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Objective:To summarize the prenatal diagnosis features, classification and pregnancy outcome of anomalous origin of one pulmonary artery branch from the aorta (AOPA).Methods:This study involved 14 cases who were prenatally diagnosed with AOPA in Guangzhou Women and Children's Medical Center between June 2016 and August 2022. Prenatal and postnatal echocardiographic features, postpartum diagnosis, surgical treatment and pregnancy outcome in these cases were summarized and analyzed by descriptive analysis.Results:Out of the 14 fetuses, there were seven fetuses with proximal-type AOPA (including three isolated AOPA, three Berry syndrome and one with interruption of the aortic arch, aorticopulmonary septal defect and ventricular septal defect) and another seven with isolated distal-type of AOPA. Among the seven cases of proximal-type AOPA, two were terminated and five were born alive. The postpartum diagnosis was consistent with the prenatal diagnosis in the five babies who later underwent surgical treatment with good outcomes. Among the seven cases of distal-type AOPA, one was terminated; two were initially diagnosed as AOPA in the neonatal period but then as unilateral absence of pulmonary artery (UAPA) due to tapering or closure of the ductus arteriosus during follow-up; the other four were confirmed with UAPA after delivery. All of the six neonates underwent surgical treatment with good outcomes.Conclusions:Prenatal diagnosis and classification of AOPA should be as accurate as possible. It is recommended that the distal-type of AOPA could be diagnosed as UAPA after delivery and treated according to UAPA. Both kinds of patients should be treated with surgery timely after delivery to ensure a good prognosis.
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Endogenously eliminating the hematoma is a favorable strategy in addressing intracerebral hemorrhage (ICH). This study sought to determine the role of retinoid X receptor-α (RXR-α) in the context of hematoma absorption after ICH. Our results showed that pharmacologically activating RXR-α with bexarotene significantly accelerated hematoma clearance and alleviated neurological dysfunction after ICH. RXR-α was expressed in microglia/macrophages, neurons, and astrocytes. Mechanistically, bexarotene promoted the nuclear translocation of RXR-α and PPAR-γ, as well as reducing neuroinflammation by modulating microglia/macrophage reprograming from the M1 into the M2 phenotype. Furthermore, all the beneficial effects of RXR-α in ICH were reversed by the PPAR-γ inhibitor GW9662. In conclusion, the pharmacological activation of RXR-α confers robust neuroprotection against ICH by accelerating hematoma clearance and repolarizing microglia/macrophages towards the M2 phenotype through PPAR-γ-related mechanisms. Our data support the notion that RXR-α might be a promising therapeutic target for ICH.
Subject(s)
Humans , Anilides/pharmacology , Cerebral Hemorrhage/drug therapy , Hematoma/drug therapy , Macrophages , Microglia , Neuroprotection , PPAR gamma , Retinoid X Receptor alphaABSTRACT
Endogenously eliminating the hematoma is a favorable strategy in addressing intracerebral hemorrhage (ICH). This study sought to determine the role of retinoid X receptor-α (RXR-α) in the context of hematoma absorption after ICH. Our results showed that pharmacologically activating RXR-α with bexarotene significantly accelerated hematoma clearance and alleviated neurological dysfunction after ICH. RXR-α was expressed in microglia/macrophages, neurons, and astrocytes. Mechanistically, bexarotene promoted the nuclear translocation of RXR-α and PPAR-γ, as well as reducing neuroinflammation by modulating microglia/macrophage reprograming from the M1 into the M2 phenotype. Furthermore, all the beneficial effects of RXR-α in ICH were reversed by the PPAR-γ inhibitor GW9662. In conclusion, the pharmacological activation of RXR-α confers robust neuroprotection against ICH by accelerating hematoma clearance and repolarizing microglia/macrophages towards the M2 phenotype through PPAR-γ-related mechanisms. Our data support the notion that RXR-α might be a promising therapeutic target for ICH.
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Objective To assess the application value of juxtaposition of the great vessels in diagnosis of right atrial isom-erism( RAI) by echocardiography.Methods We retrospectively analyzed 73 patients with right atrial isomerism in Guangzhou Women and Children's Medical Center from 2010-2017.All these patients were performed by echocardiography and compu-ted tomography(CT).We used the results of CT as the golden standard.Results 73 cases of RAI were diagnosed by CT.60 cases of RAI were diagnosed by echocardiography.Juxtaposition of the descending aorta(DAO) and inferior vena(IVC) was demonstrated in all these 60 patients.29 of 60 were on the left side of the spine(39.73％).31 of 60 were on the right side of spine(42.47％).In the other 13 patients of echocardiography missed diagnosis, the arrangement of the great vessels was nor-mal in 10 cases, and inverse in 3 cases.Conclusion Juxtaposition of the great vessels is one of the most important findings in patients with RAI.It is quick and simple and has a higher application value in diagnosis of RAI.
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Objective To explore the diagnostic value of transthoracic echocardiography (TTE) for anomalous origin of right pulmonary artery (AORPA).Methods Echocardiographic data of 26 patients with AORPA proved by surgical operation were analyzed retrospectively.Results TTE showed that the pulmonary trunk and the left pulmonary artery were displayed normally,and the right pulmonary artery originated from the ascending aorta.AORPA in all 26 patients were diagnosed,and the coincidence rate was 100％.Among 26 patients,9 associated with Berry's syndrome,1 combined with coarctation of aorta,22 combined with patent ductus arteriosus,23 combined with atrial septal defect or patent foramen ovale,25 were found with severe pulmonary artery hypertension,and the diagnostic accuracy rate of TTE was 100％ (26/26),100％ (26/26),96.15％ (25/26),92.31％ (24/26) and 100％ (26/26),respectively.Conclusion TTE can early and accurately diagnose AORPA,also has high accuracy in diagnosis of other concomitant malformations.Multi-section scan can reduce misdiagnosis.TTE can be taken as the preferred inspection method for diagnosing AORPA.
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Purpose Unilateral absence of pulmonary artery is a rare congenital pulmonary arterydysplasia whose untypical symptom often leads to missed diagnosis or misdiagnosis.Diagnostic and follow-up values of ultrasound cardiogram to pediatric congenital unilateral absence of pulmonary artery (UAPA) are explored in this paper.Materials and Methods Ultrasound cardiogram images of 35 UAPA patients confirmed by surgery or CT angiography in Guangzhou Women and Children's Medical Center from May 2009 to July 2016 were analyzed retrospectively,and images characteristics of the correctly diagnosed and missed diagnosis or misdiagnosed ultrasound cardiogram were analyzed.Results Among these 35 UAPA patients,21 cases were on the right side,14 cases on the left side,16 cases single UAPA and 19 cases combined with other intracardiac malformations.27 cases were initially diagnosed by ultrasound cardiogram,7 cases were missed diagnosed and 1 case misdiagnosed,with 77.1％ diagnosis accuracy.UAPA ultrasound cardiogram showed that normal bifurcation structure of pulmonary artery disappears.Main pulmonary artery continued to be one-side pulmonary artery,running to left or right and forming left branch or right branch of pulmonary artery.Pulmonary artery of the one-side absence of pulmonary segment supplied blood through aorta or other branches or vessels on the other side.After receiving unilateral pulmonary artery reconstruction surgery,ultrasound cardiogram of 8 patients of one month indicated that average flow rate of close-end anastomosis was (1.22± 0.17) m/s and far-end (2.17± 0.56) m/s.Conclusion Ultrasound cardiogram is efficient for UAPA preoperative diagnosis.Meanwhile,it is convenient,noninvasive and efficient in assessing anastomosis and pressure of pulmonary artery after surgery.
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Objective To discuss the value of color Doppler echocardiography in diagnosis of scimitar syndrome .Methods The echocardiographic results of 6 patients with a diagnosis of scimitar syndrome were reviewed retrospectively .Their sonographic and hemodynamic characteristics were also analyzed connected with the reports in the literature .Results Three cases had dextrocardia and the others had mesocardia .All cases got right ventricular dimension enlargement .Total or partial of right pulmonary venous connection to the inferior vena cava were 3 cases respectively .All cases had right pulmonary artery hypoplasia .All of 6 cases echocardiographic results were in accordance with the findings by CT angiography and 4 cases were confirmed by operation .Conclusions The sonographic features of scimitar syndrome were obvious ,and echocardiography was contribute to early diagnosis of scimitar syndrome .
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Purpose To evaluate the carotid artery structure and diastolic function of the brachial artery in children with coarctation of aorta (CoA) using high-frequency ultrasound. Materials and Methods Twenty-four cases of children with CoA were selected, among them seven cases were simple CoA and 17 cases were combined with other cardiac malformations, and 20 cases of simple ventricular septal defect (VSD) children and 20 healthy children over the same period were selected as the control groups. Carotid artery intima-media thickness (IMT) and brachial artery flow-mediated vasodilation (FMD) in all subjects were evaluated using high-frequency ultrasound, vascular structure and functional changes were compared before CoA correction and VSD repair treatment. Results Carotid artery IMT of CoA group was (0.47±0.12) mm, which was significantly larger than the VSD group (0.41±0.10) mm and the control group (0.40±0.09) mm, and the difference was statistically significant (F=4.275, P0.05) between the VSD group and the control group for carotid artery IMT and brachial artery FMD. Conclusion In children with CoA, besides the structure abnormalities of the narrowing vascular, there is also the combination of structural and fuctional changes such as carotid artery intima-media complex thickening and brachial artery flow-mediated dilation function decrease.