W3 Congenital Heart Disese (Quiz 2. See Study Guide) Flashcards
Review Fetal circulation
What are the 3Ds for Acyanotic? [know]
3Ds
—atrial septal defect
—ventricular septal defect
—patent ductus ateriosus
blood contains enough oxygen but it is pumped abnormally around the body
What are the 5Ts for cyanotic [know]
Cyanotic defects are defects in which blood pumped to the body contains less-than-normal amounts of oxygen, resulting in a condition called cyanosis.
5Ts
—truncus arteriosus
—transposition of the great vessels
—tricuspid insufficiency
—tetralogy of Fallot
—total anomalous pulmonary venous return
What are the risks of congenital HD?
—teratongenic medication (phenytoin, ACE inhibitors, lithium, statins, isotretinoin)
—toxins: smoking, drugs, alcohol
—infections: rubella, toxoplasmosis, CMV, HSV, Hep B
—family history/genetic
—diabetes
Patent foramen ovale (PFO)
What is normal/abnormal?
SX if present? 3
DX?
TX?
—required for oxygenated flow from RA to LA
—closes several days after birth
—if it remains open, usually asymptomatic as LA pressure is higher than the right (L to R shunt)
SX:
—😵💫cryptogenic stroke (cryptogenic = uncertain origin)
—💨 🫁 air embolism
—😮💨platypnea-orthodeoxia (SOB sitting up)
DX:
—echo
TX:
—closed percutaneously
Atrial septal defect (ASD) [know]
What are the types? 4
Associated w/ which conditions
Diagnosed in which decade of life?
What is the patho?
defect in embryological formation of missing tissue
Types based on location:
1. Secundum
—70% involves the region of the fossa ovalis
2. Primum
—15-20% within the spectrum of the atrioventricular (AV) septal defects
3. Sinus venous defect
—SVC and IVC, located at the junction of the right atrium and superior vena cava, a/w partial anomalous pulmonary venous return
4. Coronary sinus defect <1%
—F>M
—a/w Down Syndrome, Fetal Alcohol Syndrome, Holt Oram Syndrome
—dx in 4th decade of life
—L ➡️ R shunt
—RV thinner wall accommodates greater blood volume at same pressure than LV
—volume burden ➡️ volume overload and enlargement of RV
—Pulm artery pressure and volume increase ➡️ development of pulm HTN and increased resistance ➡️ Reversal of shunt right to left (Eisenmenger Syndrome. Understand that this is the disease progression of uncorrected congenital heart disease
[SKILLS OSCE]
ASD
Symptoms kids (3) vs adults (4)
What is the classic finding on PE/auscultation (3) [know this]
What is THE classic finding on EKG? [know this]
Kids
—asymptomatic
—murmur, low pitched
—DOE, fatigue
Adults
—afib, flutter and SVT
—3rd decade of life
—exercise intolerance
—decreased stamina/palps
PE
—RV heave d/t dilated RV
—2/6 systolic ejection murmurs
—wide and fixed split S2 in inferior leads (volume overload, inc. flow across pulmonary valve)
EKG
—crochetage sign
A thrill is a palpable murmur whereas a heave can be a sign of right ventricular hypertrophy. A thrill feels like a vibration and a heave feels like an abnormally large beating of the heart
[SKILLS OSCE]
ASD
CXR findings 4
Think right side…
—enlargement (right side)
—RV forms apex of heart
—increase pulmonary vascular markings
—prominent pulmonary artery
ASD
What are 4 other diagnostics?
—echocardiogram
—TEE
—cardiac cath
—MRI
ASD
Treatment
—fix them w/ or w/o symptoms
—percutaneous closure (secundum)
—surgical repair/closure (primum or sinus venous)
VSD
What is it?
What is the patho?
—anywhere along septum
—muscular 60%
—can close spontaneously
—present w/ multiple defects
—shunt creation between R and L ventricles
—amount of blood and direction determines significance of VSD
—classified according to location and size
—in L to R shunts, pulmonary vascular endothelium undergoes irreversible changes resulting in persistent PAH
—when pressure in pulm circ exceeds pressure in systemic circ, shunt reverses to a right to left shunt = Eisenmenger
VSD
Symptoms
PE findings [know] - esp murmur characteristics
—similar to CHF: tachypnea, poor feeding, failure to thrive, RT infections
—dyspnea and cyanosis
—bacterial endocarditis
PE [know]
—harsh holosystolic murmur
—left sternal border
—systolic thrill palpated over murmur
—mid-diastolic rumble can be heard at the apex
—RV heave and cyanosis can be evident if pulmonary vascular disease develops
[SKILLS OSCE]
What is this?
Enlarged cardiac silhouette
In the case of VSD
—large shunts will show cardiomegaly, prominent vascular markings and enlarged pulmonary arteries
VSD treatment?
50% will close spontaneously by age 2
Kids/babies w/symptoms of heart failure or vascular disease → surgical correction in first few months of life
Patent Ductus Arteriosus
—connects left pulmonary artery to the descending aorta during Fetal life
—constricts after birth normally (d/t BP rising)
—failure to close causes a persistent shunt between aorta and L pulmonary artery
—common in preemies, high altitudes, congenital rubella
—can occur with other heart lesions, genetic syndromes
Patho
—blood from aorta → pulmonary circulation
—L → R shunting occurs in pulm army circulation. LA, LV become volume overloaded
—symptoms relate to size and length of PDA
— →LV dilatation and left HF
—causes progressive rise in pulmonary artery pressures
—if significant, shunt flow reverses and R → L shunting occurs → cyanotic HD
Symptoms
—small PDA → asymptomatic detected accidentally
—moderate PDA → present in adult life. fatigue, exercise intolerance, dyspnea and palpitations
—large → poor feeding, failure to thrive, delayed growth, tachy, resp infections
[SKILLS OSCE]
PDA
PE findings
—continuous machine life murmur → doesn’t change w/ position
—displaced LV apex, possible thrill
—low diastolic BP, wide pulse pressure
—bounding pulse
ECG
—can be normal
—can show LA enlargement or LVH
CXR
—prominent pulmonary artery segment, prominent aortic knob along left sternal border
—larger PDA: enlarged LA and LV w/ inc pulm vasc markings
Tetralogy of Fallot [know]
What are the 4 defects
—most common cyanotic congenital HD
—associated with Down Syndrome or Digeorge Syndrome or other defects
tetralogy
—right ventricular outflow tract obstruction
—RVH (d/t high pressure load on RV from pulmonic stenosis)
—VSD (d/t anterior mal alignment of interventricular septum)
—overriding aorta (receives blood from both ventricles)
Tetralogy of Fallot symptoms and presentation
And PE [know]
Tetralogy of Fallot [know]
DX
TX
Transposition of great arteries
What is it?
Transposition of great arteries
Exam
Diagnostics
Treatment
Egg shape on CXR
What is Eisenmenger Syndrome? [know]
Eisenmenger (I-sun-meng-ur) syndrome is a long-term complication of an unrepaired heart problem present at birth (congenital heart defect). Eisenmenger syndrome is life-threatening.
In Eisenmenger syndrome, there is irregular blood flow in the heart and lungs. This causes the blood vessels in the lungs to become stiff and narrow. Blood pressure rises in the lungs’ arteries (pulmonary arterial hypertension). Eisenmenger syndrome permanently damages the blood vessels in the lungs.
[SKILLS OSCE]
What is this?
Tetralogy of Fallot
[SKILLS OSCE]
What is this?
X-ray of transposition of great arteries
