2. Congenital Heart Disease Prof. Pavlyshyn H.A.

3. Differential cyanosis 1. pink upper, blue lower CoA (Coarctation of the aorta), IAA (Interrupted aortic arch), Pulm HtnCoarctation of the aortaInterrupted aortic arch 2. blue upper, pink lower Transposition of the great vessels Transposition of the great vessels d-TGA with pulm Htn dextro-Transposition of the great arteries dextro-Transposition of the great arteries *indicates serious underlying cardiac or lung disease* Recognition of Cyanosis

5. Patent Ductus ArteriosusHemodynamics As a result of higher aortic pressure, blood shunts L to R through the ductus from Aorta to PA.As a result of higher aortic pressure, blood shunts L to R through the ductus from Aorta to PA.

6. Patent Ductus Arteriosus Clinical Signs & Symptoms tachycardiatachycardia respiratory problems - shortness of breathrespiratory problems - shortness of breath shortness of breath shortness of breath Poor growthPoor growth Differential cyanosis - cyanosis of the lower extremities but not of the upper body.Differential cyanosis - cyanosis of the lower extremities but not of the upper body.

7. Patent Ductus Arteriosus Classic continuous machine-like murmurClassic continuous machine-like murmurmurmur It begins soon after onset of the 1st sound, reaches maximal intensity at the end of systole, and wanes in late diastole.It begins soon after onset of the 1st sound, reaches maximal intensity at the end of systole, and wanes in late diastole. prominent apical impulse enlarged heart,prominent apical impulse enlarged heart, enlarged heart enlarged heart Left subclavicular thrillLeft subclavicular thrill Bounding pulseBounding pulseBounding pulseBounding pulse Widened pulse pressureWidened pulse pressure Clinical Signs & Symptoms

8. Ventricular Septal Defect During systole some of the blood from the LV leaks into the RV, passes through the lungs and reenters the LV via the pulmonary veins and LA.During systole some of the blood from the LV leaks into the RV, passes through the lungs and reenters the LV via the pulmonary veins and LA. Such circuitous route of blood causes volume overload on the LV.Such circuitous route of blood causes volume overload on the LV. The LV normally has a much higher systolic pressure (~100 mm Hg) than the RV (~85 mm Hg) and through VSD blood leaks into the RV and elevates RV pressure and volume, causing Pulm HTN.The LV normally has a much higher systolic pressure (~100 mm Hg) than the RV (~85 mm Hg) and through VSD blood leaks into the RV and elevates RV pressure and volume, causing Pulm HTN. These changes lead to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV.These changes lead to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV.

9. Ventricular Septal Defect Clinical Signs & Symptoms Small - moderate VSD, 3-6mm, are usually Small - moderate VSD, 3-6mm, are usually asymptomatic. asymptomatic. Small defects located predominantly in the muscular septum with slight hemodynamic impairment (Tolochinov-Roge disease) Moderate – large VSD, almost always have Moderate – large VSD, almost always have symptoms and will require surgical repair. symptoms and will require surgical repair.

10. Listen at the back for radiation of murmurs Pansystolic/holosystolic murmur - loud, harsh, blowing heard best over the LLSB, frequently is accompanied by thrill (depending upon the size of the defect) +/- Pansystolic/holosystolic murmur - loud, harsh, blowing heard best over the LLSB, frequently is accompanied by thrill (depending upon the size of the defect) +/- more prominent with small VSD, may be absent with a very large VSD. more prominent with small VSD, may be absent with a very large VSD. Ventricular Septal Defect

11. І ІІ ІІІ AVR AVL AVF V1 V2 V3 V4 V5 V6 ECG: overload of LV and RV LA, LV or biventricular hypertrophy. RV hypertrophy predominates when pulmonary vascular resistance is high.

12. Atrial Septal Defect - ASD is a form of CHD that enables blood flow between the left and right atria via the interatrial septum (it is possible for blood to travel from the left side to the right side of the heart).is a form of CHD that enables blood flow between the left and right atria via the interatrial septum (it is possible for blood to travel from the left side to the right side of the heart).interatrial septuminteratrial septum Seen in 10% of all CHD.Seen in 10% of all CHD. There are 3 major types: Secundum ASDSecundum ASD Primum ASD – low in the septumPrimum ASD – low in the septum Sinus Venosus ASD Sinus Venosus ASD Sinus Venosus

13. ASD with left-to-right shunt In the case of a large ASD (>9mm), may result in left-to-right shunt, blood will shunt from the LA to the RA. This extra blood may cause a volume overload of both the right atrium and the right ventricle. Ultimately the RV must push out more blood than the LV due to the L-to-R shunt. This condition can result in eventually RV-failure (dilatation and decreased systolic function) and Pulm Htn.

14. Listen carefully Systolic ejection murmur – its medium pitched, seldom accompanied by a thrill, and best heard at the LSB (left middle and upper sternal border); Short, rumbling mid-diastolic murmur produced by the increased volume of blood flow across the tricuspid valve is often audible at the LLSB (lower left sternal border).

15. Atrial Septal Defect Diagnosis X-ray chest: pulmonary vascularity is increased X-ray chest: pulmonary vascularity is increased ECG: right-axis deviation;ECG: right-axis deviation; Echo-CG: RV is enlarged, defect is visualized;Echo-CG: RV is enlarged, defect is visualized;

16. Coarctation of the Aorta Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation.Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation. Male: Female ratio 3:1.Male: Female ratio 3:1. Accounts for 7 % of all CHD.Accounts for 7 % of all CHD.

17. Coarctation of the Aorta Hemodynamics Obstruction of left ventricular outflow  LV afterload increases  pressure hypertrophy of the LV.Obstruction of left ventricular outflow  LV afterload increases  pressure hypertrophy of the LV.

18. Coarctation of the Aorta Clinical Signs & Symptoms Sings of low cardiac output, poor peripheral perfusion - LE hypoperfusion, acidosis, HF and shock.Sings of low cardiac output, poor peripheral perfusion - LE hypoperfusion, acidosis, HF and shock. Decreased and delayed pulses in lower extremities.Decreased and delayed pulses in lower extremities. Systolic ejection murmur @ LSB.Systolic ejection murmur @ LSB. Cardiomegaly, rib notching on X-ray.Cardiomegaly, rib notching on X-ray.

19. Pulmonary Stenosis Pulmonary Stenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract.Pulmonary Stenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract. Accounts for 7-10% of all CHD.Accounts for 7-10% of all CHD. Most cases areMost cases are isolated lesions

20. Pulmonary Stenosis Hemodynamics RV pressure hypertrophy  RV failure.RV pressure hypertrophy  RV failure. RV pressures maybe > systemic pressure.RV pressures maybe > systemic pressure. Post-stenotic dilation of main PA.Post-stenotic dilation of main PA. W/intact septum & severe stenosis  R-L shunt through FO  cyanosis.W/intact septum & severe stenosis  R-L shunt through FO  cyanosis. Cyanosis is indicative of Critical PS.Cyanosis is indicative of Critical PS.

21. Pulmonary Stenosis Clinical Signs & Symptoms Depends on the severity of obstruction.Depends on the severity of obstruction. Asymptomatic w/ mild PS < 30mmHg.Asymptomatic w/ mild PS < 30mmHg. Mod-severe: 30-60mmHg, > 60mmHgMod-severe: 30-60mmHg, > 60mmHg Prominent jugular a-waveProminent jugular a-wave RV lift, RV heaveRV lift, RV heave Split 2 nd hrt soundSplit 2 nd hrt sound Ejection click, followed by systolic murmur.Ejection click, followed by systolic murmur. Heart failure & cyanosis not relieved by inhaled oxygen seen in severe cases.Heart failure & cyanosis not relieved by inhaled oxygen seen in severe cases.

22. Right sided obstruction 1.Obstruction of RV outflow (Pulmonary stenosis); 2.VSD; 3.Dextroposition of the aorta with override of the ventricular septum; 4.RV hypertrophy Tetralogy of Fallot

23. Assessment Findings with Tetralogy of Fallot Symptoms are variable depending of degree of obstruction Cyanosis – is variable (isn’t present at the birth, occurs later in the 1 st yr of life)Cyanosis – is variable (isn’t present at the birth, occurs later in the 1 st yr of life) Digital clubbing and hyperpnea at rest are directly related to the degree of cyanosis Digital clubbing and hyperpnea at rest are directly related to the degree of cyanosis TachycardiaTachycardia Mental retardationMental retardation Retarded growth and developmentRetarded growth and development RV heaveRV heave Systolic ejection murmur is heard along the left sternal borderSystolic ejection murmur is heard along the left sternal border

24. Assessment Findings with Tetralogy of Fallot Paroxymal dyspnea Paroxymal dyspnea Severe dyspnea on exertion Severe dyspnea on exertion Squatting position for the relief of dyspnea caused physical effort,Squatting position for the relief of dyspnea caused physical effort, “Blue” spells, “tet” spells, paroxysmal hypercyanotic attacks – infant becomes hyperpnea, restless, cyanosis increases, gasping respirations, syncope “Blue” spells, “tet” spells, paroxysmal hypercyanotic attacks – infant becomes hyperpnea, restless, cyanosis increases, gasping respirations, syncope

25. Hypercyanotic Spells/Blue Spells/Tet Spells Clinical Manifestations ٭ Most often occurs in morning after feedings, defecation, or crying ٭ Acute cyanosis ٭ Hyperpnea ٭ Inconsolable crying ٭ Hypoxia which leads to acidosis

26. Chest X-Ray Decreased pulmonary vascular marking Decreased pulmonary vascular marking “Boot-shaped heart” “Boot-shaped heart”

27. Treatment of the Child with TOF Decrease cardiac workloadDecrease cardiac workload Prevention of intercurrent infectionPrevention of intercurrent infection Prevention of hemoconcentrationPrevention of hemoconcentration Surgical repair – palliative or corrective surgerySurgical repair – palliative or corrective surgery

28. Pathophysiology – –Cyanosis due to failure of delivery of pulmonary venous blood to the systemic circulation – –Two parallel circulations with no mixing – –Open atrial septum (fossa ovalis) allows some left-to- right shunt, enhanced by a left-to-right ductus arteriosus shunt – –Presence of ventricular septal defect facilitates mixing d-Transposition of the Great Arteries

29. Transposition of the Great Arteries Aorta from right ventricle, pulmonary artery from left ventricle.Aorta from right ventricle, pulmonary artery from left ventricle. Cyanosis from birth, hypoxic spells sometimes present.Cyanosis from birth, hypoxic spells sometimes present. Heart failure often present.Heart failure often present. Cardiac enlargement and diminished pulmonary artery segment on x-ray.Cardiac enlargement and diminished pulmonary artery segment on x-ray.

30. Transposition of the Great Arteries Anatomic communication must exist between pulmonary and systemic circulation, VSD, ASD, or PDA.Anatomic communication must exist between pulmonary and systemic circulation, VSD, ASD, or PDA. Untreated, the vast majority of these infants would not survive the neonatal period.Untreated, the vast majority of these infants would not survive the neonatal period.

31. Transposition of the Great Arteries Clinical Manifestations Cyanosis, tachypnea are most often recognized within the 1st hrs or days of life.Cyanosis, tachypnea are most often recognized within the 1st hrs or days of life. Hypoxemia is usually moderate to severe, depending on the degree of atrial level shunting and whether the ductus is partially open or totally closed. Hypoxemia is usually moderate to severe, depending on the degree of atrial level shunting and whether the ductus is partially open or totally closed. Physical findings, other than cyanosis, may be remarkably nonspecific. Physical findings, other than cyanosis, may be remarkably nonspecific. Murmurs may be absent, or a soft systolic ejection murmur may be noted at the midleft sternal border.Murmurs may be absent, or a soft systolic ejection murmur may be noted at the midleft sternal border.