1. ‘ The Pedi-Cardiac Lecture ’ Part 2 Pediatric Cardiovascular Disorders Jerry Carley MSN, MA, RN, CNE

2. Concept Map: Pediatric Cardiac Conditions

3. Distribution of Congenital Heart Defects by anatomical location

4. PDA

6. Persistent Ductus Arteriosus PDA Incidence 10% of all reported CHDs One of the most common benign defects Ductus normally closes within hours of birth Connection between the pulmonary artery (low pressure) and aorta (high pressure) High risk for pulmonary hypertension

7. Ductus Arteriosus Fetal Structure, Connecting Function Pulmonary Artery Aorta Blood from (R)) Ventricle Pulmonary Artery Reenters Aorta Allows Blood to Bypass Lungs Effects / Symptoms Tachypnea Dyspnea Bounding Pulse Recurrent Pneumonia Heart Failure Pulmonary Hypertension Cardiomegaly Murmurs Difficulty Feeding FTT Tires Easily Infective Endocarditis Treatments Spontaneous Closure MedicationIndomethacin (Indocin) Ibuprofen (Motrin) Surgery Heart Catheterization Usually by 2 years Nursing Care Closed Heart Surgery

8. Diagnosis and Treatment Diagnosis by  Chest x-ray – enlarged heart and dilated pulmonary artery  Echo-cardiogram – show the opening between pulmonary artery and aorta

9. Treatment Indomethocin (Indocin) given po – constricts the muscle in the wall of the PDA and promotes closure Cardiac Catheterization – coil is placed in the open duct and acts like a plug Closed heart surgery – small incision made between ribs on left hand side and PDA is ligated or tied and cut

10. ASD

11. Atrial Septal Defect ASD 10% of defects Blood in left atrium flows into right atrium Pulmonary hypertension Reduced blood volume in systemic circulation If left untreated may lead to pulmonary hypertension, congestive heart failure or stroke as an adult.

12. Pathophysiology Lower Pressure In ® Atrium Effects / Symptoms Short Stature Heat Murmur Dyspnea Pulmonary Hypertension Cardiomegaly Arrhythmia Treatments Large ASDs: Surgical Closure Heart Catheterization Patching Nursing Care Oxygenated blood From lungs shunted To ® Atrium from (L) d/t ASD Blood recirculates back to the lungs Via pulmonary arteries Transcatheter Balloon R L Suturing

13. ASD LeftRight

14. Diagnosis and Treatment Diagnosis: heart murmur may be heard in the pulmonary valve area because the heart is forcing an unusually large amount of blood through a normal sized valve. Echocardiogram is the primary method used to diagnose the defect – it can show the hole and its size and any enlargement of the right atrium and ventricle in response to the extra work they are doing.

15. Treatment Surgical closure of the atrial septal defect After closure in childhood the heart size will return to normal over a period of four to six months. No restrictions to physical activity post closure

16. VSD

17. Ventricular Septal Defect VSD 30% of congenital heart defects Opening in the ventricular septum Left-to-right shunt Right ventricular hypertrophy Deficient systemic blood flow

19. Pathophysiology Hole in Ventricular Septum R L Shunting Increased Pulmonary Flow & Pressure Effects / Symptoms Tachypnea Tachycardia Enlarged Pulmonary Arteries Recurrent Pneumonia Pulmonary Hypertension Cardiomegaly Murmurs Treatments Medication Diuretics Captopril (ACE Inhibitor) Surgery Heart Catheterization Nursing Care Open-Heart Septal Plasty Pulmonary Edema Dyspnea Paleness FTT Sweating While Feeding Frequently seen With other anomalies, e.g., TOF Congestive Heart Failure Digoxin Eventually, will become R L Shunt if Not Treated !

20. VSD Small holes generally are asymptomatic Medium to moderate holes will cause problems when the pressure in the right side of the heart decreases and blood will start to flow to the path of least resistance (from the left ventricle through the VSD to the right ventricle and into the lungs) This will generally lead to CHF

21. Diagnosis and Treatment Diagnosis – heart murmur – clinical pearl a louder murmur may indicate a smaller hole due to the force that is needed for the blood to get through the hole. Electrocardiogram – to see if there is a strain on the heart Chest x-ray – size of heart Echocardiogram – shows size of the hole and size of heart chambers

22. Treatment VSD CHF: diuretics of help get rid of extra fluid in the lungs Digoxin if additional force needed to squeeze the heart FTT or failure to grow may need higher calorie concentration Will need prophylactic antibiotics before dental procedures if defect is not repaired

23. Surgical Repair Over a period of years the vessels in the lungs will develop thicker walls – the pressure in the lungs will increase and pulmonary vascular disease If pressure in the lungs becomes too high the un-oxygenated blood with cross over to the left side of the heart and un-oxygenated blood with enter the circulatory system.(Becomes a Right Left Shunt) If the large VSD is repaired these changes will not occur.

24. COA

25. Coarctation of Aorta COA 7 % of defects Congenital narrowing of the descending aorta 80% have aortic-valve anomalies Difference in BP in arms and legs (severe obstruction)

27. Diagnosis and Treatment In 50% the narrowing is not severe enough to cause symptoms in the first days of life. When the Ductus Arteriosis closes a higher resistance develops and heart failure can develop. Pulses in the groin and leg will be diminished Echocardiogram will show the defect in the aorta

28. Treatment Prostaglandin may be given to keep the DA open to reduce the pressure changes The most common repair is resection of the narrowed area with re-anastomosis of the two ends Surgical complications – kidney damage due to clamping off of blood flow during surgery High blood pressure post surgery – may need to be on antihypertensives Antibiotic prophylactic need due to possible aortic valve abnormalities.

29. PS

30. Pulmonary Stenosis PS 7% of defects Obstruction of blood flow from right ventricle Hypertrophy of right ventricle If severe cyanosis due to right-to-left shunt

32. Pulmonary Valvular Stenosis In pulmonary valvar stenosis the pulmonary valve leads to narrowing and obstruction between the right ventricle and the pulmonary artery. Thickened tissue become less pliable and increases the obstruction Right ventricle must work harder to eject blood into the pulmonary artery.

33. Pathophysiology Abnormality of Pulmonary Valve Leaflets Effects / Symptoms Asymptomatic (usually) ® Ventricular Hypertrophy Dilated Pulmonary Artery Feeding Problems Pulmonary Hypertension Dyspnea Potential ® Ventricular Failure FTT S/S ® Heart Failure Treatments Indomethacin (Indocin) Ibuprofen (Motrin) Surgery Heart Catheterization Usually by 2 years Nursing Care Sometimes part of DiGeorge Syndrome Leakage of Pulmonary Valve When closed Tires Easily Transcatheter Balloon Stenting

34. Diagnosis and Treatment Diagnosis: heart murmur is heard – clicking sound when the thickened valve snaps to an open position. Electrocardiogram would be normal Echocardiogram most important non-invasive test to detect and evaluate pulmonary stenosis Cardiac Catheterization – to measure pressures and measure the stenosis

35. Treatment Cardiac Catheterization to dilate the valve and open up the obstruction. Open- heart procedure would only needed for more complex valve anomaly.

36. TOF

37. Tetralogy of Fallot (TOF) 6% of all CHD defects Most common cardiac malformation responsible for cyanosis in a child over 1 year

38. 1. Narrowing of the Pulmonic Valve 2.Thickening of Right Ventricular Wall 3. Displacement of Aorta over ventricular septal defect 4. Ventricular Septal defect Right Left

39. Pathophysiology Pulmonic Valve Narrowing (R) Ventricle Hypertrophy Displacement of Aorta Effects / Symptoms Tachypnea Dyspnea Central CYANOSIS Heart Failure Cardiomegaly Harsh Systolic Ejection Murmur Difficulty Feeding FTT Tires Easily Treatments Surgery Nursing Care Ventricular Septal Defect (VSD) R L Tet Spells Usually Self- Limiting Close VSD Relieve ® Ventricular Outflow Finger Clubbing

40. TOF Four Components  VSD  Pulmonary stenosis – narrowing of pulmonary valve  Overriding of the aorta – aortic valve is enlarged and appears to arise from both the left and right ventricles instead of the left ventricle  Hypertrophy of right ventricle – thickening of the muscular walls because of the right ventricle pumping at high pressure

42. Clinical Manifestations Dependent on degree of right ventricular outflow obstruction. Right-to-left shunt Clubbing of digits “tet” spells - ‘hyper-cyanotic episodes’ treated by flexing knees forward and upward Severe irritability due to low oxygen levels

43. Children with T.O.F. exhibit cyanosis during episodes of crying or exertion.

44. Knee-chest Position Child with a cyanotic heart defect squats (assumes a knee-chest position) to relieve cyanotic spells. (“tet spells “) Nurse puts infant in knee-chest position.

45. Diagnosis Cyanosis (central) Oxygen will have little effect on the cyanosis Loud heart murmur Echocardiogram – demonstrates the four defects characteristic of tetralogy

46. Treatment If oxygen levels are extremely low prostaglandins may be administered IV to keep the PDA open Complete repair is done when the infant is about 6 months of age Correction includes  Closure of the VSD with dacron patch  The narrowed pulmonary valve is enlarged  Coronary arteries will be repaired  Hypertrophy of right heart should remodel within a few months when pressure in right side is reduced

47. Long Term Outcomes Leaky pulmonic valve that can lead to pulmonary insufficiency Arrhythmias after surgery Heart block – occasionally a pacemaker is necessary Periodic echocardiogram and exercise stress test or Holter monitor evaluation

48. End of Part 2