PDA,AP WINDOW, TA.pptx

PDA,AP WINDOW, TA,nursing, cardiovascular nursing

1. COLLEGE OF NURSING MADRAS MEDICAL COLLEGE, CHENNAI-03 MEDICAL SURGICAL NURSING-II BY EDWIN JOSE.L MSC(N) II YEAR, COLLEGE OF NURSING, MADRAS MEDICAL COLLEGE, CHENNAI

2. introduction Congenital heart disease is a general term for a range of birth defects that affect the normal way the heart works. The term "congenital" means the condition is present from birth. According to the American Heart Association, about 9 of every 1,000 babies born in the U.S. have a congenital heart defect. This is a problem that occurs as the baby's heart is developing during pregnancy, before the baby is born. Congenital heart defects are the most common birth defects. Patent ductus arteriosus (PDA) is a congenital heart defect – a structural heart problem that is present at birth. Patent ductus arteriosus is an abnormal connection between the aorta and the pulmonary artery in the heart. The pulmonary artery carries blood from the heart’s right lower ventricle to the lungs, where it is loaded up with oxygen. 

3. Definition – Patent Ductus Arteriosus P atent ductus arteriosus (PDA) is a vascular structure that connects the proximal descending aorta to the roof of the main pulmonary artery near the origin of the left branch pulmonary artery which is persistent after birth. -American Heart Association   Ductus arteriosus is essential fetal structure normally closes spontaneously after birth. After the first few weeks of life, persistence of ductal patency is abnormal. The physiological impact and clinical significance of the PDA depend largely on its size and the underlying cardiovascular status of the patient. The PDA may be “silent” (not evident clinically but diagnosed incidentally by echocardiography done for a different reason), small, moderate, or large.

4. Embryology In normal cardiovascular development, the proximal portions of the sixth pair of embryonic aortic arches persist as the proximal branch pulmonary arteries, and the distal portion of the left sixth arch persists as the ductus arteriosus, connecting the left pulmonary artery with the left dorsal aorta. Normally, the distal right sixth aortic arch loses its connection to the dorsal aorta and degenerates. This transformation is completed by 8 weeks of fetal life.

5. DUCTUS ARTERIOSUS IN FETAL LIFE In the normal fetal heart, blood from the right ventricle traverses the DA and provides most of the blood flow to the lower body via the descending aorta. This diverts blood to the relatively low-resistance placenta and away from the lungs, with their inherent increased vascular resistance. Ductal patency is maintained by the presence of a certain neurohormonal milieu in the fetus. Continuous production of prostaglandin E2 within the vessel wall help maintain patency of the DA. Nitric oxide also plays a role in maintaining ductal patency. It is also maintained both by inhibition of intracellular release of calcium ions and by a level of insensitivity of the vascular smooth muscle cells to calcium ions.

6. NORMAL POSTNATAL CLOSURE OF THE DUCTUS ARTERIOSUS There are two processes involved in the postnatal closure of the DA functional closure anatomic closure Functional closure is normally initiated by the constriction of spiral muscles within the medial muscle layer. During functional closure, there is shortening and thickening of the ductal wall along with disruption of the intimal layer which results in the formation of intimal cushion. A subsequent process of migration of smooth muscle into the subintimal layer results in hemorrhage and necrosis.

7. Cont …… Initial closure occurs at the pulmonary end and extends toward the aortic end. Patency of the conical aortic end may persist for weeks after closure. Functionally complete closure occurs within 24–48 hours after birth in the full-term Anatomical closure - During the next 2–4 weeks, involution of the endothelium as well as changes in the subintimal layer (that is, structural/anatomic closure) occurs that is a fibrous band called the ligamentum arteriosum.

8. Incidence of Pda Patent ductus arteriosus (PDA) occurs in approximately 20-50% of neonates born before 32 weeks gestation and in up to 60% of neonates born before 29 weeks gestation 1 in every 2,500 to 5000 infants The incidence of an isolated PDA in term infants ranges from 0.03 to 0.08 percent There is a 2:1 female to male predominance Increased incidence in infants born at high altitude compared to sea level  Increased incidence in infants with congenital rubella Having a sibling with a PDA increases the chance of having a PDA by 2-4% 

9. TYPES OF PDA There are five types of patent ductus arteriosus determined by angiographic appearance ( Krichenko classification ): 1. Type A (conical): prominent aortic ampulla with a constricted pulmonary end         2.   Type B (window): large width with a very short length          3.   Type C (tubular): long and without any evidence of constriction         4.  Type D (complex): complicated course with potentially multiple areas of constriction         5.  Type E (elongated): extended length with a more remote constriction

10. Risk factors

11. Causes of pda Prematurity PDA is most prevalent in premature neonates, probably as a result of abnormalities in oxygenation. Prostaglandin E The relaxant action of prostaglandin E prevents ductal spasm and contracture necessary for closure. Other congenital defects PDA commonly accompanies rubella syndrome and may be associated with other congenital defects, such as coarctation of the aorta, ventricular septal defect, and pulmonary and aortic stenoses.

12. PATHOPHYSIOLOGY OF PATENT DUCTUS ARTERIOSUS PRETERM IMMATURE AD Immature expression of o2 in kv channels Activity of cAMP PDEs Activity of cAMP PDEs Sensitivity of oxygen O2 induced contration of smooth muscle Degradation of cAMP SENSITIVITY OF PGEs Degradation of cAMP Relaxation of vascular smooth muscle Sensitivity of NO PDA

13. CLINICAL MANIFESTATIONS Degree of left to right shunt, which depends on the size and length of the PDA The difference between pulmonary and systemic vascular resistance CLINICAL MANIFESTATIONS: MURMUR - medium pitched high-grade continuous murmur  heard best at the pulmonic position, with a harsh  machine like quality  that often radiates to the left clavicle. The first heart sound is normal but the  second heart sound is obscured by a continuous crescendo-decrescendo murmur , which runs from the start of systole to the end of diastole .

14. CONT….. Respiratory distress Heart failure Low immune system Slow motor development Physical underdevelopment Bounding peripheral pulses Widened pulse pressure

15. DIAGNOSTIC EVALUATION History collection – family history, maternal infections, Prematurity, perinatal distress, and hypoxia  Physical examinations : Patients usually appear well and have normal respirations and heart rates  A widened pulse pressure  Suprasternal or carotid pulsations may be prominent   tachypnea Tachycardia The apical impulse is laterally displaced; a thrill may be present in the suprasternal notch or in the left infraclavicular region

16. The first heart sound (S 1 ) is typically normal, and the second heart sound (S 2 ) is often obscured by the murmur paradoxical splitting of S 2  related to premature closure of the pulmonary valve and a prolonged ejection period across the aortic valve The murmur may be only a systolic ejection murmur, or it may be a crescendo/decrescendo systolic murmur that extends into diastole Occasionally, auscultation of the patent ductus arteriosus (PDA) reveals numerous clicks or noises resembling shaking dice or a bag of rocks

17. CONT….. Pulse oximetry/ABG usually demonstrate normal saturation because of pulmonary over circulation. A large ductus arteriosus could cause hypercarbia and hypoxemia from congestive heart failure (CHF) and air space disease (atelectasis or intra-alveolar fluid/pulmonary edema ). CHEST X –RAY: range from normal to those consistent for congestive heart failure (CHF). Cardiomegaly may be present with or without CHF. the pulmonary arteries, pulmonary veins, left atrium, and left ventricle are enlarged on chest films. the ascending aorta may be prominent. peripheral pulmonary vascular markings and increased pulmonary venous markings may be noted

18. Marked cardiomegaly with dilatation of the main pulmonary artery. Bilateral pulmonary plethora. 

19. Electrocardiography With a small patent ductus arteriosus (PDA), the electrocardiographic (ECG) findings are typically normal. Left ventricular hypertrophy may be present with a larger PDA. Left atrial enlargement may also be present with large shunts. Doppler Echocardiography  High velocity jets of turbulent flow in the pulmonary artery

20. Complications of pda Pulmonary hypertension Congestive cardiac failure Infective endarteritis Aneurismal dilatation of the pulmonary Artery Calcification of the ductus Non infective thrombosis Pulmonary or systemic emboli Paradoxical emboli

21. MANAGEMENT Pharmacological therapy: Prostaglandin analogs.  The ductus arteriosus can be induced to remain open by administering prostaglandin analogs such as  alprostadil (a prostaglandin E 1  analog). Indomethacin.  Indomethacin is a prostaglandin inhibitor that’s an alternative to surgery in premature neonates and induces ductus spasm and closure. Ibuprofen - Prophylactic ibuprofen is also widely used. The dose used for ibuprofen is 10 mg/kg bolus followed by 5 mg/kg/d for 2 additional days. Antibiotics –  because of the risk of bacterial endocarditis associated with the open structure.  Diuretic agents digoxin

22. Surgical management Cardiac Catheterization The use of the percutaneous route to close the patent ductus arteriosus (PDA) is becoming more common. Transcatheter occlusion is an effective alternative to surgical intervention and is becoming the treatment of choice for most cases of patent ductus arteriosus (PDA) in children and adults.   Gianturco spring occluding coils Amplatzer duct occluder Rashkind ductus occlusion device

23. SURGICAL LIGATION SURGICAL CLIPPING LIGATION

24. AORTOPULMONARY WINDOW

25. INTRODUCTION Aortopulmonary window (APW) is a rare congenital heart defect. It occurs as an isolated cardiac lesion or in association with other cardiac anomalies and rarely with abnormal coronary arteries. Aortopulmonary window (APW) was first described in the third decade of last century by Elliotson . It accounts for (0.2% - 0.6%) of all congenital heart lesions

26. DEFINITION- AP WINDOW Aortopulmonary window is a rare congenital heart disease characterized by a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves arising from separate sub arterial ventricular outflow tracts. -American thoracic society

27. EMBRYOLOGY The aortopulmonary septum develops as a wedge of tissue (capped by neural crest cells) that grows ventrally from the dorsal wall of the aortic sac between the origins of the fourth and sixth aortic arches.  This structure fuses with the distal margins of the embryonic outflow cushions (also capped by neural crest cells) to close the embryonic aortopulmonary foramen. Failure to close this foramen by fusion of the neural crest cells results in an aortopulmonary window. 

28. Classification of ap window Type I Type II Type III

29. Type I It is also called as proximal defects which occur in the proximal part of the aortopulmonary septum This defect is more proximally located between the origin of the main pulmonary artery and the ascending aorta immediately above the sinus of Valsalva with little inferior rim separating the AP window from the semilunar valves. These defects tend to be large, round or oval shaped, and are more often seen.

30. TYPE II It is also called as distal defects which occur in the distal part of the aortopulmonary septum adjacent to the right pulmonary artery This defect is more distal, between the ascending aorta and the origin of the right pulmonary artery These defects are more rare and tend to be smaller in size.

31. Type III It is a combination of types I and II. Consists of extension of the defect into the right pulmonary artery with anomalous origin of the right pulmonary artery from the ascending aorta with a well-formed inferior rim but little superior rim and involves the majority of the ascending aorta. This type is more frequently linked with other cardiac anomalies

32. INCIDENCE Aortopulmonary window is a very rare defect accounting for 0.2% to 0.6% of all congenital malformation There is a male preponderance. No specific genetic abnormalities have correlations with AP window, although Berry syndrome, a combination of AP window, interrupted aortic arch, and right pulmonary artery originating from the aorta is a defined entity.

33. ASSOCIATED CONDITIONS This condition can develop by itself or in conjunction with other cardiac conditions such as 1. Tetralogy of Fallot 2. Pulmonary atresia 3. Truncus arteriosus 4. Atrial septal defect 5. Patent ductus arteriosus 6. Interrupted aortic arch

34. pathophysiology

35. CLINICAL MANIFESTATIONS The clinical features of APW are not specific, but majority of patients have the manifestations of a large left to right shunt. Patients with small defects may be asymptomatic. Patients with large APW usually have symptoms of pulmonary hypertension and congestive heart failure (tachypnea, diaphoresis, failure to thrive, and recurrent respiratory difficulty) in the first weeks of life. Severe pulmonary vascular hypertension can occur in the first months of life.

36. Cont …. Tachypnea Diaphoresis Poor feeding Poor growth Delayed growth Rapid heartbeat Respiratory infections Minimal cyanosis present Heart failure symptoms generally emerge in early childhood, The cardiac murmur is usually systolic with a mid-diastolic rumble as a result of increased blood flow over the mitral valve.

37. Diagnostic evaluvation Echocardiography – left or biventricular hypertropy X-Rays – prominence of pulmonary artery and intrapulmonary vasculature Computerized tomography electrocardiogram - left and right ventricular hypertrophy

38. management Initial management: Medical therapy is focused on preoperative stabilization. It will retrogress spontaneously during follow up in majority of cases. The only effective therapy for aortopulmonary window is surgical repair (APW). However there are reports of transcatheter occlusion of simple APW.

39. First line treatment Intravenous prostaglandins (e.g., alprostadil) may be required in persons with an interrupted aortic arch to maintain the ductus arteriosus open and enable blood flow to the lower half of the body. The increased pulmonary blood flow may be exacerbated by the accompanying pulmonary arterial vasodilation. Digoxin and furosemide are widely used to treat heart failure and volume overload associated by this lesion. Inotropic drugs (for example, dopamine and dobutamine) may also be an effective therapy for babies with severe heart failure and low cardiac output due to myocardial dysfunction.

40. Surgical management Aortopulmonary window is generally treated with surgery. Surgery should be done as soon as feasible after initial stabilization and correction of acidosis. Surgery is performed with the use of cardiopulmonary bypass. The aortopulmonary window, the major pulmonary artery, or the anterior portion of the aorta can all be incised. Associated lesions are generally treated at the same time as the primary lesion. In patients with associated lesions, more complicated repairs and myocardial protection methods are necessary, increasing the morbidity and mortality associated with the surgery Transverse aortotomy – an autologous pericardial patch was used

41. Truncus arteriosus

42. introduction Truncus arteriosus (TA) is a rare form of congenital heart disease occurring in 1-3% of patients with congenital heart disease. During fetal development, the embryonic truncus arteriosus gives rise to the aorta and the pulmonary trunk.   Persistent truncus arteriosus results from incomplete or failed septation. It is characterized by a single great artery arising from the heart with a single semilunar valve that overrides the right and left ventricles. The common trunk gives rise to the pulmonary arteries, providing systemic, pulmonary and coronary perfusion.

43. definition Truncus arteriosus (TA) is an uncommon congenital cardiovascular anomaly that is characterized by a single arterial trunk arising from the normally formed ventricles by means of a single semilunar valve . T runcus arteriosus (TA) is a rare, congenital, cyanotic heart defect characterized by a ventricular septal defect (VSD), a single truncal valve, and a common ventricular outflow tract (OT).

44. EMBRYOLOGY During fetal development, persistent truncus arteriosus represents an arrest in the normal embryological separation of the anterior pulmonary artery and posterior aorta, resulting in a single great artery. Because these neural crest cells are known to play a role in the normal formation of the pulmonary artery and aorta, experimental ablation of these cells results in the specific congenital heart defect of persistent truncus arteriosus. In addition, since neural crest cells contribute to thymus and parathyroid development, there is an association between truncus arteriosus and DiGeorge syndrome

45. CLASSIFICATION The  Collett and Edwards  system is the earliest form of classification, developed in 1949. It is based on where pulmonary arteries arise from the common trunk. Type I : The main pulmonary is present and bifurcates into the left and right pulmonary arteries Type II : the right and left branches arise adjacent to each other from the posterolateral segment of the common trunk Type III : The right and left branches originate separately from the right and left lateral segments of the common trunk. Type IV : Neither of the branches arise from the common trunk, but are perfused by aortopulmonary collaterals. This type is now categorized as a form of pulmonary atresia with a ventricular septal defect rather than TA.

46. The  Van Praagh  classification system is based on where the branch pulmonary arteries arise from the trunk as well as the development of the aortic arch and the presence of a patent ductus arteriosus (PDA). Each type may include a modifier “A” (with VSD) of “B” (without VSD). Type A1 : The main pulmonary is present and bifurcates into the left and right pulmonary arteries (same as Collette and Edwards classification). Type A2 : The right and left branch pulmonary arteries arise from the common trunk. Type A3 : One branch pulmonary artery (typically the right) arises from the common trunk and the other arises from a PDA or the aorta. Type A4 : This type is defined by presence of aortic arch hypoplasia, coarctation or interrupted aortic arch and a large PDA.

47. ASSOCIATED CONDITIONS Ventricular Septal Defect - A VSD is present in the vast majority of cases. It is usually large and cono ventricular. Aortic arch anomalies - Approximately 30% of patients with TA have a right aortic arch and 12% have aortic arch hypoplasia or an interrupted aortic arch. Truncal valve anomalies - The leaflets are usually thickened. The valve is most commonly tricuspid, but may also be bicuspid, quadri cuspid or penta cuspid (rare) valve. Coronary artery anomalies - atypical origin, single coronary, or narrowed ostia resulting in coronary stenosis

48. ETIOLOGY Unknown cause chromosomal and genetic abnormalities - including duplication of chromosome arm 8q and mutation of the  NKX2.6  and  GATA6  genes. Genes - including  Tbx20, ALK2, Cited2,  and Semaphorin 3c,  children of mothers with significant diabetes mellitus during pregnancy had an increased incidence of truncus arteriosus  teratogens - eg , retinoic acid, bis-diamine

49. PATHOPHYSIOLOGY The VSD allows oxygenated and deoxygenated blood to mix before it is ejected through a common truncal valve to a single great artery, subsequently supplying the coronary, pulmonary and systemic circulations. The common semilunar valve may have 1 to 4 cusps with tricuspid most frequently seen. The presence of a single arterial trunk can be associated with several cardiac, aortic, and pulmonary abnormalities. These abnormalities include right-sided, interrupted, or hypoplastic aortic arches, abnormal origins of the coronary arteries, pulmonary artery stenosis, and patent ductus arteriosus.

50. CLINICAL MANIFESTATION rapid breathing (tachypnea) lethargy Cyanosis poor feeding, difficulty breathing (dyspnea) broadening of the fingertips (clubbing). abnormal accumulations of fluid in the face, arms, and/or legs (edema), an abnormally rapid heartbeat, slow weight gain, failure to thrive, recurrent respiratory infections, poor physical development, growth delays.

51. Diagnostic evaluation History collection Physical examination Arterial blood gas analysis- to determine the degree of acidosis Serum electrolytes ECG- Biventricular hypertrophy, A normal sinus rhythm, normal intervals, and either a normal QRS axis or minimal right-axis deviation are generally observed. Biventricular hypertrophy is a characteristic finding .

52. X ray chest- show moderate  cardiomegaly  with  pulmonary plethora  (mainly as a result of collateral formation) and widened mediastinum. Echocradiography - Allows direct visualization of a single trunk. Outflow tract views are the most useful. Color Doppler may additionally show flow across both ways through an associated VSD Magnatic resonance imaging- Allows direct display of anomalous anatomy.  SSFP  cine sequences can offer an additional functional assessment.

53. management Medical management: Intravenous prostaglandin Ionotropic agents e.g. Dopamine - Stimulates adrenergic and dopaminergic receptors Diuretic agents e.g . frusemide - Increases excretion of water by interfering with chloride-binding cotransport system Cardiac glycoside, antiarrhythmic e.g. digoxin - Acts directly on cardiac muscle, increasing myocardial systolic contractions. ACE inhibitor e.g. captopril - Inhibits activity of the angiotensin-converting enzyme, preventing conversion of angiotensin I to angiotensin II, which is a potent vasoconstrictor. 

54. Surgical management The Rastelli procedure involves creating a “baffle” to close the ventricular septal defect (VSD), separating the right & left ventricles. The baffle directs blood flow from the left ventricle to the aorta. During this surgery, a right ventricle to pulmonary artery (RV-PA) conduit is also placed to supply blood flow to the lungs During surgery, a median sternotomy (incision through the middle of the chest) is done. The patient is placed on cardiopulmonary bypass (heart–lung machine).

55. Cont ….. Depending on the patient’s anatomy and surgical plan, an incision is made on either on the right atrium or right ventricle to view the VSD. A Dacron patch is cut to the appropriate size. The patch is then sewn over the VSD to “baffle” blood flow from the left ventricle to the aorta. Once this is completed, if not already done, incisions are made on the pulmonary artery and right ventricle. An appropriate sized right ventricle-to -pulmonary artery conduit is selected. One end of the conduit is sewn onto the incision on the pulmonary artery and the other end is sewn onto the incision on the right ventricle.

56. Nursing management Nursing Assessment Assessment should focus on: Activity and rest.  The  nurse  should assess for weakness,  fatigue , dizziness, a sense of pulsing, and even  sleep  disorders. Circulation.  Circulatory  assessment should include history trigger conditions, history of heart murmurs and palpitations, BP, and pulse pressure. Food and fluids.  The nurse should assess for  dysphagia  and changes in body weight

57. Nursing Diagnosis Decreased Cardiac Output related to Structural factors of congenital heart defect Compromised Family Coping related to Situational and developmental crises of family and child Risk for Injury related to Cardiac function compromised by congenital defects and  medication administration Risk for Infection related to Chronic illness Activity intolerance  related to imbalance between oxygen consumption of the body and supply of oxygen to the cells. Anxiety  related to hospital care or lack of support system. Deficient knowledge  related to the condition and treatment needs

58. Nursing Care Planning & Goals: Maintain adequate cardiac output. Reduce the increase in pulmonary vascular resistance. Maintain adequate levels of activity. Provide support for growth and development. Maintain appropriate weight and height development.

59. Nursing Interventions Signs and symptoms Monitoring Adverse effects of indomethacin Preoperative instructions Postoperative procedures

60. Discharge and home care instruction Instructions.  Review instructions with parents about activity restrictions based on the child’s tolerance and energy levels. Activities.  Advise the parents not to be overprotective as the child’s tolerance for physical activity increases. Follow-up checkups.  Stress the need for regular follow-up examinations. History.  Advise parents to inform any practitioner who treats his child about his history of surgery for PDA-even if the child is treated for an unrelated medical problem.

61. conclusion Congenital Heart Defects are very common in our setup and early detection of CHD is increasing. Overall burden of CHD is also increasing therefore a proper population based study on a large scale is needed to estimate the prevalence accurately.