{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T12:11:04Z","timestamp":1791461464113,"version":"4.3.4"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Cardiol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Children with congenital diaphragmatic hernia (CDH) may require cardiac catheterization for evaluation of pulmonary hypertension. The extent of abnormal systemic venous return complicating access to the right heart in this population has not been well described.\u00a0We identified children with CDH who underwent cardiac catheterization at the University of California, San Francisco, at less than or equal to 2 years from 2006 to 2025. Non-invasive imaging, catheterization reports, and angiography were reviewed.\u00a0Nine children with right CDH (three with hepatopulmonary fusion) and twenty-one children with left CDH underwent cardiac catheterization. Initial catheterization occurred at 4 weeks-2 years of age and 1.9-13\u00a0kg. The right heart was directly accessed from the inferior vena cava (IVC) via the femoral vein in all patients with left CDH. Patients with right CDH had abnormal venous anatomy precluding direct IVC-right atrial access in several. This was likely secondary to IVC compression at the diaphragm. Fluoroscopy times in the latter group were shorter with superior vena cava access via the internal jugular vein compared to inferior vena cava access via the femoral vein.\u00a0Abnormal systemic venous anatomy complicating venous access to the right heart should be considered for patients with right CDH, especially those with hepatopulmonary fusion. If not already delineated by non-invasive imaging, initial angiography from the femoral vein can guide the approach to cardiac catheterization.<\/jats:p>","DOI":"10.1007\/s00246-026-04229-9","type":"journal-article","created":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T15:25:10Z","timestamp":1776698710000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Finding a Path to the Heart: Right Heart Catheterization in Patients with Right Congenital Diaphragmatic Hernia and Anomalous Venous Anatomy"],"prefix":"10.1007","author":[{"given":"Teresa A.","family":"Evans","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta L.","family":"Keller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita J.","family":"Moon-Grady","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Teitel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena K.","family":"Amin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,20]]},"reference":[{"key":"4229_CR1","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1002\/ajmg.c.30126","volume":"145C","author":"BR Pober","year":"2007","unstructured":"Pober BR (2007) Overview of epidemiology, genetics, birth defects, and chromosome abnormalities associated with CDH. Am J Med Genet C Semin Med Genet 145C:158\u2013171","journal-title":"Am J Med Genet C Semin Med Genet"},{"key":"4229_CR2","doi-asserted-by":"publisher","first-page":"1903","DOI":"10.1016\/j.jpedsurg.2019.09.037","volume":"55","author":"DM Ferguson","year":"2020","unstructured":"Ferguson DM (2020) Hepatopulmonary fusion: a rare variant of congenital diaphragmatic hernia. J Pediatr Surg 55:1903\u20131907","journal-title":"J Pediatr Surg"},{"key":"4229_CR3","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1016\/j.jpedsurg.2009.10.090","volume":"45","author":"JW Gander","year":"2010","unstructured":"Gander JW et al (2010) Hepatic pulmonary fusion in an infant with a right-sided congenital diaphragmatic hernia and contralateral mediastinal shift. J Pediatr Surg 45:265\u2013268","journal-title":"J Pediatr Surg"},{"key":"4229_CR4","doi-asserted-by":"publisher","first-page":"5278","DOI":"10.1177\/0300060518759892","volume":"46","author":"HH Almaramhy","year":"2018","unstructured":"Almaramhy HH (2018) Hepatopulmonary fusion associated with right-sided congenital diaphragmatic hernia: management of this rare anomaly and a review of the literature. J Int Med Res 46:5278\u20135284","journal-title":"J Int Med Res"},{"key":"4229_CR5","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1016\/j.jpeds.2014.10.024","volume":"166","author":"LA Lusk","year":"2015","unstructured":"Lusk LA, Wai KC, Moon-Grady AJ, Steurer MA, Keller RL (2015) Persistence of pulmonary hypertension by echocardiography predicts short-term outcomes in congenital diaphragmatic hernia. J Pediatr 166:251-256.e1","journal-title":"J Pediatr"},{"key":"4229_CR6","doi-asserted-by":"publisher","first-page":"670","DOI":"10.1002\/ppul.25258","volume":"56","author":"NP Varghese","year":"2021","unstructured":"Varghese NP, Tillman RH, Keller RL (2021) Pulmonary hypertension is an important co-morbidity in developmental lung diseases of infancy: Bronchopulmonary dysplasia and congenital diaphragmatic hernia. Pediatr Pulmonol 56:670\u2013677","journal-title":"Pediatr Pulmonol"},{"key":"4229_CR7","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1016\/j.siny.2014.09.008","volume":"19","author":"M Pierro","year":"2014","unstructured":"Pierro M, Th\u00e9baud B (2014) Understanding and treating pulmonary hypertension in congenital diaphragmatic hernia. Semin Fetal Neonatal Med 19:357\u2013363","journal-title":"Semin Fetal Neonatal Med"},{"key":"4229_CR8","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1159\/000434643","volume":"39","author":"SC Derderian","year":"2016","unstructured":"Derderian SC et al (2016) Mass effect alone may not explain pulmonary vascular pathology in severe congenital diaphragmatic hernia. Fetal Diagn Ther 39:117\u2013124","journal-title":"Fetal Diagn Ther"},{"key":"4229_CR9","doi-asserted-by":"publisher","first-page":"290","DOI":"10.1038\/pr.2013.98","volume":"74","author":"S Fleck","year":"2013","unstructured":"Fleck S et al (2013) Fetal production of growth factors and inflammatory mediators predicts pulmonary hypertension in congenital diaphragmatic hernia. Pediatr Res 74:290\u2013298","journal-title":"Pediatr Res"},{"key":"4229_CR10","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.jpeds.2018.02.040","volume":"197","author":"JP Kinsella","year":"2018","unstructured":"Kinsella JP et al (2018) The left ventricle in congenital diaphragmatic hernia: implications for the management of pulmonary hypertension. J Pediatr 197:17\u201322","journal-title":"J Pediatr"},{"key":"4229_CR11","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1016\/j.jacc.2015.07.032","volume":"66","author":"ML O\u2019Byrne","year":"2015","unstructured":"O\u2019Byrne ML et al (2015) Predictors of catastrophic adverse outcomes in children with pulmonary hypertension undergoing cardiac catheterization: a multi-institutional analysis from the pediatric health information systems database. J Am Coll Cardiol 66:1261\u20131269","journal-title":"J Am Coll Cardiol"},{"key":"4229_CR12","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2022.11.028","author":"PD Maia","year":"2022","unstructured":"Maia PD et al (2022) Hemodynamic Characterization of Neonates with Congenital Diaphragmatic Hernia-Associated Pulmonary Hypertension by Cardiac Catheterization. J Pediatr. https:\/\/doi.org\/10.1016\/j.jpeds.2022.11.028","journal-title":"J Pediatr"},{"key":"4229_CR13","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1053\/j.sempedsurg.2017.04.001","volume":"26","author":"N Patel","year":"2017","unstructured":"Patel N, Kipfmueller F (2017) Cardiac dysfunction in congenital diaphragmatic hernia: pathophysiology, clinical assessment, and management. Semin Pediatr Surg 26:154\u2013158","journal-title":"Semin Pediatr Surg"},{"key":"4229_CR14","doi-asserted-by":"publisher","first-page":"808","DOI":"10.1213\/ANE.0000000000004324","volume":"131","author":"D Chatterjee","year":"2020","unstructured":"Chatterjee D, Ing RJ, Gien J (2020) Update on congenital diaphragmatic hernia. Anesth Analg 131:808","journal-title":"Anesth Analg"},{"key":"4229_CR15","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1016\/j.jpeds.2019.10.034","volume":"217","author":"PT Levy","year":"2020","unstructured":"Levy PT et al (2020) Risk assessment and monitoring of chronic pulmonary hypertension in premature infants. J Pediatr 217:199-209.e4","journal-title":"J Pediatr"},{"key":"4229_CR16","doi-asserted-by":"publisher","first-page":"1063","DOI":"10.1016\/j.radcr.2019.06.003","volume":"14","author":"Y Mandato","year":"2019","unstructured":"Mandato Y, Pecoraro C, Gagliardi G, Tecame M (2019) Azygos and hemiazygos continuation: an occasional finding in emergency department. Radiol Case Rep 14:1063\u20131068","journal-title":"Radiol Case Rep"},{"key":"4229_CR17","doi-asserted-by":"publisher","first-page":"1659","DOI":"10.2214\/ajr.172.6.10350311","volume":"172","author":"TE Geley","year":"1999","unstructured":"Geley TE, Unsinn KM, Auckenthaler TM, Fink CJ, Gassner I (1999) Azygos continuation of the inferior vena cava: sonographic demonstration of the renal artery ventral to the azygos vein as a clue to diagnosis. AJR Am J Roentgenol 172:1659\u20131662","journal-title":"AJR Am J Roentgenol"},{"key":"4229_CR18","doi-asserted-by":"publisher","first-page":"482","DOI":"10.21037\/cdt.2016.11.18","volume":"6","author":"A Ghandour","year":"2016","unstructured":"Ghandour A, Partovi S, Karuppasamy K, Rajiah P (2016) Congenital anomalies of the IVC-embryological perspective and clinical relevance. Cardiovasc Diagn Ther 6:482\u2013492","journal-title":"Cardiovasc Diagn Ther"},{"key":"4229_CR19","doi-asserted-by":"publisher","first-page":"304","DOI":"10.1148\/84.2.304","volume":"84","author":"RW Petersen","year":"1965","unstructured":"Petersen RW (1965) Intrahepatic interruption of the inferior vena cava with azygos continuation (persistent right cardinal vein). Radiology 84:304\u2013307","journal-title":"Radiology"},{"key":"4229_CR20","doi-asserted-by":"publisher","first-page":"438","DOI":"10.2214\/ajr.180.2.1800438","volume":"180","author":"RL Keller","year":"2003","unstructured":"Keller RL, Aaroz PA, Hawgood S, Higgins CB (2003) MR imaging of hepatic pulmonary fusion in neonates. AJR Am J Roentgenol 180:438\u2013440","journal-title":"AJR Am J Roentgenol"},{"key":"4229_CR21","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1055\/s-0042-1749213","volume":"32","author":"GMD Rocha","year":"2022","unstructured":"Rocha GMD (2022) Congenital hepatopulmonary fusion. Eur J Pediatr Surg 32:477\u2013496","journal-title":"Eur J Pediatr Surg"}],"container-title":["Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00246-026-04229-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00246-026-04229-9","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00246-026-04229-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T15:25:11Z","timestamp":1776698711000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00246-026-04229-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,20]]},"references-count":21,"alternative-id":["4229"],"URL":"https:\/\/doi.org\/10.1007\/s00246-026-04229-9","relation":{},"ISSN":["0172-0643","1432-1971"],"issn-type":[{"value":"0172-0643","type":"print"},{"value":"1432-1971","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,20]]},"assertion":[{"value":"7 October 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 April 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}