Outcomes following simple and complex (Damus-Kaye-Stansel takedown) Ross operations

in 62 consecutive pediatric patients

Alejandra Bueno MD, David Zurakowski PhD, Vijayakumar Raju MD, Michele J. Borisuk MSN, CPNP, Suyog A. Mokashi MD, Sitaram Emani, MD, Gerald R.

Marx MD, Pedro J. del Nido MD, and Christopher W. Baird MD

No disclosures

Introduction

The Ross operation has good outcomes in selected pediatric patients with a biventricular circulation.

However, there have been no reports in patients with uni-ventricular circulation who have undergone single ventricle palliation followed by biventricular conversion with Ross operation.

Objectives

Review outcomes following simple and complex Ross operations including patients undergoing biventricular conversion with Damus-Kaye-Stansel (DKS) takedown.

To determine predictors for successful Ross operation based on echocardiographic measurements.

Methodology

A retrospective review on 62 patients who underwent Ross operations at Boston Children's Hospital from March 2000 and October 2014.

Simple (50 pts.) – Ross operation in patients who had a biventricular circulation.

Complex (12 pts.) –Patients with pre-operative uni-ventricular circulation with hypoplasia of the left heart structures who then underwent biventricular conversion with DKS takedown and a Ross operation.

Data collectionClinical data

IRB approval was obtained.Medical records were reviewed.

EchocardiographyData was measured independently by a single reviewer

and a random sample was reviewed by a second reviewer.

Echocardiography

Aortic Annulus Ascending Aorta

Sinus of Valsalva

Measurement of Vena Contracta

Aortic regurgitation was estimated from the vena contracta width indexed to the square root of the body surface area.

Patient Characteristics

Follow-up All patients (n=62 patients)

Follow-up was available in 54 patients (87%) At a median of 2.7 years (IQR, 8m-5.2 years)

Simple group (n=50 patients) Follow-up was available in 45 patients At a median of 42 months (IQR, 8m-66m)

Complex group (n=12 patients) Follow-up was available in 11 patients At a median of 18 months (IQR, 9m-41m)

Time Since Ross Operation (months)

0 6 12 18 24 30 36 42 48 54 60 66

Pa

tie

nt

Su

rviv

al

(%)

0

10

20

30

40

50

60

70

80

90

100

Complex Group (2 deaths in 12 patients)

Simple Group (0 deaths in 50 patients)

Group difference: P = .004, log-rank test = 8.30

(50) (36) (34) (28) (23) (16)(12) (8) (5) (4) (3) (2)

Median (IQR) Follow-up:Simple Group: 42 months (8-66)Complex Group: 18 months (9-41)

Patient Survival

Time Since Ross Operation (months)

0 6 12 18 24 30 36 42 48 54 60 66

Fre

ed

om

fro

m n

eo

-Ao

Va

lve

R

e-i

nte

rve

nti

on

(%

)

0

10

20

30

40

50

60

70

80

90

100

Complex Group (0 events in 12 patients)

Simple Group (2 events in 50 patients)

No group difference: P = .63, log-rank test

Median (IQR) Follow-up:Simple Group: 42 months (8-66)Complex Group: 18 months (9-41)

Freedom from Neo-aortic Valve Re-intervention

Pre

op

era

tive

Ec

ho

Z-s

co

res

-4

-3

-2

-1

0

1

2

3

4

P = .25

P = .04

P = .13

Simple GroupComplex Group

Sinus of Valsalva Aortic Annulus Ascending Aorta

Pre-operative Echocardiography Z-scores

Aortic annulusEchocardiographic Z-scores

Sinus of Valsalva Echocardiographic Z-scores

Aortic regurgitation – Vena contracta - Indexed to BSA (mm/m)

Baseline Pulmonary Valve Z-score

-3 -2 -1 0 1 2 3 4 5

Lat

e F

oll

ow

-up

Ao

rtic

Reg

urg

itat

ion

(

Ven

a C

on

trac

ta -

In

dex

ed t

o B

SA

)

0

1

2

3

4

5

6

Complex Group, r = -0.15, P = .67

Simple Group, r = -0.08, P = .64

Lack of correlation between initial pulmonary valve

Z-scores and late aortic regurgitation.

Pre-operative Pulmonary Valve Z-score

LimitationsRetrospective

Single center study

Patient selection bias - as patients with large dilated aortic roots and ascending aortas were generally not operated with a Ross procedure.

Complex group of patients with multiple additional lesions.

ConclusionsThe aortic valve complex including the aortic annuli, the

sinus of Valsalva and ascending aorta increased in size without development of late aortic regurgitation.

Neither pulmonary valve size or native pulmonary and aortic annular size discrepancy should be a contraindication for Ross procedure.

The Ross operation should be considered in patients with significant aortic valve disease undergoing biventricular conversion with DKS takedown.

Baseline Pulmonary Valve Z-score

-3 -2 -1 0 1 2 3 4 5

Lat

e F

oll

ow

-up

Ao

rtic

Reg

urg

itat

ion

(

Ven

a C

on

trac

ta -

In

dex

ed t

o B

SA

)

0

1

2

3

4

5

6

Complex Group, r = -0.15, P = .67

Simple Group, r = -0.08, P = .64

No correlation between base line PV Z-score and late follow-up aortic regurgitation.

Figure 3C

Time Since Ross Procedure (months)

0 6 12 18 24 30 36 42 48 54 60 66 72

Fre

ed

om

fro

m R

VO

T R

e-in

terv

enti

on

(%

)

0

10

20

30

40

50

60

70

80

90

100

Kaplan-Meier 5-Year Freedom from Re-intervention (95% CI) RVOT Surgical: 83% (72-94%) RVOT Cath: 87% (76-98%) RVOT Surgical or Cath: 71% (58-84%)

RVOT Surgical Re-interventions (n = 7)RVOT Cath Re-interventions (n = 6)RVOT Surgical or Cath Re-interventions (n = 13)

(62) (44) (39) (32) (26) (18) (10)

Time Since Ross Operation (months)

0 6 12 18 24 30 36 42 48 54 60 66

Fre

edo

m f

rom

RV

OT

Su

rgic

al

or

Ca

th

Re-

inte

rven

tio

n (

%)

0

10

20

30

40

50

60

70

80

90

100

(50)(12)

(36)(8)

(34)(5)

(28)(4)

(23)(3)

(16)(2)

Figure 3D

Complex Group (3 events in 12 patients)

Simple Group (10 events in 50 patients)

No group difference: P = .22, log-rank test

Figure 4A

Ascending Aorta Z-score (Pre-Echo)

-6 -4 -2 0 2 4 6

Asc

end

ing

Ao

rta

Z-s

core

(L

ate-

Ech

o)

-6

-4

-2

0

2

4

6

Simple Group Complex Group r = 0.00, P = .98

Aortic Root Z-score (Pre-Echo)

-6 -4 -2 0 2 4 6

Ao

rtic

Ro

ot

Z-s

core

(L

ate-

Ech

o)

-4

-2

0

2

4

6

8

10

Simple Group Complex Group r = 0.38, P = .007

Figure 4B

Online table 2. Echo measurements.

Online table 3. Late outcomes.

Preop D/C Follow-up Preop D/C Follow-up

AA

Z-s

core

-3

-2

-1

0

1

2

3

4

5

6

Complex Group

Simple Group

P = .22

P = .74

Improvement in AA Z-score between discharge and late follow-up

Preop D/C Follow-up Preop D/C Follow-up

AA

Z-s

core

-3

-2

-1

0

1

2

3

4

5

6

Complex Group

Simple Group

P = .22

P = .74

Improvement in AA Z-score between discharge and late follow-up

P = .27