Eisenmenger’s syndrome medical therapy: Difference between revisions

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If surgical intervention is not available, treatment is mostly [[palliative]], using anticoagulants, pulmonary vasodilators such as [[bosentan]], antibiotic [[prophylaxis]] to prevent endocarditis, [[bloodletting|phlebotomy]] to treat polycythemia, and maintaining proper fluid balance.  These measures can prolong lifespan and improve quality of life.
If surgical intervention is not available, treatment is mostly [[palliative]], using anticoagulants, pulmonary vasodilators such as [[bosentan]], antibiotic [[prophylaxis]] to prevent endocarditis, [[bloodletting|phlebotomy]] to treat polycythemia, and maintaining proper fluid balance.  These measures can prolong lifespan and improve quality of life.
===ACC / AHA Guidelines- Recommendations for Medical Therapy of Eisenmenger Physiology (DO NOT EDIT)===
===ACC / AHA Guidelines- Recommendations for Medical Therapy of Eisenmenger Physiology (DO NOT EDIT)===
{{cquote|


===[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]===
{| class="wikitable"
1. It is recommended that patients with Eisenmenger
|-
syndrome avoid the following activities or exposures,
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
which carry increased risks:
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' It is recommended that patients with Eisenmenger syndrome avoid the following activities or exposures, which carry increased risks:


a. Pregnancy. (Level of Evidence: B)
a. Pregnancy. '([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>


b. Dehydration. (Level of Evidence: C)
b. Dehydration. '([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>


c. Moderate and severe strenuous exercise, particularly
c. Moderate and severe strenuous exercise, particularly isometric exercise. '([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
isometric exercise. (Level of Evidence: C)


d. Acute exposure to excessive heat (eg, hot tub or
d. Acute exposure to excessive heat (eg, hot tub or sauna). ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
sauna). (Level of Evidence: C)


e. Chronic high-altitude exposure, because this causes
e. Chronic high-altitude exposure, because this causes further reduction in oxygen saturation and increased risk of altitude-related cardiopulmonary complications (particularly at an elevation greater than 5000 feet above sea level). ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
further reduction in oxygen saturation and increased
risk of altitude-related cardiopulmonary
complications (particularly at an elevation greater
than 5000 feet above sea level). (Level of Evidence:
C)


f. Iron deficiency. (Level of Evidence: B)
f. Iron deficiency. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>'


2. Patients with Eisenmenger syndrome should seek
|-
prompt therapy for arrhythmias and infections. (Level
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.''' Patients with Eisenmenger syndrome should seek prompt therapy for arrhythmias and infections. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
of Evidence: C)
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''3.''' Patients with Eisenmenger syndrome should have hemoglobin, platelet count, iron stores, creatinine, and uric acid assessed at least yearly. '([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>


3. Patients with Eisenmenger syndrome should have hemoglobin,
|-
platelet count, iron stores, creatinine, and
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''4.''' Patients with Eisenmenger syndrome should have assessment of digital oximetry, both with and without supplemental oxygen therapy, at least yearly. The presence of oxygen-responsive hypoxemia should be investigated further. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
uric acid assessed at least yearly. (Level of Evidence: C)


4. Patients with Eisenmenger syndrome should have assessment
|-
of digital oximetry, both with and without
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''5.'''. Exclusion of air bubbles in intravenous tubing is recommended as essential during treatment of adults with Eisenmenger syndrome. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
supplemental oxygen therapy, at least yearly. The
presence of oxygen-responsive hypoxemia should be
investigated further. (Level of Evidence: C)


5. Exclusion of air bubbles in intravenous tubing is
|-
recommended as essential during treatment of adults
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''6.''' Patients with Eisenmenger syndrome should undergo noncardiac surgery and cardiac catheterization only in centers with expertise in the care of such patients. In emergent or urgent situations in which transportation is not feasible, consultation with designated caregivers in centers with expertise in the care of patients with Eisenmenger syndrome should be performed and sustained throughout care. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
with Eisenmenger syndrome. (Level of Evidence: C)


6. Patients with Eisenmenger syndrome should undergo
|}
noncardiac surgery and cardiac catheterization only in
centers with expertise in the care of such patients. In
emergent or urgent situations in which transportation
is not feasible, consultation with designated caregivers
in centers with expertise in the care of patients with
Eisenmenger syndrome should be performed and sustained
throughout care. (Level of Evidence: C)


===[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]===
{| class="wikitable"
1. All medications given to patients with Eisenmenger
|-
physiology should undergo rigorous review for the
| colspan="1" style="text-align:center; background:LemonChiffon"| [[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
potential to change systemic blood pressure, loading
|-
conditions, intravascular shunting, and renal or hepatic
| bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' All medications given to patients with Eisenmenger physiology should undergo rigorous review for the potential to change systemic blood pressure, loading conditions, intravascular shunting, and renal or hepatic flow or function. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
flow or function. (Level of Evidence: C)


2. Pulmonary vasodilator therapy can be beneficial for
|-
patients with Eisenmenger physiology because of the
| bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Pulmonary vasodilator therapy can be beneficial for patients with Eisenmenger physiology because of the potential for improved quality of life. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
potential for improved quality of life. (Level of Evidence:
|}
C)}}


===ACC / AHA Guidelines- Recommendations for Follow-Up (DO NOT EDIT)===
===ACC / AHA Guidelines- Recommendations for Follow-Up (DO NOT EDIT)===
{{cquote|


===[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]===
{| class="wikitable"
1. Patients with CHD-related PAH should:
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.'''  Patients with CHD-related PAH should:


a. Have coordinated care under the supervision of a
a. Have coordinated care under the supervision of a trained CHD and PAH provider and be seen by such individuals at least yearly. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
trained CHD and PAH provider and be seen by such
individuals at least yearly. (Level of Evidence: C)


b. Have yearly comprehensive evaluation of functional
b. Have yearly comprehensive evaluation of functional capacity and assessment of secondary complications.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
capacity and assessment of secondary complications.
(Level of Evidence: C)


c. Discuss all medication changes or planned interventions
c. Discuss all medication changes or planned interventions with their CHD-related PAH caregiver. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
with their CHD-related PAH caregiver. (Level
|}
of Evidence: C)


===[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]===
{| class="wikitable"
1. Endocardial pacing is not recommended in patients
|-
with CHD-PAH with persistent intravascular shunting,
| colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class III]]
and alternative access for pacing leads should be sought (the risks should be individualized).136 (Level of
 
Evidence: B)}}
|-
| bgcolor="LightCoral"|<nowiki>"</nowiki>'''1.''' Endocardial pacing is not recommended in patients with CHD-PAH with persistent intravascular shunting, and alternative access for pacing leads should be sought (the risks should be individualized).136 ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>
|}


==References==
==References==

Revision as of 14:30, 9 October 2012

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Priyamavada Singh, MMBS [[3]]Assistant Editor-In-Chief: Kristin Feeney, B.S. [[4]]

Overview

Medical Therapy

If surgical intervention is not available, treatment is mostly palliative, using anticoagulants, pulmonary vasodilators such as bosentan, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance. These measures can prolong lifespan and improve quality of life.

ACC / AHA Guidelines- Recommendations for Medical Therapy of Eisenmenger Physiology (DO NOT EDIT)

Class I
"1. It is recommended that patients with Eisenmenger syndrome avoid the following activities or exposures, which carry increased risks:

a. Pregnancy. '(Level of Evidence: B) "

b. Dehydration. '(Level of Evidence: C) "

c. Moderate and severe strenuous exercise, particularly isometric exercise. '(Level of Evidence: C) "

d. Acute exposure to excessive heat (eg, hot tub or sauna). (Level of Evidence: C) "

e. Chronic high-altitude exposure, because this causes further reduction in oxygen saturation and increased risk of altitude-related cardiopulmonary complications (particularly at an elevation greater than 5000 feet above sea level). (Level of Evidence: C) "

f. Iron deficiency. (Level of Evidence: B) "'

"2. Patients with Eisenmenger syndrome should seek prompt therapy for arrhythmias and infections. (Level of Evidence: C) "
"3. Patients with Eisenmenger syndrome should have hemoglobin, platelet count, iron stores, creatinine, and uric acid assessed at least yearly. '(Level of Evidence: C) "
"4. Patients with Eisenmenger syndrome should have assessment of digital oximetry, both with and without supplemental oxygen therapy, at least yearly. The presence of oxygen-responsive hypoxemia should be investigated further. (Level of Evidence: C) "
"5.. Exclusion of air bubbles in intravenous tubing is recommended as essential during treatment of adults with Eisenmenger syndrome. (Level of Evidence: C) "
"6. Patients with Eisenmenger syndrome should undergo noncardiac surgery and cardiac catheterization only in centers with expertise in the care of such patients. In emergent or urgent situations in which transportation is not feasible, consultation with designated caregivers in centers with expertise in the care of patients with Eisenmenger syndrome should be performed and sustained throughout care. (Level of Evidence: C) "
Class IIa
"1. All medications given to patients with Eisenmenger physiology should undergo rigorous review for the potential to change systemic blood pressure, loading conditions, intravascular shunting, and renal or hepatic flow or function. (Level of Evidence: C) "
"2. Pulmonary vasodilator therapy can be beneficial for patients with Eisenmenger physiology because of the potential for improved quality of life. (Level of Evidence: C) "

ACC / AHA Guidelines- Recommendations for Follow-Up (DO NOT EDIT)

Class I
"1. Patients with CHD-related PAH should:

a. Have coordinated care under the supervision of a trained CHD and PAH provider and be seen by such individuals at least yearly. (Level of Evidence: C) "

b. Have yearly comprehensive evaluation of functional capacity and assessment of secondary complications.(Level of Evidence: C) "

c. Discuss all medication changes or planned interventions with their CHD-related PAH caregiver. (Level of Evidence: C) "

Class III
"1. Endocardial pacing is not recommended in patients with CHD-PAH with persistent intravascular shunting, and alternative access for pacing leads should be sought (the risks should be individualized).136 (Level of Evidence: B) "

References

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