scholarly journals Perioperative Management of Pediatric Pulmonary Hypertension

2016 ◽  
Vol 15 (2) ◽  
pp. 87-91 ◽  
Author(s):  
Rebecca Johnson Kameny ◽  
Jeffrey Fineman ◽  
Ian Adatia
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Vascular Reactivity ◽  
Pediatric Patients ◽  
Perioperative Management ◽  
Preoperative Evaluation ◽  
Invasive Mechanical Ventilation ◽  
Pulmonary Vasodilator ◽  
Hypertensive Crises

Perioperative management of patients with pulmonary hypertension or those at risk for increased pulmonary vascular reactivity should focus on supporting the patient through the vulnerable period of physiologic derangements surrounding surgery, including acute alterations in pulmonary blood flow, altered pulmonary endothelial function following cardiopulmonary bypass, invasive mechanical ventilation, and adaptation to new hemodynamics following correction or palliation of congenital heart disease lesions. These patients require careful attention to each step of perioperative management by teams experienced in the care of pediatric patients with pulmonary hypertension. This article will focus on preoperative evaluation, pulmonary hypertensive crises, general principles of perioperative management, and specific pulmonary vasodilator therapies.

Increased endothelial NOS in lambs with increased pulmonary blood flow and pulmonary hypertension

1998 ◽  
Vol 275 (5) ◽  
pp. H1643-H1651 ◽  
Author(s):  
Stephen M. Black ◽  
Jeffrey R. Fineman ◽  
Robin H. Steinhorn ◽  
James Bristow ◽  
Scott J. Soifer
Keyword(s):  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Vascular Graft ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Vascular Reactivity ◽  
Heart Defects ◽  
Pulmonary Arteries ◽  
Enos Gene ◽  
Rnase Protection

Altered pulmonary vascular reactivity is a source of morbidity and mortality for children with congenital heart defects and increased pulmonary blood flow. Nitric oxide (NO) is an important mediator of pulmonary vascular reactivity. The objective of this study was to characterize potential early alterations in expression, localization, and activity of endothelial NO synthase (eNOS) induced by increased pulmonary blood flow and pulmonary hypertension. Utilizing aortopulmonary vascular graft placement in the fetal lamb, we have established a unique animal model of pulmonary hypertension that mimics congenital heart disease with increased pulmonary blood flow. Ten fetal lambs underwent in utero placement of an aortopulmonary vascular graft (shunt). RNase protection assays and Western blotting were performed on lung tissue prepared from 4-wk-old shunt lambs and age-matched controls. eNOS mRNA (2.4:1, P < 0.05) and protein (2.08:1, P < 0.05) were increased in lungs of shunt lambs. In situ hybridization and immunohistochemistry revealed that the increase was confined to the endothelium of pulmonary arteries. eNOS protein (1.55:1, P < 0.05) and tissue cGMP concentrations (2.1:1, P < 0.05) were also increased in isolated fifth-generation pulmonary arteries of shunt lambs. In addition, total lung eNOS activity was increased (2.9:1, P < 0.05). Thus we report a previously undescribed, early upregulation of eNOS gene expression and activity in lambs with increased pulmonary blood flow and pulmonary hypertension.

Importance of adhesion molecules for children with congenital heart disease

2012 ◽  
Vol 23 (1) ◽  
pp. 35-40
Author(s):  
Ayşe Yıldırım ◽  
Aysu T. Karaağaç ◽  
Fusun Güzelmeriç ◽  
Nihat Çine ◽  
Naci C. Öner
Keyword(s):  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Adhesion Molecules ◽  
Adhesion Molecule ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Cellular Adhesion ◽  
Mean Values ◽  
Cellular Adhesion Molecule

AbstractBackgroundThe aim of our study was to compare the blood levels of adhesion molecules in children with different heart diseases and pulmonary flow rates.MethodsIn this study, we evaluated the levels of soluble intercellular adhesion molecule-1 and soluble vascular cellular adhesion molecule-1 in blood samples of 65 children with different congenital heart diseases. The patients were divided into four groups according to their pulmonary blood flow. The first group had increased pulmonary blood flow with pulmonary hypertension and left-to-right shunt. The second group had increased pulmonary blood flow without pulmonary hypertension and left-to-right shunt. The third group had decreased pulmonary blood flow with cyanotic congenital heart disease and the fourth group had normal pulmonary blood flow with left ventricle outflow tract obstruction and aortic stenosis.ResultThe highest soluble intercellular and vascular cellular adhesion molecule-1 levels with the mean values of 420.2 nanograms per millilitre and 1382.1 nanograms per millilitre, respectively, were measured in the first group and the lowest levels with the mean values of 104.4 and 358.6 nanograms per millilitre, respectively, were measured in the fourth group. The highest pulmonary blood pressure levels were found in the first group.ConclusionEndothelial activity is influenced not only by left-to-right shunt with pulmonary hypertension, but also by decreased pulmonary blood flow in cyanotic heart diseases. Adhesion molecules are valuable markers of endothelial activity in congenital heart diseases, and they are influenced by pulmonary blood flow rate.

scholarly journals Efficacy of citrulline supplementation to decrease the risk of pulmonary hypertension after congenital heart disease surgery. A local experience.

2020 ◽  
Vol 77 (4) ◽  
pp. 249-253
Author(s):  
Silene Silvera Ruiz ◽  
Carola L Grosso ◽  
Margot Tablada ◽  
Marcelo Cabrera ◽  
Raquel Dodelson de Kremer ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
Reference Level ◽  
Plasma Citrulline ◽  
Post Surgery ◽  
Group A ◽  
Group B

Pulmonary hypertension (PH) is a major cause of morbi-mortality among patients with congenital heart disease (CHD) and also a potentially severe complication after surgical repair. Oral citrulline, a precursor to NO synthesis, is safe and efficacious for decreasing the risk of postoperative PH. Objective: The aim of the present study was to investigate in pediatric patients the changes of plasma citrulline, arginine, homocysteine and nitric oxide (NO) metabolites and pulmonary artery pressures (PAP) pre-post cardiac surgery in order to describe our population status with regard to the risk of pulmonary hypertension and look for potential biomarkers for early detection and treatment. Main results/Discussion: 16 Argentine pediatric patients with CHD undergoing cardiopulmonary bypass were randomized in two groups: (A) with and (B) without perioperative citrulline supplementation. We found that plasma citrulline median levels before surgery were lower in both groups respect to referential values, probably due to the poor nutritional status of our patients; only group A surpassed post-surgery the minimum recommended level to avoid PH. Furthermore, none of the patients in group A showed mean PAP higher than 20 mmHg, whereas in group B, 67% of the measurements were ≥ than the reference level. Conclusions: We reaffirm that citrulline supplementation it is effective in reducing postoperative pulmonary hypertension and biomarkers could evidence patient status as a translational medicine application.

Alterations in ET-1, not nitric oxide, in 1-week-old lambs with increased pulmonary blood flow

2003 ◽  
Vol 284 (2) ◽  
pp. H480-H490 ◽  
Author(s):  
Boaz Ovadia ◽  
Olaf Reinhartz ◽  
Robert Fitzgerald ◽  
Janine M. Bekker ◽  
Michael J. Johengen ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Endothelial Function ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Vascular Reactivity ◽  
Protein Levels ◽  
Vasodilator Response

Altered pulmonary vascular reactivity is a source of morbidity and mortality for children with congenital heart disease and increased pulmonary blood flow. Nitric oxide (NO) and endothelin (ET)-1 are important mediators of pulmonary vascular reactivity. We hypothesize that early alterations in endothelial function contribute to the altered vascular reactivity associated with congenital heart disease. The objective of this study was to characterize endothelial function in our lamb model of increased pulmonary blood flow at 1 wk of life. Eleven fetal lambs underwent in utero placement of an aortopulmonary vascular graft (shunt) and were studied 7 days after delivery. The pulmonary vasodilator response to both intravenous ACh (endothelium dependent) and inhaled NO (endothelium independent) was similar in shunted and control lambs. In addition, tissue NOx, NO synthase (NOS) activity, and endothelial NOS protein levels were similar. Conversely, the vasodilator response to both ET-1 and 4Ala-ET-1 (an ETBreceptor agonist) were attenuated in shunted lambs, and tissue ET-1 concentrations were increased ( P < 0.05). Associated with these changes were an increase in ET-converting enzyme-1 protein and a decrease in ETBreceptor protein levels ( P < 0.05). These data demonstrate that increased pulmonary blood flow induces alterations in ET-1 signaling before NO signaling and suggest an early role for ET-1 in the altered vascular reactivity associated with increased pulmonary blood flow.

scholarly journals Pulmonary hypertension in infants with congenital heart defects: are leukotrienes involved?

1997 ◽  
Vol 6 (5-6) ◽  
pp. 323-326 ◽  
Author(s):  
A. Serraf ◽  
J-P. Gascard ◽  
J. Bruniaux ◽  
C. Labat ◽  
C. Planche ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Congenital Heart Defects ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Heart Defects ◽  
Blood Levels ◽  
Blood Samples ◽  
Number Of Patients ◽  
Pulmonary Arterial

The circulating levels of leukotriene E4in infants with congenital heart defects, increased pulmonary blood flow and pulmonary arterial hypertension, were determined and compared with infants with decreased pulmonary blood flow (Tetralogy of Fallot). There was no correlation (r=0.38) between the pulmonary arterial pressure (56 ± 4 mmHg) and the leukotriene E4levels (1.37 ± 0.67 ng/ml blood) measured in peripheral blood samples from the hypertensive group prior to surgery. There was considerable variation in the detectable leukotriene E4levels in blood samples from different patients. The levels detected in the blood samples between the two groups of patients was similar. These data suggest that neither the surgical repair during cardiopulmonary bypass nor the pulmonary hypertension appeared to modify the leukotriene E4blood levels in the small number of patients studied.

Decreased surfactant proteins in lambs with pulmonary hypertension secondary to increased blood flow

2001 ◽  
Vol 281 (5) ◽  
pp. L1264-L1270 ◽  
Author(s):  
Jorge A. Gutierrez ◽  
Andrew J. Parry ◽  
D. Michael McMullan ◽  
Cheryl J. Chapin ◽  
Jeffrey R. Fineman
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Surfactant Proteins ◽  
Control Value ◽  
Mrna Content ◽  
Increased Blood Flow

Infants with increased pulmonary blood flow secondary to congenital heart disease suffer from tachypnea, dyspnea, and recurrent pulmonary infections. We have recently established a model of pulmonary hypertension secondary to increased pulmonary blood flow in lambs after in utero placement of an aortopulmonary vascular graft. The purpose of the present study was to utilize our animal model to determine the effects on the expression of surfactant proteins A (SP-A), B (SP-B), and C (SP-C). At age 4 wk, SP-A mRNA content in lambs decreased to 61.4 ± 8% of age-matched control value ( n = 5; P < 0.05). In addition, SP-A protein content was decreased to 50 ± 12% of control value ( n = 6; P < 0.0001). Although we did not observe statistically significant changes in SP-B mRNA content, SP-B protein was decreased to 74 ± 25% of control value ( n = 4; P < 0.02). There was no difference in SP-C mRNA. These data show that in a model of congenital heart disease with pulmonary hypertension secondary to increased pulmonary blood flow, there is a decrease in SP-A gene expression as well as a decrease in SP-A and SP-B protein contents.

Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection

2016 ◽  
Vol 21 (2) ◽  
pp. 138-144 ◽  
Author(s):  
Faith J. Ross ◽  
Denise Joffe ◽  
Gregory J. Latham
Keyword(s):  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Perioperative Management ◽  
Venous Flow ◽  
Venous Circulation ◽  
Pulmonary Venous Flow ◽  
Anesthetic Considerations ◽  
Neonatal Cardiac Surgery ◽  
Cardiac Lesions ◽  
Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions.

scholarly journals The diagnostic and prognostic role of amino-terminal pro-brain type natriuretic peptides in pediatric cardiac diseases and pulmonary hypertension

2021 ◽  
Vol 5 (Volume 1) ◽  
pp. 21
Author(s):  
Kayirnisa Tilemanbetova ◽  
Taalaibek Kudaiberdiev ◽  
Jalil Sheishenov ◽  
Damirbek Abibillaev
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Natriuretic Peptide ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
Heart Diseases ◽  
Congenital Heart Diseases ◽  
Amino Terminal

Objective: Several studies showed that N-terminal pro-brain type natriuretic peptide (NT-pro-BNP) as the reliable marker both in diagnostic and prognostic workup of pediatric patients with congenital heart disease complicated by pulmonary hypertension. Furthermore, it has well assisted both in therapeutic and surgical management of children with congenital heart diseases. In this review, we aimed to analyze recent studies concerning the role of the NT-pro-BNP in pediatric cardiac patients with pulmonary hypertension. Methods: PubMed, Cochrane, Science Direct and Google Scholar databases were used with the searching of the following words: “amino terminal pro brain type natriuretic peptide”, “pediatric pulmonary hypertension”, “congenital heart diseases”, emphasizing on original research articles published within recent 15 years and in English language. We critically analyzed and summarized the retrieved studies. Conclusion: NT-pro-BNP remains as the reliable diagnostic and prognostic marker of pulmonary hypertension and congenital heart diseases in pediatric patients. NT-pro-BNP and BNP remain as the universal marker of cardiovascular pathology in pediatric population. Improvement of their effectiveness in diagnostic workup in patients with CHD and PH undergoing surgery needs further clarification. Key words: pediatric pulmonary hypertension, N-terminal pro brain type natriuretic peptide, congenital heart disease

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