Transcript Ethics and HLHS - Children's Mercy Hospital

Hypoplastic Left Heart Syndrome (HLHS)
© Copyright 2010
About HLHS
Underdevelopment
of the left ventricle,
aorta, and aortic
arch, as well as
mitral atresia or
stenosis.
http://www.nlm.nih.gov/medlineplus/ency/images/ency/fullsize/22705.jpg
About HLHS
• Occurs in about 0.02% of live births
• Prevalence changing as prenatal
diagnosis allows diagnosis earlier in
pregnancy.
HLHS outcomes improving over time
Children’s Hospital, Philly, 1989-91
• 151 patients treated using three-stage approach
• 109 (72%) early survivors.
• 78 underwent hemi-Fontan operation; 69
survivors (88%).
• 27/78 patients have subsequently undergone
definitive Fontan procedures with no deaths.
• Anticipated overall survival 63%
Norwood. Ann Thoracic Surgery. 1991.
Edmonton, 1990-95
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158 patients
120/158 (76%) survivors of stage I
102/106 (96%) survivors of stage II
53/62 (85%) survivors of stage III (Fontan)
• Actuarial survival – 58% at 5 years. Better
with normal anatomy (71%)
Bove et al. Ann Surg. 1996.
Alberta results and choices
• Shift in outcomes and choices over time
– 1987-93: 25% treatment; 75% hospice; 0% survival
– 1994-96: 37% treatment; 63% hospice; 62% survival
– 1996-98:95% treatment; 5% hospice; 78% survival
• Survival to discharge improved from 0% to 78%
Osiovich et al. J Perinatol. 2000.
Outcomes better for patients with “standard risk”
compared to those at “high risk.”
High risk group: non-cardiac congenital
anomalies, pulmonary venous obstruction,
age >1 month at initial surgery
Bove et al. Ann Surg. 1996.
Actuarial survival for babies who undergo stage I – Boston Children’s, 2001-6
Pigula et al. Semin Thorac Cardiovasc Surg. 2007.
Outcomes have improved over time, but vary with anatomic subtype
Pigula et al. Semin Thorac Cardiovasc Surg. 2007.
Outcomes for 79 fetuses
diagnosed with HLHS in
Birmingham, England,
between 2000 and 2004.
25% of parents chose to
terminate the pregnancy.
Of the rest
10% stillborn
25% compassionate care
15% died before surgery
50% had surgery
2/3 of babies who had
surgery survived.
Rasiah et al. Arch Dis Child Fetal Neonatal Ed. 2008.
Neurodevelopmental outcomes
• 7/11 survivors with “major developmental
disabilities”*
• Median IQ 66, 57% had CP**
• Median IQ 86, 18% with MR, 33% in special
ed.***
• 3/31 survivors have “significant neurologic
impairment.****
* Rogers et al. J Peds. 1995.
** Miller et al. J Child Neurol. 1996.
*** Mahle et al. Peds. 2000.
**** Andrews et al. Arch Dis Child. 2001.
Neurologic problems associated with
congenital heart disease (CHD)
• 41 newborns with CHD
• MRI, MRS, and DTI (diffusion tensor
imaging)
• White matter injury in 13 (32%) newborns
with CHD, none in control newborns.
Miller et al NEJM 2007
Developmental outcomes after
heart transplantation
• 26 infants evaluated after transplant for HLHS
• Bayley Scales of Infant Development
– MDI 88 (range <50 to 102)
– PDI 86.5 (<50 to 113),
• Wechsler Preschool and Primary Scale of Intelligence or
Wechsler Intelligence Scale for Children-III
– mean verbal score of 90.5,
– mean performance score of 88.9
– full scale score of 88.5
• Vineland scales, 39% scored >1 SD below the mean on
measures of daily living scales, 22% on the socialization
subscale, 48% on the communication subscale, and
52% on the adaptive behavior scale.
Full scale, verbal, and performance IQ tests and the CELF-R
test among HLHS survivors at age 8
Mahle et al. Pediatrics. 2000.
Copyright ©2000 American Academy of Pediatrics
What would MDs and RNs do?
• Subjects: 54 pediatric residents and 543
pediatric nurses at 3 pediatric cardiac
centers in Los Angeles, 2005.
• Response – 29% (RNs) and 43% (MDs)
• Hypothetical scenarios of fetal diagnosis
and newborn diagnosis
Renella et al. Prenat Diag. 2007.
What would MDs and RNs do?
• Would you terminate a pregnancy in which
the fetus was diagnosed with HLHS?
– 43% of residents
– 50% of nurses
• Would you choose, or seriously consider,
comfort care for a newborn with HLHS
– 48% of residents
– 68% of nurses
Renella et al. Prenat Diag. 2007.
Critical care doctors
• Subjects: 454 attending physicians in the
sections of neonatology, pediatric critical care,
pediatric cardiology, and congenital cardiac
surgery at the largest pediatric cardiac surgery
centers in the United States.
• Survey sent by mail in 1999
• Asked about prenatal and neonatal
diagnosis of HLHS
Kon et al. Am J Cardiol. 2003.
Results from doctors
• 57% response rate
• 48% would terminate pregnancy, 22%
would continue, 30% were uncertain.
• For newborns, 32% would choose surgery,
28% comfort care, 33% uncertain.
• Surgeons most likely to choose surgery
• Choices not associated with perceived
postoperative outcomes.
Kon et al. Am J Cardiol. 2003.
Generalizing from physicians
• “Because a large proportion of experts
would choose nonsurgical care for their
own child, this option should be
considered reasonable and therefore
presented to parents of affected children.”
Kon et al. Am J Cardiol. 2003.
Prenatal diagnosis of HLHS
• Majority of cases occur in pregnancies
with no risk factors
• Sensitivity varies with
– Experience of the ultrasonographer
– Types of views (4-chamber vs. outflow tracts)
– Wide range of detection rates: 7-95%
Kovalchin et al. Pediatr Cardiol. 2004.
Implications of prenatal diagnosis
• Better medical/surgical management?
• Better psychological outcomes?
• More termination of pregnancy?
Better management
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Delivery in tertiary care setting
Decreased pre-op morbidity
Less acidosis
Better end organ perfusion
May improve both survival and neurologic
outcomes for survivors
Are outcomes better with fetal diagnosis?
Flow diagram depicting outcomes of patients with prenatal or postnatal diagnosis
(Dx) of HLHS.
Tworetzky. Circulation. 2001.
Prenatal vs. postnatal diagnosis
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Guy’s Hospital, London
Survival with prenatal diagnosis - 49%
Survival with postnatal diagnosis – 44%
Postnatally diagnosed babies are a
selected population who have survived a
collapse and transfer prior to surgery.
Better psychological outcomes?
• Normal fetal echocardiography decreased
maternal anxiety, increased happiness,
and increased the closeness women felt
toward their unborn children.
• Abnormal fetal echocardiography
increased maternal anxiety; mothers felt
less happy about being pregnant.
Sklansky. J Am Soc Echocardiogr. 2002.
Europe vs. US
• 32 prenatal diagnoses of HLHS, Utrecht, 19882001
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16 pregnancy terminations
4 in utero fetal deaths
5 compassionate care after birth, no surgery
7 reconstructive surgery
– 7 cases had associated extracardiac anomalies
Verheijen et al. Herz. 2003.
Outcome of 174 fetuses diagnosed antenatally at Guy's
Hospital with HLHS.
TOP, termination of pregnancy; IUD, intrauterine death.
Andrews et al. Arch Dis Child. 2001.
Comparison of parental decisions in the U.S., the UK, and the
Netherlands after prenatal diagnosis of HLHS
TOP = termination of
pregnancy
CC = compassionate care
ITT = intention to treat
Verheijen et al. Herz. 2003.
References
Andrews R, Tulloh R, Sharland G, Simpson J, Rollings S, Baker E, Qureshi S, Rosenthal E, Austin C,
Anderson D. Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following
antenatal diagnosis. Arch Dis Child. 2001 Dec;85(6):474-7.
Bove EL, Lloyd TR. Staged reconstruction for hypoplastic left heart syndrome. Contemporary Results.
Ann Surg. Sep;224(3):387-94, discussion 394-95.
Kon AA, Ackerson L, Lo B. Choices physicians would make if they were the parents of a child with
hypoplastic left heart syndrome. Am J Cardiol. 2003 Jun 15;91(12):1506-9,A9.
Kovalchin JP, Silverman NH. The impact of fetal echocardiography. Pediatr Cardiol. 2004 MayJun;25(3):299-306.
Mahle WT, Clancy RR, Moss EM, Gerdes M, Jobes DR, Wemovsky G. Neurodevelopmental outcome
and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome.
Pediatrics. 2000 May;105(5):1082-9.
Miller G, Tesman JR, Ramer JC, Baylen BG, Myers JL. Outcome after open-heart surgery in infants
and children. J Child Neurol. 1996 Jan;11(1):49-53.
Norwood WI Jr. Hypoplastic Left Heart Syndrome. Ann Thorac Surg. 1991 Sept;52(3):688-95.
Osiovich H, Phillipos E, Byrne P, Robertson M. Hypoplastic Left Heart Syndrome: “To Treat or Not to
Treat”. J Perinatol. 2000 Sep;20(6):363-5.
References – Cont'd.
Pigula FA, Vida V, Del Nido P, Bacha E. Contemporary Results and Current Strategies in the
Management of Hypoplastic Left Heart Syndrome. Semin Thorac Cardiovasc Surg. 2007
Fall;19(3):238-44.
Rasiah SV, Ewer AK, Miller P, Wright JG, Barron DJ, Brown WJ, Kilby MD. Antenatal perspective of
hypoplastic left heart syndrome: 5 years on. Arch Dis Child Fetal neonatal Ed. 2008 May;93(3):F1927. Epub 2007 Jun 29.
Renella P, Chang RK, Ferry DA, Bart RD, Sklansky MS. Hypoplastic left heart syndrome: attitudes
among pediatric residents and nurses towards fetal and neonatal management. Prenat Diagn. 2007
Nov;27(11):1045-55.
Rogers BT, Msall ME, Buck GM, Lyon NR, Norris MK, Roland JM, Gingell RL, Cleveland DC, Pieroni
DR. Neurodevelopmental outcome of infants with hypoplastic left heart syndrome. J Pediatr. 1995
Mar;126(3):496-8.
Sklansky M, Tang A, levy D, Grossfield P, Kashani I, Shaughnessy R, Rothman A. Maternal
psychological impact of fetal echocardiography. J Am Soc Echocardiogr. 2002 Feb;15(2):159-66.
Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH. Improved surgical
outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation. 2001 Mar 6;103(9):126973.
Verheijen PM, Lisowski LA, Plantinga RF, Hitchcock JF, Bennink GB, Stoutenbeek P, Mejboom EJ.
Prenatal Diagnosis of the fetus with hypoplastic left heart syndrome management and outcome. Herz.
2003 May;28(3):250-6.