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Pediatric Cardio Part 1 Cheat Sheet by

Congenital Heart Disease - both Acyanotic & Cyanotic

Cyanotic VS. Acyanotic CHD

Acyanotic heart disease
Cyanotic heart disease
L to R shunt
R to L shunt
obstru­ction occurring
  beyond lungs
blood passes through pulmonic circul­ation
blood bypasses the lungs
oxygen­ation takes place
no oxygen­ation occurs
levels of deoxyg­enated blood in systemic circul­ation
levels of deoxyg­enated blood enters systemic circul­ation
no cyanosis
Cyanosis: blue mucous membranes, nail beds, & skin secondary to an absolute conc. of deoxyg­enated Hb of at least 30 g/dL


L to R shunt lesions
- extra blood displaced through commun­ication frm L to R side of heart pulm. blood flow pulm. pressures
- shunt vol. dependent upon 3 factors:
      1) size of defect
      2) pressure gradient b/w chambers / vessels, and
      3) peripheral outflow resistance
- untreated shunts can result in pulm. vascular disease, L ventri­cular dilatation & dysfun­ction, R ventri­cular HTN & RVH, and ultimately R to L shunts
Obstru­ctive lesions
- present w/ urine output, pallor, cool extrem­ities & poor pulses, shock, or sudden collapse
- syst. venous return re-enters syst. circul­ation directly
- most prominent feature = cyanosis (O2 sat. <75%)
- hyperoxic test differ­ent­iates b/w cardiac & other causes of cyanosis
      --> obtain preductal, R radial ABG in room air, then repeat after child inspires 100% O2
      --> if PaO2 improves to >150 mmHg, cyanosis less likely cardiac in origin
- pre-ductal & post-d­uctal pulse oximetry
      --> >5% diff. suggests R to L shunt
1. Acyanotic incl.:
- Coarct­ation of aorta, Aortic stenosis, Pulm. stenosis

2. Cyanotic incl.:
- Tetralogy of Fallot
- TGA, Total Anomalous Pulm. Venous Return, Truncus Arteri­osus, Hypopl­astic L Heart Syndrome

Atrial Septal Defect

3 types:`
- ostium primum
-- common in DS
-- defect located @ mitral / tricuspid valve
- ostium secundum
-- most common type
-- 50-70%
-- defect located @ septum b/w L & R atria
- sinus venosus
-- defect located @ entry of SVC into R atrium
- 6-8% of congenital heart lesions
- common in pts. w/ certain congenital disorders (eg. DS, FAS)
Natural history
- 80-100% sponta­neous closure rate if ASD diameter <8mm
- if remains patent, CHF & pulm. HTN can develop in adult life
Clinical features
- hist.: often asymp. in childhood
- phy. exam: grade 2-3/6 pulm. outflow murmur, widely split, & fixed S2
- children w/ large ASDs may hv. signs of heart failure
      --> tachypnea, FTT, hepato­megaly, pulmo. rales/­ret­rac­tions
- ECG: RAD, mild RVH, RBBB (normal ECG doesn't rule out)
- CXR: pulmo. vascul­ature, cardiac enlarg­ement (normal ECG doesn't rule out)
- echo: diagnostic
- elective surgical / catheter closure b/w 2-5 yr, though majority req. no surgery
- size <8 mm will likely sponta­neously close

Ventri­cular Septal Defect

most common congenital heart defect
SMALL VSD (majority)
Clinical Features
- hist.: asymp., normal growth, & develo­pment
- phy. exam: early systolic to holosy­stolic murmur, best heard at LLSB, thrill
- echo to confirm diagnosis
      (ECG & CXR are normal)
- most sponta­neously close
- CHF by 2 mo
- late secondary pulm. HTN if left untreated
Clinical features
- hist.: delayed growth, exercise tolerance, recurrent URTIs or "­ast­hma­" episodes
- phy. exam: holosy­stolic murmur at LLSB,
                    mid-di­astolic rumble @ apex,
                    size of VSD inversely related to intensity of murmur,
                    loss of splitting of 2nd heart sound & loud P2 suggests pulm. HTN
- ECG: LVH, LAH, RVH (normal ECG doesn't rule it out)
- CXR: pulm. vascul­ature, cardio­megaly, CHF (normal CXR doesn't rule out)
echo: diagnostic
- tx. of CHF & surgical closure by 1 yr, if surgery req.
* Size of VSD inversely related to sound of murmur
    --> loud murmur = smaller hole

Patent Ductus Arteriosus

Patent vessel b/.w descending aorta & L pulm. artery
- normally, func. closure w/i first 15 hr of life
- anatomical closure w/i first days of life
- 5-10 % of all CHD
- delayed closure of ductus common in premature infants (1/3 of infants <1750 g)
      --> this is diff. frm PDA in term infants
Natural history
- sponta­neous closure common in premature infants
- less common in term infants
Clinical features
- hist.: aymp. , or have apneic / bradyc­ardic spells, poor feeding, accessory muscle use, CHF
- phy. exam: tachyc­ardia / gallop rhythm,
              bounding pulses,
              hypera­ctive precor­dium,
              wide pulse pressure,
              cont. "­mac­hin­ery­" murmur best heard @ L infrac­lav­icular area
- ECG: may show L atrial enlarg­ement, LVH, RVH
- echo = diagnostic
- CXR: may show normal to mildly enlarged heart, pulm. vascul­ature, prominent pulm. artery
- indome­thacin (Indocid): antago­nizes prosta­glandin E2, which maintains ductus arteriosus patency
        --> only effective in premature infants
- catheter or surgical closure if PDA causes resp. compro­mise, FTT, or persists beyond 3rd mo of life

Coarct­ation of aorta

Narrowing of aorta
- almost always at lvl. of ductus arteriosus
- commonly ass. w/ bicuspid aortic valve (50%) ; Turner synd. (35%)
Clinical features
- hist.: often asymp.
- phy. exam:
      -- BP discre­pancy b/w upper & lower extrem­ities ( sus. / severity if > 20 mmHg diff.)
      -- diminished / delayed femoral pulses relative to brachial pulses (i.e. brachi­al-­femoral delay)
      -- possible systolic murmur w/ late peak @ apex, L axilla, & L back
      -- if severe, presents w/ shock in neonatal period when ductus arteriosus closes
- ECG shows RVH early in infancy, LVH later in childhood
- echo / MRI for diagnosis
- can be compli­cated by HTN
- if ass. w/ other lesions (e.g. PDA, VSD) can lead to CHF
- give prosta­gla­ndins to keep ductus arteriosus patent for stabil­ization & perform surgical correction in neonates
- for older infants & children balloon arteri­oplasty may be an altern­ative to surgical correction

Aortic Stenosis

4 types`
- valvular (75%)
- subval­vular (20%)
- suprav­alv­ular, & idiopathic hypert­rophic subaortic stenosis (5%)
Clinical features
- hist.: often asymp. , b/ may be ass. w/ CHF, exertional chest pain, syncope, or sudden death
- phy. exam: SEM @ RUSB w/ aortic ejection click @ apex (only for valvular stenosis)
- echo for diagnosis
- valvular stenosis usually treated w/ balloon valvul­opl­asty, pts. w/ subval­vular or suprav­alvular stenosis require surgical repair, exercise restri­ction req.

Pulmonary Stenosis

3 types
- valvular (90%)
- subval­vular
- or suprav­alvular
Definition of critical Pulm. S.
Inadequate pulm. blood flow,
Dependent on ductus arteriosus for oxygen­ation,
Progre­ssive hypoxia & cyanosis
Natural history
- may be part of other congenital heart lesions (e.g. Tetralogy of Fallot)
- or in ass. w/ syndromes (e.g. congenital rubella, Noonan synd.)
Clinical features
- hist.: spectrum frm asymp. to CHF
- phy. exam: wide split S2 on expira­tion,
            SEM @ LUSB,
            pulmonary ejection click (for valvular lesions)
- ECG findings: RVH
- CXR: post-s­tenotic dilation of main pulm. artery (due to velocity jest past stenotic valve)
- echo: diagnostic
- surgical repair if critically ill or if symp. in older infant­s/c­hildren

Tetralogy of Fallot

- 10% of all CHD
- most common cyanotic heart defect diagnosed beyond infancy w/ peak incidence @ 2-4 mo
- embryo­logical defect due to ant. & superior deviation of outlet septum leading to: VSD, RVOTO (i.e. pulm. stenosis / subpulm. valve stenosis), overriding aorta, & RVH
      --> infants may initially hv. a L R shunt (∴ no cyanosis)
      --> however, RVOTO = progre­ssive, leading to R L shunting w/ hypoxemia & cyanosis
      --> degree of RVOTO determines direction & degree of shunt, & ∴, extent of clinical cyanosis & degree of RVH
Clinical features
- hist.: hypoxic "­tet­" spells
      --> during exertional states (crying, exercise), increasing pulm. vascular resistance & decrease in sys. resistance causes in R-to-L shunting
      --> clinical features incl.
                  paroxysms of rapid & deep breathing,                         irrita­bility & crying,
                intensity of murmur { flow across RVOTO),
                  pt. squatting for relief ( peripheral resist­ance, R to L shunting)
      --> if severe, can lead to lvl. of consci­ous­ness, seizures, death

phy. exam:
      --> single loud S2 due to severe pulm. stenosis (i.e. RVOTO)
      --> SEM @ LLSB
- CXR: boot-s­haped heart, pulm. vascul­ature, R aortic arch (in 25%)
- echo: diagnostic
Management of spells
O2, knee-chest position, fluid bolus, morphine sulfate, propra­nolol, phenyl­ephrine
- surgical repair @ 4-6 mo of age
- earlier if marked cyanosis or "­tet­" spells

Transp­osition of the Great Arteries

- 3-5 % of all congenital cardiac lesions
- most common cyanotic CHD in neonates
parallel pulm. & syst. circul­ations
- Systemic: body RA RV aorta body
- Pulmonary: lungs LA LV pulm. artery lungs
- survival dependent on mixing through PDA, ASD, or VSD
Physical exam
- neonates: ductus arteriosus closure causes rapidly progre­ssive severe hypoxemia unresp­onsive to O2 therapy, acidosis, & death
- VSD present: cyanosis not prominent ; CHF w/i first wks of life
- VSD absent: no murmur
- ECG: RAD, RVH, or may be normal
- CXR: egg-shaped heart w/ narrow medias­tinum ("egg on a string­")
- echo: diagnostic
- sympto­matic neonates: prosta­glandin E1 infusion to keep ductus open until balloon atrial septostomy
- surgical repair: arterial switch performed in first 2 wk in those w/o VSD while LV muscle still strong

Total Anomalous Pulmonary Venous Return

1-2% of CHD
- all pulm. veins drain into R-sided circul­ation (syst. veins, RA)
- no direct oxygenated pulm. venous return to L atrium
- often ass. w/ obstru­ction @ connection sites
- ASD must be present for oxygenated blood to shunt into LA & syst. circul­ation
surgical repair in all cases & req. urgently for severe cyanosis

Truncus Arteriosus

- single great vessel gives rise to aorta, pulm. & coronary arteries
- truncal valve overlies a large VSD
- potential for coronary ischemia w/ fall in pulm. vascular resistance
surgical repair w/i first 6 wk of life

Hypopp­lastic Left Heart Syndrome

- 1-3% of CHD
- most common cause of death frm CHD in first mo of life
- LV hypoplasia may incl.
      --> atretic / stenotic mitral and/or aortic valve
      --> small ascending aorta
      --> coarct­ation of aorta w/ resultant syst. hypope­rfusion
Systemic circul­ation dependent on ductus patency
Upon closure of ductus, infant presents w/ circul­atory shock & metabolic acidosis
- intubate & correct metabolic acidosis
- IV infusion of prosta­glandin E1 to keep ductus open
- surgical palliation (overall survival 50% to late childhood) or heart transplant


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