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Toronto Notes 2019 Cardiology Pediatrics P17
Investigations
• Echo, ECG, CXR
• preandpostductaloxygensaturations,4limbBPs,hyperoxiatest
CYANOTIC VS . ACYANOTIC CONGENITAL HEART DISEASE
• cyanosis:bluemucousmembranes,nailbeds,andskinsecondarytoanabsoluteconcentrationof deoxygenated hemoglobin of at least 30 g/dL
• acyanoticheartdisease(i.e.LtoRshunt,obstructionoccurringbeyondlungs):bloodpassesthrough pulmonic circulation → oxygenation takes place → low levels of deoxygenated blood in systemic circulation → no cyanosis
• cyanoticheartdisease(i.e.RtoLshunt):bloodbypassesthelungs→nooxygenationoccurs→high levels of deoxygenated hemoglobin enters the systemic circulation → cyanosis
Acyanotic
Congenital Heart Disease
Cyanotic (5 “T” lesions)
LR shunt ASD
VSD
PDA Atrioventricular septal defect (endocardial cushion defect)
Obstructive
Coarctation of the aorta Aortic stenosis Pulmonic stenosis
RL shunt Tetralogy of Fallot
Ebstein’s anomaly
Other Transposition of the great arteries
Truncus arteriosus Total anomalous pulmonary venous drainage Tricuspid atresia Hypoplastic left heart syndrome
Characteristic CXR Findings in CHD
• Boot-shaped heart: tetralogy of Fallot, tricuspid atresia
• Egg-shaped heart: transposition of great arteries
• “Snowman” heart: total anomalous pulmonary venous return
Figure 2. Common congenital heart diseases
Acyanotic Congenital Heart Disease
1 . LEFT-TO-RIGHT SHUNT LESIONS
• extrabloodisdisplacedthroughacommunicationfromthelefttotherightsideoftheheart→ increased pulmonary blood flow → increased pulmonary pressures
• shuntvolumeisdependentuponthreefactors:(1)sizeofdefect,(2)pressuregradientbetween chambers or vessels, and (3) peripheral outflow resistance
• untreatedshuntscanresultinpulmonaryvasculardisease,leftventriculardilatationanddysfunction, right ventricular HTN and RVH, and ultimately R to L shunts
Atrial Septal Defect
• 3types:ostiumprimum(commoninDS),ostiumsecundum(mostcommontype,50-70%),sinus venosus (defect located at entry of superior vena cava into right atrium)
• epidemiology: 6-8% of congenital heart lesions, common in patients with certain congenital disorders (e.g. Down’s syndrome, fetal alcohol syndrome)
• naturalhistory
■ 80-100% spontaneous closure rate if ASD diameter <8 mm
■ if remains patent, CHF and pulmonary HTN can develop in adult life
• clinicalpresentation
■ history: often asymptomatic in childhood
■ physical exam: grade 2-3/6 pulmonic outflow murmur, widely split, and fixed S2
■ children with large ASDs may have signs of heart failure (tachypnea, FTT, hepatomegaly, pulmonary
rales/retractions)
• investigations
■ ECG: RAD, mild RVH, RBBB
■ CXR: increased pulmonary vasculature, cardiac enlargement ■ Echo: test of choice
• management:electivesurgicalorcatheterclosurebetween2-5yrofage
Ventricular Septal Defect
• mostcommoncongenitalheartdefect(30-50%) • smallVSD(majority)
■ clinical presentation
◆ history: asymptomatic, normal growth, and development
◆ physical exam: early systolic to holosystolic murmur, best heard at LLSB, thrill
■ investigations:Echotoconfirmdiagnosis(ECGandCXRarenormal)
■ management: most close spontaneously • moderate-to-largeVSD
Moderate-to-Large VSD
Size of VSD is inversely related to intensity of murmur
■ epidemiology: CHF by 2 mo; late secondary pulmonary HTN if left untreated