keyword
Keywords Pulmonary arterial hypertensio...

Pulmonary arterial hypertension AND congenital heart disease AND pediatric

https://read.qxmd.com/read/38654425/total-anomalous-pulmonary-venous-connection-in-adults-should-we-offer-surgery
#1
JOURNAL ARTICLE
Vivek Bagaria, Badragiri Lahari, B R Jagannath, C S Hiremath
Background: We aim to determine the surgical outcomes of adult patients with total anomalous pulmonary venous connection (TAPVC) and examine the regression of pulmonary artery (PA) pressures after the procedure. Methods: We reviewed the hospital records from 2003 to 2022 and identified 49 adult patients with TAPVC. We assessed their surgical outcomes and the trend of PA pressures after the procedure. Continuous data are presented as mean ± SD or median (interquartile range) and categorical variables are presented as percentages...
April 23, 2024: World Journal for Pediatric & Congenital Heart Surgery
https://read.qxmd.com/read/38646828/operative-repair-of-aortopulmonary-window-a-25-year-experience
#2
JOURNAL ARTICLE
Alyssa B Kalustian, Richard C Tang, Michiaki Imamura
Background: Aortopulmonary window (APW) is a rare anomaly with variable morphology and associated cardiac anomalies. We evaluated impact of patient and operative factors on mid-term outcomes following APW repair. Methods: Twenty-nine patients underwent surgical APW repair at our institution from 1996 to 2022. Eight (28%) had simple APW, accompanied by only atrial septal defect or patent ductus arteriosus; 21 (72%) had complex APW with additional cardiovascular lesions, including nine with interrupted aortic arch...
April 22, 2024: World Journal for Pediatric & Congenital Heart Surgery
https://read.qxmd.com/read/38636784/congenital-diaphragmatic-hernia-patients-with-left-heart-hypoplasia-and-left-ventricular-dysfunction-have-highest-odds-of-mortality
#3
JOURNAL ARTICLE
María V Fraga, Holly L Hedrick, Natalie E Rintoul, Yan Wang, Devon Ash, Sabrina J Flohr, Leny Mathew, Tom Reynolds, Jenny L Engelman, Catherine M Avitabile
OBJECTIVES: To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease. STUDY DESIGN: Demographic and clinical variables were collected from a single-center, retrospective cohort of CDH patients from January 2017 through May 2022...
April 16, 2024: Journal of Pediatrics
https://read.qxmd.com/read/38607876/empowering-little-fighters-post-cardiotomy-pediatric-ecmo-and-the-journey-to-recovery
#4
JOURNAL ARTICLE
Alok Kumar, Sangeeth Raj, Saurabh Singh, Gurpinder S Ghotra, Nikhil Tiwari
INTRODUCTION: Extra Corporeal Membrane Oxygenation (ECMO) has long been used for cardiorespiratory support in the immediate post-paediatric cardiac surgery period with a 2-3% success as per the ELSO registry. Success in recovery depends upon the optimal delivery of critical care to paediatric patients and a comprehensive healthcare team. METHODOLOGY: The survival benefit of children placed on central veno arterial (VA) ECMO following elective cardiac surgeries for congenital heart disease (n = 672) was studied in a cohort of 29 (4...
April 1, 2024: Annals of Cardiac Anaesthesia
https://read.qxmd.com/read/38531747/perioperative-sildenafil-therapy-in-pulmonary-hypertension-associated-with-congenital-cardiac-disease-an-updated-meta-analysis
#5
REVIEW
Shajie Ur Rehman Usmani, Syed Umar Hasan, Syed Hasan Ali, Syeda Hoorulain Ahmed, Neha Pervez, Muhammad Danial, Owais Khan, Dominique L Tucker, M Mujeeb Zubair
To provide an updated meta-analysis to evaluate the efficacy and safety of sildenafil on pediatric patients with pulmonary hypertension (PH) associated with congenital heart disease (CHD). To assess the efficacy and safety of sildenafil, five outcomes, time duration of post-operative need for mechanical ventilation, time duration of post-operative ICU stay, length of hospitalization (LOH), the incidence of mortalities and pulmonary arterial pressure to aortic pressure ratio (PAP/AoP) were regarded as primary efficacy outcomes...
March 25, 2024: Asian Journal of Surgery
https://read.qxmd.com/read/38529652/diagnostic-strategy-for-suspected-unilateral-absence-of-the-pulmonary-artery
#6
JOURNAL ARTICLE
Van Luong Hoang, Viet Anh Lam, Thanh Nguyen Pham
UNLABELLED: <p>Background: Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly. OBJECTIVE: To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam. METHODS: All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue...
March 19, 2024: Current medical imaging
https://read.qxmd.com/read/38410024/the-evaluation-of-the-smad1-rs1016792-polymorphism-and-gene-expression-on-pulmonary-hypertension-due-to-congenital-heart-disease-in-children-a-preliminary-study
#7
JOURNAL ARTICLE
Adnan Selim Kimyon, Ayşegül Çetinkaya, Olgu Hallıoğlu Kılınç, Nurcan Aras
Smad Family Member (SMAD), a protein family responsible for transducing the signal induced by TGF-β into the nucleus, is thought to play a role in the pathology of many heart diseases. Therefore, we aimed to evaluate the influence of the SMAD1 rs1016792 polymorphism and gene expression on pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD) in children. A total of 90 children, 45 of whom were PAH-CHD children and 45 healthy children, were included in the study. Patients were selected from those who were diagnosed and followed in the Department of Pediatric Cardiology...
February 26, 2024: Nucleosides, Nucleotides & Nucleic Acids
https://read.qxmd.com/read/38370158/application-of-a-modified-clinical-classification-for-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-in-children-emphasis-on-atrial-septal-defects-and-transposition-of-the-great-arteries-an-analysis-from-the-topp-registry
#8
JOURNAL ARTICLE
Julie Wacker, Tilman Humpl, Rolf M F Berger, Dunbar Ivy, David Bowers, Damien Bonnet, Maurice Beghetti
AIMS: A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA). METHODS AND RESULTS: Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed...
2024: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/38361584/corrigendum-use-of-the-index-of-pulmonary-vascular-disease-for-predicting-longterm-outcome-of-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#9
Ayako Chida-Nagai, Naoki Masaki, Kay Maeda, Konosuke Sasaki, Hiroki Sato, Jun Muneuchi, Yoshie Ochiai, Hiroomi Murayama, Masahiro Tahara, Atsuko Shiono, Atsushi Shinozuka, Fumihiko Kono, Daisuke Machida, Shinichi Toyooka, Seiichiro Sugimoto, Kazufumi Nakamura, Satoshi Akagi, Maiko Kondo, Shingo Kasahara, Yasuhiro Kotani, Junichi Koizumi, Katsuhiko Oda, Masako Harada, Daisuke Nakajima, Akira Murata, Hazumu Nagata, Koichi Yatsunami, Tomio Kobayashi, Yoshikiyo Matsunaga, Takahiro Inoue, Hiroyuki Yamagishi, Naomi Nakagawa, Katsuki Ohtani, Masaki Yamamoto, Yushi Ito, Tatsunori Hokosaki, Yuta Kuwahara, Satoshi Masutani, Koji Nomura, Tsutomu Wada, Hirofumi Sawada, Masayuki Abiko, Tatsunori Takahashi, Yuichi Ishikawa, Seigo Okada, Atsushi Naitoh, Takako Toda, Tatsuya Ando, Akihiro Masuzawa, Shinsuke Hoshino, Masaaki Kawada, Yuichi Nomura, Kentaro Ueno, Naoki Ohashi, Tsuyoshi Tachibana, Yuchen Cao, Hideaki Ueda, Sadamitsu Yanagi, Masaaki Koide, Norie Mitsushita, Kouji Higashi, Yoshihiro Minosaki, Tomohiro Hayashi, Takashi Okamoto, Kenji Kuraishi, Eiji Ehara, Hidekazu Ishida, Hitoshi Horigome, Takashi Murakami, Kohta Takei, Taku Ishii, Gen Harada, Yasutaka Hirata, Jun Maeda, Shunsuke Tatebe, Chiharu Ota, Yasunobu Hayabuchi, Hisanori Sakazaki, Takashi Sasaki, Keiichi Hirono, Sayo Suzuki, Masahiro Yasuda, Atsuhito Takeda, Madoka Sawada, Kagami Miyaji, Atsushi Kitagawa, Yosuke Nakai, Nobuyuki Kakimoto, Kouta Agematsu, Atsushi Manabe, Yoshikatsu Saiki
[This corrects the article DOI: 10.3389/fcvm.2023.1212882.].
2024: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/38271986/effect-of-face-mask-on-pulmonary-artery-pressure-during-echocardiography-in-children-and-adolescents
#10
JOURNAL ARTICLE
Alireza Ahmadi, Mohammad Reza Sabri, Zohreh Sadat Navabi
BACKGROUND: Face masks have become an important tool for preventing the spread of respiratory diseases. However, we hypothesized that face masks with reduced nasal airflow may alter pulmonary artery systolic pressure (PASP). PURPOSE: This study aimed to evaluate the effect of face masks on PASP in children and adolescents. METHODS: This case-control study was conducted between March 2021 and April 2022 at the Pediatric Cardiovascular Research Center in Isfahan, Iran...
January 23, 2024: Clinical and experimental pediatrics
https://read.qxmd.com/read/38148409/lung-transplantation-for-pulmonary-vascular-disease-in-children-a-united-network-for-organ-sharing-analysis
#11
JOURNAL ARTICLE
Hosam F Ahmed, Amalia Guzman-Gomez, Malika Desai, Alia Dani, David L S Morales, Paul J Critser, Farhan Zafar, Don Hayes
Pulmonary vascular disease (PVD) represents an important clinical indication for lung transplant (LTx) in infants, children, and adolescents. There is limited information on LTx outcomes in these patients. We explored LTx volumes and post-LTx survival in children with PVD compared to other diagnoses. The UNOS Registry was queried from 1989 to 2020 to identify first-time pediatric LTx recipients (< 18 yo). PVD was categorized as idiopathic pulmonary arterial hypertension (IPAH) and non-idiopathic arterial hypertension (non-IPAH) and compared to all other patients as other diagnoses...
December 26, 2023: Pediatric Cardiology
https://read.qxmd.com/read/37963484/-new-aspects-in-pediatric-pulmonary-hypertension-commented-2022ers-esc-ph-guidelines
#12
JOURNAL ARTICLE
Christian Apitz, Rainer Kozlik-Feldmann, Christina A Eichstaedt, Matthias Gorenflo, Astrid E Lammers, Ralf Geiger
Pulmonary hypertension (PH) in childhood differs from that of adulthood particularly in the specific pathophysiology of congenital heart disease-associated pulmonary arterial hypertension, the presence of developmental lung disease, and the frequent association with chromosomal, genetic, and syndromal abnormalities. Treatment of children with PH requires a modified diagnostic algorithm tailored to childhood, as well as pathophysiologically oriented therapeutic strategies. In the current 2022 ERS/ESC-PH guidelines, the specific features of PH in children are highlighted in its own chapter and commented on by the authorship group in this article...
November 2023: Pneumologie
https://read.qxmd.com/read/37859771/covid-19-vaccination-for-children-with-pulmonary-hypertension-efficacy-safety-and-reasons-for-opting-against-vaccination
#13
JOURNAL ARTICLE
Zeying Zhang, Dan Wang, Wanyun Zuo, Xun Wang, Fan Yang, Haiyan Luo, Zhenghui Xiao, Qiming Liu, Yunbin Xiao
OBJECTIVE: To determine the reasons why pulmonary hypertension (PH) children refused vaccination against COVID-19, evaluate the safety and efficacy of COVID-19 vaccine in PH children. STUDY DESIGN: This retrospective cohort study included congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) and bronchopulmonary dysplasia associated PH (BPD-PH) children who were divided into vaccinated group and non-vaccinated group. Univariate logistic regression analysis and multivariate logistic regression analysis were conducted to explore the reasons why PH children refused COVID-19 vaccine...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/37844905/preoperative-sildenafil-in-pediatric-patients-undergoing-congenital-heart-surgeries-a-systematic-review-and-meta-analysis
#14
REVIEW
Yusuf Ananda Fikri, Eka Prasetya Budi Mulia, Faris Wahyu Nugroho
Background. Pulmonary hypertension (PH) is a common complication of congenital heart disease (CHD). With early surgical intervention, outcomes have improved over the last two decades. Persistent PH, however, may still occur following surgery. Sildenafil has been shown to be beneficial for postoperative pulmonary hypertensive crises. The role of preoperative sildenafil in controlling postoperative PH is poorly elucidated. This study aimed to evaluate the effect of preoperative sildenafil on pediatric patients undergoing congenital heart surgeries...
December 2023: Seminars in Cardiothoracic and Vascular Anesthesia
https://read.qxmd.com/read/37839791/a-surgical-mouse-model-of-neonatal-right-ventricular-outflow-tract-obstruction-by-pulmonary-artery-banding
#15
JOURNAL ARTICLE
Debao Li, Haifa Hong, Minghui Li, Xiuxia Xu, Shoubao Wang, Yingying Xiao, Sixie Zheng, Zheng Wang, Yi Yan, Hao Chen, Chunxia Zhou, Hao Zhang, Qi Sun, Lincai Ye
Diseased animal models play an extremely important role in preclinical research. Lacking the corresponding animal models, many basic research studies cannot be carried out, and the conclusions obtained are incomplete or even incorrect. Right ventricular (RV) outflow tract (RVOT) obstruction leads to RV pressure overload (PO) and reduced pulmonary blood flow (RPF), which are two of the most important pathophysiological characteristics in pediatric cardiovascular diseases and seriously affect the survival rate and long-term quality of life of many children...
October 13, 2023: Journal of Heart and Lung Transplantation
https://read.qxmd.com/read/37823242/pulmonary-hemodynamics-before-and-after-pediatric-heart-transplantation
#16
JOURNAL ARTICLE
Philipp Biedermann, Vanessa Sitte-Koch, Martin Schweiger, Anke Meinold, Daniel Quandt, Oliver Kretschmar, Christian Balmer, Walter Knirsch
BACKGROUND: Pulmonary hypertension (PH) may limit the outcome of pediatric heart transplantation (pHTx). We evaluated pulmonary hemodynamics in children undergoing pHTx. METHODS: Cross-sectional, single-center, observational study analyzing pulmonary hemodynamics in children undergoing pHTx. RESULTS: Twenty-three children (female 15) underwent pHTx at median (IQR) age of 3.9 (.9-8.2) years with a time interval between first clinical signs and pHTx of 1...
January 2024: Clinical Transplantation
https://read.qxmd.com/read/37731527/use-of-the-index-of-pulmonary-vascular-disease-for-predicting-long-term-outcome-of-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#17
JOURNAL ARTICLE
Ayako Chida-Nagai, Naoki Masaki, Kay Maeda, Konosuke Sasaki, Hiroki Sato, Jun Muneuchi, Yoshie Ochiai, Hiroomi Murayama, Masahiro Tahara, Atsuko Shiono, Atsushi Shinozuka, Fumihiko Kono, Daisuke Machida, Shinichi Toyooka, Seiichiro Sugimoto, Kazufumi Nakamura, Satoshi Akagi, Maiko Kondo, Shingo Kasahara, Yasuhiro Kotani, Junichi Koizumi, Katsuhiko Oda, Masako Harada, Daisuke Nakajima, Akira Murata, Hazumu Nagata, Koichi Yatsunami, Tomio Kobayashi, Yoshikiyo Matsunaga, Takahiro Inoue, Hiroyuki Yamagishi, Naomi Nakagawa, Katsuki Ohtani, Masaki Yamamoto, Yushi Ito, Tatsunori Hokosaki, Yuta Kuwahara, Satoshi Masutani, Koji Nomura, Tsutomu Wada, Hirofumi Sawada, Masayuki Abiko, Tatsunori Takahashi, Yuichi Ishikawa, Seigo Okada, Atsushi Naitoh, Takako Toda, Tatsuya Ando, Akihiro Masuzawa, Shinsuke Hoshino, Masaaki Kawada, Yuichi Nomura, Kentaro Ueno, Naoki Ohashi, Tsuyoshi Tachibana, Yuchen Cao, Hideaki Ueda, Sadamitsu Yanagi, Masaaki Koide, Norie Mitsushita, Kouji Higashi, Yoshihiro Minosaki, Tomohiro Hayashi, Takashi Okamoto, Kenji Kuraishi, Eiji Ehara, Hidekazu Ishida, Hitoshi Horigome, Takashi Murakami, Kohta Takei, Taku Ishii, Gen Harada, Yasutaka Hirata, Jun Maeda, Shunsuke Tatebe, Chiharu Ota, Yasunobu Hayabuchi, Hisanori Sakazaki, Takashi Sasaki, Keiichi Hirono, Sayo Suzuki, Masahiro Yasuda, Atsuhito Takeda, Madoka Sawai, Kagami Miyaji, Atsushi Kitagawa, Yosuke Nakai, Nobuyuki Kakimoto, Kouta Agematsu, Atsushi Manabe, Yoshikatsu Saiki
AIMS: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores...
2023: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/37676274/mid-systolic-notching-of-the-pulmonary-valve-doppler-signal-is-highly-associated-with-pulmonary-hypertension
#18
JOURNAL ARTICLE
Robert W Loar, Christopher Tsao, Fadeke Ogunyankin, J Kevin Wilkes, Dennis VanLoozen, Deborah Schutte, Adrian Dyer
Mid-systolic notching (MSN) of the pulmonary valve Doppler signal represents a reflected systolic pressure wave from the pulmonary vasculature and is often seen in pulmonary hypertension (PH). We hypothesize that MSN is associated with a higher pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP), and a diagnosis of PH in pediatric patients. This was a retrospective study of patients ≤ 18 years who had an echocardiogram obtained ≤ 30 days before catheterization for suspected PH...
September 7, 2023: Pediatric Cardiology
https://read.qxmd.com/read/37623346/unique-pulmonary-hypertensive-vascular-diseases-associated-with-heart-and-lung-developmental-defects
#19
REVIEW
Hidekazu Ishida, Jun Maeda, Keiko Uchida, Hiroyuki Yamagishi
Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex congenital heart disease (CHD), pulmonary atresia with ventricular septal defect, and aortopulmonary collateral arteries. Some cases of complex CHD, associated with a single ventricle after Fontan operation, show pathological changes in the small peripheral pulmonary arteries and pulmonary vascular resistance similar to those observed in pulmonary arterial hypertension (PAH)...
August 3, 2023: Journal of Cardiovascular Development and Disease
https://read.qxmd.com/read/37614831/associated-radiation-exposure-from-medical-imaging-and-excess-lifetime-risk-of-developing-cancer-in-pediatric-patients-with-pulmonary-hypertension
#20
JOURNAL ARTICLE
Malini Mahendra, Philip Chu, Elena K Amin, Hythem Nawaytou, James R Duncan, Jeffrey R Fineman, Rebecca Smith-Bindman
Pediatric patients with pulmonary hypertension (PH) receive imaging studies that use ionizing radiation (radiation) such as computed tomography (CT) and cardiac catheterization to guide clinical care. Radiation exposure is associated with increased cancer risk. It is unknown how much radiation pediatric PH patients receive. The objective of this study is to quantify radiation received from imaging and compute associated lifetime cancer risks for pediatric patients with PH. Electronic health records between 2012 and 2022 were reviewed and radiation dose data were extracted...
July 2023: Pulmonary Circulation
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