Update on Outcomes of Neonatal Heart Disease

Update on Outcomes of Neonates
with Congenital Heart Disease
Geoffrey L. Bird, MD, MSIS
Children’s Hospital of Philadelphia

no disclosures of relevant
affiliations or financial interests

• Advances in CT Surgical Techniques / how
small is too small?
• Timing/decision-making key lesions:
– TGA/HLHS/Truncus/TOF
• Early Dx/prenatal Dx/fetal intervention
• Role for transplant?
• Outcomes/ Neurodevelopmental follow-up

How small is too small?
• It depends.
• For VA ECMO, most NICUs ascribe to ~34wks
and 2kg.
• For cardiopulmonary bypass, those limits are
regularly exceeded with acceptable outcomes,
but the risks can be significant as extremes are
approached.

Stage 1 reconstruction as a model
• Three primary physiologic goals:
– Reliable systemic blood flow
– Controlled pulmonary blood flow
– Free pulmonary venous egress
• So, for Norwood Stage 1 surgery:
– Arch reconstruction
– Modified BT shunt
– Atrial septostomy

How small is too small?
• It depends.
• At concerning limits of size or age, best course
of action is a complete assessment for existing
burden of disease/injury coupled with
multidisciplinary determination of best
risk/benefit ratio to achieve optimal
physiology for the short, intermediate, and
long term.

• Rather than being totally focused on the OR,
this evaluation has important components in
prenatal risks, fetal development, other
patient specific factors, and postoperative
care and follow-up.
• Fetal Neuroprotection and Neuroplasticity
Program (cf http://heart.chop.edu)

• It’s okay to say “No,” and we do say “No,”
rarely.
• But, as a referral center for high risk patients,
we often say “Yes,” when others are unwilling.

Benchmark procedures
• ASO
• ASO/VSD
• Norwood
• Truncus
• TOF
• VSD
• CCAVC
• Glenn
• Fontan
• Heart.chop.edu
• Pennsylvania Health
Care Cost Containment
Council Feb 2015 (‘09-
’12)
• Texas Children’s
Hospital (ASD, VSD, TOF,
CCAVC, TGA, HLHS)
• Lots of others…

22
The Hybrid Procedure
Hybrid
Procedure

29
Tetralogy of Fallot, Pulmonary Atresia, MAPCAS

30
TOF – Absent Pulmonary Valve

So, what about transplantation?

Pediatric Heart Transplants
Recipient Age Distribution (Transplants: January 2000 – June 2013)
0
200
400
600
800
1,000
1,200
1,400
1,600
1,800
<1 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
NumberofTransplants
Recipient Age (Years)
2014
JHLT. 2014 Oct; 33(10): 985-995

Recipient Diagnosis (Age: < 1 Year)
41%
55%
3%
0.3%
Myopathy
Congenital
Other
ReTX
2006-6/2013
21%
76%
2%
1%
1988-1999
0
25
50
75
100
%ofCases
Myopathy Congenital
35%
62%
2%
1%
2000-2005
2014 For some retransplants diagnosis other than retransplant is
reported, so the total percentage of retransplants may be greater.
JHLT. 2014 Oct; 33(10): 985-995

Kaplan-Meier Survival (Transplants: January 1982 – June 2012)
0
20
40
60
80
100
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22
Survival(%)
Years
<1 Year (N = 2,702)
1-5 Years (N = 2,419)
6-10 Years (N = 1,552)
11-17 Years (N = 4,219)
Overall (N = 10,892)
Median survival (years): <1=20.6; 1-5=17.3; 6-10=14.6; 11-17=12.9
1-5 vs. 11-17: p = 0.0133
6-10 vs. 11-17: p = 0.0298
No other pair-wise comparisons
were significant at p < 0.05.
2014
JHLT. 2014 Oct; 33(10): 985-995

Kaplan-Meier Survival Conditional on Survival to 1 Year (Transplants: January 1982 –
June 2012)
0
20
40
60
80
100
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22
Survival(%)
Years
<1 Year (N = 1,932) 1-5 Years (N = 1,876)
6-10 Years (N = 1,270) 11-17 Years (N = 3,401)
Overall (N = 8,479)
Median survival (years): <1 = NA; 1-5 = 21.5; 6-10
= 16.7; 11-17 = 16.1
All pair-wise comparisons were significant at p <
0.05 except <1 vs. 1-5.
2014
JHLT. 2014 Oct; 33(10): 985-995

Kaplan-Meier Survival by Era
Age: < 1 Year (Transplants: January 1982 – June 2012)
0
20
40
60
80
100
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19
Survival(%)
Years
1982-1989 (N=188) 1990-1999 (N=1,088)
2000-2004 (N=471) 2005-6/2012 (N=955)
All pair-wise comparisons were significant at
p<0.05 except 1982-1989 vs. 1990-1999 and
2000-2004 vs. 2005-6/2012.
Median survival (years): 1982-1989=10.8; 1990-1999=19.3; 2000-
2004=NA; 2005-6/2012=NA
2014
JHLT. 2014 Oct; 33(10): 985-995

Kaplan-Meier Survival by Diagnosis
Age: < 1 Year (Transplants: January 2000 – June 2012)
0
20
40
60
80
100
0 1 2 3 4 5 6 7 8 9 10 11 12
Survival(%)
Years
Congenital (N=811) Cardiomyopathy (N=551)
p<0.0001
2014
JHLT. 2014 Oct; 33(10): 985-995

“Just” give them a transplant?
• CHOP 2010- YTD 65 heart transplants overall
• CHOP 2010 – YTD 15 Heart transplants in infants
(2-5/yr)
• Indication: ~50:50 split for CHD vs DCM/HCM
• For those infants successfully transplanted at
CHOP, 14:15 survived to DC and all are doing well.
• Per UNOS, infant OHT alive at >1yr post-OHT, very
good survival to >20yrs
• But for all those listed, considerable attrition
prior to transplant.

• 10 yrs (1999-2008)
• AK CH, Children’s Mem CH, St. Louis CH
• 92 infants (~3/ctr/yr)

What does listing for OHT with CHD
look like in the current era?
• Patient X prenatally diagnosed with HLHS variant
(MV/AV/LV hypoplasia with severe aortic
insufficiency and unguarded aortic valve)
• d8: Stage 1 with 3.5 mm BT shunt and oversewing
of aortic valve. Chest left open. Bicuspid
dysplastic aortic valve. Direct cardioversion for
SVT 220-240, loaded with digoxin.
• d9: attempted sternal closure aborted for
hypotension and bradycardia. SVT cardioverted/
EPI/ Dopa/ Bicarb-adenosine
• d10: sternal closure successful

• wk3: Cardiac arrest, E- CPR, initiated VA ECMO via right
neck
• 5d later: Decannulated, vessels ligated
• wk5: CPR/thrombosed shunt > emergency cath lab; 3.5
x 15mm stent placed in BT shunt. LPA noted to be
narrow
• listed Status 1A for heart transplantation @ 3mo with
severe ventricular dysfunction, AI, and AV valve
regurgitation
• 3.5mo: Tracheostomy, 3.5 Bivona,
• 4mo bradycardia, cardiac decompensation requiring
epi and atropine

• Next wk: brady/desat episodes multiple requiring hand
bag ventilation (not around cares/agitation)
• 3d later brady/desat with rising lactates, made NPO
and arterial line placed
• 3d later lactic acidosis > paralyzed/sedated, made
status 7
• Given recent and continued clinical worsening, parents
and medical team made decision to make him DNR at
~5mo. Patient's vasoactive medications were stopped.
He was disconnected from his ventilator later in the
day and died shortly after in his mother's arms.

Prenatal diagnosis
Overall trends
Outcomes

PEDIATRICS Volume 136, number 2, August 2015
• ~32,000 procedures in patients <6mo from 91
STS centers
• From 2006 – 2012, overall prenatal diagnosis
rates increased from 26% to 42%
• Highly variable rates between regions and
according to defect type

No effect on surgical outcomes
1. Copel JA, Tan ASA, Kleinman CS. Does a prenatal diagnosis of congenital heart
disease alter short-term outcome? Ultrasound Obstet Gynecol 1997; 10: 237–241.
2. Kumar RK, Newburger JW, Gauvreau K, Kamenir SA, Hornberger LK. Comparison
of outcome when hypoplastic left heart syndrome and transposition of the great
arteries are diagnosed prenatally versus when diagnosis of these two conditions is
made only prenatally. Am J Cardiol 1999; 83: 1649–1653.
3. Eapen RS, Rowland DG, Franklin WH. Effect of prenatal diagnosis of critical left
heart obstruction on perinatal morbidity and mortality. Am J Perinatal 1998; 15:
237–242.
4. MahleWT, Clancy RR, McGaurn SP, Goin JE, Clark BJ. Impact of prenatal diagnosis
on survival and early neurologic morbidity in neonates with hypoplastic left heart
syndrome. Pediatrics 2001; 107: 1277–1282.
5. Dorfman AT, Marino BS, Wernovsky G, Tabbutt S, Ravishankar C, Godinez RI,
Priestley M, Dodds KM, Rychik J, Gruber PJ, Gaynor W, Levy RJ, Nicolson SC,
Montenegro LM, Spray TL, Dominquez TE. Critical heart disease in the neonate:
presentation and outcome at a tertiary care center. Pediatr Crit Care Med 2008; 9:
193–202.
6. Kipps AK, Feuille C, Azakie A, Hoffman JIE, Tabbutt S, Brook MM, Moon-Grady
AJ. Prenatal diagnosis of hypoplastic left heart syndrome in current era. Am J Cardiol
2011; 108: 421–427.
7. Sivarajan V, Penny DJ, Filan P, Brizard C, Shekerdemian LS. Impact of antenatal
diagnosis of hypoplastic left heart syndrome on the clinical presentation and surgical
outcomes: the Australian experience. J Paediatr Child Health 2009; 45: 112–117.
8. Trento LU, Pruetz JD, Chang RK, Detterich J, Sklansky MS. Prenatal diagnosis of
congenital heart disease; impact of mode of delivery on neonatal outcome. Prenat
Diagn 2012; 32: 1250–1255.
9. Montana E, Khoury MJ, Cragan JD, Sharma S, Char P, Fyfe D. Trends and
outcomes after prenatal diagnosis of congenital cardiac malformations by fetal
echocardiography in a well defined birth population, Atlanta, Georgia, 1990–1994.
J Am Coll Cardiol 1996; 28: 1805–1809.
10. Levi S, Zhang WH, Alexander S, Viart P, Grandjean H, Eurofetus Study Group.
Short-term outcome of isolated and associated congenital heart defects in relation to
antenatal ultrasound screening. Ultrasound Obstet Gynecol 2003; 21: 532–538.
11. Levey A, Glickstein JS, Kleinman CS, Levasseur SM, Chen J, Gersony WM, Williams
IA. The impact of prenatal diagnosis of complex congenital heart disease on neonatal
outcomes. Pediatr Cardiol 2010; 31: 587–597.
12. Satomi G, Yasukochi S, Shimizu T, Takigiku K, Ishii T. Has fetal echocardiography
improved the prognosis of congenital heart disease? Comparison of patients with
hypoplastic left heart syndrome with and without prenatal diagnosis. Pediatr Int
1999; 41: 728–732.
13. Landis BJ, Levey A, Levasseur S, Glickstein JS, Kleinman CS, Simpson LL, Williams
IA. Prenatal diagnosis of congenital heart disease and birth outcomes. Pediatr Cardiol
2013; 34: 597–605.
14. Khoshnood B, DeVigan C, Vodovar V, Goujard J, Lhomme A, Bonnet D, Goffinet
F. Trends in prenatal diagnosis, pregnancy termination, and perinatal mortality of
newborns with congenital heart disease in France, 1983–2000: a population-based
evaluation. Pediatrics 2005; 115: 95–101.
15. Wright LK, Ehrlich A, Stauffer N, Samai C, Kogon B, Oster ME. Relation of prenatal
diagnosis with one-year survival rate for infants with congenital heart disease. Am J
Cardiol 2014; 113: 1041–1044.
16. Matsumoto A, Aoyagi Y, Masaki M, Endo K, Mochizuki I, Kanedo M, Fukuda
Y, Momoi N, Hosoya M. Outcome of fetal echocardiography: a 17 year
single-institution experience in Japan. Pediatr Int 2012; 54: 634–638.
17. Oster ME, Kim CH, Kusano AS, Cragan JD, Dressler P, Hales AR, Mahle WT,
Correa A. A population-based study of the association of prenatal diagnosis with
survival rate for infants with congenital heart defects. Am J Cardiol 2014; 113:
1036–1040.
18. McBrien A, Sands A, Craig B, Dornan J, Casey F. Major congenital heart disease:
antenatal detection, patient characteristics and outcomes. J Matern Fetal Neonatal
Med 2009; 22: 101–105.

Truncus Arteriosus
Swanson TM et al
Pediatric Cardiology 2009

• Meta-analysis of 8 studies
• Standard risk, planned surgery, optimal care
– Preop death in 0.5% prenatal vs 3.2% postnatal
• “Several of the most critical infants in the
postnatal group were considered too high risk
and never underwent surgery.”

• ~600pt from 43 centers surviving to discharge
after Stage 1 surgery for single ventricle dz
• 75% with prenatal dx, 66% HLHS

• Variation in rate of
prenatal diagnosis
between sites 40%-
100%.
• Those with PD had less
preop AV valve
regurgitation,
ventricular
dysfunction, acidosis,
shock, AKI, need for
mechanical ventilation.
• Unable to detect
change in LOS or
mortality

• Clinician angst isn’t always good for patients.
– E.G., Correlation with lower birth weight and
earlier gestational age.
• Might proximity to centers and modern care
reduces risk/harm/burden of “late” diagnosis?
• Prenatal diagnosis rates may co-vary with
higher SES, race/ethnicity, proximity to centers
of excellence
• Start worrying sooner vs. start processing
sooner? Effect on parental stress/coping?

Fetal intervention
• Evolving picture at several centers
• Fetal balloon aortic valvuloplasty to prevent or
reduce HLHS – promising results in select
cases.
• Pericardial tumor resection.
• Stent/balloon atrial septostomy for HLHS with
intact atrial septum.

Ensuring “Half a heart” doesn’t
mean “Half a life.”

Infant Single Ventricle Monitoring Program
• Enrollment: Any neonate with shunt
dependent pulmonary blood flow
• Goal: to augment care provided by primary
cardiologist to decrease interstage mortality
• Focus on:
– Growth
– Overall well being
– Getting them safely to Stage II or full repair

Program design
• Inpatient
– Enrolled after Stage I palliation or shunt placement
• Follow along while inpatient
– Discharge readiness
• Discharge checklist
– Importance of scheduled and documented 1st cardiology f/u visit
within 5-7d of discharge prior to patient leaving
• Teaching
• Supplies – electronic scale and pulse oximeter
– Pathway
• On intranet:
http://www.chop.edu/pathways/inpatient/single-ventricle-
fetus-newborn/

Program design
• Outpatient
– Frequent appointments with physicians (alternating
cardiology and pediatrician)
• Pre visit planning email
– Phone contact with NP
• Minimum of weekly to review weights, feedings, sats,
problems/questions
• Triage calls during the week ( ie red flags) M-F 8a-5p
– Weekly home nursing visits/PDN (optional)
– Pathway
• On intranet: http://www.chop.edu/professionals/clinical-
pathways/outpatient-clinical-pathways.html

Red Flags
Infant Single Ventricle Monitoring Program
Red Flags for seeking medical attention/advice
Call monitoring program NP 215-435-6342 Monday-Friday 8am-5pm
Call CHOP Cardiology fellow on nights, weekends, and holidays 215-590-1000
(and ask to speak to cardiology fellow)
1. Fussiness/Irritability
2. Change in behavior from “norm”
3. Change in color
4. Decreased oxygen levels (less than 75%)
5. Diarrhea, vomiting (more than usual)
6. Not eating well
7. Sweating more than usual or sweating with feeds
8. Change in breathing pattern (too fast or too slow; too hard)
9. Increased sleepiness
10.Fever greater than 100.4
11.Cold symptoms (cough, congestion, runny nose)
12.Failure to gain an average of 20grams/day in 3 days or weight loss of 30 grams in one
day
You know your baby best! We encourage you to report any changes that are of concern to you.
 ISVMP Nurse Practitioner: Alyson Stagg 215-435-6342
 Cardiologist: ___________________________

Cardiac Kids Developmental
Follow Up Program
CKDP
David Goldberg, MD (Director)
Alisa Burnham, MD (Pediatrician)
Psychology, Speech, PT, OT, Social Work
Kaitlyn Daniels, RN, BSN (Program Manager)
267-425-6616 danielsk@email.chop.edu

AHA White Paper 2012
Increased risk of
developmental delay
calls for periodic
developmental
surveillance throughout
childhood

American Academy of Pediatrics
• Policy statement released June 2014
• Read often and early
• Reduce disparities
– Increasing the number of words children hear in
the earliest years
– Disparities evident by 18 months of age1
• Read Out and Read
– Nationwide program of 20,000 pediatricians that
gives books to low-income families
1Fernald, A. (2012) SES Differences in language processing skill and vocabulary evident at 18 months.Developmental Science. 16 (2)234-248.

Enrollment
• Active screening/enrollment: Any patient
having cardiac surgery or cardiac cath
intervention under the age of 6 months
• Referrals:
– Prolonged hospitalization
– Developmental concerns
• 0 to 3 years of age
• *expanding soon to 5 years of age

Program design
• Recruited at initial inpatient stay in CCU or
immediately after discharge
• 1st visit at 3-6 months of age
• Return at 12, 24, 36 months, 4 and 5 years
*9 and 18 months if clinically indicated

Developmental Testing
• Bayley Scales of Infant and Toddler
Development (Bayley –III)
• MCHAT
• Rosetti, PLS
• PDMS, Beery VMI
• *School readiness
– CBCL, WPPSI, Bracken, ABAS, BITSEA

Opening the “Black Box” of the ICU

Closing Thoughts
• Advances:
– As a credit to years of advances in CT Surgery, to
some extent, “It’s not about the run.”
– Variability in outcomes can have more to do with
who comes to the OR and what happens before
and after the OR
• Timing/Decision-making:
– Goal and physiology directed approach for best
long term outcomes

Closing Thoughts
• Fetal Dx/Intervention:
– Exciting evolution continues in fetal diagnosis and
intervention
– Prenatal diagnosis is good – be careful how you ask
the question and hear the answer
• Transplant:
– Can be the least bad option,
– with possible good long term outcomes,
– for those who need it,
– if it goes well…

Closing Thoughts
• Outcomes and follow-up
– Prematurity is forever
– Heart disease is forever

Thank you!
• SlideShare
– http://www.slideshare.net/GeoffreyBird
• LinkedIn
– http://lnkd.in/bHP5Q5v
• Not to be ironic, I do occasionally tweet:
@GLBird
• bird@email.chop.edu

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