Effect of Earlier Atrioventricular Valve Intervention on Survival After the Fontan Operation.

Published on Dec 15, 2020in American Journal of Cardiology2.57
· DOI :10.1016/J.AMJCARD.2020.09.028
Elizabeth H. Stephens2
Estimated H-index: 2
(Mayo Clinic),
Elizabeth H. Stephens25
Estimated H-index: 25
(Mayo Clinic)
+ 3 AuthorsFrank Cetta46
Estimated H-index: 46
(Mayo Clinic)
Whereas the prevalence and impact of atrioventricular valve (AVV) regurgitation in patients with single ventricle physiology has become increasingly apparent, the optimal timing for valve intervention is unclear. To investigate this, we performed a retrospective review of all 1,167 patients from the Mayo Clinic Fontan database. Thirteen percent (153 patients) had AVV repair or replacement during their staged single ventricle palliation. We found that patients with right ventricular morphology and common AVV were at increased risk for AVV intervention. Patients who underwent AVV intervention had increased risk of death/transplant compared with those who did not (hazards ratio [HR] = 1.75, 95% CI 1.37 to 2.23, p <0.001). With respect to valve intervention timing, whereas AVV intervention before Fontan presented similar risk for death/transplant compared with no AVV intervention (HR = 0.85, 95% CI 0.32 to 2.27, p = 0.74), intervention at time of Fontan had a significantly higher risk (HR = 1.46, 95% CI 1.09 to 1.97, p = 0.01), and intervention after Fontan had a much more substantial risk (HR = 3.83, 95% CI 2.54 to 5.79, p <0.001). AVV repair failure occurred in 11% of patients. In terms of relative risk of valve repair versus replacement, in post-Fontan AVV intervention patients, AVV replacement carried a 2.9 fold risk of death/transplant compared with AVV repair. In conclusion, AVV disease remains a considerable challenge for durable Fontan physiology. This data demonstrates that earlier intervention on valve pathology improves survival with the Fontan circulation. Continued surveillance of single ventricle patients and prompt referral of those with valve pathology can improve outcomes in this challenging population.
#1Celest Dines Muntaner (Royal Children's Hospital)H-Index: 1
#2Gregory King (University of Melbourne)H-Index: 3
Last. Yves d'Udekem (University of Melbourne)H-Index: 45
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Abstract Background Outcomes of tricuspid valve repair in single ventricle palliation are poor. We sought to identify the impact of repair technique and primary mechanisms of regurgitation on outcomes following surgery. Methods A retrospective review of medical records across four centres undertaking single ventricle palliation in Australia and New Zealand was performed. Patients with a dominant right functional single ventricle undergoing tricuspid valve repair between January 1991 and March 20...
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#1Jack RychikH-Index: 69
#2Andrew M. AtzH-Index: 55
Last. Yves d'UdekemH-Index: 45
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It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array o...
119 CitationsSource
#1Gregory King (Royal Children's Hospital)H-Index: 3
#2Julian Ayer (Children's Hospital at Westmead)H-Index: 21
Last. Yves d'UdekemH-Index: 45
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Abstract Background Atrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation. Objectives This study determined the incidence of atrioventricular valve failure and its clinical impact on patients undergoing Fontan palliation. Methods A retrospective cohort longitudinal study was conducted using patient data extracted from an existing bi-national, population-based registry. Results A total ...
33 CitationsSource
#1Edward Buratto (University of Melbourne)H-Index: 12
#2Xin Tao Ye (University of Melbourne)H-Index: 6
Last. Igor E. Konstantinov (University of Melbourne)H-Index: 41
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Abstract Background Single-ventricle palliation (SVP) for children with unbalanced atrioventricular septal defect (uAVSD) is thought to carry a poor prognosis, but limited data have been reported. Methods We performed a retrospective review of children with uAVSD who underwent SVP at a single institution. Data were obtained from medical records and correspondence with general practitioners and cardiologists. Results Between 1976 and 2016, a total of 139 patients underwent SVP for uAVSD. A neonat...
25 CitationsSource
#1Kavitha N. Pundi (Mayo Clinic)H-Index: 8
#2Jonathan N. Johnson (Mayo Clinic)H-Index: 30
Last. Frank Cetta (Mayo Clinic)H-Index: 46
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Background There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation. Objectives This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic. Methods Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study. Results Overall, 10-, 20-, and 30-year survival for 1,052...
271 CitationsSource
#1Yves d'UdekemH-Index: 45
#2Ajay J. IyengarH-Index: 18
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Background—The life expectancy of patients undergoing a Fontan procedure is unknown. Methods and Results—Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975–1995), 271 lateral tunnels (1988–2006), and 532 extracardiac conduits (1997–2010). The proportion with hypoplastic left ...
300 CitationsSource
#1Victor Bautista-Hernandez (Boston Children's Hospital)H-Index: 16
#2David W. Brown (Boston Children's Hospital)H-Index: 69
Last. Christopher W. Baird (Boston Children's Hospital)H-Index: 23
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Objectives Tricuspid regurgitation (TR) remains a risk factor for morbidity and mortality through staged palliation in patients with hypoplastic left heart syndrome (HLHS). Reports on the mechanisms associated with TR in patients with HLHS are limited. Thus, we sought to describe our experience with tricuspid valve (TV) repair in these patients, focusing on the mechanisms of TR and corresponding surgical techniques. Methods We performed a retrospective single-center review (January 2000 to Decem...
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#1Junya Sugiura (Boston Children's Hospital)H-Index: 3
#2Toshihide Nakano (Boston Children's Hospital)H-Index: 14
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OBJECTIVES: Tricuspid regurgitation (TR) remains a significant risk factor affecting the survival of patients with hypoplastic left heart syndrome (HLHS). We performed this study to investigate differences in the clinical course based on the timing of the development of TR and the effects of tricuspid valve surgery (TVS). METHODS: One hundred and five patients of classic HLHS underwent staged operations from May 1991 to July 2010. Forty-four patients (41.9%) exhibited moderate or greater TR duri...
12 CitationsSource
#1Yves d'Udekem (Royal Children's Hospital)H-Index: 45
#2Mary Y. Xu (Royal Children's Hospital)H-Index: 2
Last. Christian P. Brizard (Royal Children's Hospital)H-Index: 47
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Objectives This study examined survival after surgical palliation in children with single-ventricle physiology. Background Contemporary surgical outcomes for the entire population of newborns undergoing single-ventricle palliation are unclear. Methods In a single-center review of 499 consecutive patients undergoing univentricular palliation from 1990 to 2008, predictors of mortality were determined using multivariate risk analysis, stratified for each post-operative stay and interim states. Resu...
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#1Tarek Alsaied (University of Cincinnati Academic Health Center)H-Index: 11
In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address the following: (1) transcatheter closure of sinus venous atrial septal defect which is feasible in adults with careful planning, (2) exercise echocardiography in children with hypertrophic cardiomyopathy which was found to be very helpful ...