Congenital Heart Disease

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

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

4. Patent Ductus Arteriosus Hemodynamics • As a result of higher aortic pressure, blood shunts L to R through the ductus from Aorta to PA.

5. Patent Ductus Arteriosus Clinical Signs & Symptoms • tachycardia • respiratoryproblems - shortnessofbreath • Poorgrowth • Differentialcyanosis - cyanosisofthelowerextremitiesbutnotoftheupperbody.

6. Patent Ductus Arteriosus Clinical Signs & Symptoms • Classiccontinuous machine-like murmur • It begins soon after onset of the 1st sound, reaches maximal intensity at the end of systole, and wanes in late diastole. • prominent apical impulseenlargedheart, • Leftsubclavicularthrill • Boundingpulse • Widenedpulsepressure

7. 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. • 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. • These changes lead to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV.

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

9. Ventricular Septal Defect 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) +/- • more prominent with small VSD, may be absent with a very large VSD.

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

11. 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). • Seen in 10% of all CHD. • There are 3 major types: • Secundum ASD • Primum ASD – low in the septum • •Sinus VenosusASD Sinus Venosus

12. 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.

13. 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) .

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

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

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

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

18. Pulmonary Stenosis • 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. • Most cases are isolated lesions

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

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

21. Right sided obstruction Tetralogy of Fallot • Obstruction of RV outflow (Pulmonary stenosis); • VSD; • Dextroposition of the aorta with override of the ventricular septum; • RV hypertrophy

22. 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 1st yr of life) • Digital clubbing and hyperpnea at rest are directly related to the degree of cyanosis • Tachycardia • Mental retardation • Retarded growth and development • RV heave • Systolic ejection murmur is heard along the left sternal border

23. Assessment Findings with Tetralogy of Fallot • Paroxymaldyspnea • Severe dyspnea on exertion • 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

24. 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

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

26. Treatment of the Child with TOF • Decrease cardiac workload • Prevention of intercurrent infection • Prevention of hemoconcentration • Surgical repair – palliative or corrective surgery

27. d-Transposition of the Great Arteries • 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

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

29. Transposition of the Great Arteries • 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.

30. Transposition of the Great Arteries Clinical Manifestations • Cyanosis, tachypneaare 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. • Physical findings, other than cyanosis, may be remarkably nonspecific. • Murmurs may be absent, or a soft systolic ejection murmur may be noted at the midleftsternal border.