Pediatrics- lecture 8 CV diseases

murmur within first 6 hours of life

valve problem

murmur after 6 hours of life

shunt lesion

murmur >12 months old

likely innocent

murmur and age

less likely to be significant the older the child/adolescent is

when are murmurs concerning?

1. any age- respiratory difficulties, diaphoresis (exertion), poor growth

2. infants- poor feeding or excessively irritable

3. older children/teens- chest pain and syncope

physical exam for murmurs

1. careful review of vitals

2. assess HR and rhythm

3. assess pulses

4. detailed cardiac exam

what is normal, common in children, and varies with respirations?

split S2 heart sound

workup for murmurs

1. in peds office- EKG and CXR

2. cardiology- echo, cardiac cath, cardiac MRI

key features of innocent murmurs

1. sensitive- changes with position or respiration

2. short duration- not holosystolic

3. single- no clicks or gallops

4. small- nonradiating

5. soft

6. sweet- not harsh

7. systolic- systole

grade 1 murmur

very soft, heard in quiet room with cooperative patient

grade 2 murmur

easily heard but not loud

grade 3 murmur

loud but no thrill

grade 4 murmur

loud with palpable thrill

grade 5 murmur

loud with thrill, audible with stethoscope not fully on chest

grade 6 murmur

loud with thrill, audible with stethoscope off the chest

umbilical cord clamping

baby no longer receives O2 and nutrients from mom

what happens to the lungs during first breaths of life?

lungs begin to expand; alveoli in lungs are cleared of fluid

what physiologic changes after birth lead to the closure of the ductus arteriosus?

1. increase in baby's blood pressure

2. significant reduction in pulmonary pressures

what happens to the pressure in the heart when baby's BP increases and pulmonary pressure decreases?

1. increased pressure in left atrium of heart

2. decreases pressure in right atrium

3. stimulates foramen ovale to close

innocent murmurs are aka

Still's, benign, functional, vibratory and flow murmurs

when are innocent murmurs common?

1. first 6 months

2. 3 to 6 y/o

3. early adolescence

history and PE of innocent murmurs?

1. no symptoms

2. PE otherwise unremarkable

innocent murmur characteristics

1. musical, lack of radiation, louder supine

2. normal growth and development

3. ≤2 intensity

4. short systolic

when is a seemingly innocent murmur concerning?

1. dysmorphism or syndromic features

2. FTT

3. cyanosis

4. diastolic, holosystolic, late systolic, continuous

5. thrill

6. ≥3 intensity

7. louder when upright

is venous hum pathologic or innocent sign ?

innocent

signs of heart disease in infants

1. poor feeding

2. FTT

3. diaphoresis with feeding

cyanotic congenital heart disease

right to left shunt

cyanotic congenital heart disease causes

1. transposition of great arteries

2. tetralogy of fallot

3. truncus arteriosus

4. total anomalous pulmonary venous connection

5. tricuspid valve abnormalities

acyanotic congenital heart disease

left to right shunt

causes of acyanotic congenital heart disease

1. VSD

2. ASD

3. PDA

acyanotic, obstructive, stenotic congenital heart disease

1. coarctation of aorta

2. aortic stenosis

3. pulmonic stenosis

what is the most common cyanotic congenital heart defect?

tetralogy of Fallot

4 anatomic defects of tetralogy of Fallot

1. pulmonary stenosis- R ventricular outflow tract obstruction

2. ventricular septal defect

3. overriding aorta

4. RVH

what is tetralogy of Fallot associated with?

1. Down syndrome

2. can be sporadic without any other abnormality

clinical presentation of tetralogy of Fallot

1. may initially be acyanotic- central cyanosis

2. progression of pulm. stenosis and cyanosis

pulmonary stenosis murmur

1. systolic, crescendo-decrescendo murmur with a harsh ejection quality

2. best appreciated along left mid-to-upper sternal border with radiation posteriorly

Tet spell

transient near occlusion of the RVOT with profound cyanosis

how will a child present in a Tet spell?

1. hypoxia, cyanosis

2. irritability

3. squat- increases venous return

diagnosis of tetralogy of Fallot

1. often prenatal

2. echo

workup for tetralogy of Fallot

1. EKG- RAD and RVH

2. CXR- boot-shaped heart

3. lungs may appear dark if not enough blood going to lungs

4. echo- 4 classic features

treatment for tetralogy of Fallot if severe RVOT?

have to keep ductus arteriosus open- give prostaglandin to keep open

treatment of Tet spells

O2 and knee-chest position, calming

complete surgical repair for tetralogy of Fallot?

1. indicated if Tet spells

2. can occur in infancy

if the patient has surgical repair for tetralogy of Fallot, what prophylaxis is needed?

1. bacterial endocarditis prophylaxis indicated until 6 months after complete repair

2. if residual VSD- prophylaxis as long as residual VSD

what specialist referral is needed for tetralogy of Fallot?

pediatric cardiologist with expertise in congenital heart disease

complications of tetralogy of Fallot

1. arrhythmias

2. heart failure

3. can lead to sudden cardiac death

what is the most common cyanotic lesion to present in newborn period?

1. transposition of great arteries

2. NOT associated with any genetic/family inheritance

what is dextroposed transposition?

1. aorta arises from right ventricle

2. pulmonary artery arises from left ventricle

clinical presentation of transposition of great arteries

1. cyanosis

2. quiet tachypnea

3. murmur if VSD

4. fatal unless mixing- mod./large ASD, large VSD, PDA, or combo of these

workup for transposition of great arteries

1. EKG- RAD, RVH

2. CXR- increased pulm. vascularity, egg on string cardiac shadow

3. echo-transposition of great arteries, sites of mixing and any associated lesions

diagnosis of transposition of great arteries

1. prenatal or postnatal

2. echo

3. cath

treatment of transposition of great arteries

1. initial- prostaglandin to maintain ductal patency

2. balloon atrial septostomy- improves mixing

3. complete surgical repair- arterial switch operation in first 2 weeks of life

4. f/u with cardiologist

complications of transposition of great arteries

1. developmental delays

2. pulm. stenosis

3. arrhythmia

4. HF

tricuspid atresia

1. R-> L shunt

2. absence of tricuspid valve-> hypoplastic right ventricle

3. PDA or VSD needed for pulm. blood flow and survival

clinical presentation of tricuspid atresia

1. severe cyanosis

2. single S2

3. if VSD-> murmur

workup for tricuspid atresia

1. EKG- LVH and superior QRS axis

2. CXR- normal or mild cardiomegaly

3. echo- anatomy, associated lesions, source of pulm. blood flow

diagnosis of tricuspid atresia

prenatal or postnatal echo

treatment for tricuspid atresia

1. initial-prostaglandin to maintain ductal patency if no VSD or small VSD

2. surgery-staged

3. f/u with cardiologist

surgery stages for tricuspid atresia

1. initial subclavian artery to pulmonary shunt

2. followed by 2 stage procedure

complications of tricuspid atresia

1. arrhythmia

2. thrombosis

3. ventricular dysfunction

truncus arteriosus

1. all blood pumped through a single truncal valve into a truncal artery

2. artery then splits into aorta and pulmonary artery

clinical presentation of truncus arteriosus

1. present in first few weeks after birth

2. varying cyanosis

3. signs of HF- tachypnea, difficulty feeding and poor growth

4. single S2

5. systolic murmur

workup of truncus arteriosus

1. EKG- nonspecific cardiac enlargement

2. CXR- increased pulm. blood, maybe displaced pulm. arteries

3. echo- anatomy, VSD, truncal valve function, origin of pulm. arteries

diagnosis of truncus arteriosus

echo-usually postnatal

treatment of truncus arteriosus

1. stabilize and diuretics

2. surgical correction- neonatal period

3. f/u with cardiologist

most common congenital heart defect?

VSD

most common type of VSD?

perimembranous

what is VSD associated with?

1. T21, 13, 18

2. fetal alcohol spectrum disorders

3. ASD

4. infants of diabetic mothers

clinical presentation of VSD

1. depends on size and pulm vascular resistance

2. typical murmur- harsh, blowing

3. pansystolic

4. heard best at LLSB

5. +/- thrill

large VSDs

1. not symptomatic at birth

2. over first 3-4 weeks of life, pulmonary vascular resistance decreases-> symptoms develop

3. symptoms- HF, feeding difficulty, poor weight gain

small VSDs

asymptomatic murmur

workup for VSD

1. EKG- may be normal if small VSD, left atrial and ventricular enlargement and hypertrophy

2. CXR- may be normal if small VSD, cardiomegaly, left ventricle enlargement, increased pulm. flow

3. echo- anatomy of VSD

diagnosis of VSD

echo

treatment for VSD

1. 1/3 close spontaneously if small VSD

2. small and asx- no treatment

3. large- diuretics initially

4. surgical closure depending on size

5. f/u with cardiologist

complications of VSD

1. HF if large

2. endocarditis

3. aortic regurg

4. subaortic stenosis

5. right ventricular obstruction

6. left ventricular to right atrial shunting

most common type of ASD?

secundum defect- hole in fossa ovalis

patent foramen ovale

1. other type of ASD

2. tiny hole between left and right atria

what is ASD associated with?

1. T21, 13, 18

2. fetal alcohol spectrum disorders

clinical presentation of ASD

1. rarely symptomatic in childhood

2. asx if small

3. if large- symptoms of HF, recurrent resp. infections, or FTT

4. murmur

ASD murmur

1. a soft grade (1 or 2) systolic ejection murmur heard best at 2nd ICS

2. wide, fixed split S2

ASD workup

1. EKG- RAD, RBBB, RVH

2.- CXR- cardiomegaly, RAD, prominent pulm. artery

3. echo

diagnosis of ASD

echo

treatment for ASD if asymptomatic and no significant left-to-right shunting

monitor longitudinally

treatment for ASD if significant shunt present at age 3

transcatheter device closure or surgical closure

complications of ASD

1. stroke

2. HF

3. arrhythmia

4. pulm. HTN

PDA

failure of closure of ductus arteriosus, which allows blood to flow from pulmonary artery to aorta during fetal life

what is PDA associated with?

T21, 13, 18

clinical pres of PDA

1. machinery like continuous flow murmur

2. +/- thrill

3. heard best at left 2nd ICS

small PDA

asymptomatic

large PDA

exertional dyspnea, HF

workup for PDA

1. if small, EKG and CXR may be normal

2. EKG- biventric. hypertrophy

3. CXR- heart slightly enlarged, vascular markings increased

4. echo

if small PDA and murmur, what is the management?

closure, even if asymptomatic

if moderate-large PDA and murmur, what is the management?

diuretics and eventual closure

closure for PDA

surgical- coil embolization or PDA closure device

treatment for PDA in preemie if stable

watchful waiting, repeat echo at 36 weeks postmenstrual age

treatment for PDA in preemie if unstable

closure- medication 1st and then surgical if unsuccessful

PDA complications if not corrected

1. HF

2. infective endocarditis

3. pulm. HTN

coarctation of aorta

narrowing of aortic lumen due to developmental error of aortic arch

what is coarctation of aorta associated with?

Turner syndrome

what is coarctation of aorta usually accompanied with?

1. another cardiac lesion

2. bicuspid aortic valve

3. VSD or PDA