Ross Procedure in Neonates and Infants: A European Multicenter Experience

doi:10.1016/j.athoracsur.2015.08.008

The Annals of Thoracic Surgery

Volume 100, Issue 6, December 2015, Pages 2278-2284

https://doi.org/10.1016/j.athoracsur.2015.08.008 Get rights and content

Background

Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability.

Methods

A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013.

Results

Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts.

Conclusions

Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding.

Section snippets

Patients and Methods

Institutional Review Board permission for this study was obtained in each participating center. Informed consent was obtained from parents or legal guardians.

Results

Preoperative patient characteristics are shown in Table 1. Two babies were born prematurely (gestational age 33 and 35 weeks) after induction of labor for chronic severe left ventricular dysfunction. Both underwent balloon valvuloplasty of the aortic valve shortly after birth. On day 97 and 20, respectively, the aortic valve was replaced with a pulmonary autograft. Both patients survived and remained in good clinical condition, now more than 6 years after the operation.

Comment

Replacement of the aortic root with the pulmonary autograft, with or without enlargement of the left ventricular outflow tract by septal myectomy, has become an important surgical treatment modality for infants and neonates with critical aortic stenosis. Our study has expanded the clinical knowledge of this complex patient population.

Early mortality in our cohort was substantial, especially in neonatal patients. Woods and associates [5] analyzed The Society of Thoracic Surgeons congenital heart

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Cited by (36)

Aortic insufficiency following balloon aortic valvuloplasty does not impact long-term autograft durability after the Ross procedure
2024, Journal of Thoracic and Cardiovascular Surgery
Preoperative aortic insufficiency (AI) is associated with inferior autograft durability after the Ross procedure. However, many patients with aortic stenosis (AS) undergo balloon aortic valvuloplasty (BAV) early and present with longstanding AI before Ross. We studied how BAV and subsequent valvular pathology impacts autograft durability.
Patients undergoing the Ross operation from 1993 to 2020 were identified. Those who underwent BAV before Ross were compared with patients who did not undergo BAV and underwent Ross for predominant AI (AI group) or AS (AS group). Those who underwent previous open surgical aortic valve intervention were excluded. Primary outcome of interest was autograft failure, defined as a composite of autograft reintervention or severe insufficiency.
A total of 198 patients were included. Seventy-nine (39.9%) underwent BAV and subsequently underwent the Ross for predominant AI (45.6%) or AS (54.4%). Of patients who did not undergo BAV, 66 (33.3%) presented with predominant AI and 53 (26.8%) with AS. Freedom from autograft failure at 15 years was 90%, 92%, and 62% in BAV, AS, and AI groups, respectively. The AI group was at significantly increased risk of long-term autograft failure (hazard ratio, 5.6; P = .01), whereas the AS and BAV groups had similar, low risk (hazard ratio, 1.1; P = .91). Autograft durability was similar among patients who received BAV and presented with AS or AI before the Ross (P = .84).
BAV before the Ross procedure is common in patients with AS. These patients have excellent long-term autograft durability regardless of preoperative valvular pathology and should strongly be considered for the Ross operation.
Ross Procedure in Children: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis
2023, Annals of Thoracic Surgery
Single-center studies have demonstrated excellent results for the Ross procedure in children. We aimed to evaluate national variation in clinical outcomes using The Society of Thoracic Surgeons Congenital Heart Surgery Database.
The database was used to identify 2805 children undergoing the Ross procedure from 2000 through 2018, comprising 163 neonates (<30 days, 5.8%), 448 infants (30-365 days, 16.0%), 1444 children (1-12 years, 51.5%), and 750 teenagers (13-17 years, 26.7%). Centers were divided into terciles by procedural volume. Multivariable logistic regression was used to identify predictors of a composite outcome of operative mortality, neurologic deficit, or renal failure requiring dialysis.
Neonates and infants were more likely to present with aortic stenosis than children and teenagers (61.7% [n = 377] vs 34.6% [n = 760]; P < .01) and have risk factors including preoperative shock (9.2% [n = 56] vs 0.4% [n = 8]; P < .01). Operative mortality was 24.1% (n = 39) in neonates, 11.2% (n = 50) in infants, 1.5% (n = 21) in children , and 0.8% (n = 6) in teenagers (P < .01). Independent predictors of the composite outcome in children aged <1 year included neonatal age (odds ratio [OR], 3.0; 95% CI, 1.9-4.8), low-volume center (OR, 2.1; 95% CI, 1.1-3.9), and procedure year (OR, 0.7; 95% CI, 0.5-0.9 per 5 years). In children aged ≥1 year, no association was found between center volume, procedure year, and outcome.
The Ross procedure is being performed with low mortality in children aged ≥1 year throughout North America. High-volume centers have improved outcomes in children aged <1 year, who have different anatomic characteristics and risk profiles.
Ross procedure in neonates and infants: A valuable operation with defined limits
2023, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
Potential disadvantages of the Ross procedure include putting both semilunar valves at risk with an associated increase in reintervention rates, notably for the RVOT.9 In addition, several single-center, multicenter, and meta-analysis publications have reported high early mortality rates for neonates and infants who underwent the Ross procedure.5,7-10,12-14 Our results confirm these findings with a 19% mortality rate in those younger than 1 year old and a 39% mortality rate in neonates.
The Ross procedure is an important tool that offers autologous tissue repair for severe left ventricular outflow tract (LVOT) pathology. Previous reports show that risk of mortality is highest among neonates and infants. We analyzed our institutional experience within this patient cohort to identify factors that most affect clinical outcome.
A retrospective chart review identified all Ross operations in neonates and infants at our institution over 27 years. The entire study population was analyzed to determine risk factors for mortality and define outcomes for survival and reintervention.
Fifty-eight patients underwent a Ross operation at a median age of 63 (range, 9-156) days. Eighteen (31%) were neonates. Eleven (19%) patients died before hospital discharge. Multiple regression analysis of the entire cohort identified young age (hazard ratio [HR], 1.037; P = .0045), Shone complex (HR, 17.637; P = .009), and interrupted aortic arch with ventricular septal defect (HR, 16.01; P = .031) as independent predictors of in-hospital mortality. Receiver operating characteristic analysis (area under the curve, 0.752) indicated age younger than 84 days to be the inflection point at which mortality risk increases. Of the 47 survivors, there were 2 late deaths with a mean follow-up of 6.7 (range, 2.1-13.1) years. Three patients (6%) required LVOT reintervention at 3, 8, and 17.5 years, respectively, and 26 (55%) underwent right ventricular outflow tract reintervention at a median of 6 (range, 2.5-10.3) years.
Ross procedure is effective in children less than one year of age with left sided obstructive disease isolated to the aortic valve and/or aortic arch. Patients less than 3 months of age with Shone or IAA/VSD are at higher risk for morbidity and mortality. Survivors experience excellent intermediate-term freedom from LVOT reintervention.
Ross and Ross-Konno procedure in pediatric patients: ¿is the age a risk factor?
2022, Cirugia Cardiovascular
La estenosis valvular aórtica severa en edad pediátrica supone un auténtico reto quirúrgico. Las opciones terapéuticas son altamente complejas, especialmente en niños menores de un año de edad. La cirugía de Ross está indicada en aquellos casos donde la reparación quirúrgica o la valvuloplastia con balón no han sido factibles. El objetivo del estudio es comparar los resultados postoperatorios y en el seguimiento de los pacientes menores de un año con la totalidad de pacientes intervenidos de Ross/Ross-Konno en nuestro centro.
Se recogieron de manera consecutiva todos los pacientes intervenidos en nuestro centro mediante estos dos procedimientos durante los últimos 10 años. Durante ese periodo, se intervinieron a 11 pacientes (cinco Ross y seis Ross-Konno) de los cuales tres corresponden a pacientes menores de un año.
La mortalidad hospitalaria es del 9,1% (un paciente) con una supervivencia a 10 años del 90,9%. El porcentaje de reoperaciones en el seguimiento es del 72,7% siendo mayor en los pacientes menores de un año (100 vs. 62,5%). Sólo un paciente requirió una reintervención sobre el autoinjerto en edad adulta. La necesidad de intervención sobre el tracto de salida derecho es del 64,6% a 10 años.
La mortalidad postoperatoria y supervivencia a los 10 años de los pacientes intervenidos durante el primer año de vida no difiere del resto de pacientes de mayor edad, aunque la tasa de reintervenciones en el seguimiento es mayor en este subgrupo de pacientes.
Severe aortic valve stenosis in pediatric population represents a real surgical challenge. Therapeutic options are highly complex, especially in children under one year of age. Ross procedure is indicated in those cases where surgical repair or balloon valvuloplasty has not been feasible. The aim of the study is compare the postoperative outcomes and in the follow-up of patients under one year with all the patients who underwent Ross / Ross-Konno procedure in our centre.
Consecutive patients operated in our centre with these two procedures for the last ten years were collected. During this period, 11 patients were operated (5 Ross and 6 Ross-Konno) of which 3 corresponded to patients under one year of age.
Hospital mortality is 9,1% (1 patient) with a 10-year survival of 90,9%. The reoperations percentage is 72,7%, higher in patients less than 1-year-old (100% vs. 62,5%). Only one patient required an autograft reoperation in the adulthood. The right ventricle outflow tract reoperation rate is 64,6% at 10 years.
Postoperative mortality and survival at 10 years of patients operated on during the first year of life does not differ from the rest of older patients, although the rate of reoperations in the follow-up is higher in this subgroup of patients.
Current perspectives in Ross and Ross-Konno procedures: Is it time to search for alternatives?
2022, Anales de Pediatria
Commentary: Ross procedure in neonates and infants: Withstanding the litmus test of time
2022, Journal of Thoracic and Cardiovascular Surgery

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Original articleCongenital heart surgeryRoss Procedure in Neonates and Infants: A European Multicenter Experience

Background

Methods

Results

Conclusions

Section snippets

Patients and Methods

Results

Comment

J Am Coll Cardiol

J Thorac Cardiovasc Surg

Ann Thorac Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Original article
Congenital heart surgery
Ross Procedure in Neonates and Infants: A European Multicenter Experience