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Eisenmenger Syndrome Anita Saxena Department of Cardiology, All India Institute of Medical Sciences New Delhi, India 110029 Anita Saxena Department of.

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Presentation on theme: "Eisenmenger Syndrome Anita Saxena Department of Cardiology, All India Institute of Medical Sciences New Delhi, India 110029 Anita Saxena Department of."— Presentation transcript:

1 Eisenmenger Syndrome Anita Saxena Department of Cardiology, All India Institute of Medical Sciences New Delhi, India Anita Saxena Department of Cardiology, All India Institute of Medical Sciences New Delhi, India

2 Eisenmenger Syndrome 1887 : Victor Eisenmenger described history and postmortem details of 32 year old man with VSD and pathological features of PAH

3 Eisenmenger Syndrome 1958: Paul Wood’s Croonian Lectures coined the term “Eisenmenger Syndrome”

4 Eisenmenger Syndrome Definition: Pulmonary hypertension at or near systemic level with reversed or bidirectional shunt between the pulmonary and systemic circulation and pulmonary vascular resistance above 800dyn/cm -5 (10 Wood Units) Paul Wood, Br Med J, 1958 Definition: Pulmonary hypertension at or near systemic level with reversed or bidirectional shunt between the pulmonary and systemic circulation and pulmonary vascular resistance above 800dyn/cm -5 (10 Wood Units) Paul Wood, Br Med J, 1958

5 Eisenmenger Syndrome Underlying Basic Lesions Type of lesion Somerville ‘98 Daliento et al ‘98 (n=132) (n=188) Ventricular Septal Defect4571 Atrial Septal Defect621 Patent ductus arteriosus1236 Atrio ventricular septal defect1623 Truncus arteriosus1511 Single ventricle139 Transposition of great arteries58 Others209

6 Eisenmenger Syndrome – A progressive disease

7 Eisenmenger Syndrome Mechanism of abnormal pulm vascular response  Stimulation of insulin like growth factor  Impaired relaxation of pulmonary arterioles  Increased endothelin production  Elevated plasma thromboxane B 2 Exact mechanism not clear Mechanism of abnormal pulm vascular response  Stimulation of insulin like growth factor  Impaired relaxation of pulmonary arterioles  Increased endothelin production  Elevated plasma thromboxane B 2 Exact mechanism not clear

8 Pulmonary Arterial Hypertension Hyperkinetic Obstructive (Eisenmenger’s) Heart Size Large Normal Parasternal impulse Hyperkinetic Forcible Click Absent Present S 2 ASD wide & fixed wide & fixed VSD wide & variable Single PDA paradoxic split normal split Shunt murmur present short/absent Flow murmur Present Absent

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10 Question 1 At what age a large VSD Eisenmengerize? 1.< 6 months 2.2 years 3.10 years 4.20 years At what age a large VSD Eisenmengerize? 1.< 6 months 2.2 years 3.10 years 4.20 years

11 Eisenmenger Syndrome Clinical Groups Cyanosis since birth: TGA, Truncus, Univentricular hearts Failure to thrive in infancy – A settled phase – Symptomatic adolescent: Large VSD, PDA, AVSD Insidious presentation: AP Window Cyanosis since birth: TGA, Truncus, Univentricular hearts Failure to thrive in infancy – A settled phase – Symptomatic adolescent: Large VSD, PDA, AVSD Insidious presentation: AP Window

12 Eisenmenger Syndrome Clinical Evaluation History of symptoms of L R shunt in infancy Cyanosis, erythrocytosis, headache Mildly symptomatic with dyspnoea, fatigue History of syncope, hemoptysis,CVA Clinical Evaluation History of symptoms of L R shunt in infancy Cyanosis, erythrocytosis, headache Mildly symptomatic with dyspnoea, fatigue History of syncope, hemoptysis,CVA

13 ES - Underlying CHD Which one of the following clinical sign is unlikely in VSD ES (uncomplicated) 1.Single S2 2.Palpable second sound 3.Cardiomegaly 4.Absent parasternal heave Which one of the following clinical sign is unlikely in VSD ES (uncomplicated) 1.Single S2 2.Palpable second sound 3.Cardiomegaly 4.Absent parasternal heave

14 Eisenmenger’s Physiology: Clinical Assessment Cyanosis: generally mild Absence of cardiomegaly, heart failure Minimal left parasternal lift Constant ejection click of PAH Absence of significant shunt murmurs Pulmonary regurgitation murmur may be audible Cyanosis: generally mild Absence of cardiomegaly, heart failure Minimal left parasternal lift Constant ejection click of PAH Absence of significant shunt murmurs Pulmonary regurgitation murmur may be audible

15 ES: Underlying CHD CharacteristicVSDPDAASD Usual age of ES< 2 years 20 – 40 years Differential Cyanosis -Yes (50%)- Cardiomegaly--Yes Second H S (S 2 )SingleNarrow/normalWide & fixed Parasternal heave--Yes TR murmur--Yes PR murmur-Yes-

16 Noninvasive Assessment

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18 Eisenmenger Syndrome Noninvasive Evaluation Echocardiography is very useful Defines the large defect (PDA may be difficult) Estimates PA pressure by TR/PR jets Contrast echo demonstrates R L shunting TEE is safe and may be required in adults for precise delineation of the abnormality Echocardiography is very useful Defines the large defect (PDA may be difficult) Estimates PA pressure by TR/PR jets Contrast echo demonstrates R L shunting TEE is safe and may be required in adults for precise delineation of the abnormality

19 Eisenmenger Syndrome: Invasive Evaluation Cardiac cath can be safely performed It must be done in borderline cases to assess operability Response of pulmonary vasculature to pulmonary vasodilators like 0 2, tolazoline and nitric oxide should be assessed Limit the use of contrast agent to minimal Cardiac cath can be safely performed It must be done in borderline cases to assess operability Response of pulmonary vasculature to pulmonary vasodilators like 0 2, tolazoline and nitric oxide should be assessed Limit the use of contrast agent to minimal

20 Eisenmenger Syndrome: Natural history Identify the false statement 1. Prognosis of ES is good 2. Survival better than IPAH 3. With recent advances, pregnancy better tolerated 4. Heart failure most common cause of death Identify the false statement 1. Prognosis of ES is good 2. Survival better than IPAH 3. With recent advances, pregnancy better tolerated 4. Heart failure most common cause of death

21 Eisenmenger Syndrome Natural History Life expectancy reduced by about 20 years Survival Pattern: At one year97% At 5 years87% At 10 years80% At 15 years77% At 25 years 42% Natural History Life expectancy reduced by about 20 years Survival Pattern: At one year97% At 5 years87% At 10 years80% At 15 years77% At 25 years 42%

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23 Life expectancy reduced by about 20 years Unwarranted surgical closure hastens death Policy of “non-intervention”, unless absolutely necessary Avoid destabilizing the “balanced physiology”

24 ES – Survival better than IPAH

25 Landzberg, M. J. et al. J Am Coll Cardiol 2006;47:D33-D36 Impact of left ventricular dysfunction on survival in Eisenmenger syndrome

26 Dimopoulos, K. et al. Circulation 2010;121:20-25 Cumulative mortality rate curve (with 95% CIs) Overall population (n=229) According to functional class

27 Long Term Survival in Eisenmenger physiology Diller G et al. Eur Heart J 2006;27:

28 Eisenmenger Syndrome Predictors of Poor outcome History of syncope Elevated right heart filling pressure Severe hypoxemia (Sa0 2 <85%) Lange RA et al, 1998 Predictors of Poor outcome History of syncope Elevated right heart filling pressure Severe hypoxemia (Sa0 2 <85%) Lange RA et al, 1998

29 Eisenmenger’s Syndrome Is Preventable

30 Eisenmenger Syndrome Management Strategies Drug treatment Phlebotomy Transplantation : Heart lung / lung Counsel against special risks Pregnancy Hormone contraceptives Noncardiac surgery High altitude/flying Sudden emotional upset Management Strategies Drug treatment Phlebotomy Transplantation : Heart lung / lung Counsel against special risks Pregnancy Hormone contraceptives Noncardiac surgery High altitude/flying Sudden emotional upset

31 Conventional Therapy Digitalis, diuretics Anti-arrhythmic drugs Anticoagulants Long term oxygen therapy Avoidance of dehydration, high altitude, infections and IV lines Avoidance of pregnancy Digitalis, diuretics Anti-arrhythmic drugs Anticoagulants Long term oxygen therapy Avoidance of dehydration, high altitude, infections and IV lines Avoidance of pregnancy

32 Targeted Therapy: Pulmonary Vasodilators Prostanoids: Epoprostenol infusion Phosphodiesterase-5 inhibitors: Sildenafil, tadalafil Endothelin receptor antagonists: Bosentan (BREATH-5 trial) Prostanoids: Epoprostenol infusion Phosphodiesterase-5 inhibitors: Sildenafil, tadalafil Endothelin receptor antagonists: Bosentan (BREATH-5 trial) 1.Fernandes SM, et al Chou EM, et al Mukhopadhyay S, et al Galie N, et al Gatzoulis MA, et al Fernandes SM, et al Chou EM, et al Mukhopadhyay S, et al Galie N, et al Gatzoulis MA, et al 2008

33 Dimopoulos, K. et al. Circulation 2010;121:20-25 Survival in Eisenmenger Syndrome Patients on Advanced Therapy (n=287)

34 Bosentan in ES

35 Bosentan in ES: BREATH 5 Gatzoulis MA, Int J Cardio 2008

36 Eisenmenger Syndrome: Role of Phlebotomy Indication for Isovolumic Phlebotomy  Symptomatic hyper viscosity (PCV >0.65, Hb>20gm%) Important issues to remember  Symptoms of hyper viscosity resemble those of iron deficiency  Phlebotomy may result in iron deficiency anemia and cerebrovascular accidents Discourage routine phlebotomy

37 Management of Eisenmenger Syndrome Transplantation 1982 : Combined heart-lung transplantation introduced by Reitz et al 1990 : Single lung transplantation with repair of cardiac defect successfully performed by Fremes et al Lung transplant has advantages of better donor availability Avoidance of cardiac allograft rejection Absence of coronary vasculopathy Transplantation 1982 : Combined heart-lung transplantation introduced by Reitz et al 1990 : Single lung transplantation with repair of cardiac defect successfully performed by Fremes et al Lung transplant has advantages of better donor availability Avoidance of cardiac allograft rejection Absence of coronary vasculopathy

38 Management of Eisenmenger Syndrome Lung Transplantation Actuarial survival rates : At 1 year 70-80%, At 4 years <50%, At 10 years<30% Indications for transplant History of syncope Refractory right heart failure Poor exercise tolerance Severe hypoxemia Lung Transplantation Actuarial survival rates : At 1 year 70-80%, At 4 years <50%, At 10 years<30% Indications for transplant History of syncope Refractory right heart failure Poor exercise tolerance Severe hypoxemia

39 Perioperative Risk for Noncardiac Surgery High risk conditions  Pulm hypertension  Cyanotic CHD  NYHA class III or IV  Severe ventricular dysfuntion (EF<35%)  Severe left heart obstructive obstruction Moderate risk conditions  Intracardiac shunt lesions High risk conditions  Pulm hypertension  Cyanotic CHD  NYHA class III or IV  Severe ventricular dysfuntion (EF<35%)  Severe left heart obstructive obstruction Moderate risk conditions  Intracardiac shunt lesions ACC/AHA guidelines 2008

40 Associated with a mortality rate of 14% -19% Local anesthesia is preferred to general anesthesia Prolonged fasting and volume depletion should be avoided Small air bubbles in IV lines should be removed Early ambulation is encouraged Antibodies given to prevent infective endocarditis Associated with a mortality rate of 14% -19% Local anesthesia is preferred to general anesthesia Prolonged fasting and volume depletion should be avoided Small air bubbles in IV lines should be removed Early ambulation is encouraged Antibodies given to prevent infective endocarditis Perioperative Risk for Noncardiac Surgery in Eisenmenger Syndrome

41 Risk to Fetus: if Sao 2 < 85%, chances of live fetus only 12% Caesarian section only for obstetric reasons Pregnancy and congenital heart disease

42 Complications During Pregnancy in Women with CHD Drenthen W, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007;49:

43 Complications During Pregnancy in Women with CHD

44 Management of Eisenmenger Syndrome Avoidance of Pregnancy Pregnancy is absolutely contraindicated Maternal mortality is 36%-45% Mortality often occurs in post-partum period Fetal loss occurs in over 60% Termination is indicated in early gestation Outcome of pregnant women with Eisenmenger syndrome has not changed in last three decades Avoidance of Pregnancy Pregnancy is absolutely contraindicated Maternal mortality is 36%-45% Mortality often occurs in post-partum period Fetal loss occurs in over 60% Termination is indicated in early gestation Outcome of pregnant women with Eisenmenger syndrome has not changed in last three decades

45 Eisenmenger Syndrome Management of Pregnancy Prolonged bed rest after 20th wks gestation Oxygen therapy Digoxin and diuretics if CHF present Prolonged use of anticoagulants - Heparin Careful monitoring of volume status, oxygen saturation and hematocrit is necessary Management of Pregnancy Prolonged bed rest after 20th wks gestation Oxygen therapy Digoxin and diuretics if CHF present Prolonged use of anticoagulants - Heparin Careful monitoring of volume status, oxygen saturation and hematocrit is necessary

46 Eisenmenger Syndrome Basic Events Leading to Death Right ventricular failure 30% Sudden death?vent arrhythmia 25% Cardiovascular surgery 12% Cerebrovascular accidents/abscess 10% Hemoptysis 9-15% Noncardiac surgery 6% Pregnancy related 5% Heart lung/lung transplants 4% Basic Events Leading to Death Right ventricular failure 30% Sudden death?vent arrhythmia 25% Cardiovascular surgery 12% Cerebrovascular accidents/abscess 10% Hemoptysis 9-15% Noncardiac surgery 6% Pregnancy related 5% Heart lung/lung transplants 4%

47 Is generally established by 2-4 yrs of age Accelerated onset in Down’s, Cyanotic CHD Median survival is yrs of age Anesthesia, surgery, dehydration poorly tolerated Pregnancy carries 30-50% maternal mortality Closure of the defect is detrimental once obstructive PAH has developed Is generally established by 2-4 yrs of age Accelerated onset in Down’s, Cyanotic CHD Median survival is yrs of age Anesthesia, surgery, dehydration poorly tolerated Pregnancy carries 30-50% maternal mortality Closure of the defect is detrimental once obstructive PAH has developed Eisenmenger Syndrome Carefully managed, most patients lead useful lives

48 Eisenmenger Syndrome Conclusion Patients with Eisenmenger syndrome can live upto fifth and sixth decades with informed medical care, patient education and protection from special risks 20% of death are related to avoidable errors “Doing nothing may be a positive action for good in such patients” Jane Somerville, 1998 Conclusion Patients with Eisenmenger syndrome can live upto fifth and sixth decades with informed medical care, patient education and protection from special risks 20% of death are related to avoidable errors “Doing nothing may be a positive action for good in such patients” Jane Somerville, 1998

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