Univentricular Heart: Can We Alter the Natural History?

Corno, Antonio F.; Becker, Anton E.; Bulterijs, Jan Lam Anton H.K.; Nijveld, A.; Schuller, J.; Marcelletti, Carlo

doi:10.1016/s0003-4975(10)60917-4

Cited by 35 publications

(22 citation statements)

References 53 publications

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“…The modern surgical approach to a univentricular heart is the Fontan operation [1-4]. Although the procedure offers effective hemodynamic palliation for most patients, problematic atrial tachycardias can develop as a late complication [5-10].…”

Section: Introductionmentioning

confidence: 99%

Atrial Remodeling After the Fontan Operation

Wolf

Seslar

Boer

et al. 2009

The American Journal of Cardiology

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Hemodynamics following Fontan surgery are notable for right atrial (RA) hypertension and dilation. The effect of this stress on atrial cytoarchitecture has not been studied systematically, and may be relevant to arrhythmias and their treatment. Morphologic and histopathologic analysis was performed on RA and left atrial (LA) tissue from post-mortem specimens of Fontan hearts (n = 47) and compared to control samples from young patients with normal atrial hemodynamics (n = 15). Most Fontan specimens were from young patients who expired after a relatively short duration of Fontan physiology. Tissues were analyzed for wall thickness, fibrosis content, and fibrosis pattern. Mean wall thickness for both RA (3.0±1.0 mm) and LA (2.3±0.6 mm) in Fontan hearts was significantly greater than in control hearts (RA = 1.8±0.4 mm and LA = 1.8±0.5 mm, p<0.001 and p=0.024, respectively). Predictors for RA thickening included: a) older age at Fontan surgery, b) older age at death, and c) longer duration of Fontan circulation. Fontan hearts and controls exhibited nearly identical RA and LA fibrosis patterns. Neither wall thickness nor fibrosis varied with underlying heart defect or style of Fontan connection. In conclusion, atrial remodeling after Fontan surgery for univentricular heart physiology involves increased wall thickness in both the RA and LA. Interstitial fibrosis was also observed in Fontan atria, but because a similar pattern was present in control tissue, this likely represents normal fibroelastic atrial structure rather than a specific response to Fontan hemodynamics. The degree of wall thickening observed in Fontan atria was not so excessive as to preclude transmural lesions during catheter or surgical ablation of reentrant arrhythmias.

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Section: Introductionmentioning

confidence: 99%

Atrial Remodeling After the Fontan Operation

Wolf

Seslar

Boer

et al. 2009

The American Journal of Cardiology

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“…First of all, in this review all cases with the pre-natal diagnosis of “functionally” univentricular heart have been taken into consideration. In this regard, despite decent medium and long-term results obtained from few decades with the univentricular type of repair ( 75 – 79 ), the concept of “intractable” congenital heart defect is still associated with the diagnosis of univentricular heart, because of the hazardous long-term results ( 57 , 69 ). “Intractable” ( 57 ) or “uncorrectable” ( 69 ) has been defined the heart malformations not suitable to bi-ventricular repair, and therefore where the only option available for treatment remains multi-staged complex palliation programs toward univentricular type of repair or heart transplant.…”

Section: Ethical Implications Parents Counseling Interruption Of Prmentioning

confidence: 99%

â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis

Corno

2015

Front. Pediatr.

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Within the last few decades the pre-natal echocardiographic diagnosis of congenital heart defects has made substantial progresses, particularly for the identification of complex malformation. "Functionally" univentricular hearts categorize a huge variety of heart malformations. Since no one of the patients with these congenital heart defects can ever undergo a bi-ventricular type of repair, early recognition and decision-making from the neonatal period are required in order to allow for appropriate multiple-step diagnostic and treatment procedures, either of interventional cardiology and/or surgery, on the pathway of "univentricular" heart. In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects. This review of the recent reports has been undertaken to better understand the impact of pre-natal diagnosis in "functionally" univentricular hearts taking into consideration the following topics: pre-natal screening, outcomes and survival, general morbidity, neurologic and developmental consequences, pregnancy management and delivery planning, resources utilization and costs/benefits issues, ethical implications, parents counseling, and interruption of pregnancy versus treatment.

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“…The vast majority of children with balanced forms of univentricular heart require a surgical palliation early in life (4, 24–26). After the neonatal palliation, the Fontan circulation is generally established in two stages: an end-to-side connection between the SVC and the right pulmonary artery (bi-directional Glenn) (27–31), followed later by the Fontan completion, with the connection of the IVC to the pulmonary artery.…”

Section: Introductionmentioning

confidence: 99%

Physiological Fontan Procedure

et al. 2019

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Objective: The conventional Fontan circulation deviates the superior vena cava (SVC = 1/3 of the systemic venous return) toward the right lung (3/5 of total lung volume) and the inferior vena cava (IVC = 2/3 of the systemic venous return) toward the left lung (2/5 of total lung volume). A “physiological” Fontan deviating the SVC toward the left lung and the IVC toward the right lung was compared with the conventional setting by computational fluid dynamics, studying whether this setting achieves a more favorable hemodynamics than the conventional Fontan circulation. Materials and Methods: An in-silico 3D parametric model of the Fontan procedure was developed using idealized vascular geometries with invariant sizes of SVC, IVC, right pulmonary artery (RPA), and left pulmonary artery (LPA), steady inflow velocities at IVC and SVC, and constant equal outflow pressures at RPA and LPA. These parameters were set to perform finite-volume incompressible steady flow simulations, assuming a single-phase, Newtonian, isothermal, laminar blood flow. Numerically converged finite-volume mass and momentum flow balances determined the inlet pressures and the outflow rates. Numerical closed-path integration of energy fluxes across domain boundaries determined the flow energy loss rate through the Fontan circulation. The comparison evaluated: (1) mean IVC pressure; (2) energy loss rate; (3) kinetic energy maximum value throughout the domain volume. Results: The comparison of the physiological vs. conventional Fontan provided these results: (1) mean IVC pressure 13.9 vs. 14.1 mmHg (= 0.2 mmHg reduction); (2) energy loss rate 5.55 vs. 6.61 mW (= 16% reduction); (3) maximum kinetic energy 283 vs. 396 J/m 3 (= 29% reduction). Conclusions: A more physiological flow distribution is accompanied by a reduction of mean IVC pressure and by substantial reductions of energy loss rate and of peak kinetic energy. The potential clinical impact of these hemodynamic changes in reducing the incidence and severity of the adverse long-term effects of the Fontan circulation, in particular liver failure and protein-losing enteropathy, still remains to be assessed and will be the subject of future work.

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Univentricular Heart: Can We Alter the Natural History?

Cited by 35 publications

References 53 publications

Atrial Remodeling After the Fontan Operation

Atrial Remodeling After the Fontan Operation

â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis

Physiological Fontan Procedure

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Univentricular Heart: Can We Alter the Natural History?

Cited by 35 publications

References 53 publications

Atrial Remodeling After the Fontan Operation

Atrial Remodeling After the Fontan Operation

â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis

Physiological Fontan Procedure

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â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis