Single ventricle palliation: Greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

Day, Ronald W.; Etheridge, Susan P.; Veasy, L. George; Jenson, Conrad B.; Hillman, Neal D.; Russo, G.; Thorne, J. K.; Doty, Donald B.; McGough, Edwin C.; Hawkins, John A.

doi:10.1016/j.ijcard.2005.01.039

Cited by 44 publications

(59 citation statements)

References 29 publications

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“…However, one should not use this fact as an argument when comparing outcomes between patients left with a BCPS status and those who have undergone Fontan surgery. Some have argued that a Fontan circulation would not be superior to a BCPS supplemented with some additional source of blood flow [17][18][19]. As most of the patients in this series were expected to proceed to Fontan surgery, the present study should not be used to compare these two statuses, because, in this setting, all the mortalities were automatically attributed to the group with a BCPS circulation.…”

Section: Discussionmentioning

confidence: 96%

Fontan completion rate and outcomes after bidirectional cavo-pulmonary shunt☆

Tan

Iyengar

Donath

et al. 2010

European Journal of Cardio-Thoracic Surgery

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Section: Discussionmentioning

confidence: 96%

Fontan completion rate and outcomes after bidirectional cavo-pulmonary shunt☆

Tan

Iyengar

Donath

et al. 2010

European Journal of Cardio-Thoracic Surgery

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“…BDG circulation is a particular topic of study because only the superior systemic flow is oxygenated, and the overall SO 2 is highly dependent on the division between upper and lower circulations (Salim et al, 1995; Whitehead et al, 2009). Patients who have high surgical risk factors remain at this second stage BDG physiology living with moderate cyanosis (Day et al, 2006; Salim et al, 1995). …”

Section: Introductionmentioning

confidence: 99%

Exercise capacity in the Bidirectional Glenn physiology: Coupling cardiac index, ventricular function and oxygen extraction ratio

Vallecilla

Khiabani

Trusty

et al. 2015

Journal of Biomechanics

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In Bi-directional Glenn (BDG) physiology, the superior systemic circulation and pulmonary circulation are in series. Consequently, only blood from the superior vena cava is oxygenated in the lungs. Oxygenated blood then travels to the ventricle where it is mixed with blood returning from the lower body. Therefore, incremental changes in oxygen extraction ratio (OER) could compromise exercise tolerance. In this study, the effect of exercise on the hemodynamic and ventricular performance of BDG physiology was investigated using clinical patient data as inputs for a lumped parameter model coupled with oxygenation equations. Changes in cardiac index, Qp/Qs, systemic pressure, oxygen extraction ratio and ventricular/vascular coupling ratio were calculated for three different exercise levels. The patient cohort (n=29) was sub-grouped by age and pulmonary vascular resistance (PVR) at rest. It was observed that the changes in exercise tolerance are significant in both comparisons, but most significant when sub-grouped by PVR at rest. Results showed that patients over 2 years old with high PVR are above or close to the upper tolerable limit of OER (0.32) at baseline. Patients with high PVR at rest had very poor exercise tolerance while patients with low PVR at rest could tolerate low exercise conditions. In general, ventricular function of SV patients is too poor to increase CI and fulfill exercise requirements. The presented mathematical model provides a framework to estimate the hemodynamic performance of BDG patients at different exercise levels according to patient specific data.

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“…1 Importantly, some infants who remain palliated by a SCPC fare better than after a Fontan 2 which often demonstrates significant late failures. 3 However, the durability of the SCPC is significantly limited due to progressive cyanosis observed secondary to the development of PAVM’s.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Artery Endothelial Cell Phenotypic Alterations in a Large Animal Model of Pulmonary Arteriovenous Malformations After the Glenn Shunt

Kavarana

Mukherjee

Eckhouse

et al. 2013

The Annals of Thoracic Surgery

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Background: Longevity of the superior cavopulmonary connection (SCPC) is limited by the development of pulmonary arteriovenous malformations (PAVM). The goal of this study was to determine whether phenotypic changes in pulmonary artery endothelial cells (PAEC) that favor angiogenesis occur with PAVM formation. Methods: A superior vena cava to right pulmonary artery connection was constructed in 5 pigs. Pulmonary arteries were harvested at 6-8 weeks following surgery to establish cultures of PAEC and smooth muscle cells, to determine cell proliferation, gene expression, and tubule formation. Abundance of proteins related to angiogenesis was measured in lung tissue. Results: Contrast echocardiography revealed right-to-left shunting, consistent with PAVM formation. While the proliferation of smooth muscle cells from the right pulmonary artery (RPA) (shunted side) and left pulmonary artery (LPA) (non- shunted side) were similar, right PAEC proliferation was significantly higher. Expression profiles of genes encoding cellular signaling proteins were higher in PAECs from the RPA vs. LPA. Protein abundance of angiopoietin-1, and Tie-2 (angiopoietin receptor) were increased in the right lung (both p<0.05). Tubule formation was increased in endothelial cells from the RPA compared to the LPA (404±16 vs. 199±71 tubules/mm2, respectively p<0.05). Conclusions: These findings demonstrate that PAVMs developed in a clinically relevant animal model of SCPC. This study found that PAVM development occurred concomitantly with differential changes in PAEC proliferative ability and phenotype. Moreover, there was a significant increase in the angiopoietin/Tie-2 complex in the right lung, which may provide novel therapeutic targets to attenuate PAVM formation following a SCPC.

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Single ventricle palliation: Greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

Cited by 44 publications

References 29 publications

Fontan completion rate and outcomes after bidirectional cavo-pulmonary shunt☆

Fontan completion rate and outcomes after bidirectional cavo-pulmonary shunt☆

Exercise capacity in the Bidirectional Glenn physiology: Coupling cardiac index, ventricular function and oxygen extraction ratio

Pulmonary Artery Endothelial Cell Phenotypic Alterations in a Large Animal Model of Pulmonary Arteriovenous Malformations After the Glenn Shunt

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