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TO THE EISENMENGER COMPLEX PATIENT: HOW DO WE OPTIMIZE CARE. Maria Concepcion C. Sison , MD, FPPS, FPCC Pediatric Cardiologist. Eisenmenger Complex.

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to the eisenmenger complex patient how do we optimize care

TO THE EISENMENGER COMPLEX PATIENT: HOW DO WE OPTIMIZE CARE

Maria Concepcion C. Sison, MD, FPPS, FPCC

Pediatric Cardiologist

eisenmenger complex
Eisenmenger Complex
  • Victor Eisenmenger (1897): 32 yo/male with cyanosis and dyspnea since infancy, was active until 3 years before death; succumbed to hemoptysis
    • Autopsy: large malaligned VSD, marked RVH
  • Paul Wood (1951): described pathophysiology of Eisenmenger syndrome as PULMONARY HYPERTENSION with REVERSED SHUNT
eisenmenger complex syndrome physiology definition
EISENMENGER Complex/Syndrome/Physiology: DEFINITION
  • Pulmonary vascular obstructive disease induced by uncorrected significant left-to-right shunt (any large congenital cardiac defect) causing a balanced or predominantly right to left shunt
eisenmenger complex syndrome definition
Eisenmenger Complex/Syndrome: DEFINITION
  • Hemodynamically:
    • Elevation of PVR to 12 (10) Wood units
    • Pulmonary-to-systemic resistance ratio ≥ 1.0
    • No significant respone so vasoreactivity testing

EISENMENGER SYNDROME

=INOPERABILITY

=PROGRESSIVE HEART FAILURE

= INEVITABLE PREMATURE DEATH

eisenmenger complex syndrome prognosis
Eisenmenger Complex/Syndrome: PROGNOSIS
  • LONG SYMPTOM FREE PERIOD
  • USUALLY SYMPTOMATIC AROUND 30 years old
  • USUALLY DIE BETWEEN 30-35 years old
  • Actuarial survival rate:
    • 80% at 10 years
    • 77% at 15 years
    • 42% at 25 years

Kaemmerer H et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part I: Epidemiology, Clinical Aspects and Diagnostic Options. Current Cardiology Review 2010;6:343-355

outline
OUTLINE
  • Definition of Eisenmenger Complex (EC)
  • Pathophysiology relevant to management
  • Problems and complications of EC
  • Therapeutic Objectives
  • Choose Optimal Therapy- efficacy, safety
    • “Standard or Conventional” Therapy
    • “Advanced or New” Therapy
  • Other Issues/General Measures/Supportive Treatment
pathophysiology
PATHOPHYSIOLOGY

Beghetti M and Galie N. J Am CollCardiol 2009;53:733-740

pathophysiology1
PATHOPHYSIOLOGY
  • VASOACTIVE MEDIATORS PAH-CHD
  • Endothelin-1 and endothelin receptors A and B
  • Angiotensin II and angiotensin receptors
  • Vascular endothelial growth factor and the flk1/tdr receptor
  • SIGNALING PATHWAYS PAH-CHD
  • Calcium-dependent K+ channels
  • Increased phosphodiesterase 5 activity
  • Decreased nitric oxide synthase activity
  • Angiopoietin 1
  • Tenascin
  • Diminished function of BMPR1A, BMPR2

Landberg MJ. ClinChes Med 2007;28:243-253

problems and complications
PROBLEMS AND COMPLICATIONS
  • Dyspnea on exertion, easy fatigability, shortness of breath, tiredness
  • Edema and fluid retention
  • Palpitations/Cardiac arrhythmia
  • Syncopal episodes
  • Erythrocytosis – increased blood viscosity and intravascular “sludging”
    • CVA, Renal insufficiency, pulmonary thromboembolism
problems and complications multisystem disorder
PROBLEMS AND COMPLICATIONS: MULTISYSTEM DISORDER
  • Fluid retention and elevated systemic venous pressure may alter hepatic function
  • Hyperuricemia and gout
  • Bleeding tendencies/Coagulation disorders
    • hemoptysis
  • Sudden death
therapeutic objectives
THERAPEUTIC OBJECTIVES:
  • TO IMPROVE QUALITY OF LIFE
  • TO IMPROVE, IF NOT RELIEVE, SYMPTOMS
  • TO DECREASE, IF NOT PREVENT, MORBIDITY/COMPLICATIONS
  • TO OPTIMIZE FUNCTIONAL/ EXERCISE CAPACITY
  • TO IMPROVE HEMODYNAMICS (decrease PAP, increase oxygenation)
  • TO DELAY DETERIORATION, AND PROLONG SURVIVAL, IF POSSIBLE
standard conventional therapy
STANDARD/CONVENTIONAL THERAPY
  • DIGOXIN – supportive treatment
  • DIURETICS- supportive treatment
  • ANTIARRHYTHYMICS- when appropriate
  • ANTICOAGULANTS- controversial
  • O2 THERAPY- controversial
  • IRON SUPPLEMENTATION- general measure
anticoagulation
ANTICOAGULATION

EFFICACY:

  • Prevalence of pulmonary artery thrombosis in ES ~ 20%
  • Shown to reduce morbidity and mortality in patients with IPAH

SAFETY:

  • Thrombus formation and bleeding coexist in patients with ES.
  • Risk of Fatal and life threatening and bleeding complication particularly significant hemoptysis

Oechslin E et al. Current Cardiology Review 2010;6:363-372

Beghetti M and Galie N. J Am CollCardiol2009;53:733-740

anticoagulation1
ANTICOAGULATION
  • May be CONSIDERED as supportive treatment in patients with PA THROMBOSIS in the ABSENCE of significant hemoptysis

Oechslin E et al. Current Cardiology Review 2010;6:363-372

oxygen therapy
OXYGEN THERAPY

EFFICACY:

  • In PAH: extrapolated from RCTs in COPD patients
  • Subjective benefit in patients with intense hypoxemia, dyspnea at rest and loss of vital capacity

RISK and SIDE EFFECTS:

  • desiccation of nasal mucosa, epistaxis, sleep disturbance
  • No impact of nocturnal oxygen therapy on exercise capacity, natural history and survival of the patients within a follow up period of 2 years.
  • Can be considered in cases in which it produces a consistent increase in O2 saturation and reduces symptoms

Oechslin E et al. Current Cardiology Review 2010;6:363-372

iron supplementation
IRON SUPPLEMENTATION

BASIS:

    • Erythrocytosis
    • Hyperviscosity syndrome occurs at lower Hb level in the presence of iron deficiency anemia
    • Iron deficiency may cause headache, reduced exercise tolearnce, restless leg syndrome

CONTROVERSY:

    • No studieson the role of iron store repletion in lowering the occurrence of other organ system damage or thrombosis
    • In vitro study: iron deficiency has no impact on blood viscosity
  • Iron deficiency must be avoided in ES!

Oechslin E et al. Current Cardiology Review 2010;6:363-372

conventional pharmacologic treatment
Conventional Pharmacologic Treatment
  • Conventional pharmacological treatment, including digitalis, diuretics, antiarrhythmics, anticoagulants, iron supplementation, and oxygen therapy, may be used empirically, BUT does not seem to alter survival rate
newer advanced targeted therapies
NEWER/ADVANCED/ TARGETED THERAPIES
  • For stable patients: “noli-me-tangere” is still an option due to delicate balance of many variables
  • INDICATED IN PATIENTS WITH REDUCED EXERCISE TOLERANCE, INCREASING CYANOSIS, OR INCREASING SIGNS OF HEART FAILURE
    • WHO FC III-IV

Siegrun M et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part II: Medical Treatment-Study Results. Current Cardiology Review 2010;6:356-362

advanced newer therapy
ADVANCED/NEWER THERAPY

Beghetti M and Galie N. J Am CollCardiol 2009;53:733-740

advanced newer therapy pulmonary vasodilators
ADVANCED/NEWER THERAPY: PULMONARY VASODILATORS
  • ENDOTHELIN-1 RECEPTOR ANTAGONISTS (BOSENTAN)
  • PHOSPHODIESTERASE-5 INHIBITORS (SILDENAFIL)-
  • PROSTACYCLIN and PROSTACYCLIN ANALOGS (EPOPROSTENOL)
  • TO SOME EXTENT, DEMONSTRATED IMPROVEMENT IN EXERCISE CAPACITY, QUALITY OF LIFE, AND HEMODYNAMICS

Siegrun M et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part II: Medical Treatment-Study Results. Current Cardiology Review 2010;6:356-362

et 1 antagonist bosentan
ET-1 ANTAGONIST: BOSENTAN

BOSENTAN: BREATHE-5

    • First RCT Eisenmenger patients, 16 weeks
    • Significant improvement in hemodynamics and exercise capacity (6 MWD) without compromising oxygen saturations
  • Approved for use in PAH both in adults in children
  • Maintained up to 40 wks (open-label)
    • Initial persistent improvement, decline after 1 year, reduction to baseline after 2 years (natural progression vstachyphylaxis)
pde 5 inhibitors sildenafil
PDE-5 Inhibitors- Sildenafil
  • SUPER-1: large prospective multicenter blinded and controlled:IPAH: improved EC (6 MW test), FC, HD
  • In ES: case reports, series, observational studies, few RCT placebo: Safe and improved symptoms, FC, Exercise Capacity (6MWD, Ex duration, pulmonary HD)

Tadalafil- observational study (ES)- benefits in O2 sat and mean FC

prostacyclin analog epoprostenol
PROSTACYCLIN ANALOG:EPOPROSTENOL
  • LIMITED DATA ON EFFICACY AND SAFETY IN ES
      • Case series: improved O2 and 6 MWD, FC
  • IPAH
    • RCT: improved exercise capacity, QOL, hemodynamics
  • Side effects IV Administration: CVA, infection
  • TREPROSTNIL (SC, IV)- IPAH, CTD, CHD
    • benefits on EC, HD, clinical events
    • Side effects: high frequency of injection site pain
  • Iloprost (inhalation)- IPAH
  • Beraprost- no crucial role
optimizing care in es
OPTIMIZING CARE IN ES

OTHER GENERAL MEASURES AND SUPPORTIVE TREATMENT

phlebotomy
PHLEBOTOMY
  • Phlebotomy with isovolumic replacement should be considered in the presence of moderate to severe symptoms of hyperviscosity
  • Prophylactic phlebotomy plays no role in patient management
    • Causes iron deficiency anemia, reduces exercise tolerance
hyperuricemia gout
HYPERURICEMIA/GOUT
  • Asymptomatic, secondary hyperuricemia is no indication for routine therapy to lower uric acid level because it does not have any serious impact on renal function

TREAT: Acute Gouty Arthritis

ischemic events reducing risks
ISCHEMIC EVENTS: REDUCING RISKS
  • Avoidance and treatment of volume depletion;
  • Iron supplementation in patients with iron deficiency or those undergoing repeated phlebotomies;
  • Use of air filters in all intravenous lines.

Oechslin E et al. Current Cardiology Review 2010;6:363-372

factors that may aggravate pah in eisenmenger syndrome
FACTORS THAT MAY AGGRAVATE PAH IN EISENMENGER SYNDROME
  • PREGNANCY
  • Dehydration or acute vasodilation (eg, sauna, hot tub)
  • Increased fluid volume
  • Worsened renal or hepatic function
  • Chronic environmental hypoxia
  • Increased left-sided filling pressure
  • Left ventricular diastolic dysfunction
  • Obstructive congenital lesion
  • Myocardial restriction
  • Systemic hypertension withincreased left ventricular afterload
  • Erythrocytosis and increased bloodviscosity; anemia
  • Hypercoagulability: thrombosis
other general measures
OTHER GENERAL MEASURES
  • Infective Endocarditis PROPHYLAXIS
  • Pregnancy and Contraception
    • ES is an absolute contraindication to Pregnancy
    • Maternal mortality= 30-60%
    • Spontaneous abortion=40%
    • Premature delivery 50%
    • IUGR 30% of infants
    • Perinatal infant mortality 8-28%
transplantation
Transplantation
  • Heart/Lung Transplantation or Lung Transplantation with repair of CHD
  • Option for patients with poor prognosis and poor quality of life
survival benefits
SURVIVAL BENEFITS
  • RETROSPECTIVE STUDY (systematic cohort), EISENMENGER PATIENTS RECEIVING ADVANCED THERAPY (Bosentan, Sildenafil, Epoprostenol) showed LOWER RISK OF DEATH
  • 52 PATIENTS DIED, ONLY 2 WHILE ON AT
  • CLINICAL DIFFERENCES STATISICALLY CORRECTED
    • Those on AT had more advanced disease

Dimopoulos K et al. Improved Survival Among Patients With Eisenmenger Syndrome Receiving Advanced Therapy For Pulmonary Arterial Hypertension. Circulation. 2010;121:20-25

management algorithm for pah in chd
Management algorithm for PAH in CHD

Kaemmerer H et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part I: Epidemiology, Clinical Aspects and Diagnostic Options. Current Cardiology Review 2010;6:343-355

major references
MAJOR REFERENCES:
  • Kaemmerer H et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part I: Epidemiology, Clinical Aspects and Diagnostic Options. Current Cardiology Review 2010;6:343-355
  • Siegrun M et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part II: Medical Treatment-Study Results. Current Cardiology Review 2010;6:356-362
  • Oechslin E et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part III. Specific Management and Surgical Aspects. Current Cardiology Review 2010;6:363-372
  • Dimopoulos K et al. Improved Survival Among Patients With EisenmengerSyndrome Receiving Advanced Therapy For Pulmonary Arterial Hypertension. Circulation. 2010;121:20-25
  • Landberg MJ. Congenital Heart Disease Associated Pulmonary Arterial Hypertension. ClinChes Med 2007;28:243-253
  • Beghetti M and Galie N. Eisenmenger Syndrome: A Clinical Perspective in a New Therapeutic Era of Pulmonary Arterial Hypertension. J Am CollCardiol 2009;53:733-740