years old
What are the obstructive defects? (3)
- Aortic Stenosis
- Pulmonary Stenosis
- Coarctation of the Aorta
Coarctation of the aorta:
* What it is?
* How many of the CHD?
* How many births?
* What is the gender predominance?
- Narrowing of the Aorta typically around ductus arteriosus in upper thoracic aorta.
- 5% of CHD
- 4 in 10,000 live births in the US
- 2:1 male predominance
Coarctation of the aorta:
* Risks: genetic disorders, familial risk
Turners syndrome
-workup for this very common
Coarctation of the Aorta-Clinical Manifestations (MILD)
* What happens in neonate, infant/children, adults?
- Neonate= asymptomatic, especially if PDA persistent
- Infant/Children= chest pain, cold extremities with activity
- Adults= HTN
Coarctation of the Aorta-Clinical Manifestations (MOD/SERVERE)
* What are the sxs?
irritability, sweating, poor feeding, poor weight gain, heart failure and SHOCK when PDA closes
Coarctation of the Aorta:
* What does the exam show?
- Decreased perfusion/pulses in lower extremities
- Reduced systolic BP in the lower extremities compared to upper extremities
- Continuous Systolic murmur – radiates to the back/scapula/chest
Coarctation of the Aorta- Diagnosis
* What are the dx tests? What are the results?
* What is gold standard?
- CXR – Cardiomegaly (neonate), Notching appears in ribs due to erosion by the large collateral arteries (children & adults)
- ECG – Normal; LVH
- ECHO- Gold Standard Test
What is the txt of coarctation of the aorta?
- Maintain the PDA- IV prostaglandin
- Surgery-balloon angioplasty, stenting
What are the acyanotic lesions? What does it result in?
- Patent Ductus Arteriosus (PDA)
- Atrial Septal Defect (ASD)
- Ventricular Septal Defect (VSD)
- Atrioventricular canal (AV canal)
- Pulmonary stenosis
- Aortic stenosis
- Coarctation of the Aorta
Result in pulmonary over-circulation
Cyanotic defects:
* What are the 5 Ts?
- Tetralogy of Fallot (TOF)
- Tricuspid anomalies
- Truncus arteriosus
- Total anomalous pulmonary venous return (TAPVR or TAPVC)
- Transposition of the great arteries (TGA)
Cyanotic defects:
* Results from what?
Result from mixing of deoxygenated with oxygenated blood or R->L shunting
What is the mnemonic cyanotic congenital heart defects?
- One big trunk:Truncus arteriosus
- Two interchanged vessels: Transposition of the Great Vessels
- Three:Tricuspid Atresia
- Four:Tetralogy of Fallot
- Fivewords:Total Anomalous Pulmonary Venous Return
Cyanosis in CHD:
* To be present, how much deoxyhemoglobin must be present?
* What does that equate to on room air?
* What affects level of O2 sat at which cyanosis is detected?
- To be present, 3-5 g/dL of deoxyhemoglobin must be present
- Equates to O2 sat of 80-85% on room air
- Total Hgb levels affect level of O2 sat at which cyanosis is detected
Cyanosis in CHD:
* What can happen with infants?
Infants have high fetal Hbg levels= lower O2 levels before cyanosis visible
When may Cyanosis be observed? (4)
- Normal infants have central cyanosis for 5 - 10 minutes after birth as the oxygen saturation rises from 85% to 95% by 10 minutes of age
- Persistent cyanosis is always abnormal and should be evaluated
- First few weeks of life
- Episodically through childhood if uncorrected
What is important history of cyanotic CHD?
Tetralogy of Fallot (TOF)
* Most common what?
* How many live births?
* How many of all CHD?
* WHat are the risks?
- Most common Cyanotic Congenital Heart Disease
- 4-5 in 10,000 live births in the US
- 7%-10% of all congenital heart disease
- Risks: Down’s, DiGeorge Syndrome
Tetralogy of Fallot (TOF)
* What are the 4 components?
- Large VSD
- RV outflow obstruction (Pulmonary Stenosis)
- Overriding aorta
- Right ventricular hypertrophy
Tetralogy of Fallot (TOF)-Clinical Manifestations
* When do they become symptomatic?
- Typically asymptomatic until 2-5 months of age
Tetralogy of Fallot (TOF)-Clinical Manifestations
* What is a tet spell?
hypercyanotic & hypoxic episode (increased R ->L shunting)
* acute cyanosis that develops when infant is agitated, feeding and resolves
* toddlers/older children might instinctively squat when they’re short of breath
What does the exam show in tetralogy of fallot?
- NO Murmur during Tet Spells
- Palpable RV impulse
- Systolic, crescendo-decrescendo murmur at LSB (when calm), Loud S2
Tetralogy of Fallot (TOF)
* What are the dx tests? What do they show?
* What is gold standard?
- CXR-Boot shaped heart
- EKG- RV hypertrophy
- ECHO-Gold Standard
What is the tetralogy of fallot tx?
- Tx-Surgical Repair <6 months old
Transposition of the Great Arteries (TGA)
* What is it?
* Most common what?
* How many of CHD?
* How many births?
- Aorta arises from RV & Pulmonary artery from LV
- Most common cyanotic congenital heart defect in the Newborn
- 3% of CHD (20% of all cyanotic)
- 4.7 in every 10,000 live births in the US
Transposition of the Great Arteries (TGA)
* What does the exam show?
- cyanosis, tachypnea
- systolic murmur if VSD, single S2
Transposition of the Great Arteries (TGA)
* What is the dx test? What does it show?
* What is the tx?
- CXR- egg-shaped heart (after 1st week), with narrowing of the mediastinum
- Tx-Surgical Repair (often 1st week of life), administer
prostaglandin
What does this show?
Transposition of the Great Arteries (TGA)
What does this show?
Transposition of the Great Arteries (TGA)
Tricuspid Atresia
* What is it?
* What is needed for survival?
- No tricuspid valve = no flow RA->RV->decrease pulmonary blood flow
- PDA, ASD or VSD are necessary for survival (give prostaglands)
Tricuspid atresia:
* How many of CHD?
* How many live births?
* In 25% of the cases great arteries are what?
* What is the mortality rate if untreated?
- 1-2% of CHD
- 1 in every 10,000 live births in US
- In 25% of the cases great arteries are transposed with VSD & PS
- High mortality rate if untreated <10% survival at 1 year old
Tricuspid Atresia-Clinical Manifestations
* What are the sx? When do sxs start?
- 50% present with symptoms on the first day of life
- Tachypnea, cyanosis, poor feeding
Tricuspid Atresia-
* What does the exam show?
- Cyanosis at birth, Hepatomegaly (if CHF present)
- Single S2, Grade 2/6 or 3/6 holosystolic murmur LLSB (if VSD present)
What is the txt of Tricuspid Atresia?
Tx: IV prostaglandin, Surgical Repair (before 1)
Total Anomalous Pulmonary Venous Return
(TAPVR)
* What is it?
* How many of CHD?
* How many live births?
* What is the risk?
- All four pulmonary veins fail to make their normal connection to the left atrium
- ~1% of CHD
- 1 in every 10,000 live births in US
- Risks: Heterotaxy
Total Anomalous Pulmonary Venous Return (TAPVR)
* What are the four types?
* What is enlarged?
4 types: Supracardiac, Cardiac, Infracardiac, Mixed
* entire oxygenated pulmonary venous return mixes with deoxygenated blood
RA and RV enlargement
Total Anomalous Pulmonary Venous Return (TAPVR)
* What does the exam show?
- Cyanosis, CHF in first few weeks of life
- RV Heave
- fixed split S2, systolic ejection murmur LSB
- diastolic rumble across Tricuspid valve
* if pulmonary obstruction present – cyanosis & loud single S2
Total Anomalous Pulmonary Venous Return (TAPVR)
* What is the treatment?
Tx- Surgical Repair in infancy
Truncus arteriosus:
* What is it?
* How common?
* How many births?
* What is a risk?
- Pulmonary, systemic and coronary circulations originate from single arterial trunk
- Rare <1% of CHD
- 6-10 per 10,000 live births in the US
- Risks: DiGeorge’s syndrome
Truncus Arteriosus
* What does the exam show?
* What is the tx?
- Exam-CHF, cyanosis within the first few week of life Single S2, Systolic murmur at LSB
- Tx- Surgical Repair in infancy
Key Physical Exam Components Cyanotic Heart Disease? (9)
- Vitals (Include Pulse ox, BP in upper and lower extremities)
- Weight and growth
- Document central or peripheral cyanosis
- Note location, timing and loudness of murmurs, if present
- Note gallop or splitting of S2
- Palpate chest for heaves and thrills
- Strength and quality of pulses
- Check for splenomegaly and hepatomegaly
- Lungs – rales/signs of CHF
Diagnosis of Cyanotic Heart Disease
* What is not helpful?
* What can help distinguish heart disease from other causes of cyanosis? What is the issue though with neonates?
- Labs not helpful
- PaO2 in response to breathing 100% O2 may help distinguish heart disease
from other causes of cyanosis
* neonates will not demonstrate this due to fetal Hgb
Diagnosis of Cyanotic Heart Disease
* What are the primary tests? What do they show?
- Primary tests – Chest X-ray and ECG
- CXR may show abnormal position of the aortic arch may be clue to dx of CHD
- ECG useful in evaluation of chamber size, electrical axis and conduction, right ventricular hypertrophy
What is the gold standard of sx cyanotic heart disease?
- Echo-Gold Standard
Treatment of Cyanotic CHD
* What does it depend on?
* Obstructive lesions require what?
* What is the process of etralogy of Fallot
* What happens if the neonates tolerate o2 levels in the 70s?
- Depends on age of patient, hemodynamic stability and previous interventions
- Obstructive lesions require a patent ductus arteriosus – patency may
require prostaglandins - Tetralogy of Fallot -surgically repaired in first 4-12 months of life, others in first few weeks
- Neonates tolerate oxygen saturation rates in the 70’s – administering oxygen is not always required or recommended and such decisions are made by a specialist
What patients need antimicrobial prophylaxis for the prevention of bacterial endocarditis?
- Unrepaired Cyanotic CHD
- Completely repaired CHD with prosthetic material or device the first six months after the procedure
- Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device
