Heart murmur Flashcards
Case
Patient is a 6 week old female from Laguna who presents to the
ER with respiratory distress.
She was apparently well until 2 weeks prior when she developed a
febrile illness with cough, rhinorrhea, and emesis. She
subsequently developed progressive respiratory distress. Her parents report that she sweats a lot on her forehead when
feeding. Her parents also noted lethargy and fast breathing
She is born to a 25 year old G3P2 mother, full term with
uncomplicated pregnancy. Delivery was unremarkable except for
meconium stained fluid. She did well at deliver and at nursery.
Physical exam
Mildly cachectic, acyanotic infant, with generalized pallor and
lethargy
HR 160, RR 68, T 36.8, BP 92/68, sats 99%
Anicteric sclerae, pink conjunctivae, no lymphadenopathy, no
dysmorphisms
Scattered crackles with slightly decreases aeration in the left
lower lobe
Precordium mildly active, regular rhythm, Grade 3/6 holosystolic
murmur at the mid left lower sternal border, with radiation to the apex. S3 gallop at apex
Soft abdomen, globular, non-distended, liver edge palpable at 4
cm right subcostal margin,
Symmetrically good pulses, CRT 2 secs, warm extremities
CBC
Hgb 100, Hct 0.33, WBC 15.0, Neu 0.65, Lym 0.22, Plt 350
MCV 75, MCH 20, MCHC 25, RDW 19
Electrolytes
Na 155, K 3.0, Cl 89
ABG
pH 7.44, pCO2 45, pO2 94, HCO3 31, sats 99%
CXray (refer to plates)
12 L ECG refer to plates
2D echo
situs solitus, levocardia
Av-VA concordance seen
IAS intact
IVS- large perimembranous VSD measuring 6mm left to right
shunt with IVG of 160 mmHg
Normally related great arteries
Normal Pulmonary venous drainage
Good biventricular function
Aortic arch left side - d loop
no PDA/PS/Coarctation of aorta
What is your primary working impression?
Ventricular septal defect, in failure
What is VSD?
Most common congenital heart lesion;
Failure of closure of intraventricular foramen
What are the differentials?
- Acute Renal failure
-Rule in: Fluid retention
(pulmonary edema)
- Rule out because of presence of
cardiac findings
2.Nephrotic syndrome
-Rule in: Pulmonary edema
Pneumonia Tachypnea, in mild
respiratory distress, perihilar
infiltrates, coarse crackles
-Cannot be ruled
out
- Liver failure
Rule in: hepatomegaly with possible
ascites
Rule out: Presence of cardiac findings
What are the diagnostics?
*CBC
*Electrolytes
*15L ECG – CXR
*2D echo
important to note location of defect (membranous, muscular, subpulmonic)
note associated anomalies (ASD, valvular defects)
pulmonary pressure (assess for pulmonary hypertension)
chamber enlargement and cardiac function
Management
Management
1. Insert IVF, conscientious water balance
2. No added salt in diet, abstain from foods containing large amounts of sodium
3. Diuretics: Furosemide (chlorothiazide and spironolactone
considered second line drugs)
4. Afterload reducing agents: ACEI
Beta-blockers once stable, prior to discharge
5. Assess for the need of inotropes (digitalis or dobutamine)
6. Referral to cardio for possible patch closure
Complications
Complication
Pulmonary hypertension, infective endocarditis, paradoxical
embolus, failure to thrive
General Peds Care
Gen Peds Care
Complete immunization to prevent infection
IE prophylaxis
Monitor growth (WOF FTT)
No restriction in activity
What are the congenital heart diseases with LEFT TO RIGHT SHUNTS?
ACYANOTIC
—–1. ASD (Secundum)—–
usually asymtomatic; widely split and fixed S2; systolic ejection murmur at LUSB
Dx: RA and RV enlargement on CXR (chest radiograph and ECG)
Management: depends on the type shunt, its symptoms and/or size; transcatheter closure generally preferred for ASD secundum, muscular VSD and PDA
—–2. VSD—–
holosystolic murmur in left parasternal border
Dx: LVH or biventricular enlargement on CXR and ECG
Management: depends on the type shunt, its symptoms and/or size; transcatheter closure generally preferred for ASD secundum, muscular VSD and PDA
—–3. PDA—–
continuous/machinery murmur
Dx: prominent PA, enlarged LA and LV on CXR; LVH or biventricular hypertrophy on ECG
Management: depends on the type shunt, its symptoms and/or size; transcatheter closure generally preferred for ASD secundum, muscular VSD and PDA
What are the congenital heart diseases with RIGHT TO LEFT SHUNTS?
CYANOTIC
—–1. TGA—–
single and loud S2
no murmur if with intact ventricular septum
Dx: EGG-shaped heart on CXR
management: Rashkind procedure (palliative); Jatene procedure (arterial switch); Atrial switch (Senning, Mustard)
—–2. TOF—–
systolic ejection murmur at 2nd LUSB; loud and single S2
Dx: BOOT-SHAPED heart on CXR
Management :Blalock-Taussig procedure (palliative); complete repair of TOF
—–3. Tricuspid Atresia—–
systolic regurgitant murmur at left lower sternal border (LLSB)
Dx: hypoplastic RV; LAD and LVF in ECG
Management: palliative (Rashkind, PDA stent); corrective repair: Glenn shunt followed by Fontan procedure
—–4.TAPVR—–
systolic murmur at LSB
murmurs may be absent
Dx: SNOWMAN SIGN or figure of 8 on CXR
Management:
Van Praagh procedure
What are OBSTRUCTIVE LESIONS?
—–1. COA—–
weak femoral pulses; BP in arms higher than in the legs
Dx: rib notching
Management: Primary re-anastomosis or a patch aortoplasty
—–2.PULMONIC STENOSIS—-
systolic ejection murmur at LUSB with radiation to upper back
Dx: uplifting of apex and normal or decreased pulmonary vascularity on CXR
management: Depends on symptoms and severity of stenosis; balloon valvuloplasty; valvotomy (Brock procedure)
—–3. AORTIC STENOSIS—–
Systolic ejection murmur at RUSB
Dx: prominent ascending aorta with a normal aortic knob on CXR
management: depends on symptoms and severity of stenosis; balloon valvuloplasty; Ross procedure(valve translocation)
What are the ACYANOTIC CONGENITAL HEART DISEASE?
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
What is ASD?
ATRIAL SEPTAL DEFECT (ASD)
Etiopatho
- Can occur in any portion of the atrial septum
(secundum, primum, sinus venosus)
- M<F; 7-10% of all CHDs
- Effects of ASD on the heart:
o In large defects, oxygenated blood flows
from LA to RA, which is added the usual
venous return to the RA and is pumped by
the RV to the lungs
o Enlargement of the R sided chambers of the
heart (RA, RV, PA)
o LA is usually not enlarged
o Pulmonary vascular resistance may begin to
increase in adulthood
What are the types of ASD?
Type of ASD
1. Ostium secundum – most common; defect present at
the site of the fossa ovalis; least severe
- Ostium primum – defect situated in the lower portion
of the atrial septum and overlies the MV and TV
- AV septal defect (AV canal defect or endocardial
cushion defect): consists of contiguous atrial and VSDs
with markedly abnormal AV valves
- Most severe - Sinus venosus – defect situated in the upper part of the
atrial septum close to the entry of the SVC (more
common) or IVC; rarest
What are the clinical manifestations of ASD?
CM
1. Usually asx’c (esp small ASD secundum)
2. SOB, diaphoresis, fatigue, frequent respiratory
infections, tachycardia, tachypnea, RD, poor growth
and devt
3. Subtle FTT for more significant shunt
4. Eisenmenger physiology (reversal of flow) may occur
for uncorrected large ASDs (rarely)
5. Left precordial bulge and RV systolic lift may be
present
6. Widely split and fixed S2: characteristic finding in ASD
7. SEM, grade 2-6 at the L middle and upper sternal
border – due to inc flow across the RVOT into the PA
8. CHF – intermittency of feeding (infants), easy
fatigability (child), tachypnea, diaphoresis, FTT,
recurrent URTI
o If with signs of CHF, think of L-R shunts:
occurs at 6-8wks of life when PVR decreases
Natural course of ASD
Natural course
- Rate of spontaneous closure 87% (>8mm rarely close)
- Untreated large defect leads to CHF and pulmo HTN in
adults
- Eisenmenger syndrome – long standing L to R shunt
causes pulmonary HTN & reversal into a R to L
(cyanotic) shunt
- <3mm: spontaneous closure at 1 ½ yo
- 3-8mm: spontaneous closure 80% at 1 ½ yo
- >8mm: rare
- Infective endocarditis does not occur in ASD (unless
with MR)
- Hemodynamics: RA and RV overload
What are the diagnostics for ASD?
Dx
1. CXR – RVH, RAE and enlarged PA, inc pulmonary
vascularity; obliteration of retrosternal space
2. ECG – volume overload of the RV, N or R axis deviation
of the QRS
3. 2D echo – RV overload:
§ Increased RV end-diastolic
dimension
§ Flattening or reversed (anterior)
motion of the ventricular septum
o Location and size of ASD
- Transesophageal echocardiogram: recommended
imaging modality to delineate more accurate
measurement/size of the defect
4. Cardiac catheterization – confirms the presence of the
defect; for measurement of shunt ratio and pulmonary
P/resistance
5. Cardiac MRI (CMR) – determine the presence,
dimensions & margins & degree of shunting and
effects on the R heart volume and cardiac function
Management of ASD
Mgt
1. Exercise restriction is unnecessary.
- Prophylaxis for infective endocarditis if primum ASD
- Tx for CHF if present – high success rate and spontaneous closure
- Percutaneous catheter device closure – using an atrial septal occlusion device
- Open heart surgery – to patch large defects (if not amenable for percutaneous closure)
- Timing of closure: may proceed after 1 yo if sx’c or before entry to school (optimal age 2-4yo)
- Indications for closure:
a. Sx’c patients
b. Asx’c px with Qp:Qs ratio (pulmonary vs
systemic blood flow) of at least 2:1 or with
RV enlargement
c. Any age with CHF sx - In small secundum ASD and min L to R shunts w/o RV enlargement – closure not required
What is Ventricular Septal Defect (VSD)?
VENTRICULAR SEPTAL DEFECT (VSD)
Etiopatho
- MC CHD (25% of all CHDs)
- May occur in any portion of the VSD, most are membranous type
- Effects on the heart
o Because of the shunt, occurs mainly during systole when the RV also contracts, the shunted blood goes directly to the PA rather
than remaining in the RV cavity
o Enlargement of the LA, LV, and main PA
o Size of the VSD is a major determinant of
the magnitude of the L-R shunt
- Restrictive VSD – small communication present
(<5mm); RV pressure N - Large nonrestrictive VSD - >10mm, R and L ventricular
P are equalized
What are the types of VSD?
Types of VSD
1. Membranous – beneath the AV; MC is perimembranous VSD (70% of all VSDs)
- Outlet (infundibular or conal) – 5-7% of VSDs. Defect located within the outlet (conal) septum; the aortic
and pulmonary annulus forms part of its rim; most severe - Inlet (AV canal) – 5-8% of VSDs; located posterior and
inferior to the perimembranous defect beneath the
septal leaflet of the TV - Trabecular (muscular) – 5-20% of VSDs; multiple defects
What are the clinical manifestations of VSD?
CM
1. Small VSD (MC): asx’c with N growth and devt
2. Mod to large VSD – delayed growth and devt, dec
exercise tolerance, repeated pulmo infections, HF
3. Holosystolic regurgitant murmur at the LLSB
4. Increased P2 with large shunt
5. Loud S2 and single in pulmonary HTN
6. Systolic thrill at L sternal border
What is the natural course of VSD?
Natural course
- Spontaneous closure in 40-50% of membranous and 90% in muscular VSD (esp small defect) within 6 MOL
- Eisenmenger syndrome
- May have conduction defects if perimembranous and
inlet type
- Hemodynamics:
o Small to mod VSD: LV volume overload
o Large VSD: biventricular overload (due to
pulmo HTN)
SMALL vs LARGE VSD
—–Small VSD—–
CXR: usually normal; may have minimal cardiomegaly and borderline increase in pulmonary vasculature
ECG:generally normal but may suggest LVH
2D Echo: shows the position and size of VSD; measures degree of volume overload of the LA and LV; estimated pulmonary artery pressure may be obtained
Cardiac catheterization: normal right heart pressures and pulmonary vascular resistance
Cardiac MRI: visualizes flow, defect position and size, ventricular volume and function and net shunt quantification (arterial flow)
—–LARGE VSD—–
CXR: cardiomegaly with prominence of both ventricles, LA and pulmonary artery; increased pulmonary vascular resistance; frank pulmonary edema may be present
ECG: biventricular hypertrophy; notched or peaked P waves; presence of RVH suggests larger VSD with pulmonary hypertension
2D Echo: shows the position and size of VSD; measures degree of volume overload of the LA and LV; estimated pulmonary artery pressure may be obtained
Cardiac catheterization: equal or nearly equal pulmonary and systemic systolic pressure; elevated pulmonary vascular resistance
Cardiac MRI: visualizes flow, defect position and size, ventricular volume and function and net shunt quantification (arterial flow)
Management of VSD
Mgt
1. Small VSD – parents are reassured of the relatively benign nature of the VSD
- Surgical repair not recommended
- Infective endocarditis prophylaxis (Amoxicillin 50mkd 1h before dental procedure)
- Large VSD – mgt goals: control sx of HF and prevent
development of PV disease
- Severe pulmonary vascular disease nonresponsive to
pulmonary vasodilators is a contraindication to repair
an uncorrected large VSD - CHF – meds for 2-4mos to see if growth failure improves
- No exercise restriction, unless with pulmo HTN
- Surgery – indications for closure:
a. Patients at any age with large defects when sx & FTT cannot be controlled medically
b. Infants 6-12mos with large VSD and pulmo
HTN, even if with sx are controlled by meds
c. >24 mos with Qp:Qs ratio >2:1 - Transcatheter closure – Most successful for muscular
VSDs
- Small sized perimembranous type VSD with no
associated aortic valvar pathology - Update vaccination
What is Patent Ductus Arteriosus?
PATENT DUCTUS ARTERIOSUS (PDA)
Etiopatho
- Persistent patency of N fetal structure b/n L PA and
descending aorta
- Common in PT infants
- M<F; 10% of all CHDs
- MC CHD in congenital rubella syndrome
- Effects on heart:
o Blood shunts L-R through the ductus due to
a higher aortic P
o Enlarged LA, LV, main PA
o Enlarged aorta, w/c also handles an inc
amount of blood flow
What are the clinical manifestations of PDA?
CM
1. Small PDA – usually asx’c, N peripheral pulses, N sized
heart
- Large PDA – HF, growth retardation, bounding
peripheral arterial pulses, wide pulse pressure
(50mmHg), cardiomegaly, prominent apical impulse - Tachycardia and dyspnea – due to volume overload on
LA & LV - Continuous machinery-like murmur at 2nd L ICS or LUSB
(radiating to L neck) - Hyperactive precordium
- Systolic thrill at LUSB
- Accentuated P2 if with pulmo HTN
What is the natural course of PDA?
Natural course
- Spontaneous closure in PT within 1st 3 mos; after infancy is rare
- Unlikely spontaneous closure in FT infants, a structural abN (vs in PT, due to decreased responsiveness of DA to oxygen)
- Small PDA: few complications, but late manifestations may occur
- Large PDA: HF occurs early in infancy
- Risk for infective endocarditis
- Hemodynamics: LV volume overload
What are the diagnostics for PDA?
Dx
1. CXR – prominent PA with inc pulmonary vasculature,
enlarged LA and LV (depending on degree of shunt),
aortic knob N or prominent; cardiomegaly
- ECG – small PDA: N; large PDA: LVH or biventricular hypertrophy
- 2D echo – small PDA: N sized cardiac chambers; large
PDA: enlarged LA and LV, direct visualization of PDA - Cardiac catheterization – N or inc P in the RV and PA
(depending on size of PDA) - Cardiac CT – for PDA with tortuosity
- Cardiac MRI – info regarding volumes and flow
What is the management of PDA?
Mgt
1. In PT infants, to close PDA: Indomethacin 0.2 mg/kg IV
q12h x 3 doses
- CI: elev BUN, Crea, thrombocytopenia, NEC, bleeding
d/o, hyperbili
- Alt: Ibuprofen 10mkdose SD then 2 doses 5mkdose
q24h
- Surgical or transcatheter closure
a. Closure of small PDA to prevent bacterial
endocarditis or other late complications
b. Larger PDA: to treat HF and prevent
pulmonary vascular disease
In adolescent, if previously with murmur not specified and signs of
CHF but now no murmur, no signs of CHF with loud P2 –> severe
PA HTN
In adolescent, previously with a murmur, acyanotic and signs of
CHF but now, no murmur, no signs of CHF, with cyanosis and
clubbing –> Eisenmenger syndrome
What is Eisenmenger syndrome?
EISENMENGER SYNDROME
- Complication of acyanotic CHD
- Pulmonary HTN (PA P >25mmHg at rest, >30mmHg at exercise)
- Reversal of shunt (L-Rshunt à R-L shunt): result of
pulmo vasc disease
- Cyanosis
- Usually develops before pubertyW
Triad of Eisenmenger Syndrome
Triad:
o Systemic to pulmonary CV communication
o Pulmonary arterial disease
o cyanosis
Pathophysiology of Eisenmenger Syndrome
Patho
- Inc pulmonary BF (shear stress, circumferential stretch)
–> remodeling of pulmo vasculature (endothelial
dysfunction and smooth muscle proliferation, inc ECM,
intravascular thrombosis) –> obstruction of pulmo BF
- Failure to reduce pulmonary P in the 1st 2 YOL results
to irreversible changes in the intimal smooth muscle
- L-R shunt –> inc PBF –> arteriolar pulmonary vascular
remodeling –> inc PVR –> R-L shunt –> cyanosis, dec
heart size, dec pulmo vasculature (irreversible)
What are the clinical manifestations of Eisenmenger syndrome?
CM
1. Cyanosis, dyspnea, fatigue, dysrhythmia, AF, chest
pain, HIA, syncope
2. onset of pulmonary hemorrhage – is the hallmark of
rapid progression
3. PA HTN, shunt reversal
4. Dec heart size (due to concentric hypertrophy)
5. RV heave, narrowly split S2 (loud P2), TR murmur,
pulmonary insufficiency, decrescendo diastolic murmur
at LSB
What are the complications of Eisenmenger?
- dyspnea, syncope, chest pain, stroke, CHF,
hyperviscosity syndrome, endocarditis
Management of Eisenmenger?
Mgt
1. not responsive to O2 or pulmonary vasodilators
(sildenafil). Supportive only
2. heart and lung transplant
