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diaphragmatic hernia and right ventricle

Aura A Sanchez Mejia, Nathan J Rodgers
PURPOSE OF REVIEW: This review aims to describe the assessment of pulmonary hypertension and ventricular function in neonates with congenital diaphragmatic hernia and the long-term follow-up of their pulmonary vascular disease. RECENT FINDINGS: In 2015, the pediatric pulmonary hypertension guidelines from the American Heart Association and American Thoracic Society suggested class I level of evidence B guidelines for routine evaluation of patients with congenital diaphragmatic hernia, including longitudinal care in an interdisciplinary pulmonary hypertension program and following the recommendations offered for all children with pulmonary hypertension...
February 15, 2019: Current Treatment Options in Cardiovascular Medicine
Kendall M Lawrence, Kelsey Berger, Lisa Herkert, Christine Franciscovich, Carol Lynn H O'Dea, Lindsay N Waqar, Emily Partridge, Brian D Hanna, William H Peranteau, Catherine M Avitabile, Rachel K Hopper, Natalie E Rintoul, Holly L Hedrick
BACKGROUND/PURPOSE: Prostaglandin E1 (PGE) has been used to maintain ductus arteriosus patency and unload the suprasystemic right ventricle (RV) in neonates with congenital diaphragmatic hernia (CDH) and severe pulmonary hypertension (PH). Here we evaluate the PH response in neonates with CDH and severe PH treated with PGE. METHODS: We performed a retrospective chart review of CDH infants treated at our center between 2011 and 2016. In a subset, PGE was initiated for echocardiographic evidence of severe PH, metabolic acidosis, or hypoxemia...
October 5, 2018: Journal of Pediatric Surgery
Neil Patel, Anna Claudia Massolo, Anshuman Paria, Emily J Stenhouse, Lindsey Hunter, Emma Finlay, Carl F Davis
OBJECTIVE: To assess patterns of postnatal ventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN: In this observational case-control study of cardiac function in infants with CDH in the first 5 days of life, systolic and diastolic function in the right ventricle (RV) and left ventricle (LV) were assessed using speckle tracking echocardiography-derived global strain and tissue Doppler imaging...
December 2018: Journal of Pediatrics
Paolo Ciancarella, Fabio Fazzari, Valentina Montano, Marco Guglielmo, Gianluca Pontone
A 21 year old male with no relevant medical history presented to our Institution for further assessments of a right paracardiac mass founded on a chest X-ray. Chest computed tomography revealed a wide median defect of the diaphragm at the level of xiphoid process of the sternum, with the herniation of omental fat tissue in the mediastinum. Cardiac magnetic resonance confirmed the presence of a huge hernia originating from the foramen of Morgagni (sterno-costal hiatus), displacing the heart leftwards and posteriorly and compressing the right ventricle (RV), giving to it a tubular shape...
April 2018: Journal of the Saudi Heart Association
Abdulrahman Masrani, Sebastian McWilliams, Sanjeev Bhalla, Pamela K Woodard
PURPOSE: To report the anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. MATERIALS AND METHODS: Retrospective review of the medical records between February 2001 and February 2016 was performed. To identify patients, radiological reports were queried for "Scimitar" or "partial anomalous pulmonary venous return." Patients with radiological findings of Scimitar syndrome were included. Patients without cross-sectional imaging were excluded...
July 2018: Journal of Cardiovascular Computed Tomography
Matthew Wong, Janette Reyes, Eveline Lapidus-Krol, Monping Chiang, Tilman Humpl, Malikah Al-Faraj, Greg Ryan, Priscilla P L Chiu
Prenatal observed/expected lung-to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients. METHODS: A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5years of age...
May 2018: Journal of Pediatric Surgery
Mansi Shah, Michael R Phillips, Megan Quintana, Gary Stupp, Sean E McLean
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a structural birth defect associated with pulmonary hypoplasia and pulmonary arterial hypertension (PAH). We hypothesize that echocardiography provides a method to assess real-time right ventricle (RV) function, remodeling, and pulmonary artery (PA) flow. MATERIALS AND METHODS: Slit3 wild-type (WT) (n = 6) and knockout (KO) (n = 5) mice were analyzed at 2-3 months of age. Mice were anesthetized using isoflurane...
January 2018: Journal of Surgical Research
Benjamin Wild, Marie-Eve St-Pierre, Stéphanie Langlois, Kyle N Cowan
BACKGROUND/PURPOSE: Pulmonary vascular disease (PVD) is a leading cause of congenital diaphragmatic hernia (CDH) mortality. Progression of PVD involves extracellular matrix remodeling by elastases and matrix metalloproteinases (MMP), concomitant with proliferation of smooth muscle cells in a growth factor-enriched environment. Blockade of this pathway reversed primary pulmonary hypertension and improved survival. This study was designed to determine whether a similar pathway is induced in PVD secondary to CDH...
May 2017: Journal of Pediatric Surgery
Jan Degenhardt, Christian Enzensberger, Aline Tenzer, Andrea Kawecki, Thomas Kohl, Ellydda Widriani, Roland Axt-Fliedner
Purpose  To evaluate pre- and postprocedural myocardial function in fetuses with moderate to severe congenital diaphragmatic hernia (CDH) who underwent FETO to improve survival and to reduce morbidity and to compare these data with fetuses and CDH not undergoing FETO and normal controls. Materials and Methods  8 fetuses with isolated left-sided CDH were included and underwent FETO at our center between 2012 and 2013. Prior to and after the operation, myocardial function was assessed by measuring the mitral annular plane systolic excursion (MAPSE), the tricuspid annular plane systolic excursion (TAPSE), Tei index, isovolumetric contraction time (ICT), ejection time (ET), isovolumetric relaxation time (IRT) for the left ventricle in PW Doppler ultrasound as well as ICT, ET, IRT and Tei index in pulsed wave tissue Doppler imaging (PW-TDI) for the left and right ventricle...
January 2017: Ultraschall in der Medizin
B Thébaud, A Azancot, P de Lagausie, E Vuillard, L Ferkadji, K Benali, F Beaufils
UNLABELLED: Despite regular progress in neonatal intensive care, congenital diaphragmatic hernia (CDH) diagnosed antenatally is still associated with up to 80 % mortality. It is impossible to predict which fetus with CDH will survive or not. OBJECTIVE: To identify reliable antenatal predictors of outcome and of pulmonary hypoplasia (PH) in fetuses with CDH. DESIGN: Retrospective study. SETTING: Paediatric intensive care unit of a university children's hospital...
October 1997: Intensive Care Medicine
Gloria Pelizzo, Rossana Bussani, Lorenzo Zandonà, Ana Custrin, Carlo Valerio Bellieni, Annalisa De Silvestri, Valeria Calcaterra
OBJECTIVE: Prenatal heart adaptations to congenital diaphragmatic hernia (CDH) could help define postnatal outcome. METHODS: We retrospectively analyzed post-mortem tissues from fetuses with severe CDH (n = 7). Histology and immunohistochemical distribution of desmin, muscle actin [HHF35], endothelin-1 [ET-1] and TGF-β were evaluated. RESULTS: In the atrium, desmin, HHF35, ET-1, TGF-β were found expressed only in preterm CDH. Dishomogeneous ventricular distribution of cardiac growth factors were detected in term CDH...
2016: Fetal and Pediatric Pathology
Joon-Hyung Kim, David W Roberts, David F Bauer
BACKGROUND: Thoracic complications of ventriculoperitoneal (VP) shunts have been extensively reported in the literature. Cerebrospinal fluid (CSF) hydrothorax without catheter migration, however, has been rarely described and poorly understood. CASE DESCRIPTION: We describe development of pleural effusion and respiratory distress in a 3-year-old boy with no evidence of VP shunt catheter displacement on plain radiograph and stable ventricle size on rapid sequence magnetic resonance imaging (MRI) brain...
2015: Surgical Neurology International
P H Manso, R L Figueira, C M Prado, F L Gonçalves, A L B Simões, S G Ramos, L Sbragia
This study aimed to demonstrate that congenital diaphragmatic hernia (CDH) results in vascular abnormalities that are directly associated with the severity of pulmonary hypoplasia and hypertension. These events increase right ventricle (RV) afterload and may adversely affect disease management and patient survival. Our objective was to investigate cardiac function, specifically right ventricular changes, immediately after birth and relate them to myocardial histological findings in a CDH model. Pregnant New Zealand rabbits underwent the surgical procedure at 25 days of gestation (n=14)...
March 2015: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
F A Byrne, R L Keller, J Meadows, D Miniati, M M Brook, N H Silverman, A J Moon-Grady
OBJECTIVES: To assess whether severity of congenital diaphragmatic hernia (CDH) correlates with the degree of left heart hypoplasia and left ventricle (LV) output, and to determine if factors leading to abnormal fetal hemodynamics, such as compression and reduced LV preload, contribute to left heart hypoplasia. METHODS: This was a retrospective cross-sectional study of fetuses at 16-37 weeks' gestation that were diagnosed with CDH between 2000 and 2010. Lung-to-head ratio (LHR), liver position and side of the hernia were determined from stored ultrasound images...
December 2015: Ultrasound in Obstetrics & Gynecology
Mustafa Topuz, Mehmet Cihat Ozek
Traumatic diaphragm ruptures occur frequently after motor vehicle accidents through penetrating traumas. In 90% of the patients, traumatic diaphragm rupture commonly coexists with other organ injuries. Posteroanterior chest x-ray, computed tomography, magnetic resonance imaging, upper gastrointestinal system contrast-enhanced examinations, and thoraxoscopy/laparoscopy are several modalities which can be used for diagnosing traumatic diaphragm rupture in clinical practice. A case of right ventricle collapse secondary to hepatothorax caused by diaphragm rupture was presented in this study...
November 2014: Ulusal Travma Ve Acil Cerrahi Dergisi, Turkish Journal of Trauma & Emergency Surgery: TJTES
Noboru Inamura, Akio Kubota, Ryo Ishii, Yoichiro Ishii, Yukiko Kawazu, Yuji Hamamichi, Akihiro Yoneda, Hisayuki Kawahara, Hiroomi Okuyama, Futoshi Kayatani
OBJECT: The purpose of this study is to evaluate the outcome of our therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia (ADCDH). METHODS: We treated 61 cases of ADCDH according to our strategy. Prostaglandin E1 was required to be maintained the patency of the ductus arteriosus (PDA) in 39 cases (Group I) while it was not administered in 22 cases (Group II). Left ventricular end-diastolic dimension (LVDD) and Tei index were measured with echocardiography on days 0, 2, and 7 after birth...
September 2014: Pediatric Surgery International
Philip DeKoninck, Jan D'hooge, Tim Van Mieghem, Jute Richter, Jan Deprest
OBJECTIVE: The aim of this study is to evaluate cardiac function in fetuses with congenital diaphragmatic hernia (CDH) using speckle tracking. METHOD: Case-control study assessed cardiac contractility in consecutive fetuses with CDH. Controls were anatomically normal fetuses, adjusted for gestational age. Speckle tracking software calculated ventricular peak longitudinal velocity, displacement and strain. Pulmonary hypoplasia was assessed using observed/expected lung-to-head ratio (O/E LHR)...
December 2014: Prenatal Diagnosis
Lisa A Murphy, Nicholas J Russell, Michelle I Dulake, Reid K Nakamura
A 4-year-old female spayed domestic longhair cat was referred for dyspnea. Further diagnostics revealed severe pleural effusion and a peritoneopericardial diaphragmatic hernia (PPDH). Following surgical correction of the PPDH the pleural effusion persisted. Re-check echocardiogram 4 weeks after initial evaluation revealed leftward deviation of the interventricular septum and interatrial septum occurring with inspiration. There were also exaggerated phasic changes in trans-tricuspid flow velocities suggestive of constrictive pericardial disease...
August 2014: Journal of Feline Medicine and Surgery
Hiroyuki Nagasawa, Toshinari Koyama, Hideo Sasai, Yoshinori Kohno, Yutaka Yamamoto, Masashi Kondo, Masami Sugawara, Daisuke Terazawa, Ryosuke Miura
Spondylocostal dysostosis (SCD) is a very rare syndrome characterized by vertebral malformation and rib deformity. Some of the patients with SCD have other birth defects in the central nervous system, the genitourinary tract, diaphragm or heart and so forth. There have been reported SCD with complex congenital heart disease, such as pulmonary atresia, double outlet right ventricle, and d-transposition of great arteries. However, there have been no reported SCD patients with confirmed tetralogy of Fallot (TOF)...
August 2014: Congenital Anomalies
Claudiu Ungureanu, Christophe Laruelle, Denis Pieters, Elie El Khoury, Philippe Blouard, Patrick Timmermans, Bruno Coulier, Adrien Ramboux, Pierre-Yves Etienne
No abstract text is available yet for this article.
December 2013: Acta Cardiologica
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