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Eisenmenger syndrome
- 1. Eisenmenger Syndrome Ms. Feba Geevarghese Vice Principal & Ph.D. Scholar College of Nursing, St. Stephen’s Hospital Delhi
- 2. GENERAL OBJECTIVE At the end of the teaching, students will acquire knowledge regarding Eisenmenger Syndrome, its pathophysiology, clinical manifestations, medical management and demonstrate skills in nursing management of the patient.
- 3. SPECIFIC OBJECTIVES At the end of the class, the students will be able to: define Eisenmenger Syndrome describe anatomy and physiology of the heart identify the cause of Eisenmenger Syndrome outline the pathophysiology of Eisenmenger Syndrome enumerate the clinical manifestations of Eisenmenger Syndrome identify the diagnostic evaluation of Eisenmenger Syndrome discuss the medical management of Eisenmenger Syndrome explain the nursing management of Eisenmenger Syndrome using nursing process approach describe the points for patient education
- 4. Definition Eisenmenger syndrome was so named by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described the condition in 1897. Eisenmenger's syndrome (or ES, Eisenmenger's reaction, Eisenmenger physiology, or tardive cyanosis) is defined as the process in which a long- standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right- to- left shunt.
- 5. Anatomy and Physiology of the Heart
- 6. Anatomy and Physiology of the Heart..
- 7. Anatomy and Physiology of the Heart.. The heart is a muscular organ which pumps blood through the blood vessels of the circulatory system. The heart is divided into four chambers: upper left and right atria; and lower left and right ventricles. Commonly the right atrium and ventricle are referred together as the right heart and their left counterparts as the left heart. In a healthy heart blood flows one way through the heart due to heart valves, which prevent backflow. The heart is enclosed in a protective sac, the pericardium, which also contains a small amount of fluid. The wall of the heart is made up of three layers: epicardium, myocardium, and endocardium.
- 8. Anatomy and Physiology of the Heart.. The heart pumps blood with a rhythm determined by a group of pacemaking cells in the sinoatrial node. These generate a current that causes contraction of the heart, traveling through the atrioventricvular node and along the conduction system of the heart. The heart receives blood low in oxygen from the systemic circulation, which enters the right atrium from the superior and inferior venacavae and passes to the right ventricle. From here it is pumped into the pulmonary circulation, through the lungs where it receives oxygen and gives off carbon dioxide. Oxygenated blood then returns to the left atrium, passes through the left ventricle and is pumped out through the aorta to the systemic circulation−where the oxygen is used and metabolized to carbon dioxide. The heart beats at a resting rate close to 72 beats per minute.
- 9. Anatomy and Physiology of the Heart.. The valves between the atria and ventricles are called the atrioventricular valves. Between the right atrium and the right ventricle is the tricuspid valve. The tricuspid valve has three cusps, which connect to chordae tendinae and three papillary muscles named the anterior, posterior, and septal muscles, after their relative positions. The mitral valve lies between the left atrium and left ventricle. It is also known as the bicuspid valve due to its having two cusps, an anterior and a posterior cusp. These cusps are also attached via chordae tendinae to two papillary muscles projecting from the ventricular wall.
- 10. Anatomy and Physiology of the Heart.. The papillary muscles extend from the walls of the heart to valves by cartilaginous connections called chordae tendinae. These muscles prevent the valves from falling too far back when they close. During the relaxation phase of the cardiac cycle, the papillary muscles are also relaxed and the tension on the chordae tendineae is slight. As the heart chambers contract, so do the papillary muscles. This creates tension on the chordae tendineae, helping to hold the cusps of the atrioventricular valves in place and preventing them from being blown back into the atria.
- 11. Anatomy and Physiology of the Heart.. Two additional semilunar valves sit at the exit of each of the ventricles. The pulmonary valve is located at the base of the pulmonary artery. This has three cusps which are not attached to any papillary muscles. When the ventricle relaxes blood flows back into the ventricle from the artery and this flow of blood fills the pocket-like valve, pressing against the cusps which close to seal the valve. The semilunar aortic valve is at the base of the aorta and also is not attached to papillary muscles. This too has three cusps which close with the pressure of the blood flowing back from the aorta.
- 12. Anatomy and Physiology of the Heart.. Heart tissue, like all cells in the body, needs to be supplied with oxygen, nutrients and a way of removing metabolic wastes. This is achieved by the coronary circulation, which includes arteries, veins and lymphatic vessels. Blood flow through the coronary vessels occurs in peaks and troughs relating to the heart muscle's relaxation or contraction.
- 13. Anatomy and Physiology of the Heart.. Heart tissue receives blood from two arteries which arise just above the aortic valve. These are the left main coronary artery and the right coronary artery. The left main coronary artery splits shortly after leaving the aorta into two vessels, the left anterior descending and the left circumflex artery. The left anterior descending artery supplies heart tissue and the front, outer side, and the septum of the left ventricle. It does this by branching into smaller arteries – diagonal and septal branches. The left circumflex supplies the back and underneath of the left ventricle. The right coronary artery supplies the right atrium, right ventricle, and lower posterior sections of the left ventricle. The right coronary artery also supplies blood to the atrioventricular node (in about 90% of people) and the sinoatrial node (in about 60% of people). The right coronary artery runs in a groove at the back of the heart and the left anterior descending artery runs in a groove at the front.
- 14. Anatomy and Physiology of the Heart.. The coronary sinus is a large vein that drains into the right atrium, and receives most of the venous drainage of the heart. It receives blood from the great cardiac vein (receiving the left atrium and both ventricles), the posterior cardiac vein (draining the back of the left ventricle), the middle cardiac vein (draining the bottom of the left and right ventricles), and small cardiac veins. The anterior cardiac veins drain the front of the right ventricle and drain directly into the right atrium.
- 15. Cause of Eisenmenger's syndrome A number of congenital heart defects can cause Eisenmenger syndrome, including atrial septal defects, ventricular septal defects, patent ductus arteriosus, and more complex types of acyanotic heart disease.
- 17. The larger and more muscular left side of the heart must generate the high pressure required to supply blood to the extensive, high-resistance systemic circulation. In contrast, the smaller, right side of the heart must generate a much lower pressure in order to pass blood through the low-resistance, high compliance circulation of the lungs. If a significant anatomic defect (i.e. a hole or breach) exists between the two sides of the heart, a shunt will occur, causing blood to flow down the normal pressure gradient from the left side to the right side. The left-to-right shunting of blood results in abnormally high blood flow and pressure directed to the right heart circulation, gradually leading to maladaptive changes that ultimately result in pulmonary hypertension.
- 18. Eventually, due to increased resistance and decreased compliance of the pulmonary vessels, elevated pulmonary pressures cause the myocardium of the right heart to hypertrophy(RVH) The scar tissue also provides less flexibility and compliance than normal lung tissue, causing further increases in pulmonary blood pressure, and the weakened heart must pump harder to continue supplying the lungs, leading to damage of more capillaries Increased right-sided blood volume and pressure causes a cascade of pathologic damage to the delicate pulmonary capillaries, causing them to be incrementally replaced with scar tissue
- 19. The defect, now a right-to-left shunt, causes reduced oxygen saturation in the arterial blood due to mixing of oxygenated blood returning from the lungs with the deoxygenated blood returning from systemic circulation. As a consequence, deoxygenated blood returning from the body bypasses the lungs through the reversed shunt and proceeds directly to systemic circulation, leading to cyanosis and resultant organ damage. The onset of Eisenmenger's syndrome begins when right ventricular hypertrophy causes right heart pressures to exceed that of the left heart, leading to reversal of blood flow through the shunt (i.e., blood moves from the right side of the heart to the left side)
- 20. This decreased saturation is sensed by the kidneys, in a compensatory increase in erythropoietin production and an increased production red blood cells in an attempt increase oxygen delivery As the bone marrow increases erythropoiesis, the systemic reticulocyte count and the risk for hyperviscosity syndrome increases
- 21. Clinical Manifestations of Eisenmenger syndrome Cyanosis (a blue tinge to the skin resulting from lack of oxygen) High red blood cell count Swollen or clubbed finger tips (clubbing) Fainting (also known as syncope) Heart failure Abnormal heart rhythms Bleeding disorders Coughing up blood Iron deficiency Infections (endocarditis and pneumonia) Kidney problems Stroke Gout (rarely) due to increased uric acid resorption and production with impaired excretion Gallstones
- 22. Diagnostic Evaluation Imaging tests like an X-rays, CT scan, or an echocardiogram An electrocardiogram (EKG or ECG) or a walking test (to measure your heart rate and blood flow) Blood tests (to look for abnormally high or abnormally low red blood cell counts) Cardiac catheterization (for detailed information about the heart) Lung function tests (to measure how much oxygen is getting to the bloodstream)
- 23. Medical Management Much of the therapy being used for Eisenmenger syndrome has been studied in the treatment of Idiopathic Pulmonary Arterial Hypertension (IPAH) Calcium channel blockers- These medicines lower blood pressure in the lungs and the rest of the body. Anti- coagulants- Survival is increased when patients are treated with anticoagulant therapy. Warfarin should be used, provided the patient has no contraindications to anticoagulation. Maintain an international normalized ratio (INR) of 1.5 to 2. Nitric Oxide (NO)- Nitric oxide (NO) is a potent and selective pulmonary arteriolar vasodilator produced in endothelial cells. Studies have shown inhaled NO to reduce pulmonary vascular resistance with minimal systemic effects in patients with ES and acute pulmonary hypertensive states of other etiologies.
- 24. Medical Management… Endothelin-1 (ET-1) Receptor Antagonists- ET-1 is a powerful vasoconstrictor with elevated concentrations in the plasma and lung tissue of patients with PAH. It plays a key role in the pathogenesis of PAH including in vitro effects on proliferation, fibrosis and inflammation. Bosentan- Bosentan is a non-selective endothelin receptor antagonist Sildenafil(Viagra) and tadalafil - have shown beneficial effects on pulmonary selectivity and arterial oxygenation. demonstrating an improvement in exercise capacity, as assessed according to the six-minute walking test, functional class and hemodynamics in PAH patients.
- 25. Medical Management… Prostacyclin and Prostacyclin Analogs- Prostanoids can be administered by continuously intravenous or subcutaneous infusion, by inhalation and orally. Due to their active profile with vasodilatory, antiproliferative, anti-inflammatory and anticoagulant effects, they are suitable drugs for the treatment of PAH. Epoprostenol- Prostacyclin is a potent endogenous vasodilator produced in the vascular endothelium. Epoprostenol was the first synthetic prostacyclin analog, which became standard therapy of severe PAH in many countries. Digoxin- Digoxin therapy can be used to improve right ventricular function in patients with right ventricular failure
- 26. Medical Management… Diuretics- Use diuretics to manage peripheral edema. The use of loop diuretics (eg, furosemide, bumetanide) requires potassium supplementation and close monitoring of serum potassium. Potassium-sparing diuretics may have a role in ameliorating the sometimes-intractable hypokalemia observed with daily diuretic use. Oxygen therapy- Give supplemental oxygen in patients with resting or exercise-induced hypoxemia. Therapeutic erythropheresis Prevent infections (antibipotics)
- 27. Surgical Management Surgical palliation or repair should be performed early in patients with congenital heart disease to prevent progression to Eisenmenger syndrome. No surgical care is available to correct the congenital cardiac defect that caused the pathologic pulmonary vascular changes once Eisenmenger syndrome has developed to the irreversible stage. Heart-lung transplantation and single or bilateral, sequential lung transplantation Atrial septostomy- A surgeon creates an opening between the right and left sides of the heart.
- 28. NURSING MANAGEMENT USING NURSING PROCESS APPROACH Assessment Vital signs Current weight Cardiac auscultation for a systolic murmur Pulmonary auscultation for crackles Measurement of jugular vein distention.
- 29. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… Nursing Diagnoses Decreased cardiac output related to the disease process and medical treatments Ineffective cardiopulmonary, cerebral, peripheral, and renal tissue perfusion related to decreased cardiac output Impaired gas exchange related to pulmonary congestion caused by cardiac failure Activity intolerance related to failing heart function Powerlessness related to disease prognosis
- 30. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… Planning and Goals The major goals for the patient include: improved or maintained cardiac output increased activity tolerance reduction of anxiety absence of complications
- 31. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… Nursing Interventions Improving Cardiac Output During a symptomatic episode, rest is indicated Assessing the patient’s oxygen saturation at rest and during activity Provide supplemental oxygen Ensuring that medications are taken as prescribed to preserve adequate cardiac output Determining the patient’s weight every day Keeping patient warm and frequently changing position to stimulate circulation and reduce the possibility of skin breakdown
- 32. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… Increasing Activity Tolerance Plan the patient’s activities so that they occur in cycles, alternating rest with activity periods Avoid strenuous activity and sports Provide oxygen supplementation and medications Reducing Anxiety Spiritual, psychological, and emotional support may be indicated for the patient, family, and significant others If the patient is facing death or awaiting transplantation, time must be provided to discuss these issues Nurses help the patient, family, and significant others with anticipatory grieving
- 33. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… Promoting Home and Community-Based Care (a). Teaching Patients Self-Care- Teaching patients about the medication regimen, symptom monitoring Helping patients cope with their disease status assists them in adjusting their lifestyles
- 34. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… (b). Continuing Care- The nurse reinforces previous teaching and performs ongoing assessment of the patient’s symptoms and progress Teaching patients diet and fluid restrictions Maintain a record of daily weights and symptoms Organize daily activities to increase activity tolerance can be helpful Patient’s responses to and to the medication regimen are assessed Explanations about symptoms that should be reported to the physician are emphasized Because of the risk of dysrhythmia, the patient’s family may be taught cardiopulmonary resuscitation. Women are often advised to avoid pregnancy The nurse assesses the psychosocial needs of the patient and family on an ongoing basis
- 35. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… Evaluation Expected Patient Outcomes Maintains or improves cardiac function a. Exhibits heart and respiratory rates within normal limits b. Reports decreased dyspnea and increased comfort c. Reports no weight gain d. Maintains or increases activity tolerance e. Carries out activities of daily living f. Reports increased tolerance to activity
- 36. NURSING MANAGEMENT USING NURSING PROCESS APPROACH… 2. Is less anxious a. Discusses prognosis freely b. Verbalizes fears and concerns 3. Adheres to the self-care program a. Takes medications according to prescribed schedule b. Modifies diet to accommodate sodium and fluid restrictions c. Modifies lifestyle to accommodate recommended activity and rest behaviors
- 37. Living with Eisenmenger Syndrome Pregnancy is not recommended for women with Eisenmenger’s syndrome. Pregnancy may be dangerous for the mother and may cause complications for the fetus due to effects of low oxygen levels in the blood. Any anesthesia and surgery is considered high risk and should be carefully planned. Collaboration with a cardiac specialist is recommended. Air travel and high altitude exposure requires adequate hydration (fluid intake) and supplemental oxygen to prevent complications. Severe headache, dizziness, syncope (fainting), or changes in level of consciousness should be taken seriously and evaluated emergently.
- 38. Living with Eisenmenger Syndrome… Smoking and alcohol intake are not recommended. Coughing should be controlled or prevented with a strong suppressant medication to prevent risk of pulmonary hemorrhage (bleeding from the lungs). Flu shots are recommended annually, and pneumococcal vaccine should be received according to the doctor’s recommendation. Avoid hemodynamic shifts such as dehydration