SECTION ON CARDIOLOGY 1996 ANNUAL MEETING PROGRAM

PEDIATRICS ◽  
1996 ◽  
Vol 98 (3) ◽  
pp. 518-536
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Cardiac Catheterization ◽  
Coil Embolization ◽  
Clinical Importance ◽  
Left Pulmonary Artery ◽  
Meeting Program ◽  
Vertical Vein ◽  
Subaortic Obstruction ◽  
Artery Obstruction

Background: Cardiac catheterization (CC) has been utilized with caution in children on extracorporeal membrane oxygenation (ECMO) due to concerns related to anticoagulation, however there have been no reports of procedure outcome in the literature. This retrospective review was performed to evaluate the indications and outcome of cardiac catheterization (CC) in this population. Methods: We reviewed the catheterization records, hospital charts, and follow up records of all children who have undergone this procedure at our institution from 12/90 to 12/95 (n=14). Records were reviewed to assess indications, results, and complications of CC, as well as clinical outcome. Results: Patients ranged in age from 3 days to 46 months, and diagnoses included single ventricle variants (7), tetralogy of Fallot or variant (4), and others (3). ECMO was utilized preoperatively in 2 pts, and postoperatively in 12 pts; indications for ECMO included low cardiac output or ventricular dysfunction (7), severe hypoxemia (4), and pulmonary hypertension (3). Indications for CC were diagnostic in 12 pts (coronary anatomy, pulmonary artery anatomy, pulmonary vein obstruction, pulmonary hypertension, and/or ventricular function), and interventional in 2 pts (balloon atrial septostomy and aortopulmonary collateral (APC) coil embolization). Two unplanned therapeutic procedures were also performed (coil embolization of APC in 1 pt and of a vertical vein in 1 pt). Adequate evaluation of clinical questions was accomplished in all pts. Unexpected diagnostic information of clinical importance was obtained in 5 pts, including right coronary artery obstruction, significant APC, a vertical vein from a pulmonary venous confluence, left pulmonary artery obstruction, and a restrictive ventricular septal defect causing subaortic obstruction.

scholarly journals Cinaciguat, a soluble guanylate cyclase activator, causes potent and sustained pulmonary vasodilation in the ovine fetus

2009 ◽  
Vol 297 (2) ◽  
pp. L318-L325 ◽  
Author(s):  
Marc Chester ◽  
Pierre Tourneux ◽  
Greg Seedorf ◽  
Theresa R. Grover ◽  
Jason Gien ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Guanylate Cyclase ◽  
Soluble Guanylate Cyclase ◽  
Left Pulmonary Artery ◽  
Flow Transducer ◽  
Pulmonary Vasodilation

Impaired nitric oxide-cGMP signaling contributes to severe pulmonary hypertension after birth, which may in part be due to decreased soluble guanylate cyclase (sGC) activity. Cinaciguat (BAY 58-2667) is a novel sGC activator that causes vasodilation, even in the presence of oxidized heme or heme-free sGC, but its hemodynamic effects have not been studied in the perinatal lung. We performed surgery on eight fetal (126 ± 2 days gestation) lambs (full term = 147 days) and placed catheters in the main pulmonary artery, aorta, and left atrium to measure pressures. An ultrasonic flow transducer was placed on the left pulmonary artery to measure blood flow, and a catheter was placed in the left pulmonary artery for drug infusion. Cinaciguat (0.1–100 μg over 10 min) caused dose-related increases in pulmonary blood flow greater than fourfold above baseline and reduced pulmonary vascular resistance by 80%. Treatment with 1H-[1,2,4]oxadiazolo[4,3-a]quinoxalin-1-one (ODQ), an sGC-oxidizing inhibitor, enhanced cinaciguat-induced pulmonary vasodilation by >120%. The pulmonary vasodilator effect of cinaciguat was prolonged, decreasing pulmonary vascular resistance for >1.5 h after brief infusion. In vitro stimulation of ovine fetal pulmonary artery smooth muscle cells with cinaciguat after ODQ treatment resulted in a 14-fold increase in cGMP compared with non-ODQ-treated cells. We conclude that cinaciguat causes potent and sustained fetal pulmonary vasodilation that is augmented in the presence of oxidized sGC and speculate that cinaciguat may have therapeutic potential for severe neonatal pulmonary hypertension.

scholarly journals The role of CXCR2 in systemic neovascularization of the mouse lung

2007 ◽  
Vol 103 (2) ◽  
pp. 594-599 ◽  
Author(s):  
Jesús Sánchez ◽  
Aigul Moldobaeva ◽  
Jessica McClintock ◽  
John Jenkins ◽  
Elizabeth Wagner
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Left Lung ◽  
Neutralizing Antibody ◽  
Left Pulmonary Artery ◽  
Endothelial Cell Proliferation ◽  
Antibody Treatment ◽  
Pulmonary Artery Obstruction ◽  
Artery Obstruction ◽  
Deficient Mice

We previously showed increased expression of the ELR+, CXC chemokines in the lung after left pulmonary artery obstruction. These chemokines have been shown in other systems to bind their G protein-coupled receptor, CXCR2, and promote systemic endothelial cell proliferation, migration, and capillary tube formation. In the present study, we blocked CXCR2 in vivo using a neutralizing antibody and also studied mice that were homozygous null for CXCR2. To estimate the extent of neovascularization in this model, we measured systemic blood flow to the left lung 14 days after left pulmonary artery ligation (LPAL). We found blood flow significantly reduced (67% decrease) with neutralizing antibody treatment compared with controls. However, blood flow was not altered in the CXCR2-deficient mice compared with wild-type controls after LPAL. To test for ligand availability, we measured macrophage inflammatory protein (MIP)-2 in lung homogenates after LPAL, because this is the predominant CXC chemokine previously shown to be increased after LPAL ( 22 ). MIP-2 protein was two- to fourfold higher in the left lung relative to the right lung in all treatment groups 4 h after LPAL and this increase did not differ among groups. We speculate that the CXCR2-deficient mice have compensatory mechanisms that mitigate their lack of gene expression and conclude that CXCR2 contributes to chemokine-induced systemic angiogenesis after pulmonary artery obstruction.

scholarly journals Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis

2020 ◽  
Author(s):  
Guiomar Pinheiro ◽  
Ana Margarida Alves ◽  
Isabel Neves ◽  
Teresa Sequeira
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Hypoplasia ◽  
Left Lung ◽  
Left Pulmonary Artery ◽  
Pulmonary Infections ◽  
Rare Congenital Disorder ◽  
History Of ◽  
Pulmonary Artery Hypoplasia

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved.

scholarly journals Role of ROS in ischemia-induced lung angiogenesis

2010 ◽  
Vol 299 (4) ◽  
pp. L535-L541 ◽  
Author(s):  
Julie Nijmeh ◽  
Aigul Moldobaeva ◽  
Elizabeth M. Wagner
Keyword(s):  
Pulmonary Artery ◽  
Left Lung ◽  
Left Pulmonary Artery ◽  
Wild Type ◽  
Systemic Blood Flow ◽  
Artery Ligation ◽  
Underlying Mechanisms ◽  
Pulmonary Artery Obstruction ◽  
Artery Obstruction

Pulmonary artery obstruction and subsequent lung ischemia have been shown to induce systemic angiogenesis despite preservation of normoxia. The underlying mechanisms, however, remain poorly understood. In a mouse model of lung ischemia induced by left pulmonary artery ligation (LPAL), we showed previously, the formation of a new systemic vasculature to the ischemic lung. We hypothesize that LPAL in the mouse increases reactive oxygen species (ROS) production, and these molecules play an initiating role in subsequent lung neovascularization. We used oxidant-sensitive dyes (DHE and H2DCF-DA) to quantify ROS and measured the antioxidant-reduced glutathione (GSH) and its oxidized form (GSSG) as indicators of ROS levels after LPAL. The magnitude of systemic neovascularization was determined by measuring systemic blood flow to the left lung with radiolabeled microspheres 14 days after LPAL. An increase in ROS was observed early (30 min: 55% increase in H2DCF-DA) after LPAL, with a return to baseline by 24 h. GSH/GSSG was decreased (∼50%) 4 h after LPAL, suggesting earlier ROS upregulation. Mice treated with the antioxidant N-acetylcysteine showed attenuated angiogenesis (62% of wild-type LPAL), and mice lacking Nrf2, a transcription factor important for antioxidant synthesis, resulted in increased neovascularization (207% of wild-type LPAL). Overall, GSH/GSSG was inversely associated with the magnitude of neovascularization. These results demonstrate that LPAL induces an early and transient ROS upregulation, and ROS appear to play a role in promoting ischemia-induced angiogenesis.

scholarly journals Noninvasive pulmonary artery wave intensity analysis in pulmonary hypertension

2015 ◽  
Vol 308 (12) ◽  
pp. H1603-H1611 ◽  
Author(s):  
Michael A. Quail ◽  
Daniel S. Knight ◽  
Jennifer A. Steeden ◽  
Liesbeth Taelman ◽  
Shahin Moledina ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Left Pulmonary Artery ◽  
Wave Intensity ◽  
Healthy Controls ◽  
Intensity Analysis ◽  
Wave Intensity Analysis ◽  
Pulmonary Vessel ◽  
Golden Angle ◽  
Angiographic Data

Pulmonary wave reflections are a potential hemodynamic biomarker for pulmonary hypertension (PH) and can be analyzed using wave intensity analysis (WIA). In this study we used pulmonary vessel area and flow obtained using cardiac magnetic resonance (CMR) to implement WIA noninvasively. We hypothesized that this method could detect differences in reflections in PH patients compared with healthy controls and could also differentiate certain PH subtypes. Twenty patients with PH (35% CTEPH and 75% female) and 10 healthy controls (60% female) were recruited. Right and left pulmonary artery (LPA and RPA) flow and area curves were acquired using self-gated golden-angle, spiral, phase-contrast CMR with a 10.5-ms temporal resolution. These data were used to perform WIA on patients and controls. The presence of a proximal clot in CTEPH patients was determined from contemporaneous computed tomography/angiographic data. A backwards-traveling compression wave (BCW) was present in both LPA and RPA of all PH patients but was absent in all controls ( P = 6e−8). The area under the BCW was associated with a sensitivity of 100% [95% confidence interval (CI) 63–100%] and specificity of 91% (95% CI 75–98%) for the presence of a clot in the proximal PAs of patients with CTEPH. In conclusion, WIA metrics were significantly different between patients and controls; in particular, the presence of an early BCW was specifically associated with PH. The magnitude of the area under the BCW showed discriminatory capacity for the presence of proximal PA clot in patients with CTEPH. We believe that these results demonstrate that WIA could be used in the noninvasive assessment of PH.

Cellular adaptation during chronic neonatal hypoxic pulmonary hypertension

1991 ◽  
Vol 261 (4) ◽  
pp. L97-L104 ◽  
Author(s):  
Kurt R. Stenmark ◽  
Almas A. Aldashev ◽  
Ernest C. Orton ◽  
A. G. Durmowicz ◽  
D. B. Badesch ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Smooth Muscle ◽  
Pulmonary Artery ◽  
Arterial Wall ◽  
Chronic Hypoxia ◽  
Left Pulmonary Artery ◽  
Right Atrial ◽  
Vascular Changes ◽  
Hypoxic Pulmonary Hypertension

Newbor animals develop more severe hypoxic pulmonary hypertension than do adults, their vascular changes are greater, and both the hypertension and vascular changes occur more rapidly. We hypothesize that this differential developmentally controlled response may arise from either a difference in the type or quantity of endogenously secreted mediators in response to a given injury or a difference in the replicative and/or matrix-producing response of the vascular cells to physical or chemical stimuli. We investigated the effect of chronic hypoxia (14 days) on the proliferative and matrix-producing phenotype of the neonatal (14-day-old) pulmonary artery smooth muscle cell (SMC) and examined the heterogeneity and potential mechanisms responsible for this response. In situ hybridization studies demonstrated a remarkable change in the distribution of cells hybridizing with a tropoelastin cRNA probe after 14 days of hypoxia. Studies also demonstrated a population of SMC that did not hybridize with the elastin or collagen probes, indicating that the pulmonary artery contains SMC of multiple phenotypes and that the response to hypoxic and hemodynamic stress is not uniform for the various types. Bromodeoxyuridine labeling experiments indicated a large increase in DNA synthesis in hypertensive vessels, which, again, was not uniform either across or along the arterial wall. In vitro experiments with neonatal SMC suggested that hypoxia alone could not be responsible for the proliferative or matrix changes. These observations were supported by in vivo experiments in which coarctation of the left pulmonary artery, which markedly decreased pressure and flow to the left lung in hypoxic animals (14 days), resulted in significant decreases in collagen and elastin message levels in the left pulmonary artery distal to the coarctation compared with location-matched vessels from the right lung. Finally, we noted marked decreases in B-receptor density and adenyl cyclase activity in right atrial and pulmonary artery tissue from the chronically hypoxic animals. Decreases in the ability of the cell to produce adenosine 3',5'-cyclic monophosphate could significantly affect both the proliferative and matrix-producing potential of the SMC. We conclude that in vivo adaptation of the pulmonary artery SMC to chronic hypoxia includes changes in protein synthesis, cell proliferation, receptor expression, and enzyme activity. Further, there is a marked heterogeneity of these responses both across and along the arterial wall. hypoxia; phenotype; signal transductions; smooth muscle cells

Anomalous origin of left pulmonary artery from the ascending aorta: echocardiography assessment

2019 ◽  
Vol 30 (1) ◽  
pp. 145-147
Author(s):  
Samir Atmani ◽  
Imane Bendris
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Cardiac Failure ◽  
Left Pulmonary Artery ◽  
Ascending Aorta ◽  
High Flow ◽  
Anomalous Origin ◽  
Cardiac Anomaly ◽  
Rare Anomaly

AbstractAnomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs. The purpose of this study is to demonstrate, with this rare anomaly, the accurate place of the echocardiography to establish diagnosis especially in the systemic or supra-systemic pulmonary hypertension.

scholarly journals Balloon angioplasty as an alternative to thromboendarterectomy for proximal pulmonary artery obstruction in a patient with chronic thromboembolic pulmonary hypertension: a case report

2017 ◽  
Vol 14 (1) ◽  
pp. 41-44
Author(s):  
N M Danilov ◽  
Yu G Matchin ◽  
S Yu Iarovoi ◽  
A Yu Demchenkova ◽  
I E Chazova
Keyword(s):  
Pulmonary Hypertension ◽  
Case Report ◽  
Surgical Treatment ◽  
Pulmonary Artery ◽  
Balloon Angioplasty ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Hypertension Treatment ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Artery Obstruction ◽  
Artery Obstruction

Thromboendarterectomy is undoubtedly the most widely used method in chronic thromboembolic hypertension treatment. Although surgical treatment is highly effective, it is often associated with high risk of complications. One of the main reasons for impossibility of surgery performance is distal pulmonary artery obstruction. In this case medical therapy or staged balloon angioplasty is considered the treatment of choice. This case report discusses if pulmonary artery angioplasty can be used in patients with proximal pulmonary artery obstruction.

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