ATRIAL SEPTAL DEFECT GENERAL PHYSIOLOGY

Transcription

1 The ABCs of ASDs John M. Lasala MD PhD Director Interventional Cardiology and Structural Heart Disease Professor of Medicine Washington University St Louis Mo

2 ATRIAL SEPTAL DEFECT GENERAL PHYSIOLOGY RA and RV volume overload Excess volume may result in pulmonary hypertension may develop More often in women Generally not severe May result in paradoxical emboli Brickner et al NEJM 2000

3 Hemodynamic Consequences of ASD Magnitude of and direction of flow depends on Size of the defect Relative diastolic filling properties of the left and right ventricles. Increased left-to-right shunting results from reduced LV compliance (eg, LVH) and mitral stenosis. Reduced left-to-right shunt and/or reversal of shunt (rightto-left shunt) results from reduced RV compliance (eg, pulmonary hypertension or pulmonary stenosis) and tricuspid stenosis Webb G and Gatzoulis MA. Circulation 2006;114;

4 Hemodynamic Consequences of ASD Size and Shunt As a rule, an ASD must be at least 10 mm in diameter to carry a significant left-to-right shunt CAVEAT: symptoms may develop with increasing age even with small defects owing to an increase in shunting caused by a decrease in LV compliance secondary to coronary artery disease, acquired valvular disease, or hypertension. A left-to-right atrial shunt is considered significant when the Qp/Qs ratio is greater than 1.5/1.0, or if it causes dilation of the right heart chambers. Chronic volume overload of the pulmonary vasculature may result in pulmonary arterial hypertension Webb G and Gatzoulis MA. Circulation 2006;114;

5 Rationale for Intervening Natural history of ASD diagnosed in childhood is that the ASD diameter when untreated increases in 65% of cases, and 30% will have more than a 50% increase in diameter. Only 4% of ASDs close spontaneously Although small ASDs of <5 mm and no evidence of RV volume overload do not impact the natural history of the individual and thus may not require closure Paradoxical embolism may occur Some small defects however may have progressive increase in left-to-right shunt depending on LV and LA pressures Rosas M, et al. Int J Cardiol Feb; 93(2-3): unoperated patients >40 yo followed 1.6 to 22 years. 37 events (18.5%): 5 sudden death, 7 heart failure, 13 severe pulmonary infection, 5 embolisms and 4 strokes. Predictors of poor outcome were age at presentation, pulmonary HTN or arterial O2 sat < 80% McMahon. CJ, et al. Heart Mar; 87(3):256-9.

6 Rationale for Intervening Murphy JG, et al. N Engl J Med Dec 13; 323(24): patients undergoing isolated ASD repair retrospective analysis Independent predictors of long term survival were age at operation and main pulmonary artery systolic Pressure (PASP) Attie F, et al. J Am Coll Cardiol Dec; 38(7): patients over 40 followed prospectively with a median follow up of 7.3 years. Overall mortality rate was not statistically different. There was however a higher rate of recurrent pneumonia in the medical arm. There was also a trend towards higher sudden death, CHF, and overall mortality in medical arm which did not reach statistically significance

7 Rationale for Intervening Larger defects with evidence of RV volume overload on echocardiography usually only cause symptoms in the third decade of life, and closure is usually indicated to prevent long-term complications Definite and Potential Benefits of ASD Closure RV and RA size LV size PA pressure Right-to-left shunting and embolism Exercise capacity NYHA class Atrial arrhythmias

8 ACC/AHA Guidelines Clas s 2.3. Recommendations for Evaluation of the Unoperated Patient Clas Is 2.3.ASD Recommendations for by Evaluation of the Unoperated Patient 1. should be diagnosed imaging techniques with demonstration of shunting across the defect and evidence of RV volume overload and 1. any ASDassociated should beanomalies. diagnosed by imaging techniques with demonstration C 2. Patients with unexplained RVand volume overload beoverload referred and to C of shunting across the defect evidence of RVshould volume an center for further diagnostic studies to rule out obscure anyachd associated anomalies. partial venous connection, or coronary 2. ASD, Patients withanomalous unexplained RV volume overload should besinoseptal referred to C defect. an ACHD center for further diagnostic studies to rule out obscure I IIa 1. Maximal exercise testingvenous can be connection, useful to document exercise capacity ASD, partial anomalous or coronary sinoseptal in patients with symptoms that are discrepant with clinical findings or defect. document changes in oxygen in patientsexercise with mild or IIa 1. to Maximal exercise testing can besaturation useful to document capacity moderate in patientspah. with symptoms that are discrepant with clinical findings or 2. Cardiac catheterization can be useful to rule concomitant to document changes in oxygen saturation in out patients with mild or coronary artery disease in patients at risk because of age or other moderate PAH. 2. factors. Cardiac catheterization can be useful to rule out concomitant Level of Evidenc e Level of C Evidenc e C C B B III 1. In younger patients withinuncomplicated ASD for whom B coronary artery disease patients at risk because of ageimaging or other results factors.are adequate, diagnostic cardiac catheterization is not indicated. B 2. Maximal exercise testing is not recommended in ASD with severe PAH. Warnes CA, et al, J Am Coll Cardiol 2008;52:e

9 ACC/AHA Guidelines Class Class Recommendations for Recommendations for Interventional Interventional and and Surgical Surgical Therapy Therapy II IIa IIb III Closure Closure of of an an ASD ASD either either percutaneously percutaneously or or surgically surgically is is indicated indicated for for right right atrial atrial and and RV RV enlargement with or without symptoms. enlargement with or without symptoms. A A sinus sinus venosus, venosus, coronary coronary sinus, sinus, or or primum primum ASD ASD should should be be repaired repaired surgically surgically rather rather than than by percutaneous closure. by percutaneous closure. Surgeons Surgeons with with training training and and expertise expertise in in CHD CHD should should perform perform operations operations for for various various ASD ASD closures. closures. Level Level of of Evidenc Evidenc ee B B B B C C Surgical closure of secundum ASD is reasonable when concomitant surgical repair/replacement of a tricuspid valve is considered or when the anatomy of the defect precludes the use of a percutaneous device. Closure of an ASD, either percutaneously or surgically, is reasonable in the presence of: a. Paradoxical embolism. b. Documented orthodeoxia-platypnea. C Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net left-to-right shunting, pulmonary artery pressure less than two thirds systemic levels, PVR less than two thirds systemic vascular resistance, or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). Concomitant Maze procedure may be considered for intermittent or chronic atrial tachyarrhythmias in adults with ASDs. C C B C Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not B undergo ASD closure. Warnes CA, et al, J Am Coll Cardiol 2008;52:e143

10 Nomenclature of ASD Rims Conventionally, the 5 rims of a secundum ASD are labeled as Aortic (anterosuperior) By conventional definition 1. Margin 5 mm is considered adequate 2. Margin 3 mm is considered absent Atrioventricular (AV) valve (mitral or anteroinferior) Superior venacaval (SVC or posterosuperior) Inferior venacaval (IVC or posteroinferior), Posterior (from the posterior free wall of the atria).

11 Sinus Venosus ASD: IVC Type Bicaval View

12 Sinus Venosus ASD: IVC Type Note: ASD with PFO

13 Secundum ASD Atrial septal defects Three-dimensional size Assessment of rim Percutaneous closure Assessment of anomalous pulmonary veins

14 Secundum ASD Rims Source: Journal of the American Society of Echocardiography 2011; 24: (DOI: /j.echo ) Copyright 2011 American Society of Echocardiography Terms and Conditions

15 ACC/AHA Guidelines Clas s I 2.6. Recommendations for Postintervention Follow-Up 1. Early postoperative symptoms of undue fever, fatigue, vomiting, chest pain, or abdominal pain may represent postpericardiotomy syndrome with tamponade and should prompt immediate evaluation with echocardiography. 2. Annual clinical follow-up is recommended for patients postoperatively if their ASD was repaired as an adult and the following conditions persist or develop: a. PAH. b. Atrial arrhythmias. c. RV or LV dysfunction. d. Coexisting valvular or other cardiac lesions. 3. Evaluation for possible device migration, erosion, or other complications is recommended for patients 3 months to 1 year after device closure and periodically thereafter. 4. Device erosion, which may present with chest pain or syncope should warrant urgent evaluation. Level of Evidenc e C C C C C C C Warnes CA, et al, J Am Coll Cardiol 2008;52:e

16 ACC/AHA Guidelines Class Class Recommendations for Recommendations for Interventional Interventional and and Surgical Surgical Therapy Therapy II IIa IIb III Closure Closure of of an an ASD ASD either either percutaneously percutaneously or or surgically surgically is is indicated indicated for for right right atrial atrial and and RV RV enlargement with or without symptoms. enlargement with or without symptoms. A A sinus sinus venosus, venosus, coronary coronary sinus, sinus, or or primum primum ASD ASD should should be be repaired repaired surgically surgically rather rather than than by percutaneous closure. by percutaneous closure. Surgeons Surgeons with with training training and and expertise expertise in in CHD CHD should should perform perform operations operations for for various various ASD ASD closures. closures. Level Level of of Evidenc Evidenc ee B B B B C C Surgical closure of secundum ASD is reasonable when concomitant surgical repair/replacement of a tricuspid valve is considered or when the anatomy of the defect precludes the use of a percutaneous device. Closure of an ASD, either percutaneously or surgically, is reasonable in the presence of: a. Paradoxical embolism. b. Documented orthodeoxia-platypnea. C Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net left-to-right shunting, pulmonary artery pressure less than two thirds systemic levels, PVR less than two thirds systemic vascular resistance, or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). Concomitant Maze procedure may be considered for intermittent or chronic atrial tachyarrhythmias in adults with ASDs. C C B C Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not B undergo ASD closure. Warnes CA, et al, J Am Coll Cardiol 2008;52:e143

17 Percutaneous ASD closure Majunke N, et al Am J Cardiol Feb 15; 103(4): consecutive adults with median age of 45 Procedural success in 98% 96% complete closure 22 of 25 of the incomplete closures had very small residual shunt PASP 33.3 mmhg 28.3 mmhg Intra-procedural complications consisted of 2 pts with device embolization, one pt with transient ST depression. 0.9% needed emergent CT surgery

18 Percutaneous ASD closure Surgical (n = 154) Percutaneous (n = 442) P value Size of ASD 14.2 ± 6.3 mm 13.3 ± 5.4 mm Single ASD 80.5% 89.4% Success rate 100% 95.7% NS 24.0% 7.2% p < Mortality Complication rate Length of Stay 3.4 days 1.0 day < Patients were considered to have successful ASD closure if they had no, trivial (<1 -mm color jet width) or small (color jet width 1 to 2 mm) residual shunt as assessed by color Doppler echocardiography. Patients with moderate (color jet width >2 4 mm) or large (color jet width >4 mm) residual shunts were considered to have a failed procedure. Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K, Amplatzer Investigators

19 Complications of Percutaneous ASD Closure Surgical Percutaneous 1. Pulmonary edema 1. Device or marker band 2. Large pericardial effusion embolization (requiring requiring two surgical removal) pericardiocentesis 2. Cardiac arrhythmias requiring 3. Repeat surgery due to major treatment bleeding 3. Cerebral embolism (transient) 4. Surgical wound complications Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K, Amplatzer Investigators J Am Coll Cardiol Jun 5; 39(11):

20 Currently available devices within the United States for percutaneous closure of atrial-level defects within randomized controlled trials. Currently available FDA approved devices in the United States AMPLATZER ASO Polyester material sewn into braided nitinol wires GORE HELEX Septal Occluder Inglessis I, Landzberg M. Circulation 2007;115: eptfe patch material supported by a single nitinol wire frame

21 Fenestrated Secundum ASD Source: Journal of the American Society of Echocardiography 2011; 24: (DOI: /j.echo ) Copyright 2011 American Society of Echocardiography Terms and Conditions

22 Failure to Close a Fenestrated ASD

23 Atrial Septal Defect ASD can be associated with additional malformations in nearly 30% of cases AV septal defects (primum ASD) cleft in the anterior mitral valve leaflet Sinus venosus defects frequently (rarely with secundum ASD) partial anomalous venous drainage of the right pulmonary veins Any ASD Mitral valve prolapse Any ASD Valvular pulmonic stenosis Coronary sinus septal defect partial or total anomalous pulmonary venous connection and/or a persistent left superior vena cava draining to the coronary sinus. Warnes CA, et al, J Am Coll Cardiol 2008;52:e

24 SECUNDUM ASD- ASSOCIATED DEFECTS NORMAL? NORMAL LEFT INNOMINATE VEIN INJECTION (Straight AP)

25 SECUNDUM ASD- ASSOCIATED DEFECTS

26 SECUNDUM ASD- ASSOCIATED DEFECTS: Partial Anomalous Pulmonary Venous Return to the Left Innominate Vein LPA Angio (LAO) showing split drainage of left pulmonary veins

27 OTHER DEFECTS ASSOCIATED WITH SECUNDUM ASD TO WATCH FOR : PAPVR -Left Upper Pulmonary Vein to LSVC draining to innominate ( bridging ) vein -Right Pulmonary Veins to the SVC and/or the backwall of the RA Pulmonary Valve Stenosis Balloon Valvuloplasty PSG >40mmHg

28 SINUS VENOUSUS ASD with Right PAPVR To the SVC

29 SECUNDUM ASD- ASSOCIATED DEFECTS

30 SECUNDUM ASD- ASSOCIATED DEFECTS RIGHT PARTIAL ANOMALOUS PULMONARY VENOUS RETURN: -RUPV to SVC-RA JUNCTION -RLPV to BACKWALL of RIGHT ATRIUM

31 SECUNDUM ASD- ASSOCIATED DEFECTS CHECK DRAINAGE OF LEFT PULMONARY VEINS TOO!

32 ATRIAL SEPTAL DEFECTS (occur in 5-10% of children born with CHD) Good quality 2-D and color Doppler echocardiograms were not available until early 1980s so, ASDs in adults born prior to 1980 are largely underdiagnosed

33 SECUNDUM ASD VARIANTS: SVC FOSSA ASD Also known as valve incompetent PFO

34 SECUNDUM ASD VARIANTS: MULTI-FENESTRATED TYPES they are not all the same!

35 SECUNDUM ASD VARIANTS: ANEURYSMAL ( windsock ) FENESTRATED TYPE

36 Definition of Complex Secundum ASDs Present in 28% of cases (Pedra,et al. JIC 2004;16;117) >26mm stretch diameter with a deficient rim < 4mm 2 separate ASDs with a distance > 7mm Fenestrated atrial septum Redundant or hypermobile septum

37 ASD ANATOMIC RIM DEFINITIONS:

38 So when is a paucity/absence of rim really an issue? Type of Rim Absence Acceptable Device Candidate Retro-Aortic Rim Absence Yes Posterior Rim Absence Probably NO; depends on defect size (enhanced migration risk) Inferior Rim Depends on extent & C.S. * Superior Rim Yes, except true S.V. + PAPVR AV Valve Rim Depends on overall extent & proximity of device to M.V. leaflet *

39 POOR ANATOMIC ASD CANDIDATES: Absence of significant posterior rim in defect larger than 1.5 cm: increased risk of device migration

40 Large ASDs: closure methods Rotation on delivery sheath Deployment in RUPV Deployment in LUPV Use of alternative sheaths (Mullins, Hausdorf, modified Amplatzer) Use of right coronary catheter Balloon assisted technique Use of dilator as buttress

41 Courtesy of Evan M. Zahn, MD, Miami Children s Hospital Kannan, Catheter Cardiovasc Intervent, 2003

42 ASD DEVICE TIPS & TRICKS Hausdorf Sheath (COOK Corp.) Tip: 3-D curve that keeps sheath more perpendicular to ASD plane when there is an anterior-superior ASD with absent retro-aortic rim Available in French Sizes

43 ADVANCED ASD CLOSURE Sheath Tips and Tricks: NEW AGA TorqVue Sheath Hausdorf Sheath (Cook, Inc.)

44 ASD: no rim

45 Challenging Case: Large ASD

46 Large ASD 18 y/o female referred for evaluation of asymptomatic heart murmur Transthoracic echo confirms presence of large ASD with dilation of RA and RV CXR with mild cardiomegaly and shunt vascularity

47 Large ASD Cardiac cath demonstrates normal right heart pressures and Qp:Qs 3:1 ICE demonstrates large secundum ASD

48 Large ASD

49 Large ASD Several unsuccessful attempts were made to deploy 28 mm Amplatzer device in conventional manner Attempts at left PV deployment technique also unsuccessful secondary to prolapse of LA disc through retro-aortic rim

50 Large ASD

51 Large ASD

52 Large ASD

53 Large ASD

54 Large ASD

55 Large ASD

56 Large ASDs with Deficient Anterosuperior Rims Largest percentage of deficient rims (42%) Amplatzer discs must straddle the aorta (riding bareback) Keep discs flared around the aorta to prevent erosion Avoid using a device > 1.5 times the TEE/ICE diameter Watch for encrochment on structures- MV, SVC,RUPV Know the tricks presented for appropriate placement

57 Closure of Multiple ASDs

58 Multiple ASDs Occurs in up to 6.6% of ASDs May require multiple devices to close the defects if far enough apart Single device may be adequate for closely spaced defects Occasionally balloon sizing will convert multiple defects into a single defect

59 Multiple ASDs Most fenestrated defects can be closed with the cribiform device Multiple ASDs may require more than 1 device If defects > 7 mm apart 2 devices (ASO) may be implanted May implant sequentially or simultaneously

60 Multiple ASDs

61 Multiple ASDs

62 Multiple ASDs

63 Multiple ASDs

64 2 ASDs

65 ADVANCED ASD CLOSURE FINAL DEVICE CONFIGURATION RAO LATERAL

66 FINALTIPS/POINTS Always perform a thorough multi-planar echocardiographic evaluation of the septum during balloon stop flow sizing. Repeat this multi-planar echo imaging prior to device release! Always have a high index of suspicion for additional/satellite defects.

67 Fenestrated ASD (multiple holes) Courtesy Y. Joe Woo, MD HUP Cardiac Surgery

68 Case Presentation- ASD closure Due to Hypoxia 34 yo woman with a long h/o SOB Had difficulty playing sports as a child Severe SOB with each of her 2 pregnancies Once given dx of asthma (not treated) More recently, c/o of SOB with 1 flight of stairs, carrying her baby, and also associated with some dizziness and lightheadedness Work-up including a TTE which suggested a left to right shunt.

69 OSH TEE Secundum ASD measured 0.5 cm diameter Qp:Qs ratio of 1.4 Referred for percutaneous ASD closure

70 Case Presentation Admitted for RHC and ICE with planned ASD closure Qp:Qs only 1.1:1 Normal Pa pressure

71 ICE contrast study Rest After arm exercise

72 Hypoxia in Patients with Interatrial Septal Defects (IASD) Can be persistent, intermittent, or positional Mechanism involves transient or persistent elevation in RAP>LAP, or redirection of IVC blood flow toward septum Diagnosis can be challenging - Requires documentation of R-to-L shunt while hypoxemic - Confirmed by improvement in hypoxia after closure Associated with a wide variety of conditions - Pulmonary AVM Liver Disease Chronic Lung Disease Amiodarone Toxicity Pulmonary Emboli Aortic Aneurysm - Hypovolemia Positive Pressure Ventilation Post-pneumonectomy RV Infarction Cardiopulmonary Bypass

73 Saturation (%) 96% P= % Ilkhanoff and Herrmann J Int Card 2005;18:227-32

74 Therapeutic Intervention Closure performed #25 AGA Cribiform ASO inserted in PFO #14 AGA Atrial septal occluder for ASD

75 Case Presentation In follow-up, all of her symptoms have resolved

76 Case Report cont: ASD History: Recent murmur appreciated and echo revealed a large secundum ASD with estimated diameter of 27 and 28mm. R-sided chambers were enlarged, mod Pulm HTN was present. MRI ruled out sinus venosus defect with any anomalous pulmonary drainage. He underwent initial attempt at percutaneous closure at OSH, but defect unsuccessfully closed. Patient referred to Barnes-Jewish for second attempt at percutaneous closure.

77

78 Cath Films ASD Closure:

79 Case Report cont: ASD Procedure: Fluoroscopic and TEE guidance used. 38mm Amplazter device used for procedure with successful deployment Bubble study negative immediately post-procedure

80 Cath Films ASD Closure:

81 Cath Films ASD Closure:

82 TEE Post Deployment

83

84

85 Later the Same Day Patient was transferred to rhe floor No complaints Routine TTE obtained that night per protocol 911 page sent

86 Echo Post Procedure

87 Echo post-procedure:

88 Later the Same Night. Though non-emergent CT surgery took patient to OR the same night. Percutaneous extraction considered but concern over thrombus potentional on device sitting in LA so long. Did well post op until day 3.

89 Echo post-surgical repair:

90 Echo post-tamponade drainage:

91 Deficient Posteroinferior Rims A real challenge as these tend to be larger ASDs MD beware if rim is < 3mm. Increased complication rate- IVC or Pulmonary vein obstruction, encroachment on mitral valve, frank embolization

92 The Future of Imaging for Structural Heart Disease

93 3D Imaging: Acunav 10 Fr system

94 EchoNavigator 94

95

96 ACC/AHA Guidelines Class Class Recommendations for Recommendations for Interventional Interventional and and Surgical Surgical Therapy Therapy II IIa IIa IIb III Closure Closure of of an an ASD ASD either either percutaneously percutaneously or or surgically surgically is is indicated indicated for for right right atrial atrial and and RV RV enlargement with or without symptoms. enlargement with or without symptoms. A A sinus sinus venosus, venosus, coronary coronary sinus, sinus, or or primum primum ASD ASD should should be be repaired repaired surgically surgically rather rather than than by percutaneous closure. by percutaneous closure. Surgeons Surgeons with with training training and and expertise expertise in in CHD CHD should should perform perform operations operations for for various various ASD ASD closures. closures. Level of Level of Evidenc Evidenc ee B B B B C C Surgical Surgical closure closure of of secundum secundum ASD ASD is is reasonable reasonable when when concomitant concomitant surgical surgical repair/replacement of a tricuspid valve is considered or when the anatomy repair/replacement of a tricuspid valve is considered or when the anatomy of of the the defect defect precludes the use of a percutaneous device. precludes the use of a percutaneous device. Closure Closure of of an an ASD, ASD, either either percutaneously percutaneously or or surgically, surgically, is is reasonable reasonable in in the the presence presence of: of: a. a. Paradoxical Paradoxical embolism. embolism. b. Documented b. Documented orthodeoxia-platypnea. orthodeoxia-platypnea. C C Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net left-to-right shunting, pulmonary artery pressure less than two thirds systemic levels, PVR less than two thirds systemic vascular resistance, or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). Concomitant Maze procedure may be considered for intermittent or chronic atrial tachyarrhythmias in adults with ASDs. C Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not undergo closure. Warnes ASD CA, et al, J Am Coll Cardiol 2008;52:e C C B B C B