Late Clinical Outcomes of the Fontan Operation in Patients with Tricuspid Atresia

Abstract

Objective -Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia. Methods -A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul), from August 1980 throughTricuspid atresia is a rare cardiac malformation, in which no communication exists between the right atrium and the right ventricle, bringing about complex physiological adaptations and culminating with high morbidity and mortality, thus requiring the utilization of an adequate surgical technique for its correction 1 . So, in 1971, Fontan and Baudet 2 introduced the basis for an operation that became the final palliative surgery for patients with tricuspid atresia. Basically, a communication is established between the systemic venous return and the pulmonary arterial circulation, bypassing the right ventricle flow 2,3 . As years went by, however, a number of changes were proposed to the classical Fontan operation, such as the Kreutzer technique 4 , the Björk technique 5 , and the total cavopulmonary shunt (TCPC) [6][7][8][9] , and very few studies deal with the long-term follow-up of patients who undergo these procedures [10][11][12][13][14][15][16][17][18] .Considering that Fontan surgery is a relatively recent therapeutic procedure and that, in addition to this, some important changes were added to the original surgical technique, this study aims at evaluating late survival, as well as the complications that occurred during the long-term follow-up of patients with tricuspid atresia who underwent Fontan surgery or its variants.

Paediatric and congenital heart disease in South America: an overview

“…Dr G Kreutzer and colleagues from Argentina published their innovative work in the early 1970s and, along with Dr Fontan, set the technical surgical foundations for the operation subsequently known world wide as the Fontan–Kreutzer procedure 75. After Dr Kreutzer’s initial description, several modifications have been proposed and employed in South America, with evolving results 76777879808182838485. Creation of a fenestration is still debatable 82.…”

“…Creation of a fenestration is still debatable 82. The Fontan–Kreutzer procedure is still a challenging operation and continues to stimulate surgeons and clinicians to broaden their clinical research in the field 82838485. There have been some interesting observations from centres in South America about ventricular remodelling in patients with tricuspid atresia before surgery86 and endothelial dysfunction before and after the Fontan–Kreutzer operation 8788…”

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

“…[5][6][7][8][9][10][11][12][13] Valuable data on longer-term actuarial survival are likewise emerging, with a focus on particular surgical techniques 12,14,15 or congenital diagnoses. 16 Causes and distribution of deaths remain poorly defined. As the first cohort of patients with Fontan palliation survive into adulthood and reach middle age, it is becoming increasingly important to define modes of death and to elucidate risk factors for them.…”

Parental Refusal of Surgery in an Infant With Tricuspid Atresia

“…Although it is less well studied than HLHS, 5-year postoperative survival in TA is ~80%, 22 -25 and survivors are also at risk for neurodevelopmental and other disorders. 26 Therefore, when the obstetrician argued that infants with TA should be treated similarly to those with HLHS, his reasoning had some merit.…”