An unusual cause of refractory persistent pulmonary hypertension of the newborn: anomalous origin of one pulmonary artery

2013 ◽  
Vol 24 (3) ◽  
pp. 543-545
Author(s):  
Nilufer Guzoglu ◽  
Fatma Nur Sari ◽  
Nahide Altug
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Innominate Artery ◽  
Persistent Pulmonary Hypertension ◽  
Anomalous Origin ◽  
Cardiac Malformation ◽  
Mortality And Morbidity ◽  
Congenital Cardiac Malformation

AbstractPersistent pulmonary hypertension of the newborn is a source of considerable mortality and morbidity. Anomalous origin of one pulmonary artery, an uncommon congenital cardiac malformation, is a rare cause of persistent pulmonary hypertension. Here, we report the case of a patient with an anomalous origin of one pulmonary artery from the innominate artery who presented with persistent pulmonary hypertension refractory to treatment.

scholarly journals Serotonin contributes to high pulmonary vascular tone in a sheep model of persistent pulmonary hypertension of the newborn

2013 ◽  
Vol 304 (12) ◽  
pp. L894-L901 ◽  
Author(s):  
Cassidy Delaney ◽  
Jason Gien ◽  
Gates Roe ◽  
Nicole Isenberg ◽  
Jenai Kailey ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Tryptophan Hydroxylase ◽  
Late Gestation ◽  
Persistent Pulmonary Hypertension ◽  
Sheep Model ◽  
Pulmonary Hemodynamics ◽  
Fetal Sheep ◽  
Pulmonary Arterial

Although past studies demonstrate that altered serotonin (5-HT) signaling is present in adults with idiopathic pulmonary arterial hypertension, whether serotonin contributes to the pathogenesis of persistent pulmonary hypertension of the newborn (PPHN) is unknown. We hypothesized that 5-HT contributes to increased pulmonary vascular resistance (PVR) in a sheep model of PPHN and that selective 5-HT reuptake inhibitor (SSRI) treatment increases PVR in this model. We studied the hemodynamic effects of 5-HT, ketanserin (5-HT2A receptor antagonist), and sertraline, an SSRI, on pulmonary hemodynamics of the late gestation fetal sheep with PPHN caused by prolonged constriction of the ductus arteriosis. Brief intrapulmonary infusions of 5-HT increased PVR from 1.0 ± 0.07 (baseline) to 1.4 ± 0.22 mmHg/ml per minute of treatment ( P < 0.05). Ketanserin decreased PVR from 1.1 ± 0.15 (baseline) to 0.82 ± 0.09 mmHg/ml per minute of treatment ( P < 0.05). Sertraline increased PVR from 1.1 ± 0.17 (baseline) to 1.4 ± 0.17 mmHg/ml per minute of treatment ( P = 0.01). In addition, we studied 5-HT production and activity in vitro in experimental PPHN. Compared with controls, pulmonary artery endothelial cells from fetal sheep with PPHN exhibited increased expression of tryptophan hydroxylase 1 and 5-HT production by twofold and 56%, respectively. Compared with controls, 5-HT2A R expression was increased in lung homogenates and pulmonary artery smooth muscle cell lysates by 35% and 32%, respectively. We concluded that increased 5-HT contributes to high PVR in experimental PPHN through activation of the 5-HT2A receptor and that SSRI infusion further increases PVR in this model.

Oxidant stress from uncoupled nitric oxide synthase impairs vasodilation in fetal lambs with persistent pulmonary hypertension

2007 ◽  
Vol 292 (4) ◽  
pp. H1812-H1820 ◽  
Author(s):  
Girija G. Konduri ◽  
Ivane Bakhutashvili ◽  
Annie Eis ◽  
Kirkwood Pritchard
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Nitric Oxide Synthase ◽  
Pulmonary Artery ◽  
Ductus Arteriosus ◽  
Oxidant Stress ◽  
Pulmonary Arteries ◽  
Persistent Pulmonary Hypertension ◽  
Pulmonary Vasodilation ◽  
Oxide Synthase

Persistent pulmonary hypertension of newborn (PPHN) is associated with decreased NO release and impaired pulmonary vasodilation. We investigated the hypothesis that increased superoxide (O2•−) release by an uncoupled endothelial nitric oxide synthase (eNOS) contributes to impaired pulmonary vasodilation in PPHN. We investigated the response of isolated pulmonary arteries to the NOS agonist ATP and the NO donor S-nitroso- N-acetylpenicillamine (SNAP) in fetal lambs with PPHN induced by prenatal ligation of ductus arteriosus and in sham-ligated controls in the presence or absence of the NOS antagonist nitro-l-arginine methyl ester (l-NAME) or the O2•− scavenger 4,5-dihydroxy-1,3-benzenedisulfonate (Tiron). ATP caused dose-dependent relaxation of pulmonary artery rings in control lambs but induced constriction of the rings in PPHN lambs. l-NAME, the NO precursor l-arginine, and Tiron restored the relaxation response of pulmonary artery rings to ATP in PPHN. Relaxation to NO was attenuated in arteries from PPHN lambs, and the response was improved by l-NAME and by Tiron. We also investigated the alteration in heat shock protein (HSP)90-eNOS interactions and release of NO and O2•− in response to ATP in the pulmonary artery endothelial cells (PAEC) from these lambs. Cultured PAEC and endothelium of freshly isolated pulmonary arteries from PPHN lambs released O2•− in response to ATP, and this was attenuated by the NOS antagonist l-NAME and superoxide dismutase (SOD). ATP stimulated HSP90-eNOS interactions in PAEC from control but not PPHN lambs. HSP90 immunoprecipitated from PPHN pulmonary arteries had increased nitrotyrosine signal. Oxidant stress from uncoupled eNOS contributes to impaired pulmonary vasodilation in PPHN induced by ductal ligation in fetal lambs.

scholarly journals Efficacy, safety and tolerability of bosentan as an adjuvant to sildenafil and sildenafil alone in persistant pulmonary hypertension of newborn (PPHN)

2021 ◽  
Author(s):  
J.R. Vijay Kumar ◽  
H.S. Natraj Setty ◽  
M. Jayaranganath ◽  
C.N. Manjunath
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Mode Of Delivery ◽  
Systolic Pressure ◽  
Clinical Manifestations ◽  
Persistent Pulmonary Hypertension ◽  
Pulmonary Artery Systolic Pressure ◽  
Duration Of Symptoms ◽  
Group A ◽  
Group B

AbstractBackgroundPulmonary Arterial Hypertension (PAH) carries a poor prognosis in both adult and pediatric patients. It is a life-threatening condition in newborns. Current recommendations advocate the use of targeted monotherapy as a first-line approach for the treatment of Persistent Pulmonary Hypertension of the Newborn (PPHN). In case of an inadequate clinical response to treatment, an addition of a second or third agent is considered. PAH is usually managed with a phosphodiesterase 5 inhibitor or an endothelin receptor blocker. There are limited pediatric studies that address questions like which class of therapy should be initiated first or if a combination should be initiated together. With this background, the present study was initiated to compare the efficacy, safety, and tolerability of bosentan as an adjuvant to sildenafil and sildenafil alone in PPHN.ResultsA total of 40 patients were enrolled in the study. Out of them, 26 were males (65%) and 14 were females (35%). PPHN was most commonly seen in the 29 (72.5%) of participants with a history of first order birth. Mean duration of symptoms was 14.05 ± 2.06 days. The participants were randomized to two groups. Group A consisted of total 25 participants that received both bosentan and sildenafil and group B had 15 participants that received sildenafil alone. Both groups were comparable in terms of birth weight and present weight, consanguinity, and mode of delivery. Efficacy was determined by the reduction in mean baseline Pulmonary Artery Systolic Pressure (PASP). PASP in group A was 75.56 ± 10.62 mm Hg and in group B was 64.86 ± 12.25 mm Hg which was not statistically significant (P > 0.05). PASP on the third and seventh day in group A were 43.72 ± 8.63 and 24.47 ± 3.52 mm Hg compared to 42.28 ± 9.43 and 27.276 ± 8.38 respectively in group B which was statistically significant (P < 0.05).There were two deaths each in both groups. Two participants in Group A developed liver function abnormalities. None of the participants in Group B had adverse effects.ConclusionMost common clinical manifestations were nonspecific. Cardiovocal syndrome was common in PPHN. We conclude that oral sildenafil treatment is a safe, simple and effective treatment for persistent pulmonary hypertension in newborn. Combination of bosentan with sildenafil is more effective and safe in reducing pulmonary artery (PA) pressures in high-risk patients with PPHN.

scholarly journals P1488 Anomalous origin of left coronary artery from right pulmonary artery in association with scimitar syndrome

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Aly ◽  
R Lizano Santamaria ◽  
S J Yoo
Keyword(s):  
Pulmonary Hypertension ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Right Atrial ◽  
Anomalous Origin ◽  
Coronary Perfusion ◽  
Antegrade Flow ◽  
The Right

Abstract Clinical Presentation A full-term neonate was referred to our institution because of respiratory distress. CXR was significant for right lung hypoplasia and mild cardiomegaly. ECG showed normal sinus rhythm, right atrial enlargement, and right ventricular hypertrophy with no signs of ischemia. Imaging Findings The initial echocardiogram demonstrated PAPVD with the right upper pulmonary vein draining into IVC/RA junction with flow acceleration (mean gradient= 7 mmHg), moderate ASD, small muscular VSD with left-right shunting, moderate PDA with bidirectional shunting. Forward flow was seen in the proximal part of left main coronary artery (LMCA). RV systolic pressure was supra-systemic with a qualitatively moderately reduced RV systolic function. The patient was taken to the catheterization lab where MPA angiography revealed an antegrade flow from the RPA into LMCA supplying both the anterior descending and the circumflex arteries. A selective injection within the scimitar vein showed drainage of the right lung into a vertical vein connecting with stenosis to IVC. A follow up echocardiogram to re-examine the coronary origin revealed an anomalous origin of LMCA from proximal RPA; 3 mm distal to branch pulmonary artery bifurcation with mainly antegrade low velocity flow into LMCA and LAD. (Image 1) Role of Imaging in Patient Care - Imaging of the coronary origin in patients with ALCAPA can be challenging especially if the LMCA originates from RPA. Also, the presence of pulmonary hypertension might contribute to maintain coronary perfusion and lead to misinterpretation of the antegrade flow in LMCA and its branches. - In certain situations, cardiac catheterization is essential to make the diagnosis of ALCAPA which prevented a potentially catastrophic outcome. Catheter intervention with a series of balloon dilations of the stenotic scimitar vein was successful in relieving the stenosis. Summary/Discussion Points: - Extensive review of the available literature revealed only three cases of Scimitar syndrome associated with ALCAPA. In all of these cases, the LMCA originated from the posterior sinus of MPA. Our case is the first to report ALCAPA from RPA in association with Scimitar syndrome. This presentation might have led to the initial misinterpretation of the echocardiography images. - The presence of pulmonary hypertension in our patient maintained an adequate antegrade flow across the LMCA preventing significant coronary steal and signs of myocardial ischemia. - The report highlights the challenges in making the diagnosis of ALCAPA with echocardiograms. Moreover, we discuss the role of cross-sectional and invasive imaging to rule out potential coronary arteries anomalies in patients with Scimitar syndrome, as this a rare although a very significant association that may have important implications in their outcomes. Abstract P1488 Figure. ALCAPA origin from RPA

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 Cross talk between NADPH oxidase and autophagy in pulmonary artery endothelial cells with intrauterine persistent pulmonary hypertension

2012 ◽  
Vol 302 (7) ◽  
pp. L651-L663 ◽  
Author(s):  
Ru-Jeng Teng ◽  
Jianhai Du ◽  
Scott Welak ◽  
Tongju Guan ◽  
Annie Eis ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Nadph Oxidase ◽  
Fluorescent Protein ◽  
Persistent Pulmonary Hypertension ◽  
Species Formation ◽  
Green Fluorescent ◽  
Impaired Angiogenesis

Autophagy is a process for cells to degrade proteins or entire organelles to maintain a balance in the synthesis, degradation, and subsequent recycling of cellular products. Increased reactive oxygen species formation is known to induce autophagy. We previously reported that increased NADPH oxidase (NOX) activity in pulmonary artery endothelial cells (PAEC) from fetal lambs with persistent pulmonary hypertension (PPHN) contributes to impaired angiogenesis in PPHN-PAEC compared with normal PAEC. We hypothesized that increased NOX activity in PPHN-PAEC is associated with increased autophagy, which, in turn, contributes to impaired angiogenesis in PPHN-PAEC. In the present study, we detected increased autophagy in PPHN-PAEC as shown by increased ratio of the microtubule-associated protein 1 light chain (LC3)-II to LC3-I and increased percentage of green fluorescent protein-LC3 punctate positive cells. Inhibiting autophagy by 3-methyladenine, chloroquine, and beclin-1 knockdown in PPHN-PAEC has led to decreased autophagy and increased in vitro angiogenesis. Inhibition of autophagy also decreased the association between gp91phox and p47phox, NOX activity, and superoxide generation. A nonspecific antioxidant N-acetylcysteine and a NOX inhibitor apocynin decreased autophagy in PPHN-PAEC. In conclusion, autophagy may contribute to impaired angiogenesis in PPHN-PAEC through increasing NOX activity. Our results suggest that, in PPHN-PAEC, a positive feedback relationship between autophagy and NOX activity may regulate angiogenesis.

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