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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.

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TO THE EISENMENGER COMPLEX PATIENT: HOW DO WE OPTIMIZE CARE

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

  2. 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

  3. 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

  4. 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

  5. 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

  6. “LIGHT IN THE TUNNEL: OPTIMIZING CARE OF THE EISENMENGER PATIENT”

  7. 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

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

  9. 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

  10. 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

  11. 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

  12. 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

  13. STANDARD/CONVENTIONAL THERAPY • DIGOXIN – supportive treatment • DIURETICS- supportive treatment • ANTIARRHYTHYMICS- when appropriate • ANTICOAGULANTS- controversial • O2 THERAPY- controversial • IRON SUPPLEMENTATION- general measure

  14. 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

  15. 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

  16. 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

  17. 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

  18. 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

  19. 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

  20. ADVANCED/NEWER THERAPY Beghetti M and Galie N. J Am CollCardiol 2009;53:733-740

  21. 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

  22. 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)

  23. 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

  24. 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

  25. OPTIMIZING CARE IN ES OTHER GENERAL MEASURES AND SUPPORTIVE TREATMENT

  26. 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

  27. HYPERVISCOSITY SYMPTOMS

  28. 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

  29. 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

  30. 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

  31. 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%

  32. Transplantation • Heart/Lung Transplantation or Lung Transplantation with repair of CHD • Option for patients with poor prognosis and poor quality of life

  33. 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

  34. 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

  35. “Teach us to number our days aright, that we may gain a heart of wisdom” Psalm 90:12

  36. 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

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