Pulmonary Regurgitation and Pulmonary Valve Replacement …webcast.aats.org/2013/files/Saturday/20130504_101b_0800_11.00... · Disclosure Co-Investigator Medtronic Melody® Transcatheter

Pulmonary Regurgitation and Pulmonary Valve Replacement

in the Asymptomatic Patient Stephanie Fuller, MD, MS Assistant Professor of Surgery

Perelman School of Medicine at the University of Pennsylvania

Surgical Director, Philadelphia Adult Congenital Heart Center

A Joint Program of The Children’s Hospital of Philadelphia and Penn Medicine

Pulmonary Regurgitation and Pulmonary Valve Replacement in the Asymptomatic Patient

Stephanie Fuller, MD, MS Assistant Professor of Surgery

Perelman School of Medicine at the University of Pennsylvania

Surgical Director, Philadelphia Adult Congenital Heart Center

A Joint Program of The Children’s Hospital of Philadelphia and Penn Medicine

Disclosure

Co-Investigator

Medtronic Melody® Transcatheter Pulmonary Valve Post-Approval Study

CHOP IRB Number 10-007621

No financial disclosures

STS Database Valve Procedures 2011

AVR

MVR

TVR

PVR

4,028

5,573

315

19,437 19,437

STS Adult Database 2011

160

198 546

61

STS Congenital Database 2011 – Adult only

29,353 Total Valve Replacements Pulmonary: 1.07%

965 Total Valve Replacements Pulmonary: 56.6%


AVR

MVR

TVR

PVR

4,028

5,573

315

19,437

19,437

STS Adult Database 2011

160

198 604

61


29,353 Total Valve Replacements Pulmonary: 1.07%

965 Total Valve Replacements Pulmonary: 56.6%

LESS THAN 1,000 PULMONARY VALVE REPLACEMENTS PERFORMED

ANNUALLY IN ADULTS


4,028

5,573

315

19,437 19,437

546


965 Total Valve Replacements Pulmonary: 56.6% 0

100

200

300

400

500

600

Replacement Repair Other

9.5%

Total 612

Replace 546

Repair 58

Other 8

89.2%

1980 –

1970 –

1960 –

1940 –

0 10 20 30 40

Decade

Born with

CHD

50 60 70 80 90 100

80

50

15

5

Warnes et al. J Am Coll Cardiol 2001;37:1170-75.

Van der Velde ET et al. Eur J Epidem 2005:20:549-57.

1% D-TGA and 10% of TOF to adulthood

Epidemiology Survival to 18 yrs of Age with Complex CHD

Percent Survival to 18 Years Old

1,300,000 –

–

–

1,000,000 –

–

–

700,000 –

–

500,000 –

–

300,000 –

–

100,000 –

0 –

1970 1980 1990 2000 2010

Adult

CHD

Patients

325,000

500,000

750,000

1,000,000

1,300,000

20,000 new patients/yr

5% increase/yr

Hoffman 1978 Kaiser

Fyler 1980 New England

Ferencz 1985 Baltimore-DC

Epidemiology

Pulmonary Regurgitation

Residual pulmonary regurgitation is an important determinant of outcome in patients with tetralogy of Fallot as it may contribute to:

Right ventricular (RV) enlargement

Right ventricular (RV) function

Exercise intolerance

Propensity for arrhythmias

Increased risk for sudden cardiac death

Causes of Death in Tetralogy of Fallot Survivors

Gatzoulis et al. Risk factors for arrythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356:975-81.

Indications for Pulmonary Valve Replacement




Predictors of Sudden Cardiac Death

History

Syncope

Later age at Repair

Associated morbidity

Activity level

Hemodynamic

Enlargement of RV

Severity of PR

Severity of TR

Ventricular Function

Electrophysiologic

QRS Duration

Incidence of SVT

Sustained SVT

Khairy et al. Value of programmed ventricular stimulation after tetralogy of Fallot repair: A multicenter study. Circulation 2004:109;1994-2000.


Symptoms Asymptomatic Incidental

Exercise Intolerance

Anticipation of

Symptoms

i.e. Pregnancy

Planned

Data:

Electrophysiologic

Echocardiographic

MRI

Exercise Testing

Pulmonary Stenosis

Residual VSD

Severe PA

Enlargement

Tricuspid Valve

Regurgitation


Symptoms Asymptomatic Incidental

Exercise Intolerance

Anticipation of

Symptoms

Data:

Electrophysiologic

Echocardiographic

MRI

Exercise Testing

Additional Lesions:

Pulmonary Stenosis

Residual VSD

Severe PA

Enlargement

Tricuspid Valve

Regurgitation

CMR Protocol for TOF

• Static true-FISP (Fast Imaging with Steady State Precision)

– Contiguous axial images to assess CV anatomy

• Multiplanar Reformatting – 4-chamber and short axis ventricular views for cine

– MPA and branch PA flow

• Cine Imaging

• Phase encoded velocity mapping – Aortic annulus, MPA, branch pulmonary arteries

– Stroke Volume = EDV-ESV

– Ejection Fraction = Stroke Volume/EDV

– Cardiac Output = Stroke Volume x HR

– Regurgitant Fraction =( reverse flow/forward flow) x100 *EKG gated

Are the Indications the Same for All Patients?

• Data:

– Severe PR

– Mild – Moderate TR

– RVEF 40%

– LVEF 60%

– RVEDVi 172cc/m2

Are the Indications the Same for All Patients?

• Data:

– Severe PR

– Mild – Moderate TR

– RVEF 40%

– LVEF 60%

– RVEDVi 172cc/m2

• What if your patient is:

– 19yo Male Sedentary

– 27yo Female planning

pregnancy

– 43yo Single mother of two

– 52yo Attorney

– 17yo NCAA athlete

– 15yo highschool athlete

Risk of Pulmonary Valve Replacement

Most studies suggest that restoration of pulmonary valve competency:

Decreased RV Volume and size

Improved exercise tolerance and capacity

Decreased propensity for arrhythmias

Decreased risk for sudden cardiac death

± Possible decrease in QRS duration

± Possible improvement in RV function

Indications


Parameters: RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %


Parameters:

RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %



Parameters:

RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %



Kogon et al. Risk factor for early pulmonary valve replacement after valve disruption in congenital pulmonary stenosis and tetralogy of Fallot. JTCVS 2009;138:103-8.



RVEDV > 160mL/m2

Thresholds for PVR

Does PVR Improve RV Size?

Adamson et al. ICVTS 2009;9:520-7.

0

100

200

300

400

RVEDV ml

ml/m2

Pre PVR

Post PVR

Change in NYHA Classification after Pulmonary Valve Replacement

Discigil et al. Late Pulmonary Valve Replacement after repair of Tetralogy of Fallot. JTCVS 2001;121:344-51

Change in NYHA Classification after Pulmonary Valve Replacement

Gengsakul et al. The impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison. EJCTS 2007;32:462-8.

Results after Pulmonary Valve Replacement

• Graham et al., 2008

– 93 adult patients

• Decreased RV size by 83% on echo (p<0.001)

• Systolic function increased by 36% (p=0.09)

• Ability index improved in 59% (p<0.001)

• NYHA status improved in 57%

• No improvement in arrhythmia

Surgical Options

• Repair

– RVOT Revisions and Bicuspidization

– Valve Repair

• Resuspension

• Lengthening of Leaflets

• Replacement

– Monocusp

– Homograft Valve

– Heterograft Valve – Bovine, Porcine

– Mechanical Valve

– Percutaneous Heterograft Valve

Valve Repair

Cowgill et al. Repair of Pulmonary Valve insudficiency Using an Autologous Monocusp. Ann Thorac Surg1986;42:587-9.ç

Valve Repair

Cowgill et al. Repair of Pulmonary Valve insudficiency Using an Autologous Monocusp. Ann Thorac Surg1986;42:587-9.ç

Valve Repair

Papadopoulos et al. Secondary Repair of Incompetent pulmonary Valves. Ann Thorac Surg 2009;87:1879-84

Valve Repair

Mainwaring et al. Late Repair of the Native Pulmonary Valve in Patients with Pulmonary Insufficiency After Surgery for Tetralogy of Fallot. Ann Thorac Surg 2012;93:677-9.

Valve Repair

Mainwaring et al. Late Repair of the Native Pulmonary Valve in Patients with Pulmonary Insufficiency After Surgery for Tetralogy of Fallot. Ann Thorac Surg 2012;93:677-9.

Valve Repair

Pretre et al. Recycling fo the Pulmonary Valve: An Elegant Solution for Secondary Pulmonary Regurgitation in Patients with Tetralogy of Fallot. Ann Thorac Surg 2012;94:850-3.

Valve Replacement

Pulmonary Valve Replacement – Advanced Considerations

• Strut Position – 6/10/2 o’clock

• Valve Orientation

• Need for pulmonary arterioplasty

• Possible RV Remodeling

– Resection of Transannular Patch

– Enlargement of RVOT

• Consider future operation

– Percutaneous Valve Insertion

Valve Replacement

Misbach et al. Pulmonary valve replacement for regurgitation after Repair of Tetralogy of Fallot. Ann Thorac Surg 1983;36:684-91

Valve Replacement

Misbach et al. Pulmonary valve replacement for regurgitation after Repair of Tetralogy of Fallot. Ann Thorac Surg 1983;36:684-91

Valve Replacement

Kogon et al. Adult Congenital Pulmonary Valve Replacement: A Simple, Effective and Reproducible Technique . Cong Heart Dis. 2007:2;314-8.

Valve Replacement

Kogon et al. Adult Congenital Pulmonary Valve Replacement: A Simple, Effective and Reproducible Technique . Cong Heart Dis. 2007:2;314-8.

Comparison of Bioprosthesis Durability

Zubari et al. Risk Factors for Prosthesis Failure in Pulmonary Valve Replacement. Ann Thorac Surg 2011;91:561-5.

Risk Factors for Prosthesis Failure

Zubari et al. Risk Factors for Prosthesis Failure in Pulmonary Valve Replacement. Ann Thorac Surg 2011;91:561-5.

Chen et al. Younger age and valve oversizing are predicators of structural valve deterioration after pulmonary valve replacement in tetralogy of Fallot. JTCVS 2012;143:352-60.

Risk Factors for Prosthesis Failure

Deorsola et al.

– 4 patients @ 11 years post PVR with mechanical valve 23mm

– ¾ patients compliant with Coumadin

– F/up Echocardiogram:

• Markedly improved RV function

• Mild Dilatation

• Mild residual pulmonary gradients (10-35mm Hg) with stable RV hypertrophy

Mechanical Pulmonary Valve Replacement

Deorsola et al. Pulmonary Valve Replacement with Mechnical Prosthesis: Long -term Results in 4 Patients. Annals Thoracic Surg 2010:89;2036-8.

Waterbolk et al.

– 27 mechanical PVRs

• Mortality – 1 early and 1 late due to complications of fatal anticoagulation related pulmonary hemorrhage

• Failure – one replaced due to fibrous tissue overgrowth

• Mean f/up is 5.5 yrs. (2mos – 18yrs)

Mechanical Pulmonary Valve Replacement

Waterbolk et al. Pulmonary valve replacement with a mechanical prothesis. Eur J Cardiothorac Surg; 2006;30(1):28-32.

Percutaneous Pulmonary Valves

Melody™ • Bovine jugular venous valve

segment

• Platinum-Iridium Stent

Sapien™ • Bovine pericardial tissue

leaflets

• Stainless Steel Stent

23 mm 27 mm

• Fig. 2A and 2B – Anteroposterior and lateral views of a dysfunctional right ventricle to pulmonary artery conduit demonstrating conduit stenosis and insufficiency.

• Fig. 3A and 3B – Anteroposterior and lateral views of the conduit following Melody valve insertion. A bare metal stent was introduced first to alleviate conduit stenosis. This demonstrates no residual pulmonary valve insufficiency or obstruction to forward flow.

Melody Valve Implantation

Melody Valve Implantation

Results of Melody Valve Implantation

Lurz et al. JACC 2011;57(6):724-31.

Conclusions

Longitudinal assessment of pulmonary insufficiency and RV function remain critical in the medical care of the patient with pulmonary valve disease including

tetralogy of Fallot.

The number of patients requiring pulmonic valve interventions will increase.

The optimal timing of PV replacement is still unknown.

No prospective randomized trial exists in children with PR after TOF repair.

Conclusions

Pulmonary valve repair may be a viable strategy but long term results unknown.

All choices of pulmonary valves have limited durability. With the widespread use of percutaneous pulmonary valves, earlier implantation may improve

long term results.

Considerations include:

• Durability of percutaneous valves

• Need for repeat interventions

Conclusions

Percutaneous valves will eventually be approved for use in the native RVOT.

However, candidates will be limited by:

• Size of RVOT

• Residual hemodymanically significant lesions

– Pulmonary Artery Stenosis

– Ventricular Septal defects

Factors influencing progression of PR after TOF Repair

Abnormalities in Post op TOF

Repaired TOF

• RV power output vs

RVEDV and RVESV

demonstrating

exponential decay

• Cath and CMR within

6 months of each

other

• 20% power loss

Fogel, CHOP 2010

RV Output Power Index vs. RVEDVNormalized

y = 32295x-1.20

R = 0.87

0

50

100

150

200

250

300

350

0 50 100 150 200 250 300

RVEDVNormalized

RV O

utpu

t Pow

er In

dex

RV Output Power Index vs. RVESVNormalized

y = 2168.7x-0.78

R = 0.68

0

50

100

150

200

250

300

350

0 50 100 150 200

RVESVNormalized

RV O

utpu

t Pow

er In

dex

Documents

Pulmonary Regurgitation and Pulmonary Valve Replacement …webcast.aats.org/2013/files/Saturday/20130504_101b_0800_11.00... · Disclosure Co-Investigator Medtronic Melody® Transcatheter