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Dr Ranjith MP Senior Resident Department of Cardiology Government Medical college Kozhikode.

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Presentation on theme: "Dr Ranjith MP Senior Resident Department of Cardiology Government Medical college Kozhikode."— Presentation transcript:

1 Dr Ranjith MP Senior Resident Department of Cardiology Government Medical college Kozhikode

2  Atrioventricular Septal Defects are characterized by complete absence of AV septum  Additional features  A common atrioventricular ring  A five leaflet valve that guards the common AV orifice  An unwedged left ventricular outflow tract  LV mass characterized by longer distance from apex to aortic valve than from apex to left AV valve  Also known as Endocardial cushion defect, AV canal defect, canalis atrioventricularis communis, persistent atrioventricular ostium 2

3  AVSDs account for 4% to 5% of congenital heart disease  New England Regional Infant Cardiac Program /1000 livebirths  Baltimore–Washington Infant study defined a prevalence of  The Alberta Heritage study the prevalence was and per 1000 live births using invasive or noninvasive methodology for the diagnosis respectively  Gender distribution is approximately equal or may show a slight female preponderance 3

4  Rogers, Edwards : Recognised morphology of 1 0 ASD in 1948  Wakai, Edwards : Term of partial and complete AV canal defect in 1956  Bharati & Lev : Term of Intermediate & Transitional in 1980  Rastelli: Described the of common anterior leaflet in 1966  Lillehei : 1 st repair of AVSD in 1954  Kirklin, Watkin, Gross: Open repair using oxygenator 4

5  Result from Faulty development of the endocardial cushions and of the atrioventricular septum  In partial AVSDs, incomplete fusion of the superior and inferior endocardial cushions results in a cleft in the midportion of the AML, often associated with MR  Complete AVSD associated with lack of fusion between the superior and inferior cushions 5

6  Mitral & tricuspid valves achieve the same septal insertion level because the mitral annulus is displaced toward the apex  The distance from mitral annulus to the left ventricular apex is less than the distance from the aortic annulus to the apex 6

7 7  In the normal heart, the aortic valve is wedged between the mitral and tricuspid annuli. In AVSD the aortic valve is displaced anteriorly and creates an elongated, so-called gooseneck deformity of the LVOT

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9  Based on the relationships of the anterior bridging leaflets to the crest of the ventricular septum or RV papillary muscles  Rastelli type A : the anterior bridging leaflet is tightly tethered to the crest of the IVS, occurring in 50% to 70%  Rastelli type B : (3%), the anterior bridging leaflet is not attached to the IVS; rather, it is attached to an anomalous RV papillary muscle and is almost always associated with unbalanced AV canal with right dominance  Rastelli type C : (30%) a free-floating anterior leaflet is attached to the anterior papillary muscle. 9

10 10 Kiyoshi suzuki et al J Am Coll Cardiol 1998;31:217–23

11  Partial AVSD  Most common 2 0 ASD & LSVC to CS  Less frequently- PS, TS or atresia, cor triatriatum, CoA, PDA, membranous VSD, PV anomalies, and HLV  Complete AVSD  Type A usually is an isolated defect and is frequent in patients with Down syndrome.  Type C – TOF, DORV, TGA and heterotaxy syndromes  The combination of type C complete AVSD with TOF is observed Down's syndrome, whereas DORV is a feature of patients with asplenia 11

12 12 M. Cristina Digilio et alCardiogenetics 2011; 1:e7

13 13 M. Cristina Digilio et alCardiogenetics 2011; 1:e7

14 14 M. Cristina Digilio et alCardiogenetics 2011; 1:e7

15  Anatomy of AVSD expected to demonstrate one or more of the following hemodynamic changes  Shunting across the atrial septal defect  Shunting through the ventricular septal defect  Mitral regurgitation  Tricuspid regurgitation  Patterns of shunting: obligatory shunting 15

16  Greater proportion of SVC blood with a low oxygen saturation may cross the IAS to the LA  If AV insufficiency were present blood being ejected from LV to RA  Increase the PO 2 of blood in RA, RV, PA  Slightly higher PO 2 of blood perfusing the lungs would decrease pulmonary vasoconstriction and increase pulmonary blood flow  It is possible that the lesser degree of constriction of the pulmonary arterioles may retard the development of a thick medial muscle layer, so that a more rapid decrease in PVR may occur after birth 16 Fetal physiology

17  Infants with ostium 1 0 defect usually present the same hemodynamic features as those with 2 0 ASD  As PVR falls after birth, RV after load falls & RV stroke volume increases and exceeds that of the LV. The RV fills preferentially and thus left-to right shunting occurs through the ASD  MR and LV to RA shunting are not usually prominent features in infants with 1 0 defect  If MR present, in early infancy cardiac failure develops within weeks after birth 17 Early infancy

18  Pulmonary blood flow is increased even though PVR may still be high, because shunting occurs from a high-pressure to a low- pressure chamber  The increased pulmonary blood flow and PA pressure interfere with the normal postnatal maturation of the pulmonary arterioles  The thick medial muscle layer is maintained and the fall in PR is delayed  An interesting association may develop in some infants of an obligatory left-to-right shunt through the atrioventricular septal defect and simultaneous right-to-left shunting through the ductus arteriosus  Pulmonary vascular resistance may be increased above systemic arterial resistance 18 later infancy

19  Partial AVSD  Patients with 1 0 ASD are usually asymptomatic during childhood.  Dyspnea, easy fatigability, recurrent RTI and growth retardation may be present early in life if associated with major MR or common atrium  Patients with 1 0 ASD usually have earlier and more severe symptoms than patients with 2 0 ASD  Complete AVSD  Tachypnea and failure to thrive invariably occur early in infancy & virtually all patients have symptoms by 1 year of age.  If these symptoms do not develop early on, the clinician should suspect premature development of pulmonary vascular obstructive disease 19

20 20  Usually undernourished and have signs of CHF  Hyperactive precordium with a systolic thrill at the lower left sternal border is common  S1 is accentuated. S2 narrowly splits, P2 increases in intensity.  A grade 3 to 4/6 holosystolic murmur

21  ECG  Superior” QRS axis with the QRS axis between -40 and  Most of the patients have a prolonged PR interval  More than 50% have atrial enlargement  RVH or RBBB is present in all cases (2/3 rd have rsR, RSR or Rr in lead V1, and the rest have a qR or R pattern) & many have LVH  Chest X-ray  In 1 0 ASD findings are same as 2 0 ASD except for enlargement of the LA & LV when MR is significant  In complete AVSD cardiomegaly is always present and involves all four cardiac chambers. Pulmonary vascular markings are increased, and the main PA segment is prominent 21

22  Primary imaging technique for diagnosing AVSD  The internal cardiac crux is the most consistent imaging landmark  Apical four-chamber imaging plane clearly visualizes the internal crux  The 1 0 ASD is seen as an absence of the lower IAS 22

23  Several echocardiac features are shared by all forms of AVSD:  Deficiency of a portion of the inlet ventricular septum  Inferior displacement of the AV valves  Attachment of a portion of the left AV valve to the septum  The two separate AV valve orifices are equidistant from the cardiac apex 23

24  The most common left AV valve abnormality, a cleft, is best visualized from the parasternal and subcostal short-axis imaging planes.  Rarely parachute mitral valve and double-orifice mitral valve also occur 24

25  In the transitional form of partial AVSD, there is aneurysmal replacement of a portion of the inlet ventricular septum 25

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36  Rarely required for diagnosis  In older patient it may have a role in assessing the degree of pulmonary vascular obstructive disease or CAD  A large Lt to Rt shunt at the atrial level demonstrated by a significantly higher oxygen saturation sampled from the RA compared with the blood in the IVC & SVC  In complete AVSD the PASP is invariably at or near systemic level, while in partial AVSDs, the PASP is usually <60% of systemic pressure  LV angiography - gooseneck deformation of the LVOT 36

37  Left to-right shunting increases the oxygen saturation in RA  Sample from high in the SVC usually represents the best  mixed venous oxygen saturation (normal or 40 to 50%)  Usually a further increase in oxygen saturation in the RV  Pulmonary venous oxygen saturation is frequently reduced to 93–95% in older individuals with very large L to R shunts  LA & LV O 2 saturation is often decreased to as low as 86–88% 37

38  The LV angiogram shows features characteristic of AVCD& are best revealed in the hepatoclavicular orientation  The LV outflow tract is elongated and appears narrow  A concavity of the medial border LV that extends along the outflow region to the aorta due to the abnormal attachment of the AML. If it attaches to the ventricular septum or right papillary muscle, LVOT obstruction may be evident  Detect AV valve regurgitation 38

39  The outcome of live-born patients with AVSD depends on the  specific morphology of the defect  The size of the ventricular septal defect  Degree of ventricular hypoplasia  Degree of AV valve regurgitation  Presence or absence of LVOT obstruction  Presence or absence of coarctation of aorta  Associated syndromes (cardiac and noncardiac) 39

40  Patients with the complete form of AVSD and large VSD not undergoing repair die in infancy with CHF & PAH  Those who survive without surgery into childhood usually develop pulmonary vascular obstruction and eventually die with Eisenmenger’s syndrome  Berger and his colleagues found that only 54% of patients born with a complete form of AVSD were alive at 6 months of age, 35% at 12 months, 15% at 24 months, and 4% at 5 years of age  This data would support surgical intervention in the first 3–6 months of age 40 Berger TJ,et al Ann Thorac Surg 1979; 27: 104–11.

41  Infants with 1 0 ASD presenting in infancy have a poor outcome, mainly because of the associated risk factors that bring these infants to early attention  Those with the partial form of AVSD and minimal left AV valve regurgitation seem to fare the best without surgery, although there is still likely considerable morbidity and mortality  According to Somerville, 50% die before 20 years of age and only 25% survive beyond 40 years of age  Atrial fibrillation in these patients was an important cause of late morbidity and mortality 41

42  The complete form of AVSD is the most frequent type of CHD associated with trisomy 21  70% of children with complete AVCD display this aneuploidy  Children with Down syndrome show a simple form of AVCD which is usually complete & rarely associated with additional cardiac anomalies (with the only notable exception of TOF) 42

43  Left-sided obstructive lesions are significantly rare in children with AVCD and Down syndrome compared to patients with AVCD without Down syndrome  Accordingly, some types of situs abnormalities such as l-loop of the ventricles, atresia of the AV valves and TGA are virtually absent in subjects with Down syndrome  Surgical correction of AVCD in individuals with Down syndrome results in lower mortality and morbidity rates, compared to the children without trisomy (12.6% Vs 17.8%) 43

44  Patients are at increased risk for the development of pulmonary vascular obstructive disease  These patients have a greater degree of elevation of pulmonary vascular resistance in the first year of life and more rapid progression to fixed pulmonary vascular obstruction than patients without Down syndrome  Chronic upper airway obstruction with macroglossia and an inherently small hypopharynx, hypotonia, the predisposition to chronic infection, an abnormal capillary bed morphology, and the suggestion of pulmonary hypoplasia can all adversely affect the pulmonary vascular bed  surgical correction should be carried out by 6 months 44

45  Incidence  1% in unoperated cases  Higher incidence in operated cases  10% may require reoperation to relieve LVOT obstruction  more common in partial than in complete AVSD  Etiology  Attachments of SBL to ventricular septum  Extension of the anterolateral papillary muscle into LVOT  Discrete fibrous subaortic stenosis  Tissue from an aneurysm of the membranous septum bowing into the LVOT  Septal hypertrophy 45

46 46 Systolic (left) and diastolic (right) echocardiographics demonstrating LVOT obstruction in a 17-year-old who had repair of a partial AVSD at age 15 months

47  PA banding is now performed infrequently in infants with AVSD because the surgical risks of intracardiac repair are not significantly greater than the palliative procedure  Perioperative mortality is about 5%  It is reserved for those few patients in whom intracardiac repair is likely to be associated with a high risk like  Single papillary muscle  Severe left ventricular outflow obstruction  Unbalanced commitment of the AV valve to the ventricles 47

48  Objectives - closure of the interatrial communication and restoration and preservation of left AV valve competence  These objectives can be accomplished by careful approximation of the edges of the valve cleft with interrupted nonabsorbable sutures  The repair is completed by closure of the interatrial communication (usually with an autologous pericardial patch), avoiding injury to the conduction tissue  This repair results in a two-leaflet valve  Alternatively, if the left AV valve is to be considered a trileaflet valve, with the cleft viewed as a commissure, surgical repair demands that this commissure be left unsutured and that various annuloplastic sutures be placed to promote coaptation of the three leaflets 48

49 49 A: Surgical exposure B: Closure of the mitral valve cleft C: Prosthetic patch closure of an 1 0 defect D: Repair completed

50  Surgical repair of complete forms of AVSD is indicated earlier in life than for the partial forms of AVSD  Repair should be done electively before 6 months of age & earlier repair should be considered for infants with failure to thrive  For the symptomatic infant, surgical options include palliative pulmonary artery banding and complete repair of the anomaly  In the modern era complete repair appears to be the procedure of choice 50

51  Closure of interatrial and interventricular communications, construction of two separate and competent AV valves from available leaflet tissue, and repair of associated defects  Techniques are based on the use of a single patch or double patch (separate atrial and ventricular patches) to close the ASD and VSD and then reconstruction of the left AV valve as a bileaflet valve  Some surgeons consider the cleft of the left AV valve, a true commissures and envision this valve as a trileaflet valve. This is the basis for Carpentier technique for repair of complete AVSD  The two-patch technique has become the method of choice 51

52 52 Carpentier technique for repair of complete AVSD with the double-patch technique. Concept of a trileaflet left atrioventricular valve

53  The risk of hospital death for repair is 3%  Determinants of hospital mortality include CHF, cyanosis, failure to thrive, age at operation of <4 years, and moderate to severe MR  20- and 40-yr survivals after repair is 87% and 76% respectively  Closure of the mitral cleft and age <20 years at time of operation is associated with better survival 53

54  Partial AVSD :  Regurgitation or stenosis of the left AV valve  Subaortic stenosis  Residual recurrent ASD  Reoperation for MR occurs in 10% to 15% of survivors of primary repair of partial AVSD  Risk factors for reoperation include significant residual MR as assessed intraoperatively at the time of initial repair, the presence of a severely dysplastic mitral valve, and failure to close the cleft in the AML  Repeat repair is possible if valve dysplasia is not severe or when the mechanism of regurgitation is through an unsutured cleft  Replacement of the mitral valve may be required in the presence of a severely dysplastic valve 54

55 Complete AVSD  Needed in 17% of patients during the first 20 years after surgical repair  Lesions requiring reoperation include - left and right AV valve regurgitation, left AV valve stenosis (native and prosthetic), and residual/recurrent ASDs or VSDs  Residual left AV valve regurgitation may result from inadequate surgical reconstruction  Right AV valve regurgitation requiring reoperation is rare. It is more apparent with the presence of PAH or in association with TOF with RV dysfunction owing to persistent RVOT obstruction or PR  Residual shunts are rare causes for late reoperation 55

56  Parachute Deformity of the Mitral Valve  Closure of the mitral cleft at the time of repair may result in an obstructed mitral orifice  If the patient has significant AV valve regurgitation, valve replacement may be the only suitable option  Double-Orifice Mitral Valve  The surgeon must resist the temptation of joining the two orifices by incising the intervening leaflet tissue. The combined opening of both orifices is satisfactory for adequate mitral valve function 56

57  Right or Left Ventricular Hypoplasia  The only option for definitive surgical treatment is the modified Fontan's procedure preceded by adequate pulmonary artery banding in infancy  Subaortic Stenosis  If discovered at the time of initial preoperative evaluation, subaortic stenosis tends to be of the fibromuscular membrane type and should be treated by appropriate resection during surgical repair 57

58  Common atrium is characterized by near absence of the atrial septum  In the presence of two ventricles, it always is associated with an AVSD  Most patients with common atrium present in infancy with symptoms of excess pulmonary blood flow  These patients are symptomatic earlier in life than patients with only a 1 0 ASD  The precordium is hyperactive with a prominent RV impulse. S2 is widely split and fixed 58

59  P2 intensity proportionate to the severity of pulmonary hypertension  An ESM present over the upper left sternal border. A distinct holosystolic murmur of MR may be heard at the apex. A middiastolic murmur commonly is detected over the lower left sternal border resulting from an increase in right atrial to right ventricular blood flow  The radiographic and electrocardiographic characteristics of patients with common atrium are indistinguishable from those with other forms of AVSD 59

60  Echocardiography  Subcostal four-chamber view is most suitable for accurate diagnosis  A muscle bundle or band coursing through the atrium should not be interpreted as an atrial septum  Cardiac Catheterization and Angiography  The hemodynamic diagnosis of common atrium depends on the demonstration of complete mixing of systemic and pulmonary venous blood  The oxygen saturations of pulmonary and systemic arterial blood are nearly identical  Pulmonary blood flow exceeds systemic flow, except in patients with PAH  Right ventricular pressure is increased more often than in 2 0 ASD or partial AVSD 60

61  If definitive repair is delayed, significant pulmonary vascular obstructive disease may develop more easily than in patients with secundum ASD or partial AVSD  Treatment  Medical therapy -Digoxin and diuretic therapy are traditional forms of therapy  Surgical repair, which should be performed early in life because the patient usually has symptoms and is at risk for developing pulmonary vascular obstructive disease 61

62  One ventricle and its corresponding AV valve are hypoplastic while the other ventricle receives the larger portion of the common AV valve  The most common arrangement is a dominant right ventricle with a hypoplastic left ventricle  The left-sided component of the common AV valve may be stenotic after two-ventricle repair has been performed 62

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65 1. Down syndrome most commonly associated with Type A 2. Most common form complete AVCD is Type B. 3. Free interventricular communication exists in type A 4. In type A anterior bridging leaflet has been described as free floating

66 1. Sinus rhythm is present in most patients with a 1⁰ ASD 2. P-wave changes indicating right atrial, left atrial, or biatrial enlargement are seen in 54% of patients. 3. The mean QRS axis ranges from +30⁰ to +120⁰ 4. ventricular volume overload results in the rsR or RSR pattern in the right precordial leads in 84%.

67 1. Single papillary muscle 2. Severe left ventricular outflow obstruction 3. Unbalanced commitment of the AV valve to the ventricles 4. Associated TOF

68 1. The only option for definitive surgical treatment in Right or Left Ventricular Hypoplasia is the modified Fontan's procedure preceded by adequate pulmonary artery banding in infancy 2. In double-orifice Mitral Valve, better results by joining two orifices by incising the intervening leaflet tissue 3. Closure of the mitral cleft at the time of repair may result in an obstructed mitral orifice in Parachute Deformity of the Mitral Valve 4. If discovered at the time of initial preoperative evaluation, subaortic stenosis tends to be of the fibromuscular membrane type & should be treated by appropriate resection during surgical repair 68

69 1. Oxygen saturation step up from RA to RV 2. Mixed venous sample best represented by low SVC sample 3. Oxygen saturation 93 to 95 in pulmonary vein 4. Left atrial and left ventricular oxygen saturation is often decreased to as low as 86–88% 69

70 1. Noonan syndrome 2. Down syndrome 3. CHARGE syndrome 4. Ellis-van creveld syndrome

71 and 40-yr survivals after repair is 87% and 76% respectively 2. Closure of the mitral cleft and age <20 years at time of operation is associated with better survival 3.Right AV valve regurgitation requiring reoperation is rare 4.The risk of hospital death for repair is %

72 1. Gender distribution is approximately equal or may show a slight female preponderance 2. A five leaflet valve that guards the common AV orifice 3. A wedged left ventricular outflow tract 4. LV mass characterized by longer distance from apex to aortic valve than from apex to left AV valve

73 1. Inferior displacement of the AV valves 2. The two separate AV valve orifices are not equidistant from the cardiac apex 3. The internal cardiac crux is the most consistent imaging landmark 4. Deficiency of a portion of the inlet ventricular septum 73

74 1. Reoperation for MR occurs in 10% to 15% of survivors of primary repair of partial AVSD 2. Replacement of the mitral valve may be required in the presence of a severely dysplastic valve 3. Residual shunts are common causes for late reoperation 4. The risk of hospital death for repair is 3% 74

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76 1. Down syndrome most commonly associated with Type A 2. Most common form complete AVCD is Type B. 3. Free interventricular communication exists in type A 4. In type A anterior bridging leaflet has been described as free floating

77 1. Sinus rhythm is present in most patients with a 1⁰ ASD 2. P-wave changes indicating right atrial, left atrial, or biatrial enlargement are seen in 54% of patients. 3. The mean QRS axis ranges from +30⁰ to +120⁰ 4. ventricular volume overload results in the rsR or RSR pattern in the right precordial leads in 84%.

78 1. Single papillary muscle 2. Severe left ventricular outflow obstruction 3. Unbalanced commitment of the AV valve to the ventricles 4. Associated TOF

79 1. The only option for definitive surgical treatment in Right or Left Ventricular Hypoplasia is the modified Fontan's procedure preceded by adequate pulmonary artery banding in infancy 2. In double-orifice Mitral Valve better results by joining two orifices by incising the intervening leaflet tissue 3. Closure of the mitral cleft at the time of repair may result in an obstructed mitral orifice in Parachute Deformity of the Mitral Valve 4. If discovered at the time of initial preoperative evaluation, subaortic stenosis tends to be of the fibromuscular membrane type & should be treated by appropriate resection during surgical repair 79

80 1. Oxygen saturation step up from RA to RV 2. Mixed venous sample best represented by low SVC sample 3. Oxygen saturation 93 to 95 in pulmonary vein 4. Left atrial and left ventricular oxygen saturation is often decreased to as low as 86–88% 80

81 1. Noonan syndrome 2. Down syndrome 3. CHARGE syndrome 4. Ellis-van creveld syndrome

82 and 40-yr survivals after repair is 87% and 76% respectively 2. Closure of the mitral cleft and age <20 years at time of operation is associated with better survival 3.Right AV valve regurgitation requiring reoperation is rare 4.The risk of hospital death for repair is %

83 1. Gender distribution is approximately equal or may show a slight female preponderance 2. A five leaflet valve that guards the common AV orifice 3. A wedged left ventricular outflow tract 4. LV mass characterized by longer distance from apex to aortic valve than from apex to left AV valve

84 1. Inferior displacement of the AV valves 2. The two separate AV valve orifices are not equidistant from the cardiac apex 3. The internal cardiac crux is the most consistent imaging landmark 4. Deficiency of a portion of the inlet ventricular septum 84

85 1. Reoperation for MR occurs in 10% to 15% of survivors of primary repair of partial AVSD 2. Replacement of the mitral valve may be required in the presence of a severely dysplastic valve 3. Residual shunts are common causes for late reoperation 4. The risk of hospital death for repair is 3% 85


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