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Dying of a Broken Heart: Ethics and Law in a Case of Hypoplastic
Left Heart Syndrome
Chet K. Pager, MD
The short life of Baby P is reviewed. Baby P was an infant girl who was born
with hypoplastic left heart syndrome and died at the age of 30 days.
Effective, aggressive surgical treatment for this condition is rapidly
developing in some countries, but is rarely available or successful in most.
Baby P died without intervention. After presenting the history and
background, the legal and ethical issues raised by this case regarding
decision - making, futility, and best interests are reviewed.
Journal of Perinatology 2000; 20:535 ± 539.
CASE REPORT
Baby P was born at term without complications, and weighing 3140
gm, in an Australian rural hospital. On day 2, Baby P's condition
began to deteriorate, involving tachypnea, pallor, oliguria, and poor
oxygen saturation. Treatment with lasix and morphine was
commenced, and at 45 hours she was intubated and transferred by
air ambulance to a tertiary referral hospital.
Echocardiogram and cardiac catheterization confirmed critical
aortic stenosis (pressure gradient 55 mm Hg) and a hypoplastic left
ventricle. A balloon valvotomy was performed. Postoperatively, Baby
P remained in cardiac failure. Further surgery was thought not to be
appropriate, and after discussion with Baby P's parents, their
agreement was obtained. However, the doctors saw no real treatment
alternative within Australia, and none was offered to them overseas.
On day 28, Baby P was returned to the rural hospital where she was
born. By this time her weight had fallen to 2873 gm, and her
medications included fursemide, spironolactone, digoxin, and
chloral hydrate. Baby P's cardiac failure worsened despite maximal
medical therapy, and her chest X-rays showed increasing
cardiomegaly with pulmonary edema.
Baby P remained in cardiac failure, with a heart rate of 140
to 150 and marked pulsus alternans, no palpable pulses, and
virtually nonexistent capillary refill. Her liver was palpable 4 cm
below the coastal margin, and X-ray revealed gross
cardiomegaly and patchy lung consolidation. On day 30, Baby P
was noted to be increasingly unsettled, requiring more frequent
doses of chloral hydrate for ``sedation and comfort.'' At 6:25 PM,
she suddenly ceased breathing. Resuscitation was not attempted,
Royal Prince Alfred Hospital, Sydney, Australia.
Address correspondence and reprint requests to Chet K. Pager, MD, C /- 67 Missenden Road PO,
Camperdown, NSW 2050, Sydney, Australia.
and the cause of death was thought to be a sudden arrhythmia
or cardiac arrest.
Medical Overview: Hypoplastic Left Heart Syndrome
(HLHS)
HLHS accounts for 25% of all deaths from congenital heart disease in
the first month of life,1 with an incidence of 1:6000 births,2 although
this is decreasing now that 37% of cases are detected prenatally with
ultrasound.3 Until recently, HLHS was considered to be a uniformly
fatal condition.1,4,5 Untreated, the median age of death is 4 to 5 days;2
and with maximal medical therapy median survival is only 60 days.6
Recent years have seen tremendous advancement in surgical
technique and outcome for the surgical treatment of HLHS in
specialized centers, using the Norwood and Fontan procedures.
Hospital survival rates from large patient series in the United States
are now routinely quoted well above 70%,4,5,7 with 5-year survivals in
the range of 58% to 70%.5,8 ± 10 However, staged reconstruction does
require multiple operations, and the infant would spend most of his
or her first year of life in hospital.
Neonatal heart transplantation has also significantly developed
in recent years. Five-year survival rates now approach 80% for
cardiac transplantation. Although a quarter of all HLHS patients will
die waiting for transplantation,2,7 even with this mortality factored in,
the survival rates for transplant are comparable with Norwood and
Fontan-staged reconstructions, with improved quality of life and
physical development.7,11 HLHS accounts for 60% of all pediatric heart
transplantations in the United States.12
Both staged repair and transplantation are exceedingly complex
procedures, and success is correlated directly with ongoing
experience13; neither operation could be considered a routine or
successful intervention except for specialist centers in select
countries. Australian hospitals only receive about 50 cases of HLHS
per year, and although some Australian surgeons are willing to
attempt the Norwood and Fontan procedures, outcomes have
generally been quite poor and the HLHS population insufficient to
develop or maintain a satisfactory level of skill.
Autonomy and Decision-Making
Contemporary medical ethics is firmly placed within the
individualist tradition of western philosophy, in which the
increasingly dominant principle (both legally and ethically) is
autonomy. However, the inability of the infant to express any
preferences, the difficulty in accurately estimating prognosis, the
long potential life span that might be enjoyed, and the family
context in which the child exists all make such best-interest
estimations quite difficult.14
Journal of Perinatology 2000; 20:535 ± 539
# 2000 Nature America Inc. All rights reserved. 0743-8346/00 $15
www.nature.com/jp
535
Pager
Most societies acknowledge the preeminence of the parents'
role in determining their child's best interests and their right to
make decisions on their children's behalf. The important
distinction is that parents have a responsibility to ensure their
children's welfare; children are not their parents' possessions. The
right of parents to make decisions for their children, therefore, is
not absolute, and can be overridden in cases of parental
incompetence, abuse, neglect, or decisions deemed inappropriate
or contrary to the child's best interests. This latter point is the
most difficult to define, and must be considered in the context
of socially shared values; the most classic example being the
Jehovah's Witness parent who refuses a life-saving blood
transfusion for his or her child. This has been widely judged in
law and ethics to be a decision that should be overturned.15
In fact, even some very reasonable decisions are denied parents in
order that the state, parens patriae, may protect the best interests of
its not-competent children with caution and disinterest. For
instance, the decision to sterilize an intellectually disabled minor is
one that should not be made by the child's parents or medical
practitioners, but rather by the family court.16
In the case of Baby P, doctors appropriately related to her
parents as the primary decision-makers. The parents were in
agreement with her doctors' treatment plan. However, as the
situation was presented to them, there was no real involvement
in the decision-making process or true exercise of autonomy,
only an after-the-fact acquiescence. It was suggested that there
was really only one appropriate course of action; it was further
hinted that should they make what was felt to be an
inappropriate decision (for surgery), it might be refused to
protect their baby's best interests. No mention was made of
treatment alternatives available overseas.
Although the decisions made may well have been in Baby P's best
interests, the handling of this case was lacking in key elements of
informed consent, disclosure, and voluntarism. Although the parent's
role as Baby P's surrogate decision-makers was never subverted,
neither was it exercised in any real sense: the parents had assented
rather than consented. According to the fairly stringent legal and
ethical standards for autonomy and informed consent, the
management of this case was therefore lacking. The perinatologists
involved argued that because no Australian insurance scheme
covered overseas surgery and the parents clearly could not afford it,
advising them of this option would only increase the parents' feelings
of guilt. However, in cultures in which individual autonomy has
primacy above beneficence, medical indications, or the professional
role, this well-meaning paternalism, or even nationally based
parochialism, is no longer appropriate.
Futility and Best Interests
If a treatment is considered futile, doctors may legally and ethically
withhold or withdraw the treatment, even contrary to the patient's
wishes.17 ± 19 Furthermore, doctors are under no obligation to even
inform their patients of the existence of therapies they consider
536
Ethics and Law in a Case of HLHS
futile.20,21 This view has been a longstanding and well-established
principle. Even Hippocrates advised physicians to ``refuse to treat
those who are overmastered by their diseases, realizing that in such
cases medicine is powerless.''14 Although applying the concept of
futility encounters difficulties, it is by no means an uncommon
practice, particularly in neonatal settings. In fact, the most common
mode of death in neonatal intensive care units is the limitation or
withdrawal of treatment.20,22 ± 25
Unlike many areas, the courts have in most cases left the decision
regarding a treatment's futility to the treating doctors. But although
it seems clear that a doctor should be released from a duty of care
where no good can be offered to the patient, arriving at formulae for
what futility must entail is no easy matter.18
One well-known quantitative definition was proposed by
Schneidermann26 which asserts that if the intervention does not work
in more than 1% of attempts, it should be futile. Schneidermann uses
physiological outcome: that treatment will fail ``to reverse a
physiological disturbance that will lead to the child's proximate
cause of death.''27 However, we do not judge worth by physiology
alone. When faced with an imminently and irrevocably dying infant,
one can well agree that aggressive treatment such as artificial
ventilation should be considered futile, although such a treatment
would certainly be physiologically successful, in that it would
maintain breathing, if nothing else.
This objection has yielded a second major approach to
defining futility, namely that a treatment is futile if its intent
or effect is to prolong dying without much benefit to the
patient. However, the moment one deviates from strict
physiological or mortality criteria, futility becomes an
intrinsically value-laden idea that merely designates the far end
of the burdens versus benefits continuum.18 Nonetheless, this
broader yet more difficult conception of futility is in line with
the reasoning of many ethicists and appears to better match
patients' actual use and understanding of the word. Yet while
strict (physiological) futility is poorly applicable to most
ethical and medical decisions, by expanding one's definition to
include ``benefit,'' two further problems are immediately
encountered.
The first problem is that one is forced to ask whether any
considerations might outweigh the goal of preserving or extending
life, even if this means achieving little else save a prolonged death.
Although the principle of sanctity of life has its roots in diverse
religious traditions, can be derived from the United States
Constitution's guarantee of the ``right to life'' or from Kantian ethics,
and comes perilously close to formal expression in recent United
States case law,27 it is clear to many that relentless treatment to
preserve life at all costs is not consistent with any but the most
extreme doctrines of religion or ethics.
Acknowledging this, Catholic theologians have traditionally
distinguished ordinary treatments from extraordinary treatments
(i.e., those that cannot be obtained or used without excessive
expense, pain, or other inconvenience, or which, if used, would not
Journal of Perinatology 2000; 20:535 ± 539
Ethics and Law in a Case of HLHS
offer a reasonable hope of benefit). Doctors are not obligated to
provide, nor patients to accept, extraordinary treatments.28
Instead of focusing on the nature of the treatment, one could
focus on the subject being treated, and ask to what extent severely
and permanently disabled patients ``can be regarded as being
humanly alive in the sense in which we usually understand those
words.''14 In a controversial argument rejecting the blanket
sanctity of life, Singer29 has developed a system of ethics based on
the idea of personhood, emphasizing that human rights and the
importance of human life reside in those qualities that make us
human beings, rather than merely human bodies. Tooley30
enumerated these as the capacity to have a concept of self; the
actuality of being a conscious subject of experiences: a capacity
for self-consciousness; and the actuality of being a continuing
subject of experiences and other mental states. McCormick31 has
added the capacity for relational potential to these criteria. This
viewpoint is extremely contentious, but many would agree that
ethical considerations depend both on the nature of the treatment
and on the individual case at hand. Within Catholic theology, the
concept of proportionality between the proposed treatment and the
individual situation is replacing the ordinary versus extraordinary
dichotomy.
What remains, then, are difficult and case-dependent questions of
burdens and benefits, best interests, and quality of life, ideas which are
difficult to define or cleanly distinguish from one another. It may be
difficult to admit that the severity of illness and burden of existence for
some infants makes death preferable to continued life, but many
would recoil from imposing painful treatment on an infant that at best
will yield a few years' survival in constant and severe pain, unable to
participate, even minimally, in human existence.32,33 As even Plato said,
if a treatment would at best leave a patient with a ``useless life,'' then
``medicine was not intended for them and they should not be treated
even if they were richer than Midas.''14
The English court's position on futility was best expressed by Lord
Goff in the 1993 case of Airedale National Health Service Trust v
Bland (AC789): ``I cannot see that medical treatment is appropriate
or requisite simply to prolong a patient's life, when such treatment
has no therapeutic purpose of any kind, as where it is futile because
the patient is unconscious and there is no prospect of any
improvement in his condition.''34 Likewise, the American Academy of
Pediatrics recognizes that life-sustaining treatment, including
artificial nutrition and hydration, may be withdrawn ``if treatment
only preserves biologic existence.''35
The second problem involved with considerations beyond strictly
physiological futility is one of decision-making. Futility, although
imperfect, is in essence a matter for professional judgment. Questions
of burdens, quality of life, and best interests are more explicitly value
judgments. The courts do not easily recognize quality of life as a
legal concept,34 and in the United States have seriously criticized the
concept.35 Furthermore, in the case of never-competent individuals,
decision-making is extremely difficult, and research has shown that
doctors (and often surrogate decision-makers) consistently
Journal of Perinatology 2000; 20:535 ± 539
Pager
underestimate patients' subjective assessments of their own quality of
life.34
It is for this reason that most recent publications on this subject
have focused not on what futile or burdensome treatments are, but
rather the process by which such decisions are made or conflicts
resolved, through clear communication and consensus-building
between the patients (or guardians) and doctors.17,35,36
However, there is a growing ethical and legal disquiet with any
decisions, based on the value judgments of others, that result in an
infant's death. The difficulties in adjudicating an infant's ``best
interests'' have already been discussed. Other critics raise the
symbolic value (to family, community and the medical profession)
of providing treatment, even where the actuarial value of the
intervention may be negligible. Some highlight the curious
foundations of any ``universal'' ethical principle (such as futility)
whose application varies with culture, technology, and geography.
Certainly, in the case of Baby P, the ``futility'' of the Norwood and
Fontan procedures depends entirely on whether she was born in
America, Australia, or Africa, and under Schneidermann's definition,
is further dependent on precisely what physiological goal one hopes
to achieve.
The differences in treatment, referral, information, and outcome
Baby P and her parents might have received in the United States, be it
due to organ availability, surgical skill, cost, ignorance, or other
factors, raises problematic issues concerning the management and
development of regional expertise, recently brought to the public eye,
in part, by the Bristol case in the United Kingdom. In 1998, a group
of pediatric cardiac surgeons at a peripheral hospital in Bristol
attracted widespread notoriety for continuing to perform complex
and high-risk surgery despite achieving outcomes significantly below
the national standard.37 This situation, like the Baby P case,
highlights the perennial problem imposed by an ever-increasing
medical armament without concomitant advances in guidelines
concerning their use and distribution.38
Responding to these ambiguities in both best interests and futility,
it is one advocate's opinion that the courts ``leave vast discretion to
the medical profession while both courts and doctors pay lip service
to the sanctity of life.''35 In the United States, however, as science
pushes at the bounds of the meaning of ``medical futility,'' the
sanctity of life position has gained strength and is relied on more
frequently by the judiciary.39,40 This position is best exemplified by
aspects of the Baby Doe legislation, the Emergency Medical
Treatment and Active Labor Act, the Americans with Disabilities Act,
and the most recent Baby K Case.38 In this last case, ``treatment'' and
futility as defined in the Emergency Medical Treatment and Active
Labor Act were construed in their most narrow physiological sense,
leading to a court-mandated ventilation of an anencephalic baby.
An anencephalic baby lacks a functioning cerebral cortex, is
permanently unconscious, has no cognitive ability, and cannot see,
hear, talk, or feel pain. But it has an intact brainstem, and can emit
reflex breathing, sucking, swallowing, and pain avoidance.38
Therefore, by the current ``brainstem'' legislative definition of death,
537
Pager
anencephalic babies are alive and subject to the full protection of the
Emergency Medical Treatment and Active Labor Act, the Americans
with Disabilities Act, and other laws.
Although American cases like Baby K have little bearing on and
would not be expected from most other jurisdictions, they do
highlight a general international trend for medical bodies, such as
the American Medical Association, to increasingly focus on questions
of quality of life, while the courts are called on to increasingly
address sanctity of life. As the medical armament grows, this
divergence will produce increasing tensions between medical practice
and the law.
CONCLUSIONS
It is a peculiarity of philosophy that the conclusions reached are
often less significant than the arguments used along the way,
and this study has covered a large amount of territory. However,
likely consensus positions regarding the issues raised through this
case would include: (1) Baby P's parents will make all
decisions so long as the parents are competent and the decisions
appropriate and in Baby P's best interests. (2) Futile or
extraordinary treatment need not be provided, even when parents
request it. Burdensome treatment may be withdrawn or withheld,
although court intervention may be required when agreement
cannot be reached between parents and physicians. Within
Australia, in the case of Baby P, surgical treatment of HLHS
could reasonably be considered as futile.
However, in specialist centers outside of Australia, surgical
treatment of Baby P would by no means be futile, calling into
question the adequacy of the informed consent for nontreatment in
this case. The increasing complexity and fragmentation of medical
competency further calls into question the universality of ethical
principles when applied to medical decisions that are becoming
increasingly dependent on geography, culture, technology, and
individual circumstances.
As medicine advances, along with geographical inequalities,
concepts of futility and autonomy will become less applicable, and
tensions between the sanctity and quality of life will increase. To
reduce further divergence between medical practice and law, greater
emphasis on the collaborative decision-making processes and
conflict resolution are required.
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