SHA24/042001

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Joint Gulf Heart Association - Saudi Heart Association meeting Riyadh, February 13-16, 2013

DEBATE Surgery Should be Offered to Certain Types of Congenital Heart Disease in Gulf Region: PRO Roberto M. Di Donato Prince Sultan Cardiac Center, Riyadh


Should surgery still be denied to certain Should surgery be offered to “certain� CHD? CHD?

patients selected by natural history

more complex lesions at earlier age


CHD “perceived” by many to be better suited for palliative care • • • • •

Hypoplastic left heart syndrome Hypoplastic right heart syndrome Heterotaxies Pulmonary atresia/VSD, with MAPCAs Total anomalous pulmonary venous connection (with obstruction) • Genetic syndromes, including Down’s s.


The grey zone BEST INTEREST OF THE CHILD burden > benefit

Uncertain

burden < benefit

LIFE-SUSTAINING THERAPY unreasonable

investigational

optional

Parental authority

Grey Greyzone zone Reasonable people can have different opinions Berger, TM. J. Pediatr 2010;156:7

mandatory


Decision Making Matrix Jonsen, Siegler, Winslade Clinical Ethics, Third Edition, 1992

Medical Indications (Beneficence, Nonmaleficence)

Patient Preferences (Autonomy)

Quality of Life (Utility, Futility)

Contextual Features (Justice)


MEDICAL INDICATIONS BENEFICENCE - NONMALEFICENCE Medical necessity – appropriateness of therapy … …. medical activities that may be justified as reasonable, necessary, and/or appropriate, based on evidence-based clinical “standards of care” according to: – credible scientific evidence published in peer-reviewed, medical literature generally recognized by the relevant medical community – Physician Specialty Society recommendations – views of Physicians practicing in the relevant clinical area – any other relevant factors.


Hospital Discharge Mortality for Patients Undergoing Stage I Palliation, 2002–2009 Year 2002 2003

Pts undergoing Deaths prior to stage 1 palliation, n discharge, n 303 95 391 119

Discharge survival, % 68.60 69.60

2004 2005 2006

297 658 1.155

75 140 234

74.70 78.70 79.70

2007 2008 2009

1.536 1.879 2.320

276 334 432

82.00 82.20 81.40

From the Society of Thoracic Surgeons, Congenital Heart Database, courtesy of Dr. Jeff Jacobs.


PATIENT’S PREFERENCE AUTONOMY • • • •

Paramount Relying on thorough and unbiased counseling Being self-governing Allowed free will in making a personal decision - FAMILY determines what is in the ‘BEST interest’ of the Child as an individual • A right to withhold consent • What about the baby’s opinion?


QUALITY OF LIFE FUTILITY • Treatment(s) believed not likely to accomplish intended goal • Disagreement re. intended goals of treatment: – Appropriateness of goals – Qualitative goals: poor likelihood of benefit – Quantitative goals: low grade outcome virtually certain

• Conflicts of “perceived” values, rather than facts: “life not worth living”

Consensus statement of the Society of Critical Care Medicine’s Ethics Committee regarding futile and other possibly inadvisable treatments Crit Care Med 25;1997:887-891


Current estimates of survival and predicted sequelae after surgery for complex CHD Anomaly

Survival at adolescence

Risk of reops

Exercise ability

75%

3 planned + 15% unplan.

 in most 75% of NV

Heterotaxia

50-85%

3 planned + 15% unplan.

 in most 75% of NV

PA/IVS

60-75%

multiple procedures

 in most

PA/VSD/MA PCAs

50-75%

multiple procedures

 in most

TAPVC

90%

10-15%

DOWNCAVC

96%

4%

HLHS

Arrhythmia

 cognitive & neurodev.

SND, SVT

20-25%

rare, PLE, pl. bronchitis

20-25%

rare, PLE, pl. bronchitis, malrotation

VE, CHB

not described

sudden d, subAS,V dysf

VE, CHB

not described

PH, reactive airway dis.

rare

10-15%

PH, pulm. vein obstr.

CHB, atrial arrhythmias

∼ 100%

∼ normal life

SND, SVT, CHB

Other

Wernovsky G. The paradigm shift … Arch Pediatr Adolesc Med. 2008;162:849-854


CONTEXTUAL FEATURES JUSTICE (ETHICS) TELEOLOGICAL • Consequentialism an action is“good” or “bad” according to its outcome • Utilitarianism the greatest good for the greatest number • Social Darwinism – survival of the fittest

DEONTOLOGICAL • Duty-based – universal moral laws • Acts themselves are “good” or “bad” irrespectively of the consequences • It is our duty to take right action when we can identify it


Contextual Features • • • •

Terminal illness Dying role vs sick role Disposition: home, hospice, hospital Available resources – – – –

Emotional Physical Fiscal/economic (both at family and society level) Fairness and equality in distribution

• Who does what? • Is everyone comfortable with this plan?


GDP for healthcare by country in the Gulf area Country

GDP GDP/capite ($) % GDP for health

Qatar UAE Kuwait Bahrain Oman Saudi Arabia Yemen USA

1 9 12 36 37 44 154 8

98,900 47,700 41,700 27,700 27,600 24,400 2,300 48,300

1.80% 3.70% 2.60% 5.00% 2.80% 4.30% 5.20% 17.9%


The Right to Health • Not the right to be healthy • It is the right to have the highest attainable standard of health ↓ Actual or predicted disabilities do not justify considering any “life not worth living“


Conclusion: our task as physicians • “Un-ethicality” of the decision of therapy withdrawal by the physicians only (Ethics Cmte) – Doctors can’t (always) predict life expectancy

• … when we withhold or withdraw extraordinary treatment – We aim to dispense with the treatment • Because the treatment is useless or burdensome

– We do not aim to dispense with the patient’s life • Because we judge the life to be useless or burdensome


REBUTTAL Surgery SHOULD be offered to all forms of congenital cardiac anomalies in Gulf Region Roberto M. Di Donato Prince Sultan Cardiac Center, Riyadh


Survival and sequelae after intervention for various critical neonatal and childhood illnesses surv.

Sequelae following intervention

HLHS

80%

3(+)ops, cognitive/ND deficit 25%, HF, PLE, arrhythmias

Very LBW, prematurity

80%

Cognit./ND def. ≤50%, NS def., CLD, metabolic syndr.

Cng. diaphr. hernia

70%

Cognit./ND/NS def., CLD, PH, GE reflux,  exercise tol.

Leukemia/lymphoma

80%

Sec. malign., CMP, psychol adjust, sterility, short stat.

CNS malignancies

70%

Sec. malign., cognit./ND/NS def., seizures, NE disorders

Cystic fibrosis

>90% Growth failure,  exercise tol., sterility, psychol. prob.

Meningococcemia

90%

Necr. digits/extrem., growth deform, deafness, arthritis

Near drowning

85%

Chronic neuro-, psychologic, psychiatric abnormalities

Diabetes mellitus

>90% Retinopathy, CAD, neuropathies, eating disord, depress

Wernovsky G. The paradigm shift … Arch Pediatr Adolesc Med. 2008;162:849-854


Mideast healthcare investment to rise to USD 60 bn by 2025 ISLAMIC FINANCE GATEWAY COMMUNITY, Jan 11, 2012


Community presumption/perception regarding palliative care of HLHS There is an inverse relationship between the offered choice of palliative care and the overall prognosis after intervention.

The paradigm shift

Ross LF, Frader J. Hypoplastic left heart syndrome: a paradigm case for examining conscientious objection in pediatric practice. J Pediatr. 2009;155(1):12-15


Web-based poll of pediatric cardiologists Yates AR, Hoffman TM, Boettner B, Feltes TF, Cua CL. Initial counseling prior to palliation for hypoplastic left heart syndrome. Congenit Heart Dis. 2011 Jul-Aug;6(4):347-58


Number of fetal patients with hypoplastic left heart syndrome and their perinatal course Rychik J, Szwast A, Natarajan S, et al. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound Obstet Gynecol. 2010;36(4):465-70


Web-based poll of 52 pediatric cardiac surgical centers Wernovsky G, Ghanayem N, Ohye RG, Bacha EA, Jacobs JP, Gaynor JW, Tabbutt S. Hypoplastic left heart syndrome: consensus and controversies in 2007. Cardiol Young. 2007 Sep;17 Suppl 2:75-86.


Current approaches of society towards disability


CONCLUSION: Surgery Should be Offered to Certain Types of CHD in Gulf Region • Avoid making judgments on the basis of obsolete prognostic data • Avoid discriminating complex CHD vs equally dismal congenital or acquired conditions • Need of paradigm shift – at medical level (improve health care) – at society level (avoid discriminating disabilities)

• The doctor: when possible to cure, always to care, never to kill or let die (Hippocratic oath)


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