Congenital Heart Disease.pptxyyyyyyyyyyy

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1. Congenital Heart Disease

2. OUTLINE Definition Etiology and risk factors Classification (types) Pathophysiology Clinical presentation Lab investigations Differential diagnosis Management Nursing interventions Current (existing) evidence on the area 10/16/2024 2

3. Over view Fetal circulation In the fetus, the placenta provides for gas and metabolite exchange. Since the lungs do not provide gas exchange, the pulmonary vessels are vasoconstricted , diverting blood away from the pulmonary circulation. Three cardiovascular structures unique to the fetus are important for maintaining this parallel circulation: The ductus venosus Foramen ovale, and Ductus arteriosus. 10/16/2024 3

4. Over view Fetal circulation… Blood flows from t he placenta IVC RA through the FO LA LV ascending aorta head & upper extremities returns via the SVC 10/16/2024 4

5. From the SVC RA RV pulmonary artery through the PDA descending aorta lower extremities and placenta 10/16/2024 5

6. Immediately after delivery With the first breaths at birth, the fetal circulation changes. A larger amount of blood is sent to the lungs to pick up oxygen. Because the ductus arteriosus (the normal connection between the aorta and the pulmonary valve) is no longer needed, it begins to wither and close off. (72 hrs.) The circulation in the lungs increases and more blood flows into the left atrium of the heart pressure causes the foramen ovale to close and blood circulates normally. What If this normal physiology altered ? =========CHD. 10/16/2024 6

7. 1. Congenital Heart Disease Definition: Congenital heart disease or congenital heart defect (CHD) is a problem of the heart's structure and function present at birth, affecting the heart or adjacent great blood vessels, detected either at the time of birth or detected later in life. Most common cause of childhood heart disease Cause either shunting of blood between right and left circulation or cause obstruction Range from asymptomatic to rapidly fatal 10/16/2024 7

8. Epidemiology A . Left to Right shunts (Acyanotic or Late Cyanotic group) cyanosis months or years after birth Ventricular septal defect (VSD) -25-30% Atrial septal defect (ASD)-10-15% Patent ductus arteriosus (PDA)-10-20% B . Right to Left shunts (Cyanotic group ) Tetralogy of Fallot (TOF)-6-15% Transposition of great arteries -4-10% Persistent truncus arteriosus – 2% Tricuspid atresia and stenosis 1% C. Obstructions Coarctation of Aorta 5-7% Aortic stenosis and atresia 4-6% Pulmonary stenosis and atresia 5-7% Congenital heart disease occurs in approximately 8-10 per 1000 births congenital heart diseases account for 3% of all infant deaths In premature infants about 2% excluding patent ductus arteriosus [PDA]) In stillborns (3–4%) and in spontaneous abortuses (10–25%) 10/16/2024 8

9. Etiology The cause of most (80%) congenital heart defects is still unknown . But many cases of congenital heart disease are multifactorial and result from a combination of genetic predisposition and environmental stimulus. Genetic(20%) - single gene defect and Chromosomal abnormality (down syndrome and turner syndrome) Adverse maternal conditions (environmental) Maternal infections – rubella Maternal diseases – DM Drugs – lithium, ethanol, warfarin, thalidomide, antimetabolites, vitamin A derivatives, anticonvulsant agents) Advance maternal age 10/16/2024 9

10. Assessment : history Medical history of: Illness -LE, DM Medication- cardiotoxic drugs Rheumatic fever Abortion and still birth Immunization hx : because pertussis and rubella are dangerous for these patients. Pregnancy history: viral infection, drug exposure, maternal disease Family History: CHD and Syndromes in parents Sex: In female PDA and VSD are common In male CoA, AS and transposition of great arteries are common 10/16/2024 10

11. HX cont … Birth history: Progress of labor, Method of delivery, Apgar score, Asphyxia and Delay in clamping umbilical cord Birth weight hx : CHD patients has low birth weight distribution e.g PDA and VSD. And also Baby with large for gestational age (baby of diabetic mother) has 3-4 times higher risk of CHD Endurance and exercise tolerance: this screens for cardiac diseases involving obstructive lesions such as aortic or pulmonic stenosis 10/16/2024 11

12. Laboratory investigations Blood test: to determine Arterial blood gases like the oxygen tension (PaO2), carbon dioxide tension (PaCO2), acidity (pH ), Electrolyte, oxyhemoglobin saturation (SaO2), and bicarbonate (HCO3) concentration in arterial blood 10/16/2024 12

13. Differences in infants and children Infants: feeding difficulties, easy fatigability, sweeting while breast feeding, tachypnea, poor weight gain and cyanosis Older children: shortness of breath, dyspnea on exertion, dizziness and palpitation 10/16/2024 13

14. Classification of CHD Generally, congenital heart disease is divided into cyanotic or acyanotic heart disease, a classification which is based on the level of hemoglobin saturation in the systemic circulation. 1. Acyanotic (L R shunt) Increased pulmonary blood flow Atrial septal defects Ventricular septal defect Patent ductus arteriosus Obstructed blood from the ventricles • Coarctation of the aorta • Aortic stenosis • Pulmonic stenosis 10/16/2024 14

15. Classification cont … 2. Cyanotic (R– L shunt) • Decreased pulmonary blood flow • Tetralogoy of Fallot • Tricuspid atresia • Mixed blood flow • TGA • Truncus arteriosus 10/16/2024 15

16. 1 Acyanotic Congenital Heart Disease A congenital heart defect in which the children has no cyanosis because there is no mixing of unoxyginated blood in systemic circulation. Most common type of CHD and present 3:1 ratio of females to males Blood is shunted (flows) from the left side of the heart to the right side of the heart due to a structural defect (hole) in the inter-ventricular septum. Communication between the systemic and pulmonary sides of the circulation, which results in shunting of fully oxygenated blood back into the lungs. 10/16/2024 16

17. 1.1 Increased pulmonary blood flow Ventricular Septal Defect (VSD) The defects that occur in any portion of the ventricular septum, but most are of the membranous type. Has three types: membranous, supracrystal and muscular ( single/multiple) Single most common congenital heart malformation, accounting for almost 30% of all CHD Pathophysiology Large VSDs recognized early in life Left to right shunt right side volume overload pulmonary HTN right to left shunt late onset cyanosis 10/16/2024 17

18. Clinical Manifestation Small defects - Most common - Asymptomatic - Loud, harsh holosystolic M at LLSB Large defects Excessive pulmonary blood flow Pulmonary hypertension Dyspnea , feeding difficulties, poor growth, perspiration, recurrent plum. infection , heart failure Less harsh but more blowing holosystolic M. Accentuated 2 nd heart sound(2 component sound at aortic and pulmonic valve closure) Mid-diastolic apical M when shunt/flow ratio of Qp : Qs > 2:1 10/16/2024 18

19. VSD cont.… Diagnosis - Clinical - CXR - Cardiomegaly - Plethoric lung - ECG - to show left ventricular hypertrophy. - Echocardiography – position and size of the VSD and shunt Treatment Small defects - reassurance - Prophylaxis against IE Large defects - medical treatment (Lasix and Aldactone to control CHF and Digoxin to increase effectiveness of myocardial function, prevent IE, prevent development of p. venous HTN) - Surgical repair between 6-12m 10/16/2024 19

20. Complications Infective endocarditis Recurrent lung infection Heart failure Pulmonary HTN Acquired pulmonary stenosis 10/16/2024 20

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22. Atrial Septal Defect 10-15 % of CHD and 1/1500 live births Major types Ostium Secundum --- 75% Most common In the middle of the septum in the region of the foramen ovale Ostium Primum - -- 15% associated with other endocardial cushion defects (cleft AV valves, inlet type VSD) Low position Form of AV septal defect Sinus Venosus --- 10% large, associated with anomalous pulmonary venous drainage Least common Positioned high in the atrial septum Frequently associated with partial anomalous pulmonary venous return (PAPVR) Coronary sinus (rare ) 10/16/2024 22

23. ASD Cont.… Pathophysiology Left to right shunt of: - Trans atrial in OS & SV - Trans atrial & trans ventricular in OP Clinical Manifestations Most are asymptomatic Hyperactive precordium Dyspnea, SOB and palpitations Wide & fixed split of 2 nd heart sound Systolic ejection murmur Mid- diostolic murmur at tricuspid area Holosystolic murmur at mitral area in OP 10/16/2024 23

24. Cont… Diagnosis Clinical By Ultrasound during pregnancy CXR - Right. V & A enlargement - Large pulm . artery - ↑ ed pulm . vascularity ECG - volume overload, Rt ventricle & atrium Echocardiography Prognosis - Well tolerated Complications - pulm . Hypertension Eismenger syndrome, Arrhythmia & Heart failure Treatment -Surgery -for all symptomatic - Medical Treatment -Digoxin - strengthen the heart muscle and diuretics to help the kidneys remove excess fluid from the body 10/16/2024 24

25. Atrial Septal Defect 10/16/2024 25

26. Patient Ductus Arteriosus Persistence of the normal fetal vessel that joins the PA to the Aorta . Accounts for 10% of all CHD with Female : Male ratio of 2:1 and common in preterm The ductus arteriosus connects the pulmonary artery to the descending aorta during fetal life. PDA results when the ductus fails to close after birth. In fetal life, ductus serves as a functioning connection between the pulmonary artery and aorta. After birth, the partial pressure of o 2 rises and the pulmonary arterioles dilate causing the ductus to close . Closes spontaneously in normal term infants at 3-5 days of age Leads to increased pulmonary vascular disease. 10/16/2024 26

27. PDA cont. Pathophysiology Blood flows from aorta to the pulmonary artery, creating a left to right shunt, resulting in left atrium and ventricle overload. Increased pulmonary blood flow can result in pulmonary hypertension and reversal of the shunt, which is known as Eisenmenger’s Syndrome . This results in flow of desaturated blood to the lower extremities . Clinical Manifestation Asymptomatic in small ductus Large PDA’s can result in symptoms of CHF, growth restriction, failure to thrive (FTT) Wide pulse pressure Bounding pulse Continuous or machinery mainly systolic M at 2 nd Left ICS 10/16/2024 27

28. PDA cont. Diagnosis - Clinical - Chest X-ray -enlarged cardiac silhouette - ECG - Left atrial enlargement, LVH - Echocardiography - Prognosis - Small PDA - can maintain normal life - Large PDA - CHF Complications - Infective Endocarditis/Endarteritis - CHF - Embolization - Pulmonary HTN Treatment PDA can be treated with both surgical and non-surgical methods Surgical treatment - Surgical division or ligation of the PDA Non surgical treatment - Indomethacin or a special form of  ibuprofen have been used to initiate PDA closure 10/16/2024 28

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30. 1.2 Obstructive Lesions Pulmonic Stenosis Pulmonic Stenosis is obstruction in the region of either the pulmonary valve or the sub pulmonary ventricular outflow tract . Accounts for 7-10% of all CHD . Pathophysiology - Rt outlet obstruction → Pressure work → Rt vent . hyperthropy I t has 4 types: - Valvular - Infundibular - Supra valvular - Peripheral 10/16/2024 30

31. Cont… Clinical Manifestation Mild to moderate - asymptomatic Critical stenosis - Systolic ejection murmur - Heart failure in neonates & infants - Rarely cyanosis Diagnosis - Clinical - C ardiac catheterization to inject a dye for x-ray or measure the pressure of chambers - CXR - Rt vent. Enlargement and reduced pulm . blood flow - ECG to show muscular thikening -Echocardiography to show structure narrowing and location of valve 10/16/2024 31

32. Cont… Treatment Mild PS no intervention required, close follow-up. Mod-severe – require relieve of stenosis. Balloon valvuloplasty - treatment of choice. Surgical valvotomy is also a consideration. Complications - CHF in severe Ps - rarely IE 10/16/2024 32

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34. Coarctation of the Aorta Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation. Accounts for 7 % of all CHD and Male: Female ratio 3:1 Can be Located: proximal , at , or distal to insert in of ductus. 98% of coarctations are juxtaductal 20% of cases are associated with Turner sndrome Divided in to infantile ( preductal ) & adult ( postductal ) form Infantile form proximal (pre-ductal) Associated with PDA Coarctation lies proximal to the PDA Presents as lower extremity cyanosis 10/16/2024 34

35. CoA cont. Adult, distal ( postductal ) form Coarctation lies distal to the aortic arch Presents as hypertension in upper extremity and hypotension in lower extremity Discovered in adulthood Clinical Manifestation hypertension of the upper extremities Differential cyanosis → pale upper extr . and cyanosed lower extr . Classic signs - Disparty in pulse & BP - Radio-femoral delay - Systolic M at LMSB & inter-scapular area 10/16/2024 35

36. CoA cont. Diagnosis Clinical –BP( in lower and upper extri .) and Pulse ECG- LVH CXR –Cardiomegaly, rib notching (large volume of blood in intercostal arteries) Treatment With severe coarctation maintaining the ductus with prostaglandin E is essential. Surgical intervention, to prevent LV dysfunction. Balloon angioplasty is the procedure of choice. Complications - CVA - I/E - Aneurysms 10/16/2024 36

37. Aortic Stenosis Aortic stenosis (AS) is narrowing of the aortic valve resulting in obstruction of blood flow from the left ventricle to the ascending aorta during systole. It has 3 types : Valvular - the commonest Supra valvular Subvalvular ( subaortic ) 10/16/2024 37

38. Clinical Manifestation Mild stenosis - Normal pulse & apical impulse - Systolic ejection M - Normal to enlarged heart size Critical stenosis - Left ventricular failure - pulm . edema, cardiomegaly - Weak peripheral pulses - Weak systolic M - Paradoxical split M 2 nd heart sound 10/16/2024 38

39. complication HF Stroke Blood clot Bleeding Cardiac arrhtmia Infection like endocarditis Death 10/16/2024 39

40. 2. Cyanotic Congenital Heart Disease 2.1 Decreased pulmonary blood flow Tetralogy of Fallot Tetralogy of Fallot is the most common cause of "blue baby." Defined by four findings: pulmonary stenosis Ventricular septal defect Right ventricular hypertrophy Overriding of the aorta 10/16/2024 40

41. Tof con. Pathophysiology The aorta originates from the right ventricle rather than the left, or it overrides a hole in the septum receiving blood from both ventricles. The pulmonary artery is stenosed , so that much lower than normal amounts of blood pass from the right ventricle into the lungs; Blood from the left ventricle flows either through a ventricular septal hole into the right ventricle and then into the aorta or directly into the aorta that overrides this hole. Because the right side of the heart must pump large quantities of blood against the high pressure in the aorta, its musculature is highly developed, causing an enlarged right ventricle 10/16/2024 41

42. Clinical manifestation Symptoms: Children present with cyanotic hands and feet Dyspnea on exertion or when crying Tet spells: irritability, cyanosis, hyperventilation and sometimes syncope or convulsions due to cerebral hypoxemia. Patients learn to alleviate symptoms by squatting which increases systemic resistance and decreases the right-to-left shunt and directs more blood to the pulmonary circulation. Children squat to enhance flow back to heart to oxygenate Physical exam Clubbing of the fingers and toes Delayed growth & development Systolic ejection murmur heard at the upper left sternal border created by turbulent blood flow through stenotic RV outflow tract Single 2nd heart sound 10/16/2024 42

43. ToF cont. Diagnosis CXR: - prominent RV - A boot shaped heart with narrow base & uplifted apex - decreased pulm . vascularity - Right side aortic arch seen in 20% of cases EKG: RVH, right axis deviation ECHO: displays and quantifies extent of RV outflow tract obstruction Complication - Cerebral thrombosis - in < 2 years - Brain abscess - Infective endocarditis - Polycythemia - CHF 10/16/2024 43

44. Treatment For Severe outflow obstruction Medical R x - PGE1 infusion - Prevent dehydration - Partial exchange transfusion - Patient given beta blockers for prophylaxis against Tet spells Surgery Total correction (Surgical closure of the VSD and enlargement of the pulmonary outflow tract) 10/16/2024 44

45. Tricuspid atresia It is a complete absence of the tricuspid valve No outlet from Right atrium to right vent . Usually associated with both ASD and VSD Therefore , there is an absence of right atrioventricular connection. This leads to a  hypoplastic  (undersized) or absent right ventricle Clinical Manifestation - Cyanosis at birth - Polycythemia - Easily fatiquability - Exertional dyspnea 10/16/2024 45

46. Cont… Diagnosis - EXR - Pulm . Under circulation - ECG -Lt axis deviation & Lt vent. hpertrophy - Echocardiography Treatment - PGE 1 - Surgery - Aortico - pulmonary Shunt - Bidirectional Glenn shunt(connecting SVC to pulmonary artery 10/16/2024 46

47. Cyanotic CHD With increased pulmonary blood flow Transposition of GA Transposition of the great vessels, a common cyanotic congenital anomaly , accounts for ≈5% of all congenital heart disease . Requires at least one shunt(ASD,VSD or both) A) D -TGA (uncorrected) Systemic venous return to Rt atrium Normal Pulmonary venous return to Lt atrium Aorta arises from Right ventricle Pulm . artery arises from Lt vent. Pathology 10/16/2024 47

48. TGA cont. Desaturated blood enters the right atrium, flows through the tricuspid valve into the right ventricle, and is ejected into the transposed aorta with resultant severe aortic desaturation. Fully saturated pulmonary venous blood flows into the left atrium, across the mitral valve into the left ventricle, and across the transposed pulmonary artery into the lungs. 10/16/2024 48

49. TGA cont. Clinical Manifestations - Tachypnea & cyanosis at birth - Rarely congestive heart failure B) L. TGA (corrected transposition) In l-transposition (l-TGA), the atrioventricular relationships are discordant: the right atrium is connected to the left ventricle and the left atrium to the right ventricle (also known as ventricular inversion). Clinical Manifestation Symptoms and signs are widely variable and are determined by the associated lesions. 10/16/2024 49

50. TGA cont. Diagnosis - Clinical - CXR - Cardiomegaly - Narrow mediastinum - Increased pulmonary blood flow - ECG - Echocardiography detect (RVH and left ventricular atrophy) Treatment - PGE 1 - emergency - Surgery 10/16/2024 50

51. Truncus arteriosus - Single arterial trunk for both pulm . & systemic circ. - 4 types (Type I, II, III, IV) depending the origin of pulmonary artery Clinical Manifestation - Cyanosis - CHF at 1-2 month - Systolic ejection m Diagnosis The ECG shows right, left, or combined ventricular hypertrophy. The chest x-ray also shows considerable variation like Cardiac enlargement Treatment - surgery 10/16/2024 51

52. Nursing Care Decrease Cardiac Demands Conserve energy for feeds Monitor temperature Minimize stress Minimize Respiratory Distress Elevate HOB Administer Oxygen Support Adequate Nutrition Feed infants every 3 hours High calorie formulas Monitor Fluids and Electrolytes Daily weight Strict I’s & O’s Potassium Perioperative surgical care 10/16/2024 52