Flashcards in Congential Heart Diseases: Noncyanotic Deck (82):
What are the subtypes of congenital heart anomalies?
What are the non cyanotic congenital heart diseases?
1. Atrial Septal Defect & Patent
2. Foramen Ovale
3. Ventricular Septal Defect
4. Patent Ductus Arteriosus
5. Coarctation of Aorta
Define atrial septal defect. What are the variations?
1. Opening between R & L atria
2. 80% of cases involve ostium secundum in mid septum
A. Bordered by fossa ovalis
3. Can be isolated or can be asst with other congenital heart abnormalities
4. Left-to-right shunts of varying degrees
5. Size can be variable
What are the functional consequences of Atrial septal defect related to?
1. Anatomic location
2. Size of defect
3. Presence of other cardiac abnormalities
Where are the 3 main locations of ASD?
1. Region of the fossa ovalis (ostium secundum)
2. Superior portion of the atrial septum near the junction with the SVC (sinus venosus)
3. Inferior portion of the atrial septum near the tricuspid valve annulus (ostium primum)
A. Considered part of atrioventricular septal defects (AVSDs)
What is ostium secundum ASD?
Region of the fossa ovalis
What is sinus venosus ASD?
Superior portion of the atrial septum near the junction with the SVC
What is ostium primum ASD?
Inferior portion of the atrial septum near the tricuspid valve annulus
What is the pathophys of ASD?
1. Left atrial pressure > right atrial pressure
2. Blood shunts (L) to (R)
A. Useless circuit:
RA -> RV -> PA -> Lungs -> PV -> LA -> RA
What is a consequence of ASD?
1. Leads to RVH and pulmonary artery dilation
A. Can lead to atrial arrhythmias and HF
What are the dx studies for ASD?
1. USN: in utero
What are the results of an USN in an ASD infant?
Significant defects seen in utero w/USN
What are the results of an EKG in an ASD infant?
1. NSR or atrial arrhythmias
2. RBBB common
3. May have:
A. 1st degree AV block
B. Primum ASD w/L Axis Deviation
C. Secundum ASD w/R Axis Deviation
What are the results of a CXR in an ASD infant?
Enlarged PA, RA, RV
What is an ECHO used for in a ASD infant?
Shunt volume, shunt ratios and PA pressures measured
What is the dx study of choice for an ASD infant?
What is the clinical presentation for ASD?
3. Can lead to CHF, A. Fib & stroke in untreated adults
4. Systolic Ejection Murmur @ 2nd LICS
5. Wide fixed split S2
When does an ASD need to be closed surgically?
Defects > 6 mm
What are the surgical repair options for ASD?
1. Ostium Primum or large Secundum ASD repair
2. Surgical or percutaneous transcatheter technique
A. Pericardial or Dacron patch
B. Amplatzer septal occluder
What is the normal progression of foramen ovale physiology?
1. In utero, PFO needed for oxygenated blood to flow from R→L atrium
2. After birth, pressure in (R) side of heart ↓ as the lungs begin working→ foramen ovale closes
3. Flap fusion complete by age two in 70-75% of children
What percentage of adults have a patent foramen ovale?
Defects range from 1-10 mm in diameter
What can cause the foramen ovale to remain open?
Any ↑ pressure in the pulmonary circulatory system (due to pulmonary HTN, temp. w/coughing, etc.) can cause the foramen ovale to remain open
When is interatrial shunting restricted in patent foramen ovale?
1. Usually no interatrial shunting occurs as long as LA pressure > RA pressure
2. Flap remnant of foramen ovale remains competent
When is interatrial shunting present in patent foramen ovale?
1. If large PFO (>9mm), may cause (L)to(R) shunt → volume overload of right side → Pulm HTN & Right HF
2. If RA pressure ↑ → interatrial shunting through PFO
A. Risk of right to left shunting
What can right to left shunting cause?
Risk of embolism and stroke
What is the clinical presentation of patent foramen ovale in children?
1. Generally asymptomatic in children
2. Systolic ejection murmur 2nd or 3rd ICS along LSB
3. Wide fixed split S2
What is the clinical presentation of patent foramen ovale in adults?
1. Adults-undiagnosed PFO can present as
A. Heart failure
B. Atrial arrhythmias
C. Exercise intolerance
F. Paradoxical embolic Stroke 2°to venous embolism
-. Suspect PFO in any adult < 50 yrs who has a stroke
G. May be related to migraine w/aura
H. SEM 2nd or 3rd ICS along LSB
I. Wide fixed split S2
Whta dx studies are used in patent foramen ovale?
1. TEE: Bubble study
2. Transcranial Doppler (TCD)
What are the results of a TEE in a patent foramen ovale?
1. “Bubble Study”
A. IV Saline solution w/dissolved microbubbles injected & bubbles seen in RA
B. (+) PFO → bubbles pass to the LA thru the PFO
What are the results of a Transcranial doppler in a patent foramen ovale?
1. USN probe placed on the temple
2. IV saline solution filled w/ tiny dissolved microbubbles injected
A. (-)PFO → microbubbles filtered by lungs & no change seen in the blood flow
B. (+)PFO → sharp color lines on the tracing
C. Injection is repeated while the pt takes a deep breath and then exhales forcefully to "open" the PFO
What is the medical treatment for stroke prevention in PFO pts?
1. Anti-platelet medication
2. No anticoagulants recommended
A. 2012 ACCP
3. Surgical correction as prevention is controversial
How are PFO pts treated after an embolic stroke?
1. Warfarin x 3 mo
2. Closure of defect for secondary stroke prevention is controversial and being studied
A. More likely if pt suffered 1 stroke
B. Surgical or percutaneously
Describe Eisenmenger's syndrome
1. If any ASD is left uncorrected → Pulm HTN → (R) heart pressure > (L) heart pressure
2. Reversal of the pressure gradient across ASD → (R) to (L) shunt
3. Portion of oxygen-poor blood shunted to left side of heart and ejected to the peripheral vascular system → signs of cyanosis
What is the most common congenital heart disease?
ventricular septal defect
Describe ventricular septal defect
1. Opening between ventricles
2. Can occur isolated or with other cardiac anomalies
3. Physiologic effects depend on size of the defect and peripheral vascular resistance
What is the pathophys of VSD?
1. After birth, LV contracts against high systemic vasc resistance while RV contracts against lower resistance in pulmonary circulation
2. Leads to left right shunting
3. Moderate or large defects can lead to volume overload of LA & ventricle -> HF
What is the VSD clinical presentation at birth?
Asymptomatic until a few weeks old
What sxs are present after a few weeks in a VSD infant?
3. Failure to thrive
6. +/- thrill over 3rd or 4th LICS
7. III+/VI harsh holosystolic murmur best heard @ left lower sternal border
What are the sxs of late stage or large VSD?
What are the dx studies used in VSD?
What are the results of the EKG in VSD?
1. EKG findings depend on physiologic consequences
A. LAE, LVH, RVH
What are the results of the CXR in VSD?
Prominent pulm vasculature, enlarged PA
Why is an echo used in VSD?
What is the treatment for small VSD?
1. 75% spontaneous closure w/in first 2 yrs of life, if persist into adulthood, usually benign
2. Followed by Peds Cardiologist
What is the medical treatment for mod-large VSD?
Medical management includes diuretics, ACEI, Digoxin and hospitalization for HF
What are the surgical treatment options for VSD pts? When is it performed?
1. Prior to 6 mos of age before irreversible pulm HTN develops
2. Intracardiac repair with direct patch closure or transcatheter closure
What is the normal function of the ductus arteriosus?
1. Fetal vascular connection between PA and Aorta that diverts blood away from pulmonary bed
2. After birth, DA constricts and is obliterated
DA constricts and usually closes w/in 10-15 hrs after delivery
Usually closed fully by 2-3 wks of age
What is the normal ductus arteriosus physiology in utero?
1. In the fetus, RV accommodates approx 60% of total CO
2. Unused pulm vasculature is constricted high vasc resistance w/in pulmonary bed
3. Placenta creates low vasc resistance bed
4. Majority of blood exiting RV passes right to left across DA into descending aorta to placenta
What is the normal ductus arteriosus physiology in after delivery?
1. Upon breathing, lungs expand -> pulm vasodilation -> ↓ pulm vasc resistance
2. Systemic vasc resistance ↑ with removal of placenta
3. Leads to sudden change in blood shunting from right ->left to left -> right
4. Patency of DA is maintained by circulating prostaglandin E2 produced by placenta
What is the rationale for treating PDA in preterm infants with NSAIDs?
Rationale for treating PDA in preterm infants with NSAID’s is based upon role of PG E2 in maintaining DA patency
Who has the highest risk of PDA?
preterm infants born prior to 30 wks gestation
Wha tare the early sxs of PDA?
What are the sxs by 1 yo?
1. Labored breathing/SOB
3. Bounding pulse
4. Poor growth
What are the sxs of late stage PDA?
What are the sxs of a small PDA?
What are the sxs of a mod-large PDA?
1. Exercise intolerance
2. Wide pulse pressure
3. Palpable thrill
4. Continuous machinery like III-IV/VI murmur in infraclavicular region
What are the dx studies for PDA?
3. ECHO w/ doppler flow
What are the EKG & CXR results in a small PDA?
Both may be normal
What are the EKG & CXR results in a mod/large PDA?
1. EKG may be normal
2. May have cardiomegaly & enlarged pulm vessels
What is the confirmatory test for PDA?
ECHO w/ doppler
What are the complications of PDA?
1. Lg untreated PDA can cause significant cardiac volume overload→ HF
Infants with HF present w/failure to thrive, poor feeding and resp distress
2. Infective endocarditis
3. Pulmonary HTN
What are the medical tx for PDA?
1. NSAIDS in premature infants
A. Indomethacin or ibuprofen
B. Inhibits prostagladin synthesis
What are the surgical tx options for PDA in infants?
1. Manually tied shut/surgical clip
2. Intravascular coils or plugs → formation of thrombus in DA
What are the surgical tx of choice for PDA in teens and adults?
Percutaneous catheter occlusion
When is PDA closure recommended?
pts w/ significant left to right shunting & left sided volume overload
define coarctation of the aorta?
in proximal thoracic
aorta just distal
to left subclavian
What may coarctation of the aorta be asst. with?
1. Frequently assoc w/bicuspid aortic valve
2. May have other cardiac anomalies
A. Aortic stenosis, VSD, PDA, or mitral valve abnormalities
How common is coarctation of the aorta? Who is it most common in?
1. 4-6% of all congenital heart defects
A. Except in Turner Syndrome
-10% have COA
-Females lacking X chromosome
What is the etiology of coarctation of the aorta?
1. Majority are congenital
2. Acquired cause
A. Takayasu arteritis
-Chronic vasculitis of aorta
What are the sxs of mild coarctation of the aorta?
What are the early sxs of mod/severe coarctation of the aorta?
1. Difficulty breathing
2. Poor appetite or trouble feeding
3. Failure to thrive
What are the late sxs of mod/severe coarctation of the aorta?
4. CP w/exercise
7. Intermittent claudication
What is the clinical presentation of COA?
1. Cold extremities
2. Systolic murmur LUSB & left interscapular area
3. Young adults
A. HTN may be presenting sign
4. Major clinical manifestation
A. Difference in SBP between UE and LE
B. HTN in UE
C. Diminished femoral pulses
What are the dx studies for COA?
5. Cardiac Cath
What are the EKG results in COA?
Findings may be nonspecific
What are the CXR results in COA?
1. Figure “3” sign on CXR
A. Dilated Left SCA, indentation of coarct & post-stenotic aortic dilatation form a "3
What is the confirmatory test for COA?
when is an MRI indicated fpr COA?
Most conclusive in cases where echo is not
All adults with COA should have at least 1 cardiovascular MRI or CT to assess thoracic aorta and intracranial vessels
What is the medical tx for COA?
Beta blocker for BP control
What is the gold standard for treatment of COA?
What are the benefits/risks of Percutaneous ballon angioplasty & stent placement vs surgery?
Fewer risks compared to surgery
Usually will need planned re-intervention in future