Hostname: page-component-586b7cd67f-rcrh6 Total loading time: 0 Render date: 2024-11-24T07:47:08.909Z Has data issue: false hasContentIssue false
  • English
  • Français

The effect of congenital diaphragmatic hernia on the development of left-sided heart structures

Published online by Cambridge University Press:  06 June 2019

Zachary J. Coffman ,
Eugene D. McGahren ,
Brooke D. Vergales ,
Christine H. Saunders  and
Jeffrey E. Vergales
Zachary J. Coffman
Affiliation:
Department of Pediatrics, Division of Cardiology, University of VirginiaChildren’s Hospital, P.O. Box 800386 Charlottesville, VA 22908, USA
Eugene D. McGahren
Affiliation:
Department of Surgery, Division of Pediatric Surgery, University of VirginiaChildren’s Hospital, P.O. Box 800709 Charlottesville, VA 22908-0709, USA
Brooke D. Vergales
Affiliation:
Department of Pediatrics, Division of Neonatology, University of VirginiaChildren’s Hospital, P.O. Box 800386 Charlottesville, VA 22908-0386, USA
Christine H. Saunders
Affiliation:
Department of Pediatrics, Division of Cardiology, University of VirginiaChildren’s Hospital, P.O. Box 800386 Charlottesville, VA 22908, USA
Jeffrey E. Vergales*
Affiliation:
Department of Pediatrics, Division of Cardiology, University of VirginiaChildren’s Hospital, P.O. Box 800386 Charlottesville, VA 22908, USA
*
*Author for correspondence: J. Vergales, Department of Pediatrics, Division of Cardiology, University of Virginia Children’s Hospital, P.O. Box 800386 Charlottesville, VA 22908, USA. Tel: 434-243-3697 Fax: 434-924-5656 E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Introduction:

Patients with congenital diaphragmatic hernias often have concomitant congenital heart disease (CHD), with small left-sided cardiac structures as a frequent finding. The goal of this study is to evaluate which left-sided heart structures are affected in neonates with congenital diaphragmatic hernias.

Methods:

Retrospective review of neonates between May 2007 and April 2015 with a diagnosis of a congenital diaphragmatic hernia was performed. Clinical and echocardiographic data were extracted from the electronic medical record and indexed to body surface area and compared to normative values. Univariable regression models assessed for associations between different variables and length of stay.

Results:

Data of 52 patients showed decreased mean z scores for the LVIDd (–3.16), LVIDs (–3.05), aortic annulus (–1.68), aortic sinuses (–2.11), transverse arch (–3.11), and sinotubular junction (–1.47) with preservation of the aorta at the diaphragm compared to age-matched normative data with similar body surface areas. Regression analysis showed a percent reduction in length of stay per 1 mm size increase for LVIDd (8%), aortic annulus (27%), aortic sinuses (18%), sinotubular junctions (20%), and transverse arches (25%).

Conclusions:

Patients with congenital diaphragmatic hernias have significantly smaller left-sided heart structures compared to age-matched normative data. Aortic preservation at the diaphragm provides evidence for a mass effect aetiology with increased right-to-left shunting at the fetal ductus resulting in decreased size. Additionally, length of stay appears to be prolonged with decreasing size of several of these structures. These data provide quantitative evidence of smaller left-sided heart structures in patients with congenital diaphragmatic hernias.

Keywords

Congenital diaphragmatic herniaCHDneonateleft-heart hypoplasia
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 6 , June 2019 , pp. 813 - 818
Check for updates
Copyright
© Cambridge University Press 2019 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Fauza, DO, Wilson, JM. Congenital diaphragmatic hernia and associated anomalies: their incidence, identification, and impact on prognosis. J Pediatr Surg 1994; 29: 1113–1117.CrossRefGoogle ScholarPubMed
Graziano, JN. Congenital Diaphragmatic Hernia Study G. Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 2005; 40: 1045–1049; discussion 1049–1050.CrossRefGoogle Scholar
Bianchi, S, Fesslova, V, Lista, G, Rustico, M, Torricelli, M, Pansini, L. Right congenital diaphragmatic hernia associated with a complex heart disease. J Cardiovasc Med (Hagerstown) 2006; 7: 641–644.CrossRefGoogle ScholarPubMed
Greenwood, RD, Rosenthal, A, Nadas, AS. Cardiovascular abnormalities associated with congenital diaphragmatic hernia. Pediatrics 1976; 57: 92–97.Google ScholarPubMed
Lin, AE, Pober, BR, Adatia, I. Congenital diaphragmatic hernia and associated cardiovascular malformations: type, frequency, and impact on management. Am J Med Genet C Semin Med Genet 2007; 145C: 201–216.CrossRefGoogle ScholarPubMed
Thebaud, B, Azancot, A, de Lagausie, P, et al. Congenital diaphragmatic hernia: antenatal prognostic factors: does cardiac ventricular disproportion in utero predict outcome and pulmonary hypoplasia? Intensive Care Med 1997; 23: 1062–1069.Google ScholarPubMed
Siebert, JR, Haas, JE, Beckwith, JB. Left ventricular hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg 1984; 19: 567–571.CrossRefGoogle ScholarPubMed
Okawada, M, Yanai, T, Yamataka, A, et al. Congenital diaphragamatic hernia associated with aortic coarctation: a case report. Cases J 2008; 1: 378.CrossRefGoogle ScholarPubMed
Migliazza, L, Otten, C, Xia, H, Rodriguez, JI, Diez-Pardo, JA, Tovar, JA. Cardiovascular malformations in congenital diaphragmatic hernia: human and experimental studies. J Pediatr Surg 1999; 34: 1352–1358.CrossRefGoogle ScholarPubMed
Lopez, L, Colan, SD, Frommelt, PC, et al. Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 2010; 23: 465–495; quiz 576-467.CrossRefGoogle ScholarPubMed
Pettersen, MD, Du, W, Skeens, ME, Humes, RA. Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr 2008; 21: 922–934.CrossRefGoogle Scholar
Weisberg, S. Applied Linear Regression. Wiley, Hoboken, NJ, 2014.Google Scholar
Byrne, FA, Keller, RL, Meadows, J, et al. Severe left diaphragmatic hernia limits size of fetal left heart more than does right diaphragmatic hernia. Ultrasound Obstet Gynecol 2015; 46: 688–694.CrossRefGoogle ScholarPubMed
Vogel, M, McElhinney, DB, Marcus, E, Morash, D, Jennings, RW, Tworetzky, W. Significance and outcome of left heart hypoplasia in fetal congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 2010; 35: 310–317.CrossRefGoogle ScholarPubMed
Harting, MT. Congenital diaphragmatic hernia-associated pulmonary hypertension. Semin Pediatr Surg 2017; 26: 147–153.CrossRefGoogle ScholarPubMed
Eghtesady, P, Skarsgard, ED, Smith, BM, Robbins, RC, Wexler, L, Rhine, WD. Congenital diaphragmatic hernia associated with aortic coarctation. J Pediatr Surg 1998; 33: 943–945.CrossRefGoogle ScholarPubMed
Dyamenahalli, U, Morris, M, Rycus, P, Bhutta, AT, Tweddell, JS, Prodhan, P. Short-term outcome of neonates with congenital heart disease and diaphragmatic hernia treated with extracorporeal membrane oxygenation. Ann Thorac Surg 2013; 95: 1373–1376.CrossRefGoogle ScholarPubMed
Gray, BW, Fifer, CG, Hirsch, JC, et al. Contemporary outcomes in infants with congenital heart disease and bochdalek diaphragmatic hernia. Ann Thorac Surg 2013; 95: 929–934.CrossRefGoogle ScholarPubMed