Modul TGA Transposition of Great Arteries.pptx

Transposition of Great Arteries

1. Transposition of T he G reat A rteries ( TGA ) dr. Dita Maulida Anggraini Pembimbing : dr. Charlotte J. Cool, SpJP (K) dr. Achmad Fitrah Khalid, SpJP

2. DEFINITION A orta originates from morphologically right ventricle and pulmonary artery originates from morphologically the left ventricle , while the AV connections are normal (AV concordance with ventriculoarterial discordance) Transposition of the great arteries means that the origin of the great arteries from the heart is reversed Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss & Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins; 2013 Vijayalakshmi IB, Rao PS, Chugh R. A comprehensive approach to congenital heart diseases. JP Medical Ltd; 2013.

3. EPIDEMIOLOGY Occurring in 1:2000 to 4500 births Male to female ratio is 2:1, increasing to more than 3:1 for TGA with intact ventricular septum TGA accounting for 5% to 7% of all congenital cardiac malformations Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss & Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins; 2013 Vijayalakshmi IB, Rao PS, Chugh R. A comprehensive approach to congenital heart diseases. JP Medical Ltd; 2013.

4. The prefix D, d-looping of the cardiac tube during embryogenesis so that the RV ends up on right (D-transposition) and LV on left In complete transposition of the great arteries (TGA) Aorta arises anteriorly from the RV carrying desaturated blood, and pulmonary artery arises posteriorly from the LV carrying oxygenated blood. Aorta is located anteriorly and to the right of the PA This is in contrast to L-TGA where morphologic RV ends up on the left, thus L-transposition Vijayalakshmi IB, Rao PS, Chugh R. A comprehensive approach to congenital heart diseases. JP Medical Ltd; 2013. Park MK, Salamat M. Park's Pediatric Cardiology for Practitioners E-Book. Elsevier Health Sciences; 2020.

5. Infundibulum is subpulmonary , left-sided, anterior movement of pulmonary valve: posterior to anterior on the left side aortic valve remains stationary e.c lack of development of subaortic infundibulum prevents fibrous continuity between pulmonary and tricuspid valve infundibulum is subaortic, right-sided, and anterior abnormal development of subaortic infundibulum and absence of growth of subpulmonary infundibulum aortic valve is protruded superiorly and anteriorly -> above the anterior RV prevents fibrous continuity between aortic and tricuspid valve prevents normal movement of pulmonary valve -> abnormal pulmonary to mitral valve fibrous continuity NORMAL TGA Influenced by abnormal development, growth, and absoprtion of the distal infundibulum (conus) Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss & Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins; 2013 Vijayalakshmi IB, Rao PS, Chugh R. A comprehensive approach to congenital heart diseases. JP Medical Ltd; 2013.

6. The abnormal connections in ‘double’ discordance may be present in hearts abnormal base-apex orientation, especially dextrocardia (apex of the heart pointed to the right), is common (20%)

7. Classification Defects that permit mixing of the two circulations are necessary for survival D-transposition of the great arteries (D-TGA) complete separation of the pulmonary and systemic circulations hypoxemic blood throughout the body and hyperoxemic blood in the pulmonary circuit not compatible with survival

8. MANIFESTATION (D-TGA) Usually symptomatic and diagnosed due to sianotic complaint in early after birth Common presenting symptoms are cyanosis ECG: vary depdening on age, RAD, RVH/BVH, can dysrhythmias (mostly atrial flutter during or after BAS) MANIFESTATION (L-TGA) Usually asymptomatic and diagnosed during routine cardiac evaluation Common presenting symptoms are cyanosis and dyspnea (more) Chest X-ray: dextrocardia or abnormal cardiac silhouette of L posed aorta ECG: superior QRS axis with atypical septal activation Echocardiography is diagnostic Cardiac MRI is useful to study complex LVOTO and baffling difficult VSD Vijayalakshmi IB, Rao PS, Chugh R. A comprehensive approach to congenital heart diseases. JP Medical Ltd; 2013. Park MK, Salamat M. Park's Pediatric Cardiology for Practitioners E-Book. Elsevier Health Sciences; 2020.

9. In the neonate particularly, the findings may be normal Radiologic Features D-TGA & L-TGA In TGA/IVS, the diagnostic triad: (a) oval or egg-shaped cardiac silhouette with narrow superior mediastinum (b) mild cardiomegaly (c) increased pulmonary vascular markings Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss & Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins; 2013

10. Echocardiography Features D-TGA Echocardiography has become the diagnostic method of choice in TGA Subcostal imaging provides a flexible acoustic window that visualize of the great arteries and respective ventricular connections Fetal echocardiography to early diagnose TGA can be performed Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss & Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins; 2013

11. TGA is diagnosed by transthoracic echocardiography Attention to: AV valve annulus size ventricular size subpulmonary obstruction morphology of semilunar valves size discrepancy of the main pulmonary artery versus the ascending aorta coronary artery anatomy Vijayalakshmi IB, Rao PS, Chugh R. A comprehensive approach to congenital heart diseases. JP Medical Ltd; 2013.

12. In the parasternal long-axis view, the great artery arising from the posterior ventricle (LV) has a sharp posterior angulation toward the lungs, which suggests that this artery is the PA. In contrast to the normal intertwining of the great arteries, the proximal portion of the great arteries runs parallel. Unlike in a normal heart, there is a fibrous continuity between the pulmonary and mitral valves, and subaortic conus is present. (In normal hearts, there is aortic–mitral fibrous continuity with subpulmonary conus)

13. In the parasternal short-axis view, the “circle and sausage” appearance of the normal great arteries is not visible. Instead, the great arteries appear as “double circles”. The PA is in the center of the heart, and the coronary arteries do not arise from this great artery. The aorta is usually anterior to and slightly to the right of the PA, and the coronary arteries arise from the aorta.

14. D-TGA Surgical Management Park’s Pediatric Cardiology for Practitioners 7 th edition. Myung K. Park. 2021

15. Atrial level Operation Senning Senning operation uses the patient’s own atrial septal flap and the RA free wall to redirect the venous returns Mustard Mustard operation uses a pericardial or a prosthetic baffle

16. Ventricular level Operation

17. Réparation à Iʼétage Ventriculare Rastelli Procedure Nikaidoh Procedure

18. VentricLE level Operation

19. GREAT ARTERIES LEVEL - ARTERIAL SWITCH OPERATION (ASO) Arterial switch operation is now firmly established as the procedure of choice not only for simple TGA but also TGA with other associated anomalies (e.g., VSD or PDA) The coronary arteries are transplanted to the PA, and the proximal great arteries are connected to the distal end of the other great artery, resulting in an anatomic correction Usually performed within the first 3 weeks of life Park’s Pediatric Cardiology for Practitioners 7 th edition. Myung K. Park. 2021

20. ARTERIAL SWITCH OPERATION Park’s Pediatric Cardiology for Practitioners 7 th edition. Myung K. Park. 2021

21. ARTERIAL SWITCH OPERATION Complication: Coronary artery obstruction Supravalvar PS at the anastomosis site Neoaortic valvar regurgitation and supravalvar neoaortic stenosis (rare) Park’s Pediatric Cardiology for Practitioners 7 th edition. Myung K. Park. 2021

22. LTGA / ccTGA ( Congenitally Corrected Trasnsposition of Great Arteries )

23. Congenitally corrected transposition of the great arteries ( ccTGA )

24. L-TGA Prevalency Occurs in fewer than 1% of all patients with CHDs Slight male predominance Natural History Life expectancy: if associated lesion, 50% alive at age of 40 years; without associated lesion, 50% alive at age of 60 years Die from CHF or SCD

25. Pathology The visceroatrial relationship is normal, but there is ventricular inversion Ventricular inversion  the morphologic RV with tricuspid valve is located to the left of the morphologic LV, which has the mitral valve The great arteries are transposed  with the aorta rising from the RV and the PA rising from the LV. The aorta is usually located anterior to and left of the PA.

26. Pathology No functional abnormalities exist, but unfortunately, most cases are complicated by associated intracardiac defects: VSD occurs in 80% of all cases. PS, both valvular and subvalvular , occurs in 50% of patients and is usually associated with VSD. Systemic AV valve (tricuspid valve) regurgitation occurs in 30% of patients. Varying and progressive degrees of AV block and paroxysmal supraventricular tachycardia (SVT) frequently occur The coronary arteries show a mirror-image distribution  Essentially, coronary arteries follow the ventricles.

27. Clinical Manifestation Isolated ccTGA rarely develop complication before adulthood Late complications: Systemic RV failure Progressive TR LVOTO TAVB VT History During childhood, patients are asymptomatic when L-TGA is not associated with other defects. During the first months of life, most patients with associated defects become symptomatic with cyanosis resulting from VSD and PS or CHF resulting from a large VSD.

28. Clinical Manifestation Physical Examination Cyanotic if PS and VSD are present. PS  systolic thrill, large VSD  Hyperactive precordium The S2 is loud and single at the upper left or right sternal border VSD or systemic AV valve regurgitation  grade 2 to 4 of 6 harsh holosystolic murmur along the lower left sternal border PS  grade 2 to 3 of 6 ejection systolic murmur at the upper left or right sternal border

29. Clinical Manifestation ECG The absence of Q waves in V5 and V6 or the presence of Q waves in V4R or V1  characteristic of the condition Normal P, but the QRS complexes suggest dominance or hypertrophy of the right-sided ventricle (anatomic LV). Varying degrees of AV block are common (1 st degree 50%, 3 rd degree 4%) Atrial arrhythmias and Wolff-Parkinson-White (WPW) pre-excitation are occasionally present.

30. Clinical Manifestation Radiograpghy A straight, left upper cardiac border, formed by the ascending aorta  a characteristic finding VSD  Cardiomegaly and increased pulmonary vascular markings S evere left-sided AV valve regurgitation  Pulmonary venous congestion and left atrial enlargement Positional abnormalities (e.g., dextrocardia, mesocardia) may be present.

31. Echocardiography In the parasternal short-axis scan, a “double circle” of the semilunar valves is imaged instead of the normal “circle and sausage” pattern The LA is connected to the tricuspid valve (which has a more apical attachment to the ventricular septum than the other) Looked for: type and severity of PS, size and location of VSD, straddling of the AV valve

32. Management Medical Treatment with anticongestive agents is necessary if CHF develops. Antiarrhythmic agents are used for arrhythmias. Surgical Palliative: modified Blalock- Taussig (BT) shunt is necessary for patients with severe PS, PA banding may be needed for uncontrollable CHF in early infancy. Definitive: classic repair and anatomic repair

33. Surgical Management Patients with regurgitation of the tricuspid valve (systemic AV valve) or RV dysfunction need the anatomic repair (LV is made the systemic ventricle) Classic Repair (the anatomic RV remains as the systemic ventricle) Competent tricuspid valve (or left AV valve) and good RV function are required Patients with VSD  the VSD is closed through atrial approach VSD and PS (or LVOT obstruction)  the VSD is closed and an LV-to-PA conduit is placed Park’s Pediatric Cardiology for Practitioners 7 th edition. Myung K. Park. 2021

34. Surgical Management Anatomic Repair VSD patient  A combination of Senning procedure (which is an atrial switch operation) and ASO (“double-switch” operation). Hospital mortality rate is around 10% VSD and PS patient  A combination of the Senning and Rastelli operations is performed. Hospital mortality rate is around 10% Fontan -Type Operation  p atients with complex intracardiac anatomies (hypoplasia of one ventricle, straddling AV valves, or multiple VSDs) Park’s Pediatric Cardiology for Practitioners 7 th edition. Myung K. Park. 2021

35. THERAPY L-TGA Allen et al. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents. 7 th edition. Philadelphia: Lippincott Williams & Wilkins. 2008. p1038-87

36. TERIMAKASIH