Atrial septal defect

ASD

1. ATRIAL SEPTAL DEFECT Dr. Dhanesh kumar

2. DEFINITION An ASD is a hole of variable size in the atrial septum.

3. HISTORY Galen - foramen ovale and normal postnatal closure Leonardo Da Vinci- Described ASD in human autopsy specimen

4. Murray(1948) - external suturing Charles Bailey - atrioseptopexy Tyge sondergard – purse string external suture Gross(1950) - atrial – well technique Lewis and Taufic (1953) -inflow – occlusion technique with surface cooling and circulatory arrest 1 st successful open heart ASD closure

5. Gibbon(1953) - first time used CPB to close ASD Mills and King -Device closure of (1974) ASD

6. EMBRYOLOGY Embryologically , the septum primum separates the two atria first, moving inferiorly toward the endocardial cushions. The ventricular septum forms by moving upward from the ventricles to the endocardial cushions at the same time. If the atrial septum does not make it all the way, the residual defect in the septum primum ( ostium primum ) results in the primum ASD.

7. If the septum primum makes it all the way, a hole or holes (fenestrations) form in the middle of the septum ( forming the ostium secundum ). A second septum then moves down the right side of the first and normally covers the ostium secundum hole. If it does not cover the hole, a secundum ASD is present.

8. The septum secundum normally completely covers the right side of the atrial septum except for an ovale hole in it (the foramen ovale ). If the septae do not fuse, a patent path from the RA to the LA persists (the patent foramen ovale PFO).

9. The most common form of ASD (80% of cases) is persistence of the ostium secundum in the mid septum; less commonly, the ostium primum (which is low in the septum) persists

10. Development of inter atrial septum

11. TYPE OF ATRIAL SEPTAL DEFECT OSTIUM SECUNDUM OR FOSSA OVALIS OSTIUM PRIMUM SEPTAL DEFECT SINUS VENOSUS SEPTAL DEFECT POSTERIOR DEFECT CONFLUENT DEFECT

12. ASD

13. OSTIUM SECUNDUM ASD Results either from shortening of the valve of the foramem ovale,excessive resorption of the septum primum or deficient growth of septum secundum Mc type 50-70% of all ASD APVC is present in 10% case Female to male ratio – 2 : 1

14. Located in the region of fossa ovalis bounded by limbus anteriorly, superiorly, and posteriorly

15. OSTIUM PRIMUM ASD REPRESENT ABSENCE OF THE ATRIOVENTRICULAR SEPTUM 30% of all ASD

16. Ostium Primum Next most common type Located in the lower portion of the atrial septum. Will often have a mitral valve defect associated with it called a mitral valve cleft. A mitral valve cleft is a slit-like or elongated hole usually involves the anterior l eaflet of the mitral valve.

17. Sinus Venosus Least common type of ASD Located in the upper portion of the atrial septum. Association with an abnormal pulmonary vein connection Four pulmonary veins, two from the right lung and two from the left lung, normally return red blood to the left atrium. Usually with a sinus venosus ASD, a pulmonary vein from the right lung will be abnormally connected to the right atrium instead of the left atrium. This is called an anomalous pulmonary vein. ..\asd-veno.jpg

18. SINUS VENOSUS ASD

19. CORONARY SINUS DEFECT Part of unroofed coronary sinus syndrome Uncommon Opening in the wall of distal end of coronary sinus or unroofing caused by absence of the partition b/w the coronary sinus and lt atrium. Accompanied by a left SVC

20. POSTERIOR DEFECT POSTERIOR AND INFERIOR PART OF ATRIAL SEPTUM WITH ABSENCE ,HYPOPLASIA OR ANT. DISPLACEMENT OF POS. LIMBUS

21. CONFLUENT DEFECT A confluence of two defects Fossa ovalis defect with absence of posterior limbus Fossa ovalis and coronary ASD

22. Redundancy of the valve of the foramen ovale can result in an atrial septal aneurysm. Respiratory excursion greater then 10 mm.ASA 2-4 % normal population and 70% of cases are associated with a PFO.

23. Patent foramen ovale Remnant of fetal circulation Beneath superior limbus, between it and floor of fossa ovalis Behaves like flap valve

24. Failure of septum primum and septum secundum to fuse 1. valve competent “ probe patent “ PFO 2.valvular incompetence with or without an aneurysm of septum primum component Incidence at birth (full – term ) - 60% Incidence in normal adult heart - 25% - 30% Paradoxical emboli

25. Partial anomalous pulmonary venous connection exist when one or more but not all pulmonary vein connect anomalously to the rt atrium. Total anomalous pulmonary venous connection applies when all four pulmonary vein connect anomalously to the rt atrium either directly or indirectly. 10-15% OS ASD are associated with partial anomalous PVC.

26. 80-90% SVC sinus venous defect are associated with anomalous connection of rt sup pulmonary vein to rt atrium or SVC. Approximately 90% of PAPVC join the rt upper and middle lobe pulmonary vein to the rt atrium and SVC. Anomalous connection of lt pulmonary vein is far less prevalent (about 10% ) as anomalous connection of rt pulmonary vein. b/l partial anomalous pulmonary venous connection rare.

27. A. rt pulmonary vein drain into SVC with sinus venosus ASD B . rt inferior pulmonary vein drain in to IVC C.lt pulmonary vein drain into lt innominate vein D . Lt pulmonary vein drain into coronary sinus

28. The scimitar syndrome Described by Chassinat in 1836 Rare anomaly Characterized by connection of all of the rt pulmonary vein to inferior vena cava and usually by hypoplasia of the ipsilateral lung and pulmonary artery. X ray shadow that resembles the shape of Turkish sword . Rarely involve lt side.

29. Lower portion of rt lung perfused by systematic artery from abdominal aorta. Associated anomalies ASD aortic coarctation left sided superior vena cava.

30. A –rt pulmonary vein converge to form a vascular trunk that drain in to inferior vena cava B – levophase following injection of contrast material into pulmonary trunk shows the common pulmonary venous channel C- Turkish sword

31. ASD: Associated cardiac anomalies ASD must for survival in TGA, TAPVC, and Tricuspid atresia PAPVC VSD -18% PDA PS -31% AS MS 29% COA

32. LSVC - 5% Lutembacher’s syndrome Cleft AML Mitral valve prolapse TOF

33. Cardiac chambers in ASD RT atrium enlarged and thick walled. RV diastolic size increased. Normal RV diastolic dimension are b/w .6 and 1.4 cm/ m2. in pt with atrial level large lt to rt shunts average 2.66cm/m2 ,may be large as 4 cm/m2. Cardiac apex often formed by the RV. LV normal or slightly decrease in size .

34. Mitral valve and ASD Mitral valve abnormalities consist of thickening and fibrosis of leaflets and chordae tendinae attributed to traumatic cusp movement resulting from left ventricular deformity. Mitral prolapse prevalence of prolapse is about 20%. Increasing with age and magnitude of pulmonary to systematic blood flow ratio (Qp/Qs) Schreiber and colleagues have clarified a previously confused subject by relating mitral valve prolapse to abnormalities of LV shape in pt with ASD .

35. Mitral regurgitation -prevalence of severe mitral regurgitation 2-10%. Cleft mitral leaflets -cleft anterior and posterior leaflets that cause mitral regurgitation are reported to occurs occasionally .

36. Lung and pulmonary vasculature Pulmonary artery dilated which tend to compress the smaller airway which result retention of secretion and bronchiolitis. Hypertensive pulmonary vascular disease develops infrequently in pt with ASD , usually not until the third or forth decade of life. This contrast sharply with VSD ,complete AV septal defect and PDA in which pulmonary vascular disease may be present early in life.

37. In ASD ,pulmonary vascular disease is caused mainly by secondary thrombus in the dilated pulmonary artery branches , with changes in the intima and media of vessels usually playing minor role. ASD with pulmonary HTN are relatively common in pt born at high altitude.

38. Clinical feature and diagnostic criteria The female to male ratio 2:1 in pt with an os secundum ASD while sinus venosus ASD have sex ratio that are approximately equal. OS ASD Some time familial Holt- Oram syndrome -AD

39. ASD may be go unrecognized for decades b/a symptoms are mild or absent and physical signs are subtle. An important exception is the symptomatic infant with an OS ASD in whom congestive heart failure may be followed by spontaneous closure. ASD occurs as an isolated anomaly in 5-10% of all congenital heart defect.

40. Dyspnea and fatigue are the earliest symptoms of an OS ASD. Orthopnea Platypnea –orthodeoxia rare syndrome Recurrent lower respiratory tract infection Older patient deteriorate chiefly on three counts- 1- A decrease lt ventricular destensibility associated with aging , IHD , systematic HTN or acquired calcific aortic stenosis augment the lt to rt shunt

41. 2-Age related increase in prevalence paroxysmal atrial tachycardia , atrial fibrillation and atrial flutter precipitate congestive heart failure. 3- Mild to moderate pulmonary HTN in older adult occurs in the face of a persistent lt to rt shunt Children with ASD may have a delicate gracile habitus with wt more affected then height. May have lt Precordial bulge with Harrison's grooves.

42. Cyanosis also occurs when a large Eustachian valve selectively channels inferior vena caval blood into the left atrium through an OS ASD or through an inferior vena caval sinus venosus defect.

43. Natural history survival 0.1% of individuals born with large ASD die in infancy Few die in 1 st or 2 nd decade of life 5% - 15% die in 3 rd decade of life ( PHT, Eisenmenger’s syndrome) Premature late death with CHF occurs in increasing proportion after 5 th decade Lifetime risk of mortality 25%

44. Natural history Functional status Presence and severity of functional limitation increase with age 1% with large ASD have symptoms during 1 st yr of life Most patients asymptomatic through 1 st and 2 nd decade , although many are shorter and thin. Effort intolerance and easy fatigability develop in 2 nd or 3 rd decade.

45. CHF common after 40 yr age Atrial arrhythmia incidence increase with age - 13% in >40 yr age 52%in 60 yr age Mitral incompetence - 2.5% to 10% Tricuspid regurgitation - annular dilation Bacterial endocarditis - rare Paradoxical emboli

46. Natural history Pulmonary hypertension Occur in 5% - 10% untreated ASD, predominantly female Occur after 20 yr age Higher incidence at high altitude Incidence increases with advancing age

47. Clinical features Most infants - asymptomatic Older children with moderate LTRS - asymptomatic - mild fatigue or dysponea Children with large LTRS - fatigue - dysponea on exertion - recurrent RTI - growth retardation

48. Adult patients - dysponea on exertion - palpitation - fatigue - CHF

49. Clinical features cyanosis Streaming of desaturated IVC blood across ASD, caused by persistently enlarged Eustachian valve that baffles blood flow into LA Advanced irreversible PHT

50. Physical examination Inspection Precordial bulge Hyperdynamic cardiac impulse Leftward displacement of apex

51. Clinical sign diagnostic of a large (Qp/Qs greater then 1.8 to 2.0) shunt at atrial level are as follow- Overactive left parasternal systolic lift Fixed splitting second heart sound through out the respiratory cycle A soft pulmonary mid systolic flow murmur(in second and third ICS) A mid –diastolic tricuspid flow murmur(in fourth and fifth ICS

52. Palpation Right ventricular heave A dilated pulsatile pulmonary trunk is palpable in 2 nd Lt intercostal space.

53. Auscultation First heart sound- - split at lower sternal edge and apex and the tricuspid component is loud. Pulmonary systolic murmur – - begins immediately after first heart sound

54. Wide fixed splitting S2- - wide splitting- .prolong RV systole .prolong hang out interval (decrease impedance) .delayed electrical activation of RV If associated with RBBB - Fix splitting – . The phasic changes in systemic venous return during respiration are associated with reciprocal changes in the volume of lt to rt shunt minimizing respiratory variation in the RV filling ie . (hangout interval-time interval from the cross over of the pressure to actual closer)

55. -increase venous return during inspiration is associated with no lt to rt shunt through interatrial communication -decrease venous return during expiration is associated with lt to rt shunt through interatrial communication minimizing the respiratory variation in RV filling. Mid – diastolic murmur at tricuspid area

56. The loud second component of split first heart sound(T1) is tricuspid and maximum at lower sternal edge A soft pulmonary systolic murmur in the 2 nd ICS lt side followed by wide fix splitting to second heart sound

57. ECG Sinus node dysfunction and accelerated atrial rhythm Atrial fibrillation, atrial flutter and supraventricular tachycardia PR interval prolonged The P wave axis with OS ASD is inferior and to the left with upright P waves in leads II,IIIand Avf. The P wave axis with superior vena caval sinus venosus ASD is leftward with inverted P wave in lead II,III,avf and upright P wave in lead v1.

58. rSr or rsR pattern in right Precordial leads RVH, RAD . Incomplete or complete right bundle branch block is present in nearly all cases of ASD, and superior axis deviation is noted in the AV canal defect , where complete heart block is often seen as well

59. The X ray Increase pulmonary vascularity extend periphery of lung field. Pulmonary trunk and its branch dilated Rt atrial enlargement characteristic Enlarged rt ventricle occupies the apex and form acute angle with lt hemi diaphragm.

60. Echocardiogram M - mode Enlargement of RA and RV dimensions Paradoxical ventricular septal motion – parasternal short – axis view Mitral valve motion - normal

61. Echocardiogram 2 D echocardiography Subcostal 4 – chamber view Echo – free space in mid – atrial septum Echo – free space in posterosuperior atrial septum in sinus venosus ASD Dilated coronary sinus and communication at level of orifice of coronary sinus with LA

62. 2DECHO- sub costal transducer position A ,sinus venosus defect-defect located in the posterosuperior atrial septum,beneath the orifice of SVC B . Secundum ASD – the defect is located in middle portion of the atrial septum C . Primum ASD-the defect is located in the antero inferior atrial septum just over the inflow portion of each Atrioventricular valve

63. Echocardiogram Pulsed Doppler Echocardiography Shunting typically begins in mid – systole Velocity and volume of blood progressively decrease until early diastole Early in ventricular systole – transient RTLS Determines Qp : Qs RV systolic pressure Pulmonary artery pressure

64. Echocardiography Color Doppler Echocardiography demonstrates shunt

65. Real time imaging identified a dilated hyperkinetic rt ventricle with paradoxical motion of the ventricular septum and vigorous pulsation of pulmonary trunk and its branch.

66. Cardiac catheterization Catheter course - across mid – septal portion Oxymetry - increase in oxygen saturation of 10 % or more from svc to RA in one series of blood samples or increase of 5% in two series of samples

67. Cardiac catheterization pressure measurement Equal mean pressure in RA and LA a and v waves in RA - same magnitude, appear as M – shaped RV systolic pressure slightly increased to 25 to 35 mm Hg Pulmonary artery pressure normal to slightly increased

68. Cardiac catheterization Angiocardiography cranially angulated LAO projection - defect in middle of IAS in secundum ASD,defect at the top in sinus venosus ASD,defect at bottom in AVSD For coronary sinus ASD selective injection into LSVC, pulmonary vein, or LA shows passage of contrast into LA, inferiorly to coronary sinus, and then into right atrium (in absence of increased RA pressure)

69. Cardiac catheterization- indication In infants (b/a possible associated anomalies) In many adult (for assessing possible pulmonary HTN and status of mitral valve) In any pt in whom noninvasive test suggest PAPVC Pt older then 35-40 years

70. Spontaneous closure In pt with an ASD less then 3 mm in size diagnosed before 3 month of age , spontaneous closure occurs in 100% of pt at 1 and1/2 yr of age. More 80 % in b/w 3 and 8 mm In greater then 8 mm – rarely closes spontaneously Spontaneous closure of hemodynamically significant isolated ASD occasionally occurs in 1 st year. Spontaneous closer uncommon after first year.

71. Indication of operation Elective closer of ASD is generally recommended when the Qp:Qs is 1.5:1 or greater . Ideally performed at the age 2-5 yr. An echo diagnosis of a significant defect with rt ventricular volume overload is common and sufficient indication to close ASD.

72. Contraindication Irreversible pulmonary HTN Pulmonary vascular disease of sufficient severity to raise PVR 8-12 wood/m2 at rest and prevent its decrease to less then 7 wood/m2 with a pulmonary vasodilator is contraindication for surgery.

73. Trans – catheter Device closure of ASD Indicated in- Os secundum ASD 5mm or more in diameter (but less then 32 mm) A significant Lt to Rt shunt with clinical evidence of rt ventricular overload( i.e. Qp/Qs 1.5:1 or greater or RV enlargement) There must be enough rim(4mm) of septal tissue around the defect for appropriate placement of the device

74. Defect unsuitable for device closer Failed attempted device closure Those without sufficient septal rim to engage the device Sinus venosus defect for which device closer would threaten obstruction of pulmonary veins ,IVC and SVC.

75. Trans – catheter devices Amplatzer septal occluder – FDA approved Helix septal occluder Rashkind ASD occluder Lock – USCI Clamshell occluder Cardioseal device Sideris Buttoned device ASD occlusion system (ASDOS) Das – Angel wings occlusion device Trans catheter polyurethane foam patch

76. Trans – catheter devices

77. Trans-catheter Approach Device is advance through an introducer sheath One- half of the device is deployed on left side of atrial septum, the second half is deployed on the right side A “sandwich” is formed over the defect 6-8 weeks, device as a frame work for scar tissue to form In children the new tissue formation with continue to grow

78. Trans-catheter Approach

79. Complication of Percutaneous Intervention 8% Device malposition or dislocation Device embolization Arrhythmia Atrial or ventricular perforation Aorto atrial fistula Eustachian valve entrapment RA or LA thrombus Pericardial effusion MR or TR Device erosion Sudden death

80. ostium secundum ASD :surgery Incision- -Midline skin incision - b/l fourth interspace sub mammary skin incision -Rt anterolateral fifth intercostal space incision - Small lower sternotomy approach

81. Surgical management of ostium secundum ASD 1. Direct closure 2.Patch closure

82. Care is exercised to place suture – non coronary sinus of aorta SUPERIORLY tricuspid or mitral valve ANTERIORLY the coronary sinus and AV node INFEROANTERIORLY IVC and rt lower pulmonary vein orifice INFERIORLY AND POSTERIORLY rt upper pulmonary vein and SVC SUPERIOPOSTERIORLY

83. Surgical management of sinus venosus malformation When pulmonary vein entrance is near the cavoatrial junction , a simple patch repair can be performed to channel the pulmonary vein blood across the ASD into the lt atrium . When entrance considerable distance from the cavoatial junction-double patch technique repair done

84. Lateral atriotomy extending to SVC Pericardial patch encompass the orifice sinus venosus ASD The SVC and rt atrial junction are augment with a second pericardial patch

85. V-Y atrioplasty technique- for enlarging SCV

86. WARDEN PROCEDURE When RUPV enters SVC too high to baffle Division and over sewing of SVC cephalad to entry of pulmonary vein Intra atrial patch baffling the entire SVC orifice to LA Re – implantation of cephalad SVC to RAA by direct anastomosis

87. Surgical management of coronary sinus ASD These defect can be divided into with and those with out LSVC Repair of coronary sinus ASD without an LSVC-roofing procedure

88. the fossa ovalis incised to view the left atrium, the unroof coronary sinus is shown in its position medial to four pulmonary vein. Pericardial patch is used to repair

89. Repair of coronary sinus ASD with an LSVC- when LSVC is small and particularly when there bridging vein the simplest option is usually to ligate the LSVC and perform roofing procedure. If the LSVC is large intra-atrial baffle technique done.

90. The baffle direct pulmonary venous drainage to mitral valve while the left SVC and coronary sinus drain to rt atrium

91. Repair of Scimitar syndrome Baffling of the rt pulmonary vein flow through the rt atrium across a secundum ASD to the lt atrium. Reimplantation of the anomalous pulmonary higher into rt atrium with baffling across the ASD. Reimplantation of anomalous pulmonary vein into the lt atrium with closer of ASD

92. 4. Incise the wall of the rt atrium and the rt common pulmonary vein as they travel in parallel and then sew the edge together in a side to side anastomosis and baffled into the lt atrium through ASD.

93. Repair in presence of increased pulmonary vascular resistance Indicated in high Rpi (greater then 6 u-m2 ) Using flap valve patch The open rt to lt when rt atrial pressure exceed lt atrial pressure in severe PHT Ppa and Rpi decrease late postoperatively the flap valve will close by cicatrix

94. A- flap valve patch B. – complex patch higher rt atrial pressure open flap leftward C- coronal section

95. Results Early (hospital) mortality Approached zero percent Fewer then .5% Causes of death - 1. pulmonary vascular disease 2. old age (neurologic failure, ch . CHF) 3. air embolization 4. severe supraventricular arrhythmia

96. Results Survival rate Repair during first few yrs of life - survival that of matched general population Repair later in childhood or early adult life -survival very close to that of matched general population Repair in older patient - life expectancy improves but survival is not that of matched general population

97. Results Functional status Asymptomatic children - no symptoms before or after operation Symptomatic infants - complete relief of symptoms Older symptomatic patients - improvement in symptoms

98. Results Functional status Prior to ASD closure, > 60% patients older than 40 yr NYHA class 3 or 4 After ASD closure, > 80% patients NYHA class 1 or 2. Improvement in exercise physiology but less pronounced with older age at operation

99. Results Hemodynamic Results Mean pressure in ascending aorta and mean aortic flow increase Immediate reduction in pulmonary blood flow RA pressure decreases; LA pressure increases Cardiomegaly on chest x – Ray in asymptomatic children resolve with in 2 yrs in 65% cases but not regress to normal size in 27% cases In older patients PVR drops negligibly late after operation

100. Results Ventricular function Striking decrease in RVEDV in 64% children with surgery before 10 yrs of age - RVEDV return to normal In 21% patients with age >25 yrs - RVEDV return to normal In 47% adults with impaired RV function, decreased RV wall motion and EF - less reduction in RV size and less improvement in EF

101. Results Ventricular function LV EF increases normally with maximal exercise Preoperative abnormal small LV ED dimensions increase to normal within 6 months.

102. Result Arrhythmic events Closure of ASD in children improve AV conduction, sinus node function, and decrease AVN refractory period Incidence in children - 23% Adult with preoperative AF - postoperative AF 50% patients with age >40 yrs at surgery develop AF.

103. thank you.