EARLY AND LATE PERSPECTIVES OF THE ROSS … · • Ross-Konno 955,562 ,988 ,000 ... Pasquali SK , JACC 2007 ... Early And Late Perspectives Of The Ross (Ross/Konno) Operation In Pediatric

EARLY AND LATE PERSPECTIVES OF THE ROSS(ROSS/KONNO) OPERATION IN PEDIATRIC POPULATION:AN ITALIAN MULTI-CENTRIC STUDY.

EARLY AND LATE PERSPECTIVES OF THE ROSS(ROSS/KONNO) OPERATION IN PEDIATRIC POPULATION:AN ITALIAN MULTI-CENTRIC STUDY.

XLIII National Congress SICP & SICCHPED, 16-19 October 2013, Padua.

G. Lucchese1, A. Carotti2, G. Brancaccio2, P.A. Abbruzzese3, G. Caianiello4, A.Frigiola5, G.D. Gargiulo6, L. Galletti7, S.M. Marianeschi8, A. Mazzucco1, B.Murzi9, C.N. Pace6, M. Pozzi10, L. Zannini11, G.B. Luciani1.

1Verona/IT, 2Rome/IT, 3Turin/IT, 4Monaldi H., Naples/IT, 5San Donato Milanese,Milan/IT, 6Bologna/IT, 7Bergamo/IT, 8Niguarda H., Milan/IT, 9Massa/IT,10Ancona/IT, 11Genova/IT.

None of the authors has any affiliation, financial agreement, or other involvement with any

company to disclose.

Early And Late Perspectives Of The Ross (Ross/Konno) Operation In Pediatric Population: An Italian Multi-centric Study.

Disclosure


Niguarda H. (Milan)

O.I.R.M. S. Anna (Turin)

Ospedale Pediatrico (Genova)

G.Pasquinucci H. (Massa)

O.P.B.G. (Rome)

Monaldi H. (Naples)

S. Orsola-Malpighi H. (Bologna)

Ospedali Riuniti (Ancona)

University Hospital (Verona)

Ospedali Riuniti (Bergamo)Ospedale Policlinico S. Donato (Milan)

Centers



The Ross Procedure (Indications)1. Hypoplasia of the aortic valve annulus in the neonate2. Progressive stenosis of the aortic valve in infants and

children3. Multilevel left ventricular outflow tract obstruction in

association with aortic valve stenosis not amenable to aortic valve repair that requires enlargement of the outflow tract

4. Aortic insufficiency as a complication of percutaneous balloon aortic valvuloplasty

5. Aortic valve endocarditis

Background



Background

Cox proportional hazard model, younger age among children (hazard ratio [HR] per year, 0.86; 95% CI, 0.80–0.93; P0.0001), older age

among adults (HR per year, 1.04; 95% CI, 1.02– 1.06; P0.0001), and presence of preoperative risk factors (HR, 1.38; 95% CI, 1.05–1.83;

P0.023) were associated with increased risk for late mortality.

95,0 % (1) 94,4 % (5) 92,5 % (10) 98,6 % (1) 96,9 % (5) 94,7 % (10)

Early (<30 days) mortality 1.2%.

Sievers HH, Circulation 2010Sievers HH, Circulation 2010



Background

1. A total of 24 patients younger than 1 year of

age

2. Age at operation ranged from 1 to 236 days

(median 28 days)

3. The median follow-up period was 81 months

(range 1–173 months)

4. 23 reoperations or reinterventions

performed after Ross-Konno operation

5. 14/23 consisted of RV-PA conduit

replacement.

Pulmonary autografts demonstrated gooddurability with low mortality and morbidity.

Maeda K, Ann Thorac Surg 2012Maeda K, Ann Thorac Surg 2012



Background

1. 121 patients2. Median age at

the Ross procedure was 8.2 years (4 days to 34 years)

Pasquali SK, JTCVS 2007Pasquali SK, JTCVS 2007



To define current outcome after Rossoperationfrom infancy to adolescencea multi-center observational studycovering 22 years of surgicalexperience was undertaken.

Objective



• Period of study 1990-2012

• Retrospective Study

• 11 Italian Pediatric Cardiac Units

• Descriptive and Kaplan-Meier analysis definedoutcome

• Cox regression identified risk factors

Methods



Patient’s Characteristics

N° 292

Median Age (Min.-Max.)• Age < 1 years old (%)

10 years (2 days to 18 years)35 (12%)

Indications:- Aortic stenosis (+/- sub-aortic)- Aortic regurgitation- Combined disease

10310485

Prior cardiac surgery 35% (104/292)

Techniques of operation:- Root- Root inclusion- Sub-coronary- Ross-Konno

196131667

Methods



The Ross Procedure Technique (Root)

Background



The Ross Procedure Technique (Variants)

Ross Operation: Root Inclusion Technique

Ross Operation: Sub-Coronary Technique

Background

Ross - Konno



Results

Early deaths N°10 (3.4%)

Median follow-up (min. – max.): 80.93 months (1.67 – 267.33 months)

5 years 93.2±1.5%

10 years 92.0±1.7%

15 years 82.8±8.9%

Late deaths N°12



Survival Stratified By Age

Median follow-up (min. – max.): 80.93 months (1.67 – 267.33 months)

Results

Neonates/Infants

Children/Adolescents

p=0,000

Deaths

Neonates/Infants

N° 14/24

Deaths

Children/Adolescents

N° 8/268



Results

5 years 95.7±1.3%

10 years 87.7±2.5%

15 years 73.3±7.2%

Left Heart Reoperations



Results

5 years 92.6±1.7%

10 years 83.1±2.9%

15 years 72.6±9.0%

• Overall 35 (12%)right-heart conduitreplaced

• Overall 35 (12%)right-heart conduitreplaced

Right Heart Reoperations



Results

SE Sig. HR

Technique: • Sub-Coronary ,606 ,044 ,296 • Root Inclusion 852,248 ,987 ,000 • Ross-Konno 955,562 ,988 ,000

Indication:

• Aortic Stenosis ,520 ,868 1,091 • Aortic Regurgitation ,735 ,798 1,206

Prior Cardiac Operation

,514 ,906 1,063

Age < 4 months

,511 ,000 17,147

Hazard Cox Analysis Risk Factors



Results



(*)The most of patients do not assume anticoagulation therapy

(**) 2,6% of women had pregnancy

• Ross operation is associated with excellent very long-term survival in a population of high risk infants and children with congenital aortic valve and complex LVOT disease

• Contrary to expectations, risk of autograft reoperation increases significantly 15 years after Ross and is similar to risk of right heart reoperation

• When performed in neonates and young infants (< 4 months), the Ross or Ross-Konno carries incremental hospital risk

• Ross operation is associated with excellent very long-term survival in a population of high risk infants and children with congenital aortic valve and complex LVOT disease

• Contrary to expectations, risk of autograft reoperation increases significantly 15 years after Ross and is similar to risk of right heart reoperation

• When performed in neonates and young infants (< 4 months), the Ross or Ross-Konno carries incremental hospital risk

Conclusions



• Ross operation should be reserved to children without feasible repair options

• Modifications of the root technique (support) and avoidance of the subcoronary technique in children may improve autograft durability

• The Ross or Ross-Konno should be delayed in neonates and infants whenever feasible

• High risk or repeat aortic valve balloon dilation should be discouraged to avoid need for neonatal/infant urgent Ross procedures

• Ross operation should be reserved to children without feasible repair options

• Modifications of the root technique (support) and avoidance of the subcoronary technique in children may improve autograft durability

• The Ross or Ross-Konno should be delayed in neonates and infants whenever feasible

• High risk or repeat aortic valve balloon dilation should be discouraged to avoid need for neonatal/infant urgent Ross procedures

Inferences



Discussion II

1. Six patients were younger than 3

months

2. Median age at follow-up was 15.6 years

3. Aortic valve/left ventricular outflow

4. tract (LVOT) anomalies were congenital

in 49 (92%)

5. Seventy percent had previous surgery

or balloon valvuloplasty

6. Root replacement was used in all

53 consecutive Ross procedures have been

performed in patients younger than 18 years.

Hazekamp MH, EJCTS 2005Hazekamp MH, EJCTS 2005




Discussion II




Discussion II



No patient developed significant autograftinsufficiency or required autograft reinterventionduring the follow-up period. Dilatation of theautograft occurred during the first year aftersurgery and stabilized thereafter.

• The Ross procedure performed in 27 infants < 18 m. of age (median age 5.7 months) (indication: all AS).

• Median follow-up was 6.1 years (range 0.2 to 12.9).

Williams IA, Circulation 2005Williams IA, Circulation 2005

Discussion II



Data on neo-aortic valve function and growth after the Ross procedure in children are limited.

• 74 Patients• 74 Patients

• Median age at Ross was 9 years (range 3 days to 34 years).

• At mid-term follow-up after the Rossprocedure, neo-aortic root size increasessignificantly out of proportion to so-matic growth, and neo-AI is progressive.

• Prior VSD repair and aortic valvereplacement were associated with neo-AIand reintervention.

Pasquali SK, JACC 2007Pasquali SK, JACC 2007

Discussion II



EARLY AND LATE PERSPECTIVES OF THE ROSS … · • Ross-Konno 955,562 ,988 ,000 ... Pasquali SK , JACC 2007 ... Early And Late Perspectives Of The Ross (Ross/Konno) Operation In Pediatric

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