PROMOTING PULMONARY ARTERIAL GROWTH VIA RIGHT VENTRICULAR TO PULMONARY ARTERY CONDUIT PLACEMENT IN CHILDREN WITH PULMONARY ATRESIA AND HYPOPLASTIC PULMONARY ARTERIES

AbstractMajor abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

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Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135117717266 ◽

2017 ◽

Vol 8 (5) ◽

pp. 564-569 ◽

Cited By ~ 4

Author(s):

Edon J. Rabinowitz ◽

Shilpi Epstein ◽

Nina Kohn ◽

David B. Meyer

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Arterial Tree ◽

Collateral Arteries ◽

Complete Repair ◽

Pulmonary Arterial

Background: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. Methods: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Results: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Conclusion: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

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Development of the Pulmonary Arteries After the Norwood Procedure: Comparison Between Blalock-Taussig Shunt and Right Ventricular–Pulmonary Artery Conduit

Yearbook of Cardiology ◽

10.1016/s0145-4145(08)79331-6 ◽

2009 ◽

Vol 2009 ◽

pp. 138

Author(s):

T. Graham

Keyword(s):

Pulmonary Artery ◽

Right Ventricular ◽

Pulmonary Arteries ◽

Norwood Procedure ◽

Artery Conduit ◽

Blalock Taussig Shunt

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Development of the Pulmonary Arteries After the Norwood Procedure: Comparison Between Blalock-Taussig Shunt and Right Ventricular–Pulmonary Artery Conduit

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2008.06.016 ◽

2008 ◽

Vol 86 (4) ◽

pp. 1299-1304 ◽

Cited By ~ 39

Author(s):

Joseph Caspi ◽

Timothy W. Pettitt ◽

Theodorus Mulder ◽

Aluizio Stopa

Keyword(s):

Pulmonary Artery ◽

Right Ventricular ◽

Pulmonary Arteries ◽

Norwood Procedure ◽

Artery Conduit ◽

Blalock Taussig Shunt

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State-of-the-art pulmonary arterial imaging – Part 2

VASA ◽

10.1024/0301-1526/a000709 ◽

2018 ◽

Vol 47 (5) ◽

pp. 361-375 ◽

Cited By ~ 4

Author(s):

Harold Goerne ◽

Abhishek Chaturvedi ◽

Sasan Partovi ◽

Prabhakar Rajiah

Keyword(s):

Pulmonary Artery ◽

State Of The Art ◽

Therapy Monitoring ◽

Pulmonary Arteries ◽

Imaging Modalities ◽

Cross Sectional ◽

Multiple Imaging ◽

Cross Sectional Imaging ◽

Pulmonary Arterial ◽

Arterial Imaging

Abstract. Although pulmonary embolism is the most common abnormality of the pulmonary artery, there is a broad spectrum of other congenital and acquired pulmonary arterial abnormalities. Multiple imaging modalities are now available to evaluate these abnormalities of the pulmonary arteries. CT and MRI are the most commonly used cross-sectional imaging modalities that provide comprehensive information on several aspects of these abnormalities, including morphology, function, risk-stratification and therapy-monitoring. In this article, we review the role of state-of-the-art pulmonary arterial imaging in the evaluation of non-thromboembolic disorders of pulmonary artery.

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Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-02750-4 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Hyun-Hwa Cha ◽

Hae Min Kim ◽

Won Joon Seong

Keyword(s):

Pulmonary Artery ◽

Subclavian Artery ◽

Septal Defect ◽

Left Subclavian Artery ◽

Ductus Arteriosus ◽

Main Pulmonary Artery ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Left Pulmonary Artery ◽

Right Pulmonary Artery

Abstract Background Unilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. The prenatal diagnosis of isolated unilateral pulmonary artery discontinuity can also be delayed. However, undiagnosed this malformation would have an effect on further prognosis. We report our case of a prenatal diagnosis of pulmonary atresia with ventricular septal defect and left pulmonary artery discontinuity. Case presentation A 33-year-old Asian woman visited our institution at 24 weeks of gestation because of suspected fetal congenital heart disease. Fetal echocardiography revealed a small atretic main pulmonary artery giving rise to the right pulmonary artery without bifurcation and the left pulmonary artery arising from the ductus arteriosus originating from the left subclavian artery. The neonate was delivered by cesarean section at 376/7 weeks of gestation. Postnatal echocardiography and multidetector computed tomography showed a right aortic arch, with the small right pulmonary artery originating from the atretic main pulmonary artery and the left pulmonary artery originating from the left subclavian artery. Patency of the ductus arteriosus from the left subclavian artery was maintained with prostaglandin E1. Right ventricular outflow tract reconstruction and pulmonary angioplasty with Gore-Tex graft patch was performed 25th day after birth. Unfortunately, the neonate died because of right heart failure 8 days postoperation. Conclusion There is a possibility that both pulmonary arteries do not arise from the same great artery (main pulmonary artery or common arterial trunk). Therefore, clinicians should check the origin of both pulmonary arteries.

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Abstract 532: New Agonist of A2a Receptor Reduces Ventricular and Vascular Dysfunction in Monocrotaline-induced Pulmonary Arterial Hypertension in Rats

Hypertension ◽

10.1161/hyp.60.suppl_1.a532 ◽

2012 ◽

Vol 60 (suppl_1) ◽

Author(s):

Allan K Alencar ◽

Sharlene L Pereira ◽

Arthur E Kummerle ◽

Sharon S Langraf ◽

Celso Caruso-Neves ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Pulmonary Artery ◽

Arterial Hypertension ◽

Right Ventricular ◽

Vascular Reactivity ◽

Vascular Dysfunction ◽

Systolic Pressure ◽

Pulmonary Tissue ◽

A2a Receptor ◽

Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is characterized by enhanced pulmonary vascular resistance with subsequent remodeling and right ventricular hypertrophy. Vascular reactivity and ventricular function were investigated in rats with monocrotaline-induced PAH and treated with a new N-acylhydrazone derivative named as LASSBio-1359. METHODS: Protocols were approved by Animal Care and Use Committee at Universidade Federal do Rio de Janeiro. Male Wistar rats received a single i.p. injection of monocrotaline (MCT) (60 mg/kg) for PAH induction and were randomly divided in groups which were treated with: saline, vehicle and LASSBio-1359 (50 mg/kg p.o.). After 14 days of treatment, some parameters were evaluated: pulmonary acceleration time (PAT); right ventricular systolic pressure (RVSP); vascular reactivity to acetylcholine; expression of iNOS in pulmonary tissue; wall thickness of pulmonary artery (PAWT). Results: PAT (ms) was increased from 26.2 ± 2.8 to 41.3 ± 3.9 in PAH group treated with vehicle (n=8, p<0.05) and was reduced to 24.2 ± 1.7 when PAH group was treated with LASSBio-1359. RVSP (mmHg) increased from 26.0 ± 2.0 to 55.2 ± 2.3 in PAH group (p<0.05) but was similar to control after treatment with LASSBio-1359 (31.8 ± 2.3 mm Hg). Ratio of right ventricle and body weight (mg/g) was 0.66 ± 0.02, 1.63 ± 0.16 and 0.87 ± 0.10 for control, vehicle- and LASSBio-1359-treated PAH groups, respectively. PAH promoted ventricular dysfunction which was reduced by LASSBio-1359. The pulmonary artery maximum relaxation (%) was 57.3 ± 5.5, 43.6 ± 1.2 and 61.4 ± 8.4 for control, vehicle and LASSBio-1359-treated groups indicating that PAH promoted endothelium injury which was recovered by LASSBio-1359. iNOS expression in pulmonary tissue was increased from 0.48 ± 1.31 to 0.98 ± 3.14 in PAH group and reduced to 0.53 ± 1.83 in rats treated with LASSBio-1359. The PAWT (%) were increased from 74.1 ± 1.3 to 90.2 ± 2.7 in PAH group (p<0.05) but was 74.4 ± 1.3 when treated with LASSBio-1359. This compound showed an in vitro vasodilatory activity mediated by activation of adenosinergic A2A receptor. Conclusion: LASSBio-1359 reduced ventricular and vascular dysfunction in monocrotaline-induced PAH in rats indicating a possible new alternative to treat PAH.

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Effects of acute Rho kinase inhibition on chronic hypoxia-induced changes in proximal and distal pulmonary arterial structure and function

Journal of Applied Physiology ◽

10.1152/japplphysiol.00533.2010 ◽

2011 ◽

Vol 110 (1) ◽

pp. 188-198 ◽

Cited By ~ 30

Author(s):

Rebecca R. Vanderpool ◽

Ah Ram Kim ◽

Robert Molthen ◽

Naomi C. Chesler

Keyword(s):

Pulmonary Artery ◽

Pulmonary Arteries ◽

Rho Kinase ◽

Pressure Flow ◽

Kinase Inhibition ◽

Pulmonary Vasoconstriction ◽

Pulsatile Pressure ◽

Arterial Structure ◽

Pulmonary Arterial ◽

Induced Changes

Hypoxic pulmonary hypertension (HPH) is initially a disease of the small pulmonary arteries. Its severity is usually quantified by pulmonary vascular resistance (PVR). Acute Rho kinase inhibition has been found to reduce PVR toward control values in animal models, suggesting that persistent pulmonary vasoconstriction is the dominant mechanism for increased PVR. However, HPH may also cause proximal arterial changes, which are relevant to right ventricular (RV) afterload. RV afterload can be quantified by pulmonary vascular impedance, which is obtained via spectral analysis of pulsatile pressure-flow relationships. To determine the effects of HPH independent of persistent pulmonary vasoconstriction in proximal and distal arteries, we quantified pulsatile pressure-flow relationships before and after acute Rho kinase inhibition and measured pulmonary arterial structure with microcomputed tomography. In control lungs, Rho kinase inhibition decreased 0 Hz impedance (Z0), which is equivalent to PVR, from 2.1 ± 0.4 to 1.5 ± 0.2 mmHg·min·ml−1 ( P < 0.05) and tended to increase characteristic impedance (ZC) from 0.21 ± 0.01 to 0.22 ± 0.01 mmHg·min·ml−1. In HPH lungs, Rho kinase inhibition decreased Z0 ( P < 0.05) without affecting ZC. Microcomputed tomography measurements performed on lungs after acute Rho kinase inhibition demonstrated that HPH significantly decreased the unstressed diameter of the main pulmonary artery (760 ± 60 vs. 650 ± 80 μm; P < 0.05), decreased right pulmonary artery compliance, and reduced the frequency of arteries of diameter 50–100 μm (both P < 0.05). These results demonstrate that acute Rho kinase inhibition reverses many but not all HPH-induced changes in distal pulmonary arteries but does not affect HPH-induced changes in the conduit arteries that impact RV afterload.

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