Quantitative Measures of Right Ventricular Size and Function by Echocardiogram Correlate with Cardiac Catheterization Hemodynamics in Congenital Diaphragmatic Hernia

Overview

Journal J Pediatr

Specialty Pediatrics

Date 2023 Jun 17

PMID 37329980

Authors

Catherine M Avitabile

Sabrina Flohr

Leny Mathew

Yan Wang

Devon Ash

David B Frank

Jennifer E Tingo

Natalie E Rintoul

Holly L Hedrick

Affiliations

Soon will be listed here.

Abstract

Objective: To evaluate associations between cardiac catheterization (cath) hemodynamics, quantitative measures of right ventricular (RV) function by echocardiogram, and survival in patients with congenital diaphragmatic hernia (CDH).

Study Design: This single-center retrospective cohort study enrolled patients with CDH who underwent index cath from 2003 to 2022. Tricuspid annular plane systolic excursion z score, RV fractional area change, RV free wall and global longitudinal strain, left ventricular (LV) eccentricity index, RV/LV ratio, and pulmonary artery acceleration time were measured from preprocedure echocardiograms. Associations between hemodynamic values, echocardiographic measures, and survival were evaluated by Spearman correlation and Wilcoxon rank sum test, respectively.

Results: Fifty-three patients (68% left-sided, 74% liver herniation, 57% extracorporeal membrane oxygenation, 93% survival) underwent cath (39 during index hospitalization, 14 later) including device closure of a patent ductus arteriosus in 5. Most patients (n = 31, 58%) were on pulmonary hypertension treatment at cath, most commonly sildenafil (n = 24, 45%) and/or intravenous treprostinil (n = 16, 30%). Overall, hemodynamics were consistent with precapillary pulmonary hypertension. Pulmonary capillary wedge pressure was >15 mm Hg in 2 patients (4%). Lower fractional area change and worse ventricular strain were associated with higher pulmonary artery pressure while higher LV eccentricity index and higher RV/LV ratio were associated with both higher pulmonary artery pressure and higher pulmonary vascular resistance. Hemodynamics did not differ based on survival status.

Conclusions: Worse RV dilation and dysfunction by echocardiogram correlate with higher pulmonary artery pressure and pulmonary vascular resistance on cath in this CDH cohort. These measures may represent novel, noninvasive clinical trial targets in this population.

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References

Levy P, Patel M, Groh G, Choudhry S, Murphy J, Holland M . Pulmonary Artery Acceleration Time Provides a Reliable Estimate of Invasive Pulmonary Hemodynamics in Children. J Am Soc Echocardiogr. 2016; 29(11):1056-1065. PMC: 5408579. DOI: 10.1016/j.echo.2016.08.013. View

Ploegstra M, Roofthooft M, Douwes J, Bartelds B, Elzenga N, van de Weerd D . Echocardiography in pediatric pulmonary arterial hypertension: early study on assessing disease severity and predicting outcome. Circ Cardiovasc Imaging. 2015; 8(1). DOI: 10.1161/CIRCIMAGING.113.000878. View

Sitbon O, Humbert M, Jais X, Ioos V, Hamid A, Provencher S . Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005; 111(23):3105-11. DOI: 10.1161/CIRCULATIONAHA.104.488486. View

Galie N, McLaughlin V, Rubin L, Simonneau G . An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J. 2018; 53(1). PMC: 6351332. DOI: 10.1183/13993003.02148-2018. View

Levy P, Sanchez Mejia A, Machefsky A, Fowler S, Holland M, Singh G . Normal ranges of right ventricular systolic and diastolic strain measures in children: a systematic review and meta-analysis. J Am Soc Echocardiogr. 2014; 27(5):549-60, e3. PMC: 4031687. DOI: 10.1016/j.echo.2014.01.015. View

Koestenberger M, Nagel B, Ravekes W, Urlesberger B, Raith W, Avian A . Systolic right ventricular function in preterm and term neonates: reference values of the tricuspid annular plane systolic excursion (TAPSE) in 258 patients and calculation of Z-score values. Neonatology. 2011; 100(1):85-92. DOI: 10.1159/000322006. View

Rottier R, Tibboel D . Fetal lung and diaphragm development in congenital diaphragmatic hernia. Semin Perinatol. 2005; 29(2):86-93. DOI: 10.1053/j.semperi.2005.04.004. View

OByrne M, Kennedy K, Kanter J, Berger J, Glatz A . Risk Factors for Major Early Adverse Events Related to Cardiac Catheterization in Children and Young Adults With Pulmonary Hypertension: An Analysis of Data From the IMPACT (Improving Adult and Congenital Treatment) Registry. J Am Heart Assoc. 2018; 7(5). PMC: 5866335. DOI: 10.1161/JAHA.117.008142. View

Avitabile C, Zhang X, Ampah S, Wang Y, Ash D, Nilan K . Factors associated with discontinuation of pulmonary vasodilator therapy in children with bronchopulmonary dysplasia-associated pulmonary hypertension. J Perinatol. 2022; 42(9):1246-1254. DOI: 10.1038/s41372-022-01421-6. View

10.

Koestenberger M, Ravekes W, Everett A, Stueger H, Heinzl B, Gamillscheg A . Right ventricular function in infants, children and adolescents: reference values of the tricuspid annular plane systolic excursion (TAPSE) in 640 healthy patients and calculation of z score values. J Am Soc Echocardiogr. 2009; 22(6):715-9. DOI: 10.1016/j.echo.2009.03.026. View

11.

Maia P, Gien J, Kinsella J, Zablah J, Morgan G, Ivy D . Hemodynamic Characterization of Neonates With Congenital Diaphragmatic Hernia-Associated Pulmonary Hypertension by Cardiac Catheterization. J Pediatr. 2022; 255:230-235.e2. DOI: 10.1016/j.jpeds.2022.11.028. View

12.

Himebauch A, Yehya N, Wang Y, McGowan F, Mercer-Rosa L . New or Persistent Right Ventricular Systolic Dysfunction Is Associated With Worse Outcomes in Pediatric Acute Respiratory Distress Syndrome. Pediatr Crit Care Med. 2019; 21(2):e121-e128. PMC: 11215761. DOI: 10.1097/PCC.0000000000002206. View

13.

Lusk L, Wai K, Moon-Grady A, Steurer M, Keller R . Persistence of pulmonary hypertension by echocardiography predicts short-term outcomes in congenital diaphragmatic hernia. J Pediatr. 2014; 166(2):251-6.e1. PMC: 4308510. DOI: 10.1016/j.jpeds.2014.10.024. View

14.

Koestenberger M, Friedberg M, Nestaas E, Michel-Behnke I, Hansmann G . Transthoracic echocardiography in the evaluation of pediatric pulmonary hypertension and ventricular dysfunction. Pulm Circ. 2016; 6(1):15-29. PMC: 4860554. DOI: 10.1086/685051. View

15.

DiLorenzo M, Elci O, Wang Y, Banerjee A, Sato T, Ky B . Longitudinal Changes in Right Ventricular Function in Tetralogy of Fallot in the Initial Years after Surgical Repair. J Am Soc Echocardiogr. 2018; 31(7):816-821. PMC: 6035101. DOI: 10.1016/j.echo.2018.02.013. View

16.

Himebauch A, Yehya N, Wang Y, Conlon T, Kilbaugh T, McGowan F . Early Right Ventricular Systolic Dysfunction and Pulmonary Hypertension Are Associated With Worse Outcomes in Pediatric Acute Respiratory Distress Syndrome. Crit Care Med. 2018; 46(11):e1055-e1062. PMC: 6185756. DOI: 10.1097/CCM.0000000000003358. View

17.

Patel N, Massolo A, Kipfmueller F . Congenital diaphragmatic hernia-associated cardiac dysfunction. Semin Perinatol. 2019; 44(1):151168. DOI: 10.1053/j.semperi.2019.07.007. View

18.

Chinoy M . Pulmonary hypoplasia and congenital diaphragmatic hernia: advances in the pathogenetics and regulation of lung development. J Surg Res. 2002; 106(1):209-23. DOI: 10.1006/jsre.2002.6390. View

19.

Chao P, Huang C, Liu C, Chung M, Chen C, Chen F . Congenital diaphragmatic hernia in the neonatal period: review of 21 years' experience. Pediatr Neonatol. 2010; 51(2):97-102. DOI: 10.1016/S1875-9572(10)60018-6. View

20.

Kinsella J, Steinhorn R, Mullen M, Hopper R, Keller R, Ivy D . The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension. J Pediatr. 2018; 197:17-22. DOI: 10.1016/j.jpeds.2018.02.040. View