Regional Ventricular and Global Atrial Strain Characteristics in Patients with Hypoplastic Left Heart Syndrome and Right Ventricular Dysfunction: An Echocardiography, Cardiac MRI, and Invasive Hemodynamics Study

Newburger JW, Sleeper LA, Gaynor JW, Hollenbeck-Pringle D, Frommelt PC, Li JS, Mahle WT, Williams IA, Atz AM, Burns KM, Chen S, Cnota J, Dunbar-Masterson C, Ghanayem NS, Goldberg CS, Jacobs JP, Lewis AB, Mital S, Pizarro C, Eckhauser A, Stark P, Ohye RG, Pediatric Heart Network Investigators (2018) Transplant-Free Survival and Interventions at 6 Years in the SVR Trial. Circulation 137(21):2246–2253. https://doi.org/10.1161/CIRCULATIONAHA.117.029375Epub 2018 Feb 1

Article  PubMed  PubMed Central  Google Scholar 

Fruitman DS (2000) Hypoplastic left heart syndrome: Prognosis and management options. Paediatr Child Health 5(4):219–225. https://doi.org/10.1093/pch/5.4.219

Article  CAS  PubMed  PubMed Central  Google Scholar 

Selenius S, Ilvesvuo J, Ruotsalainen H, Mattila I, Pätilä T, Helle E, Ojala T (2023) Risk factors for mortality in patients with hypoplastic left heart syndrome after the Norwood procedure. Interdiscip Cardiovasc Thorac Surg 37(2):ivad127. https://doi.org/10.1093/icvts/ivad127

Article  PubMed  PubMed Central  Google Scholar 

Lin LQ, Conway J, Alvarez S, Goot B, Serrano-Lomelin J, Colen T, Tham EB, Kutty S, Li L, Khoo NS (2018) Reduced right ventricular fractional area change, strain, and strain rate before bidirectional cavopulmonary anastomosis is associated with medium-term mortality for children with hypoplastic left heart syndrome. J Am Soc Echocardiogr 31(7):831–842. https://doi.org/10.1016/j.echo.2018.02.001Epub 2018 Apr 12

Article  PubMed  Google Scholar 

Ruotsalainen HK, Bellsham-Revell HR, Bell AJ, Pihkala JI, Ojala TH, Simpson JM (2017) Right ventricular systolic function in hypoplastic left heart syndrome: a comparison of manual and automated software to measure fractional area change. Echocardiography 34(4):587–593. https://doi.org/10.1111/echo.13470Epub 2017 Feb 13

Article  PubMed  Google Scholar 

Kanngiesser LM, Freitag-Wolf S, Boroni Grazioli S, Gabbert DD, Hansen JH, Uebing AS, Voges I (2022) Serial Assessment of right ventricular deformation in patients with hypoplastic left heart syndrome: a cardiovascular magnetic resonance feature tracking study. J Am Heart Assoc 11(9):e025332. https://doi.org/10.1161/JAHA.122.025332Epub 2022 Apr 27

Article  CAS  PubMed  PubMed Central  Google Scholar 

Peck D, Alsaied T, Pradhan S, Hill G (2021) Atrial reservoir strain is associated with decreased cardiac index and adverse outcomes post fontan operation. Pediatr Cardiol 42(2):307–314. https://doi.org/10.1007/s00246-020-02484-yEpub 2020 Oct 13

Article  PubMed  Google Scholar 

Stines JR, Hershenson JA, Hayes J, Stefaniak CA, Texter KM, Zaidi AN, Cua CL (2011) Echocardiographic assessment of atrial properties in single ventricles vs. normal controls. Congenit Heart Dis 6(3):247–252. https://doi.org/10.1111/j.1747-0803.2011.00512.xEpub 2011 Apr 19

Article  PubMed  Google Scholar 

Matsuda Y, Toma Y, Ogawa H, Matsuzaki M, Katayama K, Fujii T, Yoshino F, Moritani K, Kumada T, Kusukawa R (1983) Importance of left atrial function in patients with myocardial infarction. Circulation 67(3):566–571. https://doi.org/10.1161/01.cir.67.3.566

Article  CAS  PubMed  Google Scholar 

Hasselberg NE, Kagiyama N, Soyama Y, Sugahara M, Goda A, Ryo-Koriyama K, Batel O, Chakinala M, Simon MA, Gorcsan J III (2021) The prognostic value of right atrial strain imaging in patients with precapillary pulmonary hypertension. J Am Soc Echocardiogr 34(8):851–861e1. https://doi.org/10.1016/j.echo.2021.03.007Epub 2021 Mar 24

Article  PubMed  Google Scholar 

Alenezi F, Mandawat A, Il’Giovine ZJ, Shaw LK, Siddiqui I, Tapson VF, Arges K, Rivera D, Romano MMD, Velazquez EJ, Douglas PS, Samad Z, Rajagopal S (2018) Clinical utility and prognostic value of right atrial function in pulmonary hypertension. Circ Cardiovasc Imaging 11(11):e006984. https://doi.org/10.1161/CIRCIMAGING.117.006984

Article  PubMed  PubMed Central  Google Scholar 

Meng X, Li Y, Li H, Wang Y, Zhu W, Lu X (2018) Right atrial function in patients with pulmonary hypertension: a study with two-dimensional speckle-tracking echocardiography. Int J Cardiol 255:200–205. https://doi.org/10.1016/j.ijcard.2017.11.093

Article  PubMed  Google Scholar 

Colquitt JL, Loar RW, Morris SA, Feagin DK, Sami S, Pignatelli RH (2019) Serial strain analysis identifies hypoplastic left heart syndrome infants at risk for cardiac morbidity and mortality: a pilot study. J Am Soc Echocardiogr 32(5):643–650. https://doi.org/10.1016/j.echo.2019.01.006Epub 2019 Feb 22

Article  PubMed  Google Scholar 

Wang AP, Polsen C, Penk J, Husain N, Hauck A, Jone PN (2024) Common atrial reservoir strain during the interstage period is a predictor of poor outcomes prior to Fontan completion in hypoplastic left heart syndrome. Echocardiography 41(8):e15910. https://doi.org/10.1111/echo.15910

Article  PubMed  Google Scholar 

Muraru D, Haugaa K, Donal E, Stankovic I, Voigt JU, Petersen SE, Popescu BA, Marwick T (2022) Right ventricular longitudinal strain in the clinical routine: a state-of-the-art review. Eur Heart J Cardiovasc Imaging 23(7):898–912. https://doi.org/10.1093/ehjci/jeac022

Article  PubMed  Google Scholar 

Szabó G, Soós P, Bährle S, Radovits T, Weigang E, Kékesi V, Merkely B, Hagl S (2006) Adaptation of the right ventricle to an increased afterload in the chronically volume overloaded heart. Ann Thorac Surg 82(3):989–995. https://doi.org/10.1016/j.athoracsur.2006.04.036

Article  PubMed  Google Scholar 

van de Veerdonk MC, Marcus JT, Westerhof N, de Man FS, Boonstra A, Heymans MW, Bogaard HJ, Vonk Noordegraaf A (2015) Signs of right ventricular deterioration in clinically stable patients with pulmonary arterial hypertension. Chest 147(4):1063–1071. https://doi.org/10.1378/chest.14-0701

Article  PubMed  Google Scholar 

Goh ZM, Balasubramanian N, Alabed S, Dwivedi K, Shahin Y, Rothman AMK, Garg P, Lawrie A, Capener D, Thompson AAR, Alandejani F, Wild JM, Johns CS, Lewis RA, Gosling R, Sharkey M, Condliffe R, Kiely DG, Swift AJ (2022) Right ventricular remodelling in pulmonary arterial hypertension predicts treatment response. Heart 108(17):1392–1400. https://doi.org/10.1136/heartjnl-2021-320733

Article  PubMed  PubMed Central  Google Scholar 

van de Veerdonk MC, Huis In T, Veld AE, Marcus JT, Westerhof N, Heymans MW, Bogaard HJ, Vonk-Noordegraaf A (2017) Upfront combination therapy reduces right ventricular volumes in pulmonary arterial hypertension. Eur Respir J 49(6):1700007. https://doi.org/10.1183/13993003.00007-2017

Article  CAS  PubMed  Google Scholar 

Vonk Noordegraaf A, Chin KM, Haddad F, Hassoun PM, Hemnes AR, Hopkins SR, Kawut SM, Langleben D, Lumens J, Naeije R (2019) Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update. Eur Respir J 53(1):180–1900. https://doi.org/10.1183/13993003.01900-2018. Erratum in: Eur Respir J. 2025;65(6):2050414. doi: 10.1183/13993003.50414-2020

Article  Google Scholar 

Rösner A, Bharucha T, James A, Mertens L, Friedberg MK (2019) Impact of right ventricular geometry and left ventricular hypertrophy on right ventricular mechanics and clinical outcomes in hypoplastic left heart syndrome. J Am Soc Echocardiogr 32(10):1350–1358 Epub 2019 Jul 24

Article  PubMed  Google Scholar 

Loke YH, O’Hara R, Contento J, Marella N, Kollar S, Vegulla R, Gaviria S, Mehta R, Balaras E, Fetch A, d’Udekem Y, Alsaied T, Olivieri L, Truong U, Anwar S, Rathod RH, Capuano F, FORCE Investigators (2025) Shape Variations in RV 3D geometry are associated with adverse outcomes in hypoplastic left heart syndrome patients: a fontan outcomes registry using CMR examination (FORCE) Study. Circ Cardiovasc Imaging. https://doi.org/10.1161/CIRCIMAGING.125.018455Epub ahead of print

Article  PubMed  Google Scholar 

Wisler J, Khoury PR, Kimball TR (2008) The effect of left ventricular size on right ventricular hemodynamics in pediatric survivors with hypoplastic left heart syndrome. J Am Soc Echocardiogr 21(5):464–469. https://doi.org/10.1016/j.echo.2007.09.003Epub 2007 Oct 25

Article  PubMed  Google Scholar 

Schlangen J, Fischer G, Steendijk P, Petko C, Scheewe J, Hart C, Hansen JH, Ahrend F, Rickers C, Kramer HH, Uebing A (2013) Does left ventricular size impact on intrinsic right ventricular function in hypoplastic left heart syndrome? Int J Cardiol 167(4):1305–1310. https://doi.org/10.1016/j.ijcard.2012.03.183Epub 2012 Apr 23

Article  PubMed  Google Scholar 

Kovács A, Lakatos B, Tokodi M, Merkely B (2019) Right ventricular mechanical pattern in health and disease: beyond longitudinal shortening. Heart Fail Rev 24(4):511–520. https://doi.org/10.1007/s10741-019-09778-1

Article  PubMed  PubMed Central  Google Scholar 

Haddad F, Hunt SA, Rosenthal DN, Murphy DJ (2008) Right ventricular function in cardiovascular disease, part I: anatomy, physiology, aging, and functional assessment of the right ventricle. Circulation 117(11):1436–1448. https://doi.org/10.1161/CIRCULATIONAHA.107.653576

Article  PubMed  Google Scholar 

Pettersen E, Helle-Valle T, Edvardsen T, Lindberg H, Smith HJ, Smevik B, Smiseth OA, Andersen K (2007) Contraction pattern of the systemic right ventricle shift from longitudinal to circumferential shortening and absent global ventricular torsion. J Am Coll Cardiol 49(25):2450–2456. https://doi.org/10.1016/j.jacc.2007.02.062Epub 2007 Jun 11

Article  PubMed  Google Scholar 

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