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Anne-Marie Anagnostopoulos, MD Non-Invasive Conference December 9, 2009.

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Presentation on theme: "Anne-Marie Anagnostopoulos, MD Non-Invasive Conference December 9, 2009."— Presentation transcript:

1 Anne-Marie Anagnostopoulos, MD Non-Invasive Conference December 9, 2009

2 Outline  History and Epidemiology  Anatomy and Embryology  Spectrum of TOF  Surgical Repair  Imaging The Adult with Repaired TOF  Summary

3 Special Thanks  Special thanks to Dr. Anne Marie Valente who helped me enormously

4 History of Tetralogy of Fallot  1671: Stenson first describes pathology of what would later be confirmed as TOF  1888: Etienne-Louis Fallot first recognizes a group of complex cardiac malformations that leads to cyanosis and identifies 4 abnormalities: pulmonary stenosis, VSD, dextroposition of the aorta, and RVH  Fallot postulated that these abnormalities resulted from abnormal development of the subpulmonary infundibulum and pulmonary valve.  1924: Abbott and Dawson name the malformation “Tetralogy of Fallot”

5 History  From Wikipedia:  E. L. A. Fallot. Contribution à l’anatomie pathologique de la maladie bleue (cyanose cardiaque). Marseille médical, 1888, 25: 77-93, , , , ,

6 Epidemiology  Overall, congenital heart disease is rare  However, of the cyanotic congenital heart abnormalities, TOF is the most common  TOF has an incidence of approximately 32.6 per 100,000 live births  The success of early surgical repair has led to a large population of adults with repaired TOF

7 Anatomy and Embryology  The fundamental embryologic malformation in TOF is abnormal development of the cono- truncus (also known as: conal septum, subpulmonary infundibulum)  There is hypoplasia of the conotruncus and anterior/superior displacement of the infundibular septum  This results in failure of ventricular septation, subpulmonary and/or pulmonary valve stenosis and overriding aorta

8 The Worlds Best Anatomical Charts. Anatomical Chart Company Skokie, IL. ISBN

9 Anatomy and Embryology in Tetralogy of Fallot Figures Emily Flynn, Echocardiography in Pediatric and Congenital Heart Disease Editors Lai, Mertens, Cohen, Geva 2009

10 Anatomy and Embryology – Simplified Diagram

11 2D Echo TOF Source: Feigenbaum’s 6 th Ed.

12 Spectrum of Tetralogy  There is a spectrum of anatomy in TOF with an associated variation in clinical presentation  Children with minimal pulmonary stenosis are at one end and can be “pink”  At the other extreme is a form of TOF with pulmonary valve atresia and VSD (severely blue)  In the latter case, life is sustained by PDA or aorto-pulmonary collateral vessels

13 TOF:Pulmonary Atresia and VSD Obliterated subpulmonary infundibulum Marked anterior/left shift of conal septum Figure Emily Flynn, Echocardiography in Pediatric and Congenital Heart Disease Editors Lai, Mertens, Cohen, Geva 2009

14 Anatomy and Embryology: Coronary Anomalies  Because the aortic root is rotated in TOF, coronary artery anomalies can occur  Most common (3%) is origin of LAD from RCA  Double LAD occurs 1.8% of time  Least common anomalies are single RCA or LCA (0.3% and 0.2% respectively)

15 Surgical Repair  Symptomatic infants are repaired early – can be palliated with a variety of shunts  Asymptomatic children are usually electively repaired early as well  Surgery involves repair of the VSD and enlargement of the RVOT with infundibular septum resection +/- use of a transannular patch  This can usually be performed in one step as long as pulmonary artery and its main branches are of adequate size  The surgery uniformally results in pulmonic regurgitation

16 Palliative Shunts Glenn Shunt

17 2D Echo Glenn Shunt: SVC->PA Source: Feigenbaum’s 6 th Ed.

18 Surgical Repair – Transannular Patch

19 Patients post-repair do well up to ~25 yrs post- operatively Modes of death: Sudden cardiac death Arrhythmias Congestive heart failure Natural History Nollert G. JACC 1997; 30:1374

20 The Adult with Repaired TOF  Patients often remain asymptomatic  Although decreased exercise capacity can often be elicited with objective testing  Clinical Presentation: heart failure, dyspnea on exertion, atrial and ventricular arrhythmias, syncope, sudden death  ECG findings include RAD, RVH/RAA and RBBB; QRS duration can be prolonged (>180ms is important to note)

21 Sequelae of TOF Repair  Residual lesions:  Ventricular septal defect  Branch pulmonary artery stenosis  Tricuspid regurgitation  Pulmonary regurgitation  Progressive RV dilation and dysfunction  Progressive LV dysfunction  Aortic root dilation  Exercise intolerance, heart failure, arrhythmias and sudden cardiac death Courtesy A. Valente MD

22 Imaging in Repaired TOF  Non-invasive imaging is the mainstay of longitudinal follow-up in previously repaired TOF  Echocardiography is used to evaluate: residual VSD/PS, Ao Root size and associated AR, PR, and RV/LV function  CMR is used to determine RV volumes and severity of PR  Often these modalities are used in a complementary fashion

23 Pulmonary Regurgitation  Nearly universal  Severity is dictated by:  compliance of the RV  capacitance of the pulmonary arteries  Early: presence of RVH ( ↓ RV compliance) and small PAs ( ↓ capacitance) →↓ PR  Late: dilation and thinning of the RV ( ↑ compliance) and dilation of the PAs ( ↑ capacitance) →↑ PR Courtesy A. Valente MD

24 Pulmonary Regurgitation Courtesy A. Valente

25 Effects of Chronic PR  Adaptive mechanisms in chronic PR  increased RV end-diastolic volume  increased RV stroke volume  These mechanisms compensate for the hemodynamic burden placed on the RV for many years  Studies in the 1970’s – 1980’s on survivors of TOF repair were largely asymptomatic (based on self-reporting)

26 Geva T. STCVS 2006; 9:11.

27 Effects of Chronic PR Compensatory mechanisms exist up to a certain point, but ultimately these mechanisms fail Courtesy A. Valente MD

28 Effects of Chronic PR Good RV FunctionPoor RV Function Courtesy A. Valente

29 Severity of Pulmonary Regurgitation Prospective study of 34 adults with repaired TOF Echocardiogram & cardiac MRI within 3 months Median age 33 yrs (  12 yrs) Mean time since initial surgical repair 25  8 yrs 13 subjects had undergone transannular patch 6 subjects had undergone bioprosthetic PVR Silversides C. JASE 2003; 16: 1057

30 Pulmonary Regurgitation % PR and volume are inversely related to the pressure half-time: r = -0.6, p <0.001 Mild PR Severe PR Silversides C. JASE 2003; 16: 1057

31 Pulmonary Regurgitation Silversides C. JASE 2003; 16: 1057 In addition, PHT 20%)

32 Geva T. JACC 2004; 43(6): 1068 Biventricular Interaction  Median age from repair 21 years  Unfavorable ventricular- ventricular interaction  Confirmatory data that RV mechanics are only part of the problem  Patients repaired at older age, more likely to have poor clinical status later

33 RV Function by Echocardiography  Often adults with repaired TOF cannot undergo CMR due to devices  Myocardial Performance Index (MPI) has been shown to correlate with MRI RVEF  Retrospective study of 57 adults (repaired TOF) with a CMR and Echo within 6 months of each other  RV MPI = (Doppler duration of TR-RV ejection time)/RV ejection time Schwerzmann, M. AJC 2007;99:1593

34 RV MPI Correlation With CMR RVEF Schwerzmann, M. AJC 2007;99:1593 MPI = (a-b)/b

35 RV MPI Correlation With CMR RVEF Schwerzmann, M. AJC 2007;99:1593

36 Regional Wall Motion Abnormality Davlouros et al. JACC 2002; 40: subjects repaired TOF underwent MRI  RVOT outflow aneurysm/akinesia present in 57%  No significant difference in the type of repair  Aneurysm/akinesia negative effect on RVEF

37 Pulmonary Valve Replacement  Operative risk is small: mortality < 2%   What criteria should we use in patient selection?  Traditional indication: patient symptoms  Is there a risk to waiting until patients develop symptoms? *Oosterhof T. Heart 2007; 93: 506 Patients may not detect subtle changes in exercise capacity Patients may not detect subtle changes in exercise capacity By the time patients notice symptoms, problems may be severe and irreversible By the time patients notice symptoms, problems may be severe and irreversible

38 Predictors of Adverse Outcome  88 subjects with repaired TOF  Surgical repair between  CMR between  Median follow-up from MRI 4.2 yrs  22 subjects had a major clinical event  4 deaths  8 sustained VT  10 change in NYHA class from good to poor  Larger RVEDV, LVEF<50%, RVEF<45% by CMR predicted adverse events Knauth A. Heart 2008; 94:

39 Proposed Criteria for PVR  Balance between patient’s clinical status (exercise capacity, heart failure symptoms, arrhythmia) and quantitative information  Decision to do PVR is quite variable center to center  Repaired TOF with moderate or severe PR (PR RF >25% by CMR) and > 2 criteria  RVEDVi > 160 cc/m 2 ( z > 5)  RVESVi > 70 cc/m 2  LVEDVi < 65cc/m 2  RVEF < 45%  RVOT aneurysm  LVEF < 50% Geva T. STCVS 2006; 9:11.

40 Aortic Root Dilation  Aortic root dilation occurs in a subset of repaired TOF adults and can lead to significant AR  May be a result of R  L shunt prior to repair though not fully understood why it progresses after  A small retrospective study identified risk factors for Ao root dilation (defined as Ao root size observed:expected >1.5)  Therefore it is important to closely follow Ao root size with imaging longterm

41 Aortic Root Dilation Niwa, K. Circulation 2002;106:1374

42 Predictors of Arrhythmia and SCD  A study in England evaluated data from 793 repaired TOF patients  QRS duration >180ms was found to be predictive of SCD and ventricular arrhythmias  Older age at repair was associated with Afib/AFlutter and SCD  QRS duration rate of change may also be significant predictor of SCD Gatzoulis, M. Lancet 2002; 356:95

43 Recommendations  ECG (QRS duration): every 12 months  Exercise Testing: every months  Echo: every 24 months  CMR (RVEDVi, RV/LV EF): every 24 months  EP testing: when clinically indicated  Echo and CMR are used together  Authors from CHB Geva T. STCVS 2006; 9:11.

44 Summary  Because of successful childhood repair, larger population of adults with repaired TOF exists and can present to adult cardiologists  Pulmonary Regurgitation is predominant hemodynamic abnormality leading to RV dilation and dysfunction  Timing of surgery for PR is an area of great interest as clinical symptoms do not always correlate with severity of PR and RV dysfunction.  Echo and CMR are used together to follow repaired patients long term  Aortic root dilation occurs in a subset of patients and must also be followed closely  QRS duration >180 ms is an important predictor of ventricular arrhythmias and SCD

45 References  Feingenbaum’s Echo Textbook, 6 th Ed.  Echocardiography in Pediatric and Congenital Heart Disease Editors Lai, Mertens, Cohen, Geva 2009  Yale Congenital Heart Disease website:  Braunwald’s Textbook Heart Disease

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