Congenital Heart Disease - both Acyanotic & Cyanotic
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Acyanotic heart disease
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Cyanotic heart disease
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L to R shunt
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R to L shunt
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obstruction occurring
beyond lungs |
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blood passes through pulmonic circulation
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blood bypasses the lungs
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oxygenation takes place
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no oxygenation occurs
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levels of deoxygenated blood in systemic circulation
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levels of deoxygenated blood enters systemic circulation
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no cyanosis
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cyanosis
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ACYANOTIC
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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 |
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Obstructive lesions
- present w/ urine output, pallor, cool extremities & poor pulses, shock, or sudden collapse
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CYANOTIC
- 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 |
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3 types:`
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- ostium primum
-- common in DS
-- defect located @ mitral / tricuspid valve |
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- ostium secundum
-- most common type
-- 50-70% -- defect located @ septum b/w L & R atria |
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- sinus venosus
-- defect located @ entry of SVC into R atrium
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Epidemiology
- 6-8% of congenital heart lesions
- common in pts. w/ certain congenital disorders (eg. DS, FAS) |
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Natural history
- 80-100% spontaneous closure rate if ASD diameter <8mm
- if remains patent, CHF & pulm. HTN can develop in adult life |
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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 |
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Investigations
- ECG: RAD, mild RVH, RBBB (normal ECG doesn't rule out)
- CXR: pulmo. vasculature, cardiac enlargement (normal ECG doesn't rule out) - echo: diagnostic |
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Management
- elective surgical / catheter closure b/w 2-5 yr, though majority req. no surgery
- size <8 mm will likely spontaneously close |
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most common congenital heart defect
(30-50%)
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SMALL VSD (majority)
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Clinical Features
- hist.: asymp., normal growth, & development
- phy. exam: early systolic to holosystolic murmur, best heard at LLSB, thrill |
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Investigations
- echo to confirm diagnosis
(ECG & CXR are normal) |
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Management
- most spontaneously close
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MODERATE-TO-LARGE VSD
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Epidemiology
- CHF by 2 mo
- late secondary pulm. HTN if left untreated |
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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 |
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Investigations
- ECG: LVH, LAH, RVH (normal ECG doesn't rule it out)
- CXR: pulm. vasculature, cardiomegaly, CHF (normal CXR doesn't rule out) echo: diagnostic |
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Management
- tx. of CHF & surgical closure by 1 yr, if surgery req.
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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 |
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Epidemiology
- 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 |
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Natural history
- spontaneous closure common in premature infants
- less common in term infants |
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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 |
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Investigations
- ECG: may show L atrial enlargement, LVH, RVH
- echo = diagnostic - CXR: may show normal to mildly enlarged heart, pulm. vasculature, prominent pulm. artery |
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Management
- 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 |
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Narrowing of aorta
- almost always at lvl. of ductus arteriosus
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Epidemiology
- commonly ass. w/ bicuspid aortic valve (50%) ; Turner synd. (35%)
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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 |
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Investigations
- ECG shows RVH early in infancy, LVH later in childhood
- echo / MRI for diagnosis |
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Prognosis
- can be complicated by HTN
- if ass. w/ other lesions (e.g. PDA, VSD) can lead to CHF |
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Management
- 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 |
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4 types`
- valvular (75%)
- subvalvular (20%) - supravalvular, & idiopathic hypertrophic subaortic stenosis (5%) |
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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) |
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Investigations
- echo for diagnosis
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Management
- valvular stenosis usually treated w/ balloon valvuloplasty, pts. w/ subvalvular or supravalvular stenosis require surgical repair, exercise restriction req.
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3 types
- valvular (90%)
- subvalvular - or supravalvular |
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Definition of critical Pulm. S.
Inadequate pulm. blood flow,
Dependent on ductus arteriosus for oxygenation, Progressive hypoxia & cyanosis |
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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.) |
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Clinical features
- hist.: spectrum frm asymp. to CHF
- phy. exam: wide split S2 on expiration, SEM @ LUSB, pulmonary ejection click (for valvular lesions) |
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Investigations
- ECG findings: RVH
- CXR: post-stenotic dilation of main pulm. artery (due to velocity jest past stenotic valve) - echo: diagnostic |
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Management
- surgical repair if critically ill or if symp. in older infants/children
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Epidemiology
- 10% of all CHD
- most common cyanotic heart defect diagnosed beyond infancy w/ peak incidence @ 2-4 mo |
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Pathophysiology
- 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 |
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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, cyanosis, 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 |
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Investigations
- ECG: RAD, RVH
- CXR: boot-shaped heart, pulm. vasculature, R aortic arch (in 25%) - echo: diagnostic |
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Management of spells
O2, knee-chest position, fluid bolus, morphine sulfate, propranolol, phenylephrine
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Treatment
- surgical repair @ 4-6 mo of age
- earlier if marked cyanosis or "tet" spells |
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Epidemiology
- 3-5 % of all congenital cardiac lesions
- most common cyanotic CHD in neonates |
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Pathophysiology
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 |
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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 |
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Investigations
- ECG: RAD, RVH, or may be normal
- CXR: egg-shaped heart w/ narrow mediastinum ("egg on a string") - echo: diagnostic |
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Management
- 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 |
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Epidemiology
1-2% of CHD
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Pathophysiology
- 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 |
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Management
surgical repair in all cases & req. urgently for severe cyanosis
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Pathophysiology
- 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 |
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Management
surgical repair w/i first 6 wk of life
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Epidemiology
- 1-3% of CHD
- most common cause of death frm CHD in first mo of life |
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Pathophysiology
- LV hypoplasia may incl.
--> atretic / stenotic mitral and/or aortic valve --> small ascending aorta --> coarctation of aorta w/ resultant syst. hypoperfusion |
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Systemic circulation dependent on ductus patency
Upon closure of ductus, infant presents w/ circulatory shock & metabolic acidosis
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Management
- 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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