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Congenital Heart Disease (Patent Ductus Arteriosus (Epidemiology:
F…
Congenital Heart Disease
ATRIAL SEPTAL DEFECTS
Types:
- Ostium Secundum: most common, middle
- Ostium Primum: lower part
- Sinus venosus: Rare, upper part
Epi:
Etiology:
- idiopathic
- genetic? holt-oram syndrome?
S&S:
- infant: no sx
- older kid & adult: exercise intolerance, easily fatigued, HF
- does not cause cyanosis unless RA pressure > LA
PE:
- RV heave
- Fixed widely split S2
- Early systolic flow murmur best heard at left 2nd-3rd ICS
- Later is low-pitched diastolic murmur at upper left sternal border
Diagnostic:
- CXR: ht enlargement, dilated pulm art & incr pulm vasc
- EKG: RAD, RV hypertrophy
- Echo: TOC dilated RA & RV. See ASD and shunt by color-flow doppler
- Cardiac cath (CATH): O2 sat in RA
Tx:
- <4 mm likely spont closure
- CATH closure > 5mm & sy, enlarg, embolism
- Surgical closure > 40 mm, OP or SV ASDs, poor rim, other ht dz
- Contra: severe irreversible pulm HTN
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Patent Ductus Arteriosus
Epidemiology:
- F 2x> than M
- 20-50% of pre-term infants < 1500g
Etiology:
- idiopathic
- maternal Rubella
S&S:
- can be asymptomatic
- sweating w/ crying or eating
- breathlessness
PE:
- Split S2
- Rough machinery murmur, best at 2nd ICS
Diagnostic:
- CXR: prom pulm art, RA&RV enlarg
- EKG: LV hypertrophy
- Echo: TOC
Tx:
- Medical: Indomethacin
- Spontaneous closure up to 1 yr
- Surgical: cath plug/coil b4 1 yr
Meds:
- Prostaglandins (Alprostadil), maintain PDA (relaxes art smooth muscle)(contra - apnea in 10-12% neonates)
- Indomethacin (NSAID) reduces prostaglandin and throm synthesis (promotes PDA closure) contra - CVD, clots, GI bleeds
Pulmonary Valve Stenosis
Pulm valve annulus is small and obstructs blood flow across the pulm valve. Caused by failure of leaflets to form correctly or thickened valve.
PE:
- Palpable RV heave
- Systolic thrill in pulmonary area
Severe:
- Systolic ejection CLICK @ 3rd Lt ICS > w/ exh
- S2 splitting > w/ more stenosis
Diagnose:
- CXR
- EKG
- Echo: confirms dx
- Cath
Tx:
- Medical: keep PFO open until repair
- Surgical: balloon valvuloplasty (POC) or valvulotomy
Aortic Valve Stenosis
Congenital
- Unicuspid (ped dx)
- bicuspid (adult dx)
- abnormal tricuspid
Acquired
- Degen calcification
- Rheumatic Ht dz
S&S
- Only severe has sx in neonate (sx of HF)
- Gradual development as adult (cp, syncope...)
PE:
- systolic thrill
- narrowed pulse pressure
- LV heave or lift
- cres-decres systolic murmur @ 2nd ICS, Rt sternal border w/ radiation
- Early systolic ejection click
- Split S2
Tx:
- valve rpl
- balloon valvuloplasty
Coarctation of the Aorta
- associated with bicuspid aortic valve 80-85% of the time
- Turner syndrome
- decreased femoral pulses, LE cyanosis
- Systolic murmur Lt infraclavicular area & under the left scapula
- CXR: notching of ribs, middle 3 sign
- cath rare for dx
- Tx: prosta to open DA
- Tx before 5 yoa or HTN happens
-
tetralogy of fallot
- Pulmonary Infundibular Stenosis
- RV hypertrophy
- Overriding aorta
- VSD
PROV
-
- few children are asymptomatic
- "Tet spells" - cyanosis with crying or feeding, worse after DA closure
- Older kids naturally squat to increase blood flow
- CXR: Boot-shaped heart
- EKG: RAD, RVH
- Echo: dx
- Tx: shunt blood to lungs, Open ht sx
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