Impact of Aortic Atresia After Fontan Operation in Patients With Hypoplastic Left Heart Syndrome

King, Gregory; Buratto, Edward; Daley, Michael; Iyengar, Ajay J.; Alphonso, Nelson; Grigg, Leeanne; Cordina, Rachael; d’Udekem, Yves; Konstantinov, Igor E.

doi:10.1016/j.athoracsur.2022.09.018

Cited by 7 publications

(4 citation statements)

References 34 publications

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“…However, the outcomes of TV surgery have not been speci cally discussed by the investigators. More recently, in a sub-analysis of the Australia and New Zealand Fontan Registry, patients with HLHS exhibited poor long-term freedom from atrio-ventricular valve failure, which was demonstrated to be associated with RV contractile dysfunction and failure of Fontan circulation [17]. Interestingly, the observed inferior prognosis of Fontan patients requiring atrio-ventricular valve surgery resulted to be mainly driven by a disproportionate effect that atrio-ventricular valve regurgitation displayed in the RV-dominant population only [10].…”

Section: Discussionmentioning

confidence: 99%

“…Right ventricular (RV) dominance exposes univentricular patients to an increased risk of clinically signi cant atrioventricular valve regurgitation, which translates into poor early and long-term transplant-free survival [7][8][9][10]. Hypoplastic left heart syndrome (HLHS) represents the prototype of this condition [11] and 15-25% of affected patients are expected to require surgical management of TV regurgitation (TVR) during their palliation course [12][13][14][15][16][17].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Tricuspid valve repair can restore the prognosis of patients with hypoplastic left heart syndrome and tricuspid valve regurgitation: a meta-analysis

Ponzoni

Azzolina

Vedovelli

et al. 2023

Preprint

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Objectives To date, evidence supporting the efficacy of tricuspid valve (TV) repair in interrupting the progression of systemic right ventricular (RV) adverse remodeling in hypoplastic left heart syndrome (HLHS) is conflicting. We performed a systematic review and meta-analysis of scientific literature to assess the impact of TV repair in effectively modifying the prognosis of patients with HLHS. Methods We conducted a systematic review of PubMed, Web of Science, and Scopus databases. A random-effect meta-analysis was performed and transplant-free survival, freedom from TV regurgitation, and TV reoperation data were reconstructed using the published Kaplan–Meier curves. Results Nine studies were included, for a total of 203 HLHS patients undergoing TV repair and 323 HLHS controls. The estimated transplant-free survival at 1, 5, and 10 years of follow-up was 75.5% [95% confidence interval (CI) = 67.6–84.3%], 63.6% [95% CI = 54.6–73.9%], and 61.9% [95% CI = 52.7–72.6%], respectively. Transplant-free survival was comparable to HLHS peers without TV regurgitation (p = 0.59). Five-year freedom from recurrence of TV regurgitation and freedom from TV reoperation was 57% [95% CI = 46.7–69.7%] and 63.6% [95% CI = 54.5–74.3%], respectively. Younger age and TV repair at the time of Norwood operation increased the risk of TV regurgitation recurrence and the need for TV reoperation. Conclusions Our meta-analysis supports the efficacy of TV repair in modifying favorably the prognosis of patients with HLHS and TV regurgitation, reestablishing a medium-term transplant-free survival which is comparable to HLHS peers. However, durability of surgery and long-term fate of TV and RV performance are still unclear.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Tricuspid valve repair can restore the prognosis of patients with hypoplastic left heart syndrome and tricuspid valve regurgitation: a meta-analysis

Ponzoni

Azzolina

Vedovelli

et al. 2023

Preprint

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“…The Norwood operation is typically performed 1-2 weeks after birth, the Glenn operation is typically performed 4-6 months after birth and the Fontan operation is performed 3-4 years after birth. During the Fontan operation, the inferior vena cava is separate from the heart and connected to the pulmonary artery using an inert Gore-Tex conduit (Roeleveld et al, 2018;Arunamata et al, 2020;Danton, 2021;King et al, 2022). This conduit only serves to provide a length of inert tubing to support blood flow and does not have a contractile function (Hoashi et al, 2020;Daley and d'Udekem, 2021;Lee et al, 2007;Ochiai et al, 2009;Perez-Caballero et al, 2022).…”

Section: Applications For Biological Pumpsmentioning

confidence: 99%

Recent advances in biological pumps as a building block for bioartificial hearts

Brimmer

Ji²,

Birla³

et al. 2023

Front. Bioeng. Biotechnol.

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The field of biological pumps is a subset of cardiac tissue engineering and focused on the development of tubular grafts that are designed generate intraluminal pressure. In the simplest embodiment, biological pumps are tubular grafts with contractile cardiomyocytes on the external surface. The rationale for biological pumps is a transition from planar 3D cardiac patches to functional biological pumps, on the way to complete bioartificial hearts. Biological pumps also have applications as a standalone device, for example, to support the Fontan circulation in pediatric patients. In recent years, there has been a lot of progress in the field of biological pumps, with innovative fabrication technologies. Examples include the use of cell sheet engineering, self-organized heart muscle, bioprinting and in vivo bio chambers for vascularization. Several materials have been tested for biological pumps and included resected aortic segments from rodents, type I collagen, and fibrin hydrogel, to name a few. Multiple bioreactors have been tested to condition biological pumps and replicate the complex in vivo environment during controlled in vitro culture. The purpose of this article is to provide an overview of the field of the biological pumps, outlining progress in the field over the past several years. In particular, different fabrication methods, biomaterial platforms for tubular grafts and examples of bioreactors will be presented. In addition, we present an overview of some of the challenges that need to be overcome for the field of biological pumps to move forward.

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“…Even if the outcome of SV patients is steadily improving, particularly their largest subgroup—i.e., SV patients with aortic atresia and a hypoplastic left heart (HLHS, 40% of cases [ 5 ]) and a morphologically right ventricle (RV) serving as the subsystemic ventricle—performs worse than patients with a morphologically left ventricle (LV) [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 ], particularly with respect to atrioventricular valve failure, impaired ventricular function and/or failure of the Fontan circulation with upstream issues such as liver cirrhosis or protein-losing enteropathy [ 15 , 16 , 17 ]. Ventricular dysfunction is currently considered to be inevitable for SV patients [ 18 ].…”

Section: Introductionmentioning

confidence: 99%

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

Renaud,

Scholl-Bürgi,

Karall

et al. 2023

Metabolites

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Most studies on single ventricle (SV) circulation take a physiological or anatomical approach. Although there is a tight coupling between cardiac contractility and metabolism, the metabolic perspective on this patient population is very recent. Early findings point to major metabolic disturbances, with both impaired glucose and fatty acid oxidation in the cardiomyocytes. Additionally, Fontan patients have systemic metabolic derangements such as abnormal glucose metabolism and hypocholesterolemia. Our literature review compares the metabolism of patients with a SV circulation after Fontan palliation with that of patients with a healthy biventricular (BV) heart, or different subtypes of a failing BV heart, by Pubmed review of the literature on cardiac metabolism, Fontan failure, heart failure (HF), ketosis, metabolism published in English from 1939 to 2023. Early evidence demonstrates that SV circulation is not only a hemodynamic burden requiring staged palliation, but also a metabolic issue with alterations similar to what is known for HF in a BV circulation. Alterations of fatty acid and glucose oxidation were found, resulting in metabolic instability and impaired energy production. As reported for patients with BV HF, stimulating ketone oxidation may be an effective treatment strategy for HF in these patients. Few but promising clinical trials have been conducted thus far to evaluate therapeutic ketosis with HF using a variety of instruments, including ketogenic diet, ketone esters, and sodium-glucose co-transporter-2 (SGLT2) inhibitors. An initial trial on a small cohort demonstrated favorable outcomes for Fontan patients treated with SGLT2 inhibitors. Therapeutic ketosis is worth considering in the treatment of Fontan patients, as ketones positively affect not only the myocardial energy metabolism, but also the global Fontan physiopathology. Induced ketosis seems promising as a concerted therapeutic strategy.

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Impact of Aortic Atresia After Fontan Operation in Patients With Hypoplastic Left Heart Syndrome

Cited by 7 publications

References 34 publications

Tricuspid valve repair can restore the prognosis of patients with hypoplastic left heart syndrome and tricuspid valve regurgitation: a meta-analysis

Tricuspid valve repair can restore the prognosis of patients with hypoplastic left heart syndrome and tricuspid valve regurgitation: a meta-analysis

Recent advances in biological pumps as a building block for bioartificial hearts

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

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