Potts Shunt in a Child With End-Stage Pulmonary Hypertension After Late Repair of Ventricular Septal Defect

Petersen, Claus Leth; Helvind, Morten; Jensen, Tim; Andersen, Henning Haahr

doi:10.1177/2150135113482739

Cited by 11 publications

(9 citation statements)

References 7 publications

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“…Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance and arterial pressure, which lead to right ventricular hypertrophy and ultimately right heart failure [1][2][3][4]. A common trait related to PAH is sustained vascular remodeling of the distal pulmonary arteries [4,5].…”

Section: Introductionmentioning

confidence: 99%

Calcineurin/NFAT Signaling Modulates Pulmonary Artery Smooth Muscle Cell Proliferation, Migration and Apoptosis in Monocrotaline-Induced Pulmonary Arterial Hypertension Rats

Liu

et al. 2018

Cell Physiol Biochem

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Background/Aims: Pulmonary arterial hypertension (PAH) is a severe and debilitating disease characterized by remodeling of the pulmonary vessels, which is driven by excessive proliferation and migration and apoptosis resistance in pulmonary artery smooth muscle cells (PASMCs). The calcineurin (CaN)/nuclear factor of activated T-cells (NFAT) signaling pathway is the most important downstream signaling pathway of store-operated Ca²⁺ entry (SOCE), which is increased in PAH. CaN/NFAT has been reported to contribute to abnormal proliferation in chronic hypoxia (CH)-induced PAH. However, the effect of CaN/NFAT signaling on PASMC proliferation, migration and apoptosis in monocrotaline (MCT)-induced PAH remains unclear. Methods: PAH rats were established by a single intraperitoneal injection of MCT for 21 days. PASMCs were isolated and cultured in normal and MCT-induced PAH Sprague-Dawley rat. PASMCs were treated with CsA targeting CaN and siRNA targeting NFATc2-4 gene respectively by liposome. We investigated the expression of calcineurin/NFAT signaling by immunofluorescence, qRT-PCR and Western blotting methods. Cell proliferation was monitored using MTS reagent or by assessing proliferating cell nuclear antigen (PCNA) expression. Cell apoptosis was evaluated with an Annexin V - FITC/propidium iodide (PI) apoptosis kit by flow cytometry. PASMC migration was assessed with a Transwell chamber. Results: MCT successfully induced PAH and pulmonary vascular remodeling in rats. CaN phosphatase activity and nuclear translocation of NFATc2-4 were increased in PASMCs derived from MCT-treated rats. In addition, CaNBβ/NFATc2-4 expression was amplified at the mRNA and protein levels. PASMC proliferation and migration were markedly inhibited in a dosedependent manner by cyclosporin A (CsA). Furthermore, siRNA targeting NFATc2 and NFATc4 attenuated the excessive proliferation and migration and apoptosis resistance in PASMCs derived from both CON and MCT-treated rats, while NFATc3 knockdown specifically affected MCT-PASMCs. Conclusion: Our results demonstrate that CaN/NFAT signaling is activated and involved in the modulation of PASMC proliferation, migration and apoptosis in MCT-induced PAH.

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Section: Introductionmentioning

confidence: 99%

Calcineurin/NFAT Signaling Modulates Pulmonary Artery Smooth Muscle Cell Proliferation, Migration and Apoptosis in Monocrotaline-Induced Pulmonary Arterial Hypertension Rats

Liu

et al. 2018

Cell Physiol Biochem

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“…[127][128][129][130][131][132] Boudjemline and colleagues successfully performed stenting of a patent ductus arteriosus in three children with IPAH and suprasystemic pulmonary artery pressures. 133,134 In another series of four patients, our group was able to confirm the usefulness and safety of stenting of a patent ductus arteriosus in neonates and infants with variant forms of severe PH. 13 Further development of the technical aspects of the Potts procedure include the implantation of a unidirectional valve within the Potts anastomosis, which can be considered for patients with PH and subsystemic or isosystemic pulmonary artery pressures who exhibit suprasystemic pulmonary artery pressures during exercise, 135 and in patients in whom bidirectional shunting occurs after decompression of the right ventricle.…”

Section: New Developmentsmentioning

confidence: 57%

Treatment of pulmonary arterial hypertension in children

Latus,

Delhaas,

Schranz

et al. 2015

Nat Rev Cardiol

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Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality in children. Improved understanding of the pathophysiological mechanisms of the underlying diseases has resulted in the development of effective, but not yet curative, therapies. Currently, drugs from three main pharmacological groups targeting specific aberrant pathways (endothelin, nitric oxide, and prostacyclin) and four routes of administration (inhaled, intravenous, oral, and subcutaneous) have been approved for adult patients with PAH. However, only a minority of these drugs has been officially approved for children, mainly because randomized controlled studies are limited by the small number of paediatric patients with PAH worldwide. In children with progressive, severe PAH and an inadequate response to drug therapy, advances in interventional and surgical approaches have provided promising new strategies to avoid right ventricular deterioration. These techniques can delay or even avoid the need for lung transplantation. In this Review, we present an update on developments in drug therapy for patients with PAH and highlight the current status of these treatments in children. We also describe the new surgical, interventional, and hybrid procedures, as well as their practical application in children with severe PAH.

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“…In 2013, Petersen et al (17) performed Potts shunt in a patient with lately repaired VSD and end-stage PAH and obtained successful results. Postoperative sildenafil, treprostinil, and bosentan treatments were continued, and the patient was discharged 2 weeks later.…”

Section: Introductionmentioning

confidence: 99%

Surgical and transcatheter management alternatives in refractory pulmonary hypertension: Potts shunt

Kula¹,

Atasayan²

2015

Anatol J Cardiol

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Despite advances in the medical treatment of children with pulmonary arterial hypertension that have resulted in improved health quality and life expectancy, the progression of the disease is still the main problem for some patients. Because of this undesirable condition, the search for new treatment strategies continues for pediatric cardiologists. At this point, the Eisenmenger physiology is the main target because of the long-life expectancy and more stable hemodynamics of patients with Eisenmenger syndrome. Therefore, some invasive procedures may be used for conversion to Eisenmenger physiology with the aim of decompressing the right ventricle.

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Potts Shunt in a Child With End-Stage Pulmonary Hypertension After Late Repair of Ventricular Septal Defect

Cited by 11 publications

References 7 publications

Calcineurin/NFAT Signaling Modulates Pulmonary Artery Smooth Muscle Cell Proliferation, Migration and Apoptosis in Monocrotaline-Induced Pulmonary Arterial Hypertension Rats

Calcineurin/NFAT Signaling Modulates Pulmonary Artery Smooth Muscle Cell Proliferation, Migration and Apoptosis in Monocrotaline-Induced Pulmonary Arterial Hypertension Rats

Treatment of pulmonary arterial hypertension in children

Surgical and transcatheter management alternatives in refractory pulmonary hypertension: Potts shunt

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