Case 18: 2 week old; poor feeding Flashcards
define physiological hepatomegaly in infancy
when is it pathologic?
1-2cm below R costal margin in an infant
can be seen in kids with CHF ==> decreased renal blood flow ==> RAAS ==> fluid retention, systemic venous congestion, hepatomegaly
signs of CHF in infant a few weeks after birth
v. adult
-dyspnea with feedings ==feeding longer than normal; shorted d/t respiratory distress
- rapid and labored respirations
-diaphoresis with feedings
-poor growth d/t poor feeding and increased caloric expenditure
- tachycardia
+/- cyanosis
-an active precordium when placing hand on chest (+/- abnormal location for point of maximal impulse
-hepatomegaly
ADULTS
- rales, JVD, peripheral edema
pathophysiology of CHF in infant
poor cardiac fx, increased myocardial demand, shunt lesion
==> inefficient circulation ==> RAAS
1) fluid retention, systemic venous congestion, hepatomegaly
2) increased metabolic demands ==> poor weight gain, diaphoresis with any type of activity
Innocent murmurs
- epidemiology
- common features
- examples
- epidemiology: 70-80% of otherwise healthy pts (3-7yo)
- common features: nml precordial activity; nml S1/S2 split, <2/4 grade, nml O2 sat
- examples:
(1) Still’s == musical/vibratory during systole, @ LLSB in supine position
Structural heart defect: ASD
- AGE OF PRESENTATION:
- murmur:
- other sxs:
increased flow across normal pulmonic valve
- AGE OF PRESENTATION: 3-5yo
- murmur: systolic, widely split, fixed S2
- other sxs:
Structural heart defect: Coarctation of aorta
- AGE OF PRESENTATION:
- murmur:
- other sxs:
- AGE OF PRESENTATION: infancy, progressively more severe
- murmur:
- other sxs: HTN in upper extremities»_space; lower extremities
Structural heart defect: VSD
- AGE OF PRESENTATION:
- murmur:
- CXR findings:
- ECG findings:
- AGE OF PRESENTATION: infancy (days to weeks of age) –> with large defects causing more problems.
- murmur: holosystolic; blowing
- CXR findings: (L–> R shunt) == cardiomegaly, increased pulmonary vascular markings, pulmonary edema
- ECG findings: prominent biventricular forces (high voltage QRS complexes in leads V1, V2) == LV volume overload (pulmonary HTN), RV pressure overload.
- ECHO - for size of defect; L–>R shunt with LA and LV dilation, from pulmonary flow overload
Structural heart defect: Aortic stenosis
- AGE OF PRESENTATION:
- murmur:
- other sxs:
- AGE OF PRESENTATION: infancy
- murmur: systolic ejection murmur
- other sxs:
Structural heart defect: pulmonic stenosis
- AGE OF PRESENTATION:
- murmur:
- other sxs:
- AGE OF PRESENTATION: infancy
- murmur: harsh, systolic ejection click just after S1
- other sxs:
Structural heart defect: patent ductus arteriosus
- AGE OF PRESENTATION:
- murmur:
- other sxs:
- AGE OF PRESENTATION: infancy
- murmur: continuous, lower in systole
- other sxs:
Structural heart defect: tetralogy of fallot
- AGE OF PRESENTATION:
- murmur:
- other sxs:
- AGE OF PRESENTATION: infancy
- murmur: (from VSD = holosystolic)
- other sxs: cyanosis (NOT CHF)
1) VSD (w/ R–>L shunting)
2) RV outflow tract obstruction == progressive cyanosis
3) overriding aorta
4) RVH
what structural heart defects most commonly presents in infancy
- coarctation of aorta
- VSD
- aortic stenosis
- pulmonic stenosis
- PAD
- Tetralogy of Fallot
Structural heart defect: bicuspid aortic valve
- AGE OF PRESENTATION:
- murmur:
- other sxs:
==> when stenotic / regurgitant
- AGE OF PRESENTATION: later in childhood/adolescence/adulthood
- murmur: early systolic click during initial outflow
- other sxs:
which heart defects cause CHF
- VSD
- severe aortic stenosis
- coarctation of aorta
- large PDA
Ventricular septal defect
- cause:
- pathophysiology:
- clinical sxs:
- prognosis:
- cause: persistent communication b/w ventricles
- pathophysiology:
1) lack of tissue in embryologic endocardial cushion (inlet septum); conotruncus (outlet septum); trabecular septum (muscular septum)
2) lack of fusion of embryologic components at membranous septum = “peri-membranous” defect
==> L–>R shunting of blood ==> increased pulmonary blood flow, pulmonary VR –> LV volume overload, esp. as pulmonary resistance falls in first weeks of life ==> start murmur of VSD - clinical sxs: hyperactive precordium (+/- thrill) @ LLSB
- prognosis: 25-50% of defects spontaneously close (esp. small ones)
if suspecting heart defect, what to consider in physical exam
1) auscultation
2) patient’s color
3) palpate precordium
4) assess pulses ==> esp. brachial v. femoral.
diffdx for hx of respiratory distress + feeding difficulty in an infant
- CHF –> hx respiratory distress, difficulty feeding, poor weight gain (FTT)
- RESPIRATORY INFECTION (bronchiolitis, pneumonia) –> + fever, difficulty feeding
- SEPSIS –> subtle, sometimes no fever
- METABOLIC D/O - tested on newborn screening ==> heaptomegaly, poor growth, feeding difficulty at birth, poor tone.
Evaluation of congenital heart defect
1) ECG ==> chamber enlargement
2) CXR ==> heart size, prominent pulmonary vasculature markings
3) ECHO
Admission criteria for congenital heart disease
- significant congenital heart defects presenting with cyanosis or
congestive heart failure - children in shock
- increased respiratory effort, lethargy/decreased level of alertness, feeding difficulty, cyanosis
treatment of CHF in infant
- goal
- what treatments
goal = control sxs; help child grow == will NOT close VSD
1) FUROSEMIDE = for sxs; decrease fluid retention from RAAS
2) DIGOXIN = esp. for VSD-induced CHF
3) ENALAPRIL / CAPTOPRIL = to reduce afterload –> decreased SVR, increased forward flow of blood from LV rather than thru VSD to pulmonary vasculature
4) optimize feeding = fortifying expressed breast milk / formula == greater caloric density
define: failure to thrive
- infant <5%ile for weight
- infant <5%ile for length
- rate of growth decreased >2 major lines
organic causes of failure to thrive
10% of cases
- congenital heart defects
- cystic fibrosis
- GERD
- neurological disorders
- metabolic disease
nonorganic causes of failure to thrive
90% of cases
- psychosocial problems
- poverty, neglect
signs of feeding difficulties in infant
- rapid respirations, increased work of breathing (inter/subcostal retractions) with feeding
- weight loss
- decreasing vigorousness in feeding
- diaphoresis with feeding
define cyanosis
bluish coloration of the skin and mucous membranes that occurs due to inadequate oxygenation of the blood
what the cardiac exam can tell you about congenital heart defects
1) color of skin and mucus membranes, nail beds
==> esp. tetralogy of Fallot (otherwise, truncus arteriosus, transposition of great arteries, tiscuspid atresia, total anomalous pulmonary VR)
2) precordial activity
“active precordium” == increased workload
3) heart sounds = S1/S2 split, any gallops
4) murmur
HOLOSYSTOLIC == VSD, MR, TR, at onset of ventricular contraction
EJECTION MURMUR == AS, pulmonic stenosis
5) pulses
coarctation of aorta == descrepancy in sterngth of brachial/femoral pulses
diffdx of hepatomegaly in infancy
- CHF
- congenital infections
- inborn errors of metabolism
- anemias
- tumors
What is the most common cause of a murmur in childhood? Select the ONE best answer.
A Acute rheumatic fever B Infective endocarditis C Cardiomyopathy D Congenital heart disease E Innocent murmur
E
murmur + fever == acute rheumatic fever, endocarditis, cardiomyopathies
A previously undetected murmur is often heard on the examination of a pre-school age child.
While innocent murmurs are the most common murmurs in childhood, and these are often first noted around 3-5 years of age, there are structural cardiac defects that may first be detected at this age, too.
What congenital heart defects are more likely to be detected in school-aged children than in neonates? Select all that apply.
Multiple Choice Answer: A Atrial septal defect B Ventricular septal defect C Aortic stenosis D Pulmonic stenosis E Patent ductus arteriosus F Tetralogy of Fallot G Bicuspid aortic valve
ASD, bicuspid valve
what is the most commonly encountered congenital cardiac defect
isolated VSD = 15-20%
most common cyanotic heart defect
tetralogy of fallot
most common heart defect presenting with cyanosis in newborn period
transposition of great arteries
==> urgent intervention with ASD+ VSD to support mixing of de/oxygenated blod
cyanotic heart defects
= blue babies
- Tetralogy of fallot = most common
- Transposition of great arteries = most common presenting with cyanosis in newborn
- Truncus arteriosus
- Tricuspid atresia
- Total anomalous pulmonary VR
ECG findings in VSDs of different sizes
- LARGE VSD = RVH d/t RV pressure overload from pulmonary HTN; upright T save (V1). no pressure gradient from LV to RV (pressure RV = LV)
- MODERATE VSD = LVH d/t LV volume overload (==> L heart dilation d/t increase pulmonary flow returning to L heart in systole. RVH not prominent b/c LV flow going straight into pulmonary arteries
- SMALL VSD = nml ekg
why is normal voltage for EKG in infants age-dependent
Newborns and young infants: more RV voltage, more right-ward axis
==> elevated pulmonary vascular resistance from fetal life = thickened RV (> LV)
in older kids == normalized pulmonary vascular resistance ==> thinned RV, decreased Right ECG voltages
how does VSD cause CHF
- increased contractility
- elevated filling pressure d/t LV volume overload (high adrenergic state, RAAS) ==> high CO
- abnormaliteis of LV function and neuroabnormal regulation ==> effort intolerance, fluid retention, reduced longevity
why do infants found to have VSDs not have murmurs heard at birth?
NEWBORNS = elevated pulmonary vascular resistance
- when systemic / pulmonary vascular resistance nearly equal, no blood shunted thru VSD
==> appearance of VSD until PVR drops, usually a few days to weeks of age
What medications might be effective at improving Tyler’s symptoms of heart failure?
Check the appropriate medications. (Note: There may be more than one correct medication.)
Multiple Choice Answer: A acetaminophen B amiodarone C aminophylline D furosemide E dopamine F hydralazine G digoxin H enalapril I propranolol J pseudoephedrine
-furosemide 1 mg/kg/dose BID
-digoxin 5ug/kg/dose BID
==> improved respiratory effort, PO intake
-enalapril
-optimized feeding by fortifying expressed breast milk/formula
==> improved weight gain
complications of VSD
- CHF
- pulmonary HTN = respiratory distress, poor feeding
- pulmonary vascular obstructive disease (Eisenmenger’s sydnrome)
at what age do you decide to do surgery in an infant with VSD for closure?
6mo for large VSD that does not close spontaneously
- -> to eliminate risk for development of pulmonary vascular obstructive disease (Eisenmenger’s sydnrome) = now R–>L flow
- esp. if RV and PA pressures are at systemic level b/c can soon switch
pathophysiology of Eisenmenger’s syndrome from VSD
1) exposure to high pressure, high flow –> pulmonary vascular constriction
2) pulmonary HTN= high pulmonary vascular resistance»_space; systemic resistance ==> change to R–>L shunt
3) –> cyanosis, polycythemia, heart failure, death (in 20s)
how to repair large VSD
cardio-pulmonary bypass
1) incision in RA
2) retraction of septal leaflet of tricuspid valve to get to VSD
3) sew prosthetic patch over defect on R side of septum == be careful about conduction system.
Prognosis = good for even small infants
–> (+ meds) will help with weight gain and growth
A 3-week-old infant is brought to the pediatrician for failure to thrive (despite adequate, even prolonged, feedings) and respiratory distress (particularly tachypnea). EKG shows high voltage QRS complexes in leads V1 and V2. What other features does this infant most likely have?
Single Choice Answer:
Please select one answer.
A Cyanosis from a right-to-left shunt
B Systolic murmur with a widely split second heart sound
C Continuous murmur that is louder during systole
D Left-to-right shunt
D
FTT, respiratory distress, tachypnea
high voltage QRS complexes in leads V1 (R ventricle) and V2 (L ventricle)
pulmonary problems + enlarged LV
A heart murmur from a VSD is typically not appreciated in the immediate newborn period, as the pulmonary vascular resistance is still quite elevated. During this time, since the pulmonary vascular resistance equals the systemic vascular resistance, there is no shunting of blood through the open VSD. However, after a few days to weeks after birth, the pulmonary vascular resistance decreases, and the murmur appears, reflecting the shunted flow of blood through the open VSD (from left to right).
ASD usually in older children.
You have accepted a part-time tutoring job for first-year medical students. One of your students asks if you would please clarify the details of normal fetal circulation. Which of the following best describes the path of the majority of the blood that enters the right atrium?
Single Choice Answer:
Please select one answer.
A RA > foramen ovale > LA > LV > systemic circulation
B RA > RV > VSD > LV > systemic circulation
C RA > RV > pulmonary circulation > LA > LV > systemic circulation
D RA > RV > ductus arteriosus > LV > systemic circulatio
E RA > RV > ductus arteriosus > systemic circulation
E. (for most -2/3 - blood) RA > RV > ductus arteriosis > systemic circulation
Approximately 90–92% of the blood that enters the RV (two-thirds of the blood that enters the RA) travels out and through the ductus arteriosus, bypassing the pulmonary circulation and the left heart, ending up in the descending aorta. This blood is perferentially less oxygenated than that which flows through the foramen ovale. Like the foramen ovale, closure of this bypass is a normal transition from intra to extrauterine life.
A. (1/3 of blood goes through here) In fetal circulation, the foramen ovale connects the RA to the LA, allowing a portion of the blood to bypass the RV and the lungs. Approximately a third of the blood that enters the RA passes through this route (preferentially the most oxygenated which is then delivered to the brain and heart), leaving the majority of the blood to travel into the RV. Closure of the foramen ovale is a normal transition from fetal to extrauterine circulation.
B = abnormal
C is normal adult circulation
A 5-year-old boy is noted to have a grade II systolic murmur and a widely split S2 murmur on cardiac exam. His vital signs are stable and he has been asymptomatic. Which of the following statement is accurate regarding this patient’s presentation and likely condition?
Single Choice Answer:
Please select one answer.
A No further work-up for a presumed venous hum
B Chest x-ray, ECG, and echocardiogram would be indicated as next steps to work up a presumed ventricular septal defect
C This patient’s murmur is caused by flow through the pulmonary outflow tract and should be evaluated
D The patient should be scheduled now for cardiac catheterization
C
systolic, widely split S2
if grade 2, no big deal. but widely split S2 == ASD
This patient’s murmur is likely caused by an atrial septal defect, which causes flow of additional blood through the pulmonary outflow tract and should be evaluated.
A 1-month-old African-American male presents to your office for a check-up. The baby was born at term by NSVD to a 29-year-old G1P0 mother with no complications. Mother states the baby was feeding well until a week ago, when he developed increased sleepiness, prolonged feeding, and greater duration between feeds. His mother notes he stops to take breaks sometimes because he seems to be trying to catch his breath. He has 4 to 6 wet diapers per day and poopy diapers 3 or 4 times per day. Vital signs are: T: 37.6 C, RR: 68 bpm, P: 138 bpm, BP: 88/58 mmHg, and 02 saturation is 98%. The physical examination is notable for increased respiratory effort and retractions, and, upon cardiac examination, a murmur with a hyperactive precordium and no cyanosis. Abdominal exam reveals a liver edge palpable to 4 cm below the right costal margin. Which condition would be least likely to be the cause of the patient’s symptoms?
Single Choice Answer: Please select one answer. A Aortic stenosis B Coarctation of the aorta C Ventricular septal defect D Patent ductus arteriosus E Atrial septal defect
E
failure to thrive, respiratory distress
normal VS
hyperactive precordium + hepatomegaly – likely VSD
==> baby is presenting with CHF + murmur secondary to some cardiac defect == poor feeding, rapid breathing, poor urine output, and fussiness
NO cyanosis
atrial septal defects (ASDs) do not cause CHF. An ASD malformation is a left-to-right shunt, and—depending on the size of the defect—the patient may or may not present with symptoms. ASDs often go undiagnosed for decades due to subtle physical examination findings and/or a lack of appreciable symptoms. If the defect is large enough, pediatric patients may present with easy fatigability, recurrent respiratory infections, or exertional dyspnea.
