four critical cardiac problems commonly missed in office practice · 2019-07-15 · diego lara, md,...

Four Critical Cardiac Problems

Commonly Missed in Office Practice

Diego Lara, MD, MPH

Pediatric and Fetal Cardiology

Faculty Disclosure Information

In the past 12 months, I have no relevant financial relationships with the

manufacturer(s) of any commercial product(s) and/or provider(s) of

commercial services discussed in this CME activity.

I do not intend to discuss an unapproved/investigative use of a commercial

product/device in my presentation.

Goals

•Explore a wide range of

cardiovascular symptoms which

present to the pediatrician’s office

•Red flags

•Appropriate referral timing and

expectations

Case #1

“Concerned First Time Parents”

Concerned First Time Parents

• 3 day old

• Term, 6 pound, 9 ounce

• APGARs 8,9

• Passed CCHD Screening

on DOL 1, SaO2 = 95%

•Discharged home in AM

DOL 2 but last night poor

feeding and irritability

Concerned First Time Parents

•HR 180, RR 70

•Pale, lethargic

•Clear lungs

•Gallop rhythm, no

murmur

•Poor pulses all over

3 day old with concerned parents

•Differential Diagnosis?

•Additional Testing?

•Cardiology Referral?– When?

Differential Diagnosis

•Neonatal sepsis

• Inborn error of metabolism

•Neonatal myocarditis

•CHD: Left sided obstructive lesion

•Anomalous left coronary artery

from the pulmonary artery

Additional Tests

•Saturation 75%

•No other tests until transported

Initial ER Work Up

•ABG: 7.10/30/35/14/-9

Initial therapy

PGE to the Rescue


•Neonatal sepsis






Cardiology Consulted – What you want

to see…

Cardiology Consulted – What you do

see…

Hypoplastic Left Heart Syndrome


•Neonatal sepsis







•Spectrum of disease, ranging from

mitral and aortic valve stenosis to

atresia

•30 years ago, palliative care was

only option

•Staged surgical palliations

Stage 1 – Norwood

Norwood WI, J Thorac Cardiovasc Surg, 1981

Bidirectional Glenn

Glenn WW, N Engl J Med, 1958

Fontan Completion

Fontan F, Thorax, 1971


• Current expectation is that ~70% of newborn HLHS will reach adulthood

• Highest mortality rate of common CHD operations (7-19%)

• Highest cost and third longest length of stay among the 35 most common structural birth defects

Feinstein JA, JACC, 2012; Ohye RG, N Engl J Med, 2010

How can we help avoid this

scenario?

•Pulse ox screening for CCHD…

“Critical” Conditions

• Congenital Heart Defects

– ~8/1000 live births

– ~1/4 of these will have critical congenital heart disease

• These conditions are typically:

– Hypoxemic

– Likely to result in poor outcomes if not detected promptly

• Prenatal ultrasound

• Murmur evaluation

• Visible desaturation

Screening options

Estimated Costs?

How well is it implemented?

• Compared use of paper algorithm to computer-based

algorithm

• 20 scenarios

• 81.6% correct when using paper

algorithm

• 98.3% correct when using computer-

tool

Pulseoxtool.org

Case 1 Summary

1. HLHS can appear very normal

while PDA is open

2. CCHD screening helps identify,

need to perform at >24 hours

3. If you think neonatal sepsis, also

think left heart obstructive lesions

4. PGE can be life saving to restore

ductal patency

Case #2

“Johnny had to leave the game”

Urgent Chest Pain Visit

•16 year old

• “Star” wide receiver

•Exertional chest pain for the last 2 months

•Had to leave high school football game last night

•No syncopal episodes

Urgent Chest Pain Visit

• “Normal” vitals, HR 90

•No reproducible chest

pain

•Grade 2/6 crescendo /

decrescendo SEM,

loudest LUSB

•Normal pulses

Chest pain with exercise





•Musculoskeletal pain

•GERD

•Asthma

•Coronary ischemia from early CAD

•Hypertrophic cardiomyopathy

Additional information

•Murmur louder with Valsalva or

standing ( preload, obstruction)

•Careful family history…– Grandfather had “heart issues” starting in his 20’s, eventually

had a defibrillator

– Uncle passed away unexpectedly at 21 years of age


•Musculoskeletal pain

•GERD

•Asthma

•Coronary ischemia from early CAD

•Hypertrophic cardiomyopathy

Elective referral to cardiology…

What you want to see…

LA

LV AO

Septum

LVPW

What you do see…

Septum

LVPW

Hypertrophic Cardiomyopathy

• Prevalence: 1:500

• Most common cause of sudden death in athletes

• Thickened but non-dilated ventricles in the absence of

other obvious causes:

– Valvular heart disease

– Hypertension

– Coarctation

– Hyperthyroidism

– Athletes

Pathology

Myocardial Disarray

Normal myocardial histology

Clinical Presentation

•Asymptomatic

•Murmur

•Exercise intolerance, dyspnea

•Chest pain

•Dizziness / lightheadedness / syncope

•Palpitations

Sudden Death

CHOA HCM Clinic

• 83 patients (29 females, 54 males)

Family History

of HCM or

SCD – 16%

Fatigue During Exercise –

4%

SCD – 2%

Palpitation – 1%Syndrome

Associated with

HCM – 11%

Heart Murmur –

45%

HCM – Physical Exam

Systolic Ejection

Murmur

Louder : Standing or

with Valsalva

Softer: Squatting/ arm

contractions

Clinical Course

• Mortality

– 1% when diagnosed in adults

– 2% when diagnosed in children

• Most children are asymptomatic

• Sudden death: 1st manifestation

• Sudden death is rare before 10y

• Most deaths occur at rest

The most common cause of collapse

during competitive sports

Reggie Lewis

1993Eddie Barnett Jr

Collapsed on court

2005

Risk Factors for Sudden Death

• Family history of sudden death with

HCM

• Left ventricular hypertrophy > 30 mm

• Syncope

• Cardiac arrest or sustained VT

Primary Risk Factors

Risk Factors for Sudden Death

• Near-syncope with exertion

•Multiple or repetitive prolonged bursts of

non-sustained VT on Holter

• Blunted BP response to exercise

• High risk mutation

• Left ventricular outflow tract obstruction

Other Risk Factors

• Echocardiogram

• Genetics evaluation

• Holter

• Stress Test

• Cardiac Magnetic Resonance Imaging

Management

Stress Test

Cardiac MRI

RVLV

Septum

LVPW

Cardiac MRI – Scar Imaging (Delayed

Enhancement = DE)

Association between DE on CMR with VT

Adabag AS, J Am Coll Cardiol, 2008

HCM Management

•Activity restriction

•Beta-blockade (not shown to

decrease mortality in HCM)

•Calcium channel blocker

•Defibrillator

•Myectomy / septal ablation

Case 2 Summary

1. Most HCM patients are asymptomatic… until they are not

2. #1 cause of sudden cardiac death in the young

3. Most common genetically transmitted heart disease

4. Beware systolic ejection murmur which is louder with Valsalva or standing

Case #3

“Bilirubin Check”

Neonatal Bilirubin Check

• 3 day old

• Term, 6 pound, 9 ounce

• APGARs 8, 9

• Passed CCHD Screening on DOL 2

•Discharged home on DOL 2 but bilirubin borderline so told to follow up


•Mild / moderately

tachypneic

•Feeding “OK”

•Diaphoresis with

feeds?


•HR 160

•RV Tap

•Grade 3/6 crescendo / decrescendo murmur under L scapula

• Faint diastolic component

•No clicks or gallops

• “Difficult” femoral pulses

Differential?

•Pulmonary branch stenosis

•Pulmonary valve stenosis

•Aortic stenosis

•Coarctation of the Aorta

•Tetralogy of Fallot

•HLHS


•Saturation 98% on right arm, 88%

on left leg

•BP:

120/65 65/40

70/4070/40


•Saturation 98% on right arm, 88%

on left leg

•CXR:

Differential?

•Pulmonary branch stenosis

•Pulmonary valve stenosis

•Aortic stenosis

•Coarctation of the Aorta

•Tetralogy of Fallot

•HLHS

Same day referral to cardiology or

send to ER

What you want to see…

What you actually see…

Dr. Jarcho, Dublin J. Med, 1834

Coarctation of the Aorta


•5-7% of CHD (0.3-0.4 / 1000 live births)

•2-3 times more common in males

•Frequently associated with other cardiac

anomalies:– Biscuspid aortic valves (40-50%)

– VSD (30-40%)

– Valve or subaortic stenosis (8% each)

– Mitral abnormalities (4%)

Normal fetal circulation

Placenta

Normal fetal circulation

Placenta

Aortic isthmus

Symptoms

Depend on age, severity, and associated defects

• Neonates and Infants

– Asymptomatic vs Poor feeding / FTT vs CHF /

shock / acidosis

• Older children

– Asymptomatic vs Headache vs Epistaxis vs

Claudication vs Hypertension

Newborn

• Pale, tachypneic, shock

• Differential cyanosis

• Weak femoral pulses

• Systolic murmurs, gallop

Older children

• Hypertension

• Murmur

Physical Exam

Hallmark on Physical Examination

Diminished femoral pulses

Arm-leg BP gradient > 15-20 mmHg


Arm-leg gradient may be absent if:

• CHF

• Large PDA

• Collaterals

• Variations in arch branching

Treatment Options

•Surgical intervention (1944)

•Transcatheter angioplasty (1982)

•Transcatheter stent placement

(1991)

Crafoord, J Thorac Surg, 1945; Lock, Circulation, 1983; O’Laughlin, Circulation, 1991

Surgical Options

• Recoarctation-residual/recurrent

• Hypertension-rest & exercise

• Aortic aneurysm, dissection

• Intracranial hemorrhage

• Early atherosclerosis

• Endocarditis, endarteritis

Coarctation Prognosis

Case #3b

“Undulating Hypertension”

12 year old well-check

•No past medical history

•No symptoms

•Active

•Negative family history

12 year old well-check

•Wt 70 kg, Ht 158 cm

•HR 80, RR 14

• BP 175/80 mmHg

• Prominent LV impulse

•Grade 2/6 SEM over L scapula

• “Challenging” femoral pulses

Review of last 4 years BP’s

•2013: 175/80

•2012: 105/60

•2011: 160/85

•2010: 100/65

•2009: 145/75

What’s going on?

Location, Location, Location!

•2013: 175/80 – right arm

•2012: 105/60 – left arm

•2011: 160/85 – right arm

•2010: 100/65 – left arm

•2009: 145/75 – right arm

Elective Cardiology Referral

CXR

http://www.learningradiology.com/caseofweek/caseoftheweekpix/cow128cuarr.jpg

When echo isn’t clear… Cath

When echo isn’t clear… MRI

Flow Imaging

3D/4D Flow

3D Reconstruction

Transcatheter Treatment:

Baseline Angiogram

Balloon Angioplasty

Stent Placement

Case 3 Summary

1. Always make sure you can feel

good femoral pulses

2. Right arm is the only reliable pre-

ductal BP

3. Coarctation in a baby urgent

referral; Coarctation in an older child

elective referral

Case #4

“Runner collapsed at the finish line”

Marathon Runner

Other 6%CAD 2%

HCM36%

Coronary anomalies

19%

Cardiac Mass10%

Ruptured Ao 5%

Tunneled LAD 5%

AS 4%

Myocarditis 3%

Dilated C-M 3%

ARVD 3%MVP 2%

Differential?

Maron B, Circulation, 2009

Next steps

Other 6%CAD 2%

HCM36%

Coronary anomalies

19%

Cardiac Mass10%

Ruptured Ao 5%

Tunneled LAD 5%

AS 4%

Myocarditis 3%

Dilated C-M 3%

ARVD 3%MVP 2%

Differential?

Normal coronary arteries

Normal Coronaries by

Cardiac MRI

AO

PA

Coronaries by Cardiac MRI in Case 4

AO

PA

Our patient’s full history…

RED FLAG!!!

LCA off the Right Aortic Sinus = ALCA

(Anomalous LCA)

• Incidence

0.03%-0.05%

• More often found

at autopsy of

SCD pts with

exercise

• Males >

Females

Normal CA’s

ALCA

Overall risks

•Up to 60-100% of SCD in these patients associated w/exercise

•Sudden death risk appears to start in adolescence

– Most cases in teens to 20’s

When to cut?

• Relative agreement that if patients have

symptoms or evidence of myocardial damage,

then surgery is indicated

• Most centers will do surgery for anomalous left

coronaries, even if asymptomatic

• Controversial regarding asymptomatic patients

with anomalous right coronaries

Coronary Unroofing

• Advantage of relieving/bypassing ostial stenosis

• Potential damage to aortic valve

Exercise Restrictions?

Case 4 Summary

1. Syncope is common in

adolescents and young adults

2. Exertional syncope is uncommon

and merits further investigation

3. Anomalies of the origins of the

coronary arteries are the #2 cause of

SCD in young people in the US

Conclusions

1. Picking out cardiovascular pathology from the

myriad of cardiovascular complaints in the office

requires a detailed H & P and awareness of red

flags

2. If you think neonatal sepsis, think obstructed

left heart lesions

3. Ensure you can feel good femoral pulses

4. Beware exertional chest pain or syncope

References

1. Adabag AS et al. “Occurrence and frequency of arrhythmias in hypertrophic

cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic

resonance”. J Am Coll Cardiol, 2008, 51(14):1369-74.

2. Burke RP et al. “Video-assisted thoracoscopic division of a vascular ring in an infant:

a new operative technique”. J Card Surg, 1993, 8(5):537-40.

3. Chakraborty B et al. “Successful robotic-assisted division of symptomatic vascular

ring”. Heart Surg Forum, 2012, 15(6):e306.

4. Crafoord C et al. “Congenital coarctation of the aorta and its surgical management”. J

Thorac Surg, 1945, 14:347-61.

5. Feinstein JA et al. “Hypoplastic left heart syndrome: current considerations and

expectations”. J Am Coll Cardiol, 2012, 59(1 Suppl): S1-41.

6. Fontan F et al. “Surgical repair of tricuspid atresia”. Thorax, 1971, 26(3):240-8.

7. Glenn WW. “Circulatory bypass of the right side of the heart. IV. Shunt between

superior vena cava and distal right pulmonary artery; report of clinical application”. N

Engl J Med, 1958, 259(3):117-20.

8. Gross RE. “Surgical relief for tracheal obstruction from a vascular ring”. N Engl J Med,

1945, 233:586-90.

References1. Haggerty CM et al. “Comparing pre- and post-operative Fontan hemodynamic

simulations: implications for the reliability of surgical planning”. Ann Biomed Eng,

2012, 40(12):2639-51.

2. Haggstrom AN et al. “Patterns of infantile hemangiomas: new clues to hemangioma

pathogenesis and embryonic facial development”. Pediatrics, 2006, 117(3):698-703.

3. Haggstrom AN et al. “Risk for PHACE syndrome in infants with large facial

hemangiomas”. Pediatrics, 2010, 126(2):e418-26.

4. Kemper AR et al. “Strategies for implementing screening for critical congenital heart

disease”. Pediatrics, 2011, 128(5):e1259-67.

5. Leaute-Labreze C et al. “Propranolol for severe hemangiomas of infancy”. N Engl J

Med, 2008, 358(24):2649-51.

6. Lock JE et al. “Balloon dilation angioplasty of aortic coarctations in infants and

children”. Circulation, 1983, 68:109-116.

7. Mahle WT et al. “Role of pulse oximetry in examining newborns for congenital heart

disease: a scientific statement from the American Heart Association and American

Academy of Pediatrics”. Circulation, 2009, 120(5):447-58.

8. Maron BJ et al. “Sudden deaths in young competitive athletes: analysis of 1866

deaths in the United States, 1980-2006.” Circulation, 2009, 119(8):1085-92.

References

1. Metry D et al. “Consensus statement on Diagnostic Criteria for PHACE

Syndrome”. Pediatrics, 2009, 124(5):1447-56.

2. Munoz-Guijosa C et al. “Anomalous origin of right coronary artery from the left

coronary sinus: sudden death and successful surgical reimplantation”. Eur Heart J,

2012, 33(11):1308.

3. Norwood WI et al. “Experience with operations for hypoplastic left heart

syndrome”. J Thorac Cardiovasc Surg, 1981, 82(4):511-9.

4. Ohye RG et al. “Comparison of shunt types in the Norwood procedure for single-

ventricle lesions. N Engl J Med, 362(21):1980-92.

5. O’Laughlin MP et al. “Use of endovascular stents in congenital heart disease”.

Circulation, 1991, 83:1923-39.

6. Spirito P et al. “Magnitude of left ventricular hypertrophy and risk of sudden death

in hypertrophic cardiomyopathy”. N Eng J Med, 2000, 342(24):1778-85.

7. Slesnick TC et al. “Cardiac magnetic resonance in hypertrophic cardiomyopathy:

Application to pediatric patients”. J Am Coll Cardiol, 2012, 59(13):A268.

8. Soerensen DD et al. “Introduction of a new optimized total cavopulmonary

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four critical cardiac problems commonly missed in office practice · 2019-07-15 · diego lara, md,...

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