{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T06:18:25Z","timestamp":1788589105923,"version":"build-2803163510"},"reference-count":56,"publisher":"Georg Thieme Verlag KG","issue":"S 01","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am J Perinatol"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:p>Despite advances in caring for neonates with congenital diaphragmatic hernia (CDH), mortality and morbidity continues to be high. Additionally, the pathophysiology of cardiac dysfunction in this condition is poorly understood. Postnatal cardiac dysfunction in neonates with CDH may be multifactorial with origins in fetal life. Mechanical obstruction, competition from herniated abdominal organs into thoracic cavity combined with redirection of ductus venosus flow away from patent foramen ovale leading to smaller left-sided structures may be a contributing factor. This shunting decreases left atrial and left ventricular blood volume, which may result in altered micro- and macrovascular aberrations affecting cardiac development in the prenatal period. Direct mass effect from herniated intra-abdominal contents restricting cardiac growth and\/or reduced left ventricular preload may contribute independently to left ventricular dysfunction in the absence of right ventricular dysfunction and or pulmonary hypertension. With variable clinical phenotypes of cardiac dysfunction, pulmonary hypertension, and respiratory failure in patients with CDH, there is increased need for individualized diagnosis and tailored therapy. Routine use of therapy such as inhaled nitric oxide and sildenafil that induces significant pulmonary vasodilation may be detrimental in left ventricle dysfunction, whereas in a patient with pure right ventricle dysfunction, they may be beneficial. Targeted functional echocardiography serves as a real-time tool for defining the pathophysiology and aids optimization of vasoactive therapy in affected neonates.<\/jats:p><jats:p>\n            Key Points\n          <\/jats:p>","DOI":"10.1055\/a-2067-7925","type":"journal-article","created":{"date-parts":[[2023,4,3]],"date-time":"2023-04-03T23:25:31Z","timestamp":1680564331000},"page":"e1709-e1716","source":"Crossref","is-referenced-by-count":9,"special_numbering":"S","title":["\u201cHeart of the Matter\u201d: Cardiac Dysfunction in Congenital Diaphragmatic Hernia"],"prefix":"10.1055","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7009-4821","authenticated-orcid":false,"given":"Sharada H.","family":"Gowda","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Baylor College of Medicine, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Neonatology, Royal Hospital for Children, Glasgow, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2023,4,26]]},"reference":[{"issue":"06","key":"ref1","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1055\/s-0035-1569151","article-title":"The Congenital Diaphragmatic Hernia Study Group registry","volume":"25","author":"F Morini","year":"2015","journal-title":"Eur J Pediatr Surg"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.jpeds.2021.09.001","article-title":"In-hospital morbidities for neonates with congenital diaphragmatic hernia: the impact of defect size and laterality","volume":"240","author":"V Y Chock","year":"2022","journal-title":"J Pediatr"},{"issue":"03","key":"ref3","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1097\/SLA.0000000000005113","article-title":"Mortality in congenital diaphragmatic hernia: a multicenter registry study of over 5000 patients over 25 years","volume":"277","author":"V S Gupta","year":"2021","journal-title":"Ann Surg"},{"issue":"06","key":"ref4","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1055\/s-0037-1607291","article-title":"Pulmonary hypertension in patients with congenital diaphragmatic hernia: does lung size matter?","volume":"28","author":"A L Madenci","year":"2018","journal-title":"Eur J Pediatr Surg"},{"issue":"01","key":"ref5","doi-asserted-by":"crossref","first-page":"151168","DOI":"10.1053\/j.semperi.2019.07.007","article-title":"Congenital diaphragmatic hernia-associated cardiac dysfunction","volume":"44","author":"N Patel","year":"2020","journal-title":"Semin Perinatol"},{"issue":"01","key":"ref6","doi-asserted-by":"crossref","first-page":"151169","DOI":"10.1053\/j.semperi.2019.07.008","article-title":"The influence of genetics in congenital diaphragmatic hernia","volume":"44","author":"L Yu","year":"2020","journal-title":"Semin Perinatol"},{"issue":"06","key":"ref7","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/j.jpedsurg.2005.03.025","article-title":"Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group","volume":"40","author":"J N Graziano","year":"2005","journal-title":"J Pediatr Surg"},{"issue":"12","key":"ref8","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.201904-0731OC","article-title":"Ventricular dysfunction is a critical determinant of mortality in congenital diaphragmatic hernia","volume":"200","author":"N Patel","year":"2019","journal-title":"Am J Respir Crit Care Med"},{"issue":"07","key":"ref9","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1097\/PCC.0000000000002318","article-title":"Early left ventricular dysfunction and severe pulmonary hypertension predict adverse outcomes in \u201clow-risk\u201d congenital diaphragmatic hernia","volume":"21","author":"D T Dao","year":"2020","journal-title":"Pediatr Crit Care Med"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/j.jpeds.2019.12.037","article-title":"Implications of atrial-level shunting by echocardiography in newborns with congenital diaphragmatic hernia","volume":"219","author":"M Wehrmann","year":"2020","journal-title":"J Pediatr"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.jpeds.2018.07.062","article-title":"Early postnatal ventricular dysfunction is associated with disease severity in patients with congenital diaphragmatic hernia","volume":"203","author":"N Patel","year":"2018","journal-title":"J Pediatr"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"170104","DOI":"10.1183\/16000617.0104-2017","article-title":"Pulmonary vascular development in congenital diaphragmatic hernia","volume":"27","author":"D S Mous","year":"2018","journal-title":"Eur Respir Rev"},{"issue":"05","key":"ref13","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1016\/S0022-3468(84)80105-0","article-title":"Left ventricular hypoplasia in congenital diaphragmatic hernia","volume":"19","author":"J R Siebert","year":"1984","journal-title":"J Pediatr Surg"},{"issue":"06","key":"ref14","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1002\/uog.14790","article-title":"Severe left diaphragmatic hernia limits size of fetal left heart more than does right diaphragmatic hernia","volume":"46","author":"F A Byrne","year":"2015","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"03","key":"ref15","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1002\/uog.7497","article-title":"Significance and outcome of left heart hypoplasia in fetal congenital diaphragmatic hernia","volume":"35","author":"M Vogel","year":"2010","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"01","key":"ref16","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/S0022-3476(98)70178-7","article-title":"Cardiac malposition, redistribution of fetal cardiac output, and left heart hypoplasia reduce survival in neonates with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation","volume":"133","author":"S Baumgart","year":"1998","journal-title":"J Pediatr"},{"issue":"04","key":"ref17","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1016\/S0095-5108(18)30210-0","article-title":"The fetal heart in diaphragmatic hernia","volume":"23","author":"L D Allan","year":"1996","journal-title":"Clin Perinatol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"E151","DOI":"10.1055\/s-0031-1281651","article-title":"Association of intrathoracic herniation of the liver with left heart hypoplasia in fetuses with a left diaphragmatic hernia but not in fetuses with a right diaphragmatic hernia","volume":"32","author":"R Stressig","year":"2011","journal-title":"Ultraschall Med"},{"issue":"01","key":"ref19","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1186\/s13054-016-1440-0","article-title":"The right ventricle: interaction with the pulmonary circulation","volume":"20","author":"M R Pinsky","year":"2016","journal-title":"Crit Care"},{"issue":"01","key":"ref20","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1093\/eurheartj\/ehm567","article-title":"Right coronary artery flow impairment in patients with pulmonary hypertension","volume":"29","author":"S A van Wolferen","year":"2008","journal-title":"Eur Heart J"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"641310","DOI":"10.3389\/fphys.2021.641310","article-title":"Current understanding of the right ventricle structure and function in pulmonary arterial hypertension","volume":"12","author":"D Sharifi Kia","year":"2021","journal-title":"Front Physiol"},{"issue":"03","key":"ref22","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1159\/000215585","article-title":"Assessment of right ventricular function using tissue Doppler imaging in infants with pulmonary hypertension","volume":"96","author":"N Patel","year":"2009","journal-title":"Neonatology"},{"issue":"01","key":"ref23","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1097\/PCC.0b013e31829b1e7a","article-title":"Right ventricular diastolic function measured by tissue Doppler imaging predicts early outcome in congenital diaphragmatic hernia","volume":"15","author":"F Moenkemeyer","year":"2014","journal-title":"Pediatr Crit Care Med"},{"issue":"12","key":"ref24","doi-asserted-by":"crossref","first-page":"1474","DOI":"10.1093\/cvr\/cvx160","article-title":"The overloaded right heart and ventricular interdependence","volume":"113","author":"R Naeije","year":"2017","journal-title":"Cardiovasc Res"},{"issue":"01","key":"ref25","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1159\/000499347","article-title":"Ventricular dysfunction, interdependence, and mechanical dispersion in newborn infants with congenital diaphragmatic hernia","volume":"116","author":"A C Massolo","year":"2019","journal-title":"Neonatology"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.jpeds.2018.02.040","article-title":"The left ventricle in congenital diaphragmatic hernia: implications for the management of pulmonary hypertension","volume":"197","author":"J P Kinsella","year":"2018","journal-title":"J Pediatr"},{"issue":"03","key":"ref27","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1053\/j.sempedsurg.2017.04.001","article-title":"Cardiac dysfunction in congenital diaphragmatic hernia: pathophysiology, clinical assessment, and management","volume":"26","author":"N Patel","year":"2017","journal-title":"Semin Pediatr Surg"},{"issue":"10","key":"ref28","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1016\/j.echo.2011.07.014","article-title":"Targeted neonatal echocardiography in the neonatal intensive care unit: practice guidelines and recommendations for training. Writing Group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) and the Association for European Pediatric Cardiologists (AEPC)","volume":"24","author":"L Mertens","year":"2011","journal-title":"J Am Soc Echocardiogr"},{"issue":"01","key":"ref29","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1067\/mpd.2002.125004","article-title":"Influence of congenital heart disease on survival in children with congenital diaphragmatic hernia","volume":"141","author":"M S Cohen","year":"2002","journal-title":"J Pediatr"},{"issue":"05","key":"ref30","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1007\/s00246-018-1850-7","article-title":"Diminished cardiac performance and left ventricular dimensions in neonates with congenital diaphragmatic hernia","volume":"39","author":"G Altit","year":"2018","journal-title":"Pediatr Cardiol"},{"issue":"02","key":"ref31","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1055\/s-0033-1357757","article-title":"Intravenous sildenafil in the management of pulmonary hypertension associated with congenital diaphragmatic hernia","volume":"25","author":"A Bialkowski","year":"2015","journal-title":"Eur J Pediatr Surg"},{"issue":"04","key":"ref32","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1002\/ppul.23935","article-title":"Continuous intravenous sildenafil as an early treatment in neonates with congenital diaphragmatic hernia","volume":"53","author":"F Kipfmueller","year":"2018","journal-title":"Pediatr Pulmonol"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/j.jpeds.2019.09.052","article-title":"Inhaled nitric oxide is associated with improved oxygenation in a subpopulation of infants with congenital diaphragmatic hernia and pulmonary hypertension","volume":"219","author":"K M Lawrence","year":"2020","journal-title":"J Pediatr"},{"issue":"12","key":"ref34","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1001\/jamapediatrics.2016.2023","article-title":"Evaluation of variability in inhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia","volume":"170","author":"L R Putnam","year":"2016","journal-title":"JAMA Pediatr"},{"issue":"01","key":"ref35","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1097\/01.CCM.0000251123.70632.4E","article-title":"Mechanisms of a reduced cardiac output and the effects of milrinone and levosimendan in a model of infant cardiopulmonary bypass","volume":"35","author":"C F Stocker","year":"2007","journal-title":"Crit Care Med"},{"issue":"02","key":"ref36","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1159\/000339108","article-title":"Use of milrinone to treat cardiac dysfunction in infants with pulmonary hypertension secondary to congenital diaphragmatic hernia: a review of six patients","volume":"102","author":"N Patel","year":"2012","journal-title":"Neonatology"},{"issue":"02","key":"ref37","first-page":"137","article-title":"Judgment often impossible without randomized trials. Commentary on N. Patel: use of milrinone to treat cardiac dysfunction in infants with pulmonary hypertension secondary to congenital diaphragmatic hernia: a review of six patients (Neonatology 2012;102:130\u2013136)","volume":"102","author":"A Giaccone","year":"2012","journal-title":"Neonatology"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1186\/s40748-017-0066-9","article-title":"Milrinone in congenital diaphragmatic hernia - a randomized pilot trial: study protocol, review of literature and survey of current practices","volume":"3","author":"S Lakshminrusimha","year":"2017","journal-title":"Matern Health Neonatol Perinatol"},{"issue":"01","key":"ref39","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/j.jpedsurg.2018.10.039","article-title":"Use of prostaglandin E1 to treat pulmonary hypertension in congenital diaphragmatic hernia","volume":"54","author":"K M Lawrence","year":"2019","journal-title":"J Pediatr Surg"},{"issue":"07","key":"ref40","doi-asserted-by":"crossref","first-page":"e382","DOI":"10.1097\/PCC.0000000000002665","article-title":"Exploratory assessment of levosimendan in infants with congenital diaphragmatic hernia","volume":"22","author":"L Schroeder","year":"2021","journal-title":"Pediatr Crit Care Med"},{"issue":"01","key":"ref41","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.jpeds.2014.03.059","article-title":"Vasopressin improves hemodynamic status in infants with congenital diaphragmatic hernia","volume":"165","author":"S N Acker","year":"2014","journal-title":"J Pediatr"},{"issue":"05","key":"ref42","doi-asserted-by":"crossref","first-page":"1380","DOI":"10.1016\/S0003-4975(99)00188-5","article-title":"Cardiovascular effects of inhaled nitric oxide in a canine model of cardiomyopathy","volume":"67","author":"E Loh","year":"1999","journal-title":"Ann Thorac Surg"},{"issue":"11","key":"ref43","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1007\/s00383-018-4337-y","article-title":"Controversies in extracorporeal membrane oxygenation (ECMO) utilization and congenital diaphragmatic hernia (CDH) repair using a Delphi approach: from the American Pediatric Surgical Association Critical Care Committee (APSA-CCC)","volume":"34","author":"S B Cairo","year":"2018","journal-title":"Pediatr Surg Int"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"336","DOI":"10.3389\/fped.2019.00336","article-title":"Extracorporeal membrane oxygenation in congenital diaphragmatic hernia","volume":"7","author":"N Rafat","year":"2019","journal-title":"Front Pediatr"},{"issue":"07","key":"ref45","doi-asserted-by":"crossref","first-page":"1315","DOI":"10.1016\/j.jpedsurg.2008.12.021","article-title":"Factors associated with survival in infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: a report from the Congenital Diaphragmatic Hernia Study Group","volume":"44","author":"R Seetharamaiah","year":"2009","journal-title":"J Pediatr Surg"},{"issue":"03","key":"ref46","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1053\/j.sempedsurg.2017.04.006","article-title":"ECMO in CDH: is there a role?","volume":"26","author":"D W Kays","year":"2017","journal-title":"Semin Pediatr Surg"},{"issue":"10","key":"ref47","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1007\/s00383-015-3766-0","article-title":"The evaluation of diastolic function using the diastolic wall strain (DWS) before and after radical surgery for congenital diaphragmatic hernia","volume":"31","author":"T Tanaka","year":"2015","journal-title":"Pediatr Surg Int"},{"issue":"11","key":"ref48","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1513\/AnnalsATS.201910-767OC","article-title":"Right ventricular strain, brain natriuretic peptide, and mortality in congenital diaphragmatic hernia","volume":"17","author":"C M Avitabile","year":"2020","journal-title":"Ann Am Thorac Soc"},{"issue":"04","key":"ref49","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1007\/s12519-012-0380-2","article-title":"Mid-term differences in right ventricular function in patients with congenital diaphragmatic hernia compared with controls","volume":"8","author":"M J Egan","year":"2012","journal-title":"World J Pediatr"},{"issue":"02","key":"ref50","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1056\/NEJMoa2027030","article-title":"Randomized trial of fetal surgery for severe left diaphragmatic hernia","volume":"385","author":"J A Deprest","year":"2021","journal-title":"N Engl J Med"},{"issue":"04","key":"ref51","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1002\/uog.7340","article-title":"Left ventricular cardiac function in fetuses with congenital diaphragmatic hernia and the effect of fetal endoscopic tracheal occlusion","volume":"34","author":"T Van Mieghem","year":"2009","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"11","key":"ref52","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1002\/pd.5333","article-title":"The impact of fetal endoscopic tracheal occlusion in isolated left-sided congenital diaphragmatic hernia on left-sided cardiac dimensions","volume":"38","author":"G S Dhillon","year":"2018","journal-title":"Prenat Diagn"},{"issue":"01","key":"ref53","first-page":"65","article-title":"Myocardial function pre- and post-fetal endoscopic tracheal occlusion (FETO) in fetuses with left-sided moderate to severe congenital diaphragmatic hernia","volume":"38","author":"J Degenhardt","year":"2017","journal-title":"Ultraschall Med"},{"issue":"01","key":"ref54","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1159\/000356437","article-title":"Left heart structures in human neonates with congenital diaphragmatic hernia and the effect of fetal endoscopic tracheal occlusion","volume":"35","author":"L A Rocha","year":"2014","journal-title":"Fetal Diagn Ther"},{"issue":"12","key":"ref55","doi-asserted-by":"crossref","first-page":"6353","DOI":"10.3390\/ijms22126353","article-title":"Management of congenital diaphragmatic hernia (CDH): role of molecular genetics","volume":"22","author":"G Cannata","year":"2021","journal-title":"Int J Mol Sci"},{"issue":"05","key":"ref56","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1086\/513442","article-title":"Genetic factors in congenital diaphragmatic hernia","volume":"80","author":"A M Holder","year":"2007","journal-title":"Am J Hum Genet"}],"container-title":["American Journal of Perinatology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2067-7925.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T13:52:29Z","timestamp":1730814749000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2067-7925"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,3]]},"references-count":56,"journal-issue":{"issue":"S 01","published-online":{"date-parts":[[2024,5,13]]},"published-print":{"date-parts":[[2024,5]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2067-7925","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0735-1631","1098-8785"],"issn-type":[{"value":"0735-1631","type":"print"},{"value":"1098-8785","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,4,3]]},"published":{"date-parts":[[2023,4,3]]}}}