Reappraisal of lung manifestations in the setting of Fontan circulation

Fontan circulation is a well-established palliation in patients with functional single ventricles. Absence of a sub-pulmonary pumping chamber creates a unique physiology in which blood flow is mainly guided by negative intrathoracic and elevated central venous pressures. Various pulmonary anatomic or pathophysiologic changes can jeopardize optimal Fontan circulation. Long-term survival of patients who have undergone the contemporary total cavopulmonary connection is satisfactory. Thorough literature review in conjunction with accumulated clinical experience can lead clinicians to extract conclusions regarding Fontan and lung interactions indicating the purpose of this review.

Download Full-text

Risk Factors and Long-Term Prognosis for Chylothorax After Total Cavopulmonary Connection in Children: A Retrospective Study From a Single Center

Frontiers in Pediatrics ◽

10.3389/fped.2021.744019 ◽

2021 ◽

Vol 9 ◽

Author(s):

Liting Bai ◽

Zhengyi Feng ◽

Ju Zhao ◽

Shengwen Guo ◽

Yuanyuan Tong ◽

...

Keyword(s):

Risk Factors ◽

Venous Pressure ◽

Survival Rates ◽

Total Cavopulmonary Connection ◽

Term Survival ◽

Long Term Survival ◽

Hospital Patients ◽

Long Term Prognosis ◽

Cavopulmonary Connection

Background: Chylothorax is a severe complication after total cavopulmonary connection (TCPC) in children. This study was performed to evaluate the incidence, risk factors, and short- and long-term prognosis for chylothorax.Methods: We retrospectively reviewed the electronic records of patients who underwent TCPC between January 2008 and December 2020 in Fuwai Hospital. Patients were divided into two groups based on the occurrence of post-operative chylothorax. Univariate and multivariate analyses were performed to identify risk factors, and long-term survival was estimated by the Kaplan–Meier method.Results: Of 386 patients included in our study, chylothorax occurred in 60 patients (15.5%). Compared with the non-chylothorax group, the prevalence of prolonged intensive care unit (ICU) stay (p = 0.000) and post-operative hospital stay (p = 0.000) were greater in patients with chylothorax. Post-operative adverse events in terms of infection (p = 0.002), ascites (p = 0.001), prolonged pleural effusion (p = 0.000), and diaphragmatic paralysis (p = 0.026) were more frequent in chylothorax patients. The median follow-up duration was 4.0 (2.0, 6.8) years. The chylothorax group had significantly lower survival rates at 1 year (92.4 vs. 99.3%, p < 0.001) and 10 years (84.6 vs. 91.6%, p < 0.001), respectively. Having a right dominant ventricle [odds ratio (OR) = 2.711, 95% confidence interval (CI) = 1.285–5.721, p = 0.009] and a higher peak central venous pressure (CVP) on post-operative day (POD) 0 (OR = 1.116, 95% CI = 1.011–1.233, p = 0.030) were the risk factors for the development of chylothorax after TCPC operation.Conclusion: The incidence of chylothorax in patients undergoing TCPC is lower than previously reported but is associated with poor early- and long-term survival. Having a right dominant ventricle and a higher peak CVP on POD 0 are the risk factors for chylothorax after TCPC operation.

Download Full-text

Rebuttal

Cardiology in the Young ◽

10.1017/s1047951104006432 ◽

2004 ◽

Vol 14 (S1) ◽

pp. 114-114 ◽

Cited By ~ 1

Author(s):

Leonard L. Bailey

Keyword(s):

Coronary Disease ◽

Reconstructive Surgery ◽

Fontan Circulation ◽

Actuarial Survival ◽

Term Survival ◽

Long Term Survival ◽

Fontan Physiology ◽

Failing Fontan

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

Download Full-text