PDA AND EISENMENINGER SYNDROME..pptx for M.Sc., Nursing students

PDA AND EISENMENINGER SYNDROME

1. PATENT DUCTUS ARTERIOSUS AND EISENMENGER SYNDROME PRESENTED BY VENBA.E M.Sc.,(N) II YEAR COLLEGE OF NURSING MMC,CHENNAI-03.

2. DEFINITION Patent ductus arteriosus, the most common type of extracardiac shunt, represents persistent patency of the vessel that normally connects the pulmonary arterial system and the aorta in a fetus.

3. INCIDENCE PDA occurs in approximately 1 of 2,000 live births, but it is relatively uncommon among adults. In infants, it accounts for 10% to 12% of all congenital heart disease. PDAs are twice as common in female infants as in male infants. In Rubella syndrome males and females both are affected equally.

4. RISK FACTORS Maternal rubella infection Birth at high altitude Premature birth Female sex, and Genetic factors In infants born at < 28 weeks of gestation, there is a 60% incidence of PDA.

5. FETAL CIRCULATION The presence of the ductus arteriosus in the fetal circulation is essential to allow right-to-left shunting of nutrient-rich, oxygenated blood from the placenta to the fetal systemic circulation, thereby bypassing the fetal pulmonary circuit. In the fetus, the ductus arteriosus is kept open by Low arterial oxygen content and Placental prostaglandin E2 (PGE2)

6. BIRTH Several changes occur at birth to initiate normal functional closure of the ductus arteriosus within the first 15 to 18 hours of life. Spontaneous respirations result in increased blood oxygen content. Prostaglandin levels decrease because of placental ligation and increased metabolism of prostaglandins within the pulmonary circulation by prostaglandin dehydrogenase. Generally, the ductus arteriosus is hemodynamically insignificant within 15 hours and completely closed by 2 to 3 weeks

7. CLASSIFICATION Isolated PDAs are often categorized according to the degree of left-to-right shunting, which is determined by both the size and length of the duct and the difference between systemic and pulmonary vascular resistance, as follows: Silent: tiny PDA detected only by nonclinical means (usually echocardiography) Small: continuous murmur common; Qp /Qs <1.5 Moderate: continuous murmur common; Qp /Qs 1.5 to 2.2 Large: continuous murmur present; Qp /Qs >2.2 Eisenmenger: continuous murmur absent; substantial pulmonary hypertension, differential hypoxemia, and differential cyanosis (pink fingers, blue toes).

8. THE KRICHENKO CLASSIFICATION SYSTEM

9. NATURAL HISTORY Unlike PDA in premature infants, spontaneous closure of a PDA is rare in full-term infants and children. This is because the PDA in term infants results from a structural abnormality of the ductal smooth muscle rather than a decreased responsiveness of the ductal smooth muscle to oxygen. CHF or recurrent pneumonia develops if the shunt is large. Pulmonary vascular obstructive disease may develop if a large PDA with pulmonary hypertension is left untreated. Although rare, an aneurysm of PDA may develop and possibly rupture in adult life.

10. HISTORY The history of the mother’s pregnancy and perinatal events may provide clues ASSOCIATED with a high incidence of PDA, such as exposure to rubella in the first trimester in a non-immunized mother. PDA is also more common in premature infants, especially those with birth asphyxia or respiratory distress.

11. SYMPTOMS Severity of symptoms depends on the degree of left-to-right shunting; and it is determined by the size of the PDA, ductal resistance, cardiac output, as well as the systemic and pulmonary vascular resistances. Patients with small PDA are asymptomatic. With larger PDAs, symptoms may develop. The most common symptom is exercise intolerance followed by dyspnea, peripheral edema, and palpitations.

12. SIGNS Tachycardia and tachypnea may be present in infants with CHF. Bounding peripheral pulses Wide pulse pressure Hyperactive precordium: With a large shunt Systolic thrill: may be present at the upper left sternal border. The P2 is usually normal, but its intensity may be accentuated if pulmonary hypertension is present.

13. A grade 1 to 4 of 6 continuous (“machinery”) murmur is best audible at the left infraclavicular area or upper left sternal border. An apical diastolic rumble may be heard when the PDA shunt is large. Patients with small ductus do not have the above findings.

14. Cont.. The duration of the diastolic murmur reflects pulmonary artery pressures; elevated pulmonary artery pressures lead to a decreased gradient for left-to-right flow through the PDA during diastole, which results in a shorter diastolic murmur. As pulmonary pressure increases, the systolic component of the murmur shortens. Right-to-left flow may not generate a systolic murmur. Differential cyanosis: If pulmonary vascular obstructive disease develops, a right-to-left ductal shunt results in cyanosis only in the lower half of the body.

15. ECG With a small shunt the ECG is normal. Left ventricular hypertrophy of the volume overload type, with deep Q waves and increased R-wave voltage in the left precordial leads, is noted with increasing shunt size with left ventricular volume overload. Right ventricular hypertrophy is seen with pulmonary hypertension.

16. CXR Chest radiographs may be normal with a small-shunt PDA. Cardiomegaly varies in moderate-to-large-shunt of PDA with enlargement of the LA, LV, and ascending aorta. Pulmonary vascular markings are increased. With pulmonary vascular obstructive disease, the heart size becomes normal, with a marked prominence of the PA segment and hilar vessels.

17. CXR P/A view of a 2-day-old infant demonstrating the ductus bump (arrow).

18. ECHOCARDIOGRAM TTE has a 42% sensitivity and 100% specificity for the diagnosing PDA. ON 2-D echo, the left-sided chambers (LA and LV) are dilated due to increased venous return from the pulmonary circulation. This constitutes left ventricular volume overload. Due to the dilatation of the left atrium, the ratio between the size of the left atrium and proximal aorta (LA: Ao ratio) exceeds 1.3.

19. Parasternal long axis showing LA and left ventricular enlargement Echocardiogram in parasternal short axis demonstrating a patent ductus arteriosus with color-flow mapping indicating reversed flow

20. CARDIAC CATHETERIZATION Catheter trajectory: Catheter may easily pass from PA to Ao through the PDA. It gives a specific appearance “Hair pin” appearance.

21. Oxymetry : Steps up of O2 saturation in PA in comparison to RA. Pressure study: RV & PA pressure is normal, but elevated in large PDA. PVR is normal in infant & children but elevated in adult. LV graphy : to see associated VSD Aortography: to see PDA & associated CoA

22. MRI & CT Magnetic resonance imaging (MRI) and computed tomography may be useful in defining the anatomy in patients with unusual PDA geometry and in patients with associated abnormalities of the aortic arch.

23. MEDICAL Pharmacological closure of duct: For premature infants, treatment with indomethacin is usually the first-line therapy (0.2 mg/kg intravenously every 12 hours for up to three doses). Indomethacin therapy has been associated with an increased bleeding tendency resulting from platelet dysfunction, decreased urine output secondary to renal dysfunction, and necrotizing enterocolitis . Ibuprofen has achieved closure rates equivalent to those of indomethacin with less renal toxicity. Secondary measures: Prophylaxis against IE Treatment of HF

24. ACC/AHA 2008 GUIDELINES FOR THE MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE Class I Recommendations for Closure of Patent Ducts Arteriosus Closure of a PDA either percutaneously or surgically is indicated for the following: Left atrial and/or LV enlargement or if PAH is present, or in the presence of net left-to-right shunting Prior endarteritis Consultation with ACHD interventional cardiologists is recommended before surgical closure is selected as the method of repair for patients with a calcified PDA. Surgical repair by a surgeon experienced in CHD surgery is recommended when: The PDA is too large for device closure. Distorted ductal anatomy precludes device closure ( eg , aneurysm or endarteritis).

25. NONSURGICAL CLOSURE Since the early 1990s, transcatheter techniques have become the first-line therapy for most PDAs. Complications: Rare. The most common complication is embolization of the coil or device. Percutaneous coils were developed in 1992 and are the preferred treatment for older children and adults with PDAs < 3.5 mm in diameter Duct- Occlud coil

26. AMPLATZER DUCTAL OCCLUDER The ADO, a cone-shaped plug occluder made of thrombogenic wire mesh delivered with a 5F to 7F venous system, is the preferred device for percutaneous closure of moderate to large PDAs. The ADO stents the PDA, and blood is forced to flow through the center of the device, which is lined with thrombogenic wire mesh. The PDA then essentially clots off. Advantages include simple implantation, the ability to retract the ADO into the sheath and redeploy if needed, and high success rates. There is an 89% occlusion rate on post-procedure day 1 and 97% to 100% complete occlusion after 1month.

27. Amplatzer ADO I Amplatzer ADO II

28. Lateral angiogram showing coil occlusion of a patent ductus arteriosus (PDA). Small PDA allows shunting from the descending aorta to the pulmonary artery (arrow). Shunting is eliminated by an Amplatzer ductal occluder device placed in the ductus arteriosus (arrow).

29. SURGICAL CLOSURE In 1938, the first successful closure of a PDA was performed, which was the first repair of a congenital heart defect. With continued advances in percutaneous closure devices, surgery has become second-line therapy for most adults with PDAs Procedure: Ligation and division through left posterolateral thoracotomy without cardiopulmonary bypass is the standard procedure. The technique of video-assisted thoracoscopic surgery (VATS) clip ligation has become the standard of care for surgical management of ductus with adequate length (to allow safe ligation), which is performed through three small ports in the fourth intercostal space.

30. Mortality: The surgical mortality rate is <1% for both techniques. Complications: Injury to the recurrent laryngeal nerve (hoarseness) The left phrenic nerve (paralysis of the left hemidiaphragm ) The thoracic duct ( chylothorax ) is possible. Recanalization (reopening) of the ductus is possible, although rare, occurring after ligation alone (without division).

31. COMPLICATIONS OF PDA The most common complications of PDA include CHF Infective endocarditis Pulmonary hypertension

32. REPRODUCTIVE ISSUES Pregnancy is well tolerated in women with silent and small PDAs and in patients who were asymptomatic before pregnancy. In women with a hemodynamically important PDA, pregnancy may precipitate or worsen heart failure. Pregnancy is contraindicated in those with Eisenmenger syndrome because of the high maternal (≈50%) and fetal (≈60%) mortality.

33. EISENMENGER SYNDROME

34. INTRODUCTION Eisenmenger syndrome was named by Dr. Paul Wood after Dr. Victor Eisenmenger who first described the condition in 1897. Eisenmenger syndrome is pulmonary hypertension with a reversed central shunt. An uncorrected large left-to-right shunt causes an irreversible rise in PVR leading to the reversal of or bidirectional shunt flow with resultant hypoxemia. Eisenmenger syndrome is not a congenital defect, but a pathophysiologic condition.

35. DEFINITION Eisenmenger syndrome is defined as the process by which a long-standing left to right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, or less commonly, patent ductus arteriosus)causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt.

36. ETIOLOGY Around 12 different congenital intracardiac or extracardiac defects can cause Eisenmenger syndrome: Following 3 account for 70–80% of cases VSD Atrioventricular septal defect PDA Other causes ASD Truncus arteriosus Aortopulmonary window Univentricular heart without PS D-transposition of the great vessels with VSD Surgically created aorto-pulmonary connections With large shunts, the PVR develops relatively quickly, usually within first two years of life In patient with ASD may have Eisenmenger syndrome in adulthood.

37. Presentation and course in childhood Children may be asymptomatic or have only mild dyspnea. Reduced exercise capacity, dyspnea, and fatigue develop gradually as pulmonary blood flow decreases, and hypoxemia increases due to bidirectional shunting.

38. Course in adulthood Many individuals with Eisenmenger syndrome survive into adulthood with 80% survival at 10 years, 77% survival at 15 years and 42% at 25 years after diagnosis. Variables associated with poor prognosis include : - Syncope - Elevated RA pressure - Severe resting hypoxemia (<80% transcutaneous oxygen saturation) While individuals with Eisenmenger syndrome may remain relatively stable for long periods, it is essential to appreciate that their hemodynamic state is very delicately balanced. This balance is easily upset, often with disastrous results.

39. CLINICAL MANIFESTATIONS Cyanosis (a blue tinge to the skin resulting from lack of oxygen) High red blood cell count Swollen or clubbed fingertips (clubbing) Fainting (also known as syncope) Heart failure Abnormal heart rhythms Bleeding disorders U ric acid resorption and production with impaired excretion Gallstones

40. Cont … 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

41. Examination in Eisenmenger Syndrome Central cyanosis with digital clubbing May have differential cyanosis and clubbing Hypoxemia with resting oxygen saturation <90% Lungs are usually clear RV heave, palpable P2, right sided S4, and occasionally pulmonary ejection click Murmurs likely to be heard include a high-pitched diastolic decrescendo murmur of pulmonic insufficiency and a holosystolic murmur of TR Murmurs related to the defects connecting the systemic and pulmonary circulations are not usually heard

42. Diagnostic Testing Electrocardiography -RAE, RVH, right axis deviation, arrhythmia Chest X-ray -Cardiomegaly, dilated pulmonary arteries, pulmonary artery calcification Echocardiography: TEE is preferred -Heart defect, the direction of shunting, pulmonary hypertension Cardiac catheterization- Performed to establish that the PVR is elevated and responsiveness to the administration of oxygen, nitric oxide, sildenafil , Ca Channel Blockers. Open lung biopsy.

43. Expected abnormalities A number of abnormal findings are expected in Eisenmenger syndrome pts and should not raise undue concern unless they represent a significant change from past values Oxygen saturation at rest usually ranges in 80s If checked shortly after exertion , it will be lower (mid 70% range) The baseline value should be established after a few minutes of rest Hct , PLt INR and APTT are mildly prolonged Uric acid and bilirubin are elevated Proteinuria , Mildly elevated serum Cr and hematuria can also be found

44. Recommendations for Medical Therapy of Eisenmenger Physiology 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 (particularly at an elevation greater than 5000 feet above sea level). ( Level of Evidence: C) f. Iron deficiency. ( Level of Evidence: B)

45. Recommendations for Medical Therapy of Eisenmenger Physiology cont: 2. Patients with Eisenmenger syndrome should seek prompt therapy for arrhythmias and infections. ( Level of Evidence: C) 3. Should have hemoglobin, platelet count, iron stores, creatinine, and uric acid assessed at least yearly. ( Level of Evidence: C) 4. 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)

46. Recommendations for Medical Therapy of Eisenmenger Physiology cont: 5. Exclusion of air bubbles in intravenous tubing is recommended as essential during the treatment of adults with Eisenmenger syndrome. ( Level of Evidence: C) 6. These pts should undergo noncardiac surgery and cardiac catheterization only in centers with expertise in the care of such patients ( Level of Evidence: C)

47. MEDICAL MANAGEMENT

48. MEDICAL MANAGEMENT

49. MEDICAL MANAGEMENT

50. MEDICAL MANAGEMENT

51. MEDICAL MANAGEMENT

52. Medical Therapy of Eisenmenger Physiology cont: Hypoxemia: While it seems obvious that inhaled O2 would help, no studies show a mortality or morbidity benefit from chronic O2 administration Inhaled O2 can be used if the patient feels comfortable with it (reduced dyspnea, reduced fatigue, improved sleep). However, the adverse effects of mucosal dryness leading to mucous bleeding and the cumbersome equipment cause most patients to chose not to chronically use O2.

53. Hyperviscosity syndrome : Viscosity is affected by the concentration of RBCs and their deformability A high Hct alone may not cause these symptoms The major etiology for reduced deformity is thought to be iron deficiency which causes RBCs to change from deformable biconcave disks to more rigid microsphere Blood loss related to phlebotomy, hemoptysis, epistaxis and menses are common causes of iron deficiency

54. Important considerations in individuals with symptoms suggestive of hyperviscosity syndrome High Hct in the absence of symptoms does not require phlebotomy Exclude dehydration as a cause of Hct Exclude iron deficiency , If present, treat with oral iron Phlebotomy may be appropriate if symptoms are severe and none of the above factors apply

55. PHLEBOTOMY The goal of phlebotomy is to treat the symptoms of the hyperviscosity syndrome and not to obtain a specific Hct Prompt relief of symptoms after the phlebotomy confirms that hyperviscosity was the likely etiology If the symptoms do not resolve promptly, consider other alternative causes and do not repeat the phlebotomy

56. Medical Therapy of Eisenmenger Physiology cont BLEEDING: These pts are at risk of bleeding from the relatively benign easy bruising to life-threatening massive intra-pulmonary hemorrhage and hemoptysis Most bleeding is, however minor, involves the mucocutaneous tissues, and responds to conservative management Significant bleeding can be treated with vitamin K, FFPs, platelets or cryoprecipitate Phlebotomy may improve platelet function, increase platelet count and improve various coagulation abnormalities Phlebotomy can be considered prior to elective surgery to decrease the risk of bleeding

57. Cerebrovascular and other embolic events Mechanisms include hemorrhage, emboli and infection with formation of a cerebral abscess Iron deficiency is the major risk factor for cerebrovascular events The risk–benefit ratio of aspirin or warfarin needs to be considered in each patient

58. Gout Rare Pathophysiology ?? Increase resorption of uric acid Increase production of uric acid and impaired excretion Treatment Colchicine Avoid NSAIDs

59. Pulmonary hypertension Pulmonary vasodilator agents such as prostacyclin analogs, endothelin antagonists and phosphodiesterase inhibitors have been found to reduce PVR and improve functional capacity Limited data cite some individuals so responsive to these agents that surgical correction of the defect was possible Alternatively, in patients with progressive heart failure, these agents have been used as part of a bridge to transplantation

60. Non-cardiac surgery in eisenmenger patients Non-cardiac surgery in Eisenmenger patients carries a high morbidity and mortality risk (up to 19%) Surgery should be avoided when possible but is commonly needed for acute cholecystitis (due to bilirubin stone formation from the hyperbilirubinemia) Necessary operations should be done in a center familiar with the high risks of performing surgery on these patients

61. The following may lead one to consider surgical or transcatheter options: Progressive deterioration of functional class Recurrent syncope Refractory right heart failure Supraventricular tachyarrhythmias Worsening hypoxemia

62. CATHETER AND SURGICAL MANAGEMENT Once Eisenmenger physiology has developed, catheter or surgical interventions have a limited role in management Surgery to repair the underlying congenital anomaly is not recommended for two reasons: 1 - The risk of surgery is exceedingly high 2 – Those who survive the surgery have increased mortality

63. Cont … Heart–lung transplantation is an option, but long waiting is a problem In some instances, lung transplantation with repair of the intracardiac defect may be an option Lung transplantation has the advantage of better donor availability, a shorter waiting period, and avoidance of problems associated with heart transplantation (vasculopathy and rejection)

64. The causes of death in Eisenmenger patients Sudden death (30%) Congestive heart failure (25%) Hemoptysis (15 %) Pregnancy Perioperative following non-cardiac surgery Infective endocarditis Brain abscess Non-cardiac causes

65. PERIOPERATIVE MORBIDITY AND MORTALITY The mortality and morbidity are related to: Sudden fall in SVR leading to worsening hypoxemia due to progressive right to left shunting Hypovolemia and dehydration Excessive bleeding Perioperative arrhythmias Thrombophlebitis/DVT/paradoxical emboli

66. NURSING MANAGEMENT

67. NURSING DIAGNOSIS

68. Take Home Messages Eisenmenger syndrome is a pulmonary hypertensive disease caused by left-to-right shunting of blood The severity of pulmonary vascular resistance is an important prognostic factor Corrective surgery may cause pulmonary crisis. It should be performed on selected patients The principle of intervention is non-intervention For quality of life, complications must be managed Pregnancy, noncardiac surgery, traveling: be cautious Transplantation is an effective choice of treatment