Skip to main content
SpringerLink
Log in

Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain

  • Research
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Background

Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter stent placement in a residual ductus arteriosus (PDA) is one potentially effective option in critically ill infants and young children with PAH. We sought to assess recovery of RV function by echocardiographic strain in infants and young children following PDA stenting for acute PAH.

Methods

Retrospective review of patients < 2 years old who underwent PDA stenting for acute PAH. Clinical data were abstracted from the electronic medical record. RV strain (both total and free wall components) was assessed from echocardiographic images at baseline and 3, 6, and 12 months post-intervention, as well as at last echocardiogram.

Results

Nine patients underwent attempted ductal stenting for PAH. The median age at intervention was 38 days and median weight 3.7 kg. One-third (3of 9) of patients had PAH associated with a congenital diaphragmatic hernia. PDA stents were successfully deployed in eight patients. Mean RV total strain was − 14.9 ± 5.6% at baseline and improved to − 23.8 ± 2.2% at 6 months post-procedure (p < 0.001). Mean free wall RV strain was − 19.5 ± 5.4% at baseline and improved to − 27.7 ± 4.1% at 6 months (p = 0.002). Five patients survived to discharge, and four patients survived 1 year post-discharge.

Conclusion

PDA stenting for severe, acute PAH can improve RV function as assessed by strain echocardiography. The quantitative improvement is more prominent in the first 6 months post-procedure and stabilizes thereafter.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Li L, Jick S, Breitenstein S, Hernandez G, Michel A, Vizcaya D (2017) Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population. Pulm Circ 7:126–136

    Article  PubMed  PubMed Central  Google Scholar 

  2. Moledina S, Hislop AA, Foster H, Schulze-Neick I, Haworth SG (2010) Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart 96:1401–1406

    Article  CAS  PubMed  Google Scholar 

  3. van Loon RL, Roofthooft MT, Hillege HL, ten Harkel AD, van Osch-Gevers M, Delhaas T, Kapusta L, Strengers JL, Rammeloo L, Clur SA, Mulder BJ, Berger RM (2011) Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation 124:1755–1764

    Article  PubMed  Google Scholar 

  4. Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thebaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL, American Heart Association Council on Cardiopulmonary CCP, Resuscitation, Council on Clinical C, Council on Cardiovascular Disease in the Y, Council on Cardiovascular R, Intervention, Council on Cardiovascular S, Anesthesia, the American Thoracic S (2015) Pediatric Pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation 132:2037–2099

    Article  PubMed  Google Scholar 

  5. Hansmann G, Koestenberger M, Alastalo TP, Apitz C, Austin ED, Bonnet D, Budts W, D’Alto M, Gatzoulis MA, Hasan BS, Kozlik-Feldmann R, Kumar RK, Lammers AE, Latus H, Michel-Behnke I, Miera O, Morrell NW, Pieles G, Quandt D, Sallmon H, Schranz D, Tran-Lundmark K, Tulloh RMR, Warnecke G, Wahlander H, Weber SC, Zartner P (2019) 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: the European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant 38:879–901

    Article  PubMed  Google Scholar 

  6. Esch JJ, Shah PB, Cockrill BA, Farber HW, Landzberg MJ, Mehra MR, Mullen MP, Opotowsky AR, Waxman AB, Lock JE, Marshall AC (2013) Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant 32:381–387

    Article  PubMed  Google Scholar 

  7. Rosenzweig EB, Ankola A, Krishnan U, Middlesworth W, Bacha E, Bacchetta M (2021) A novel unidirectional-valved shunt approach for end-stage pulmonary arterial hypertension: early experience in adolescents and adults. J Thorac Cardiovasc Surg 161:1438–1446

    Article  PubMed  Google Scholar 

  8. Kerstein D, Levy PS, Hsu DT, Hordof AJ, Gersony WM, Barst RJ (1995) Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 91:2028–2035

    Article  CAS  PubMed  Google Scholar 

  9. Boudjemline Y, Patel M, Malekzadeh-Milani S, Szezepanski I, Levy M, Bonnet D (2013) Patent ductus arteriosus stenting (transcatheter Potts shunt) for palliation of suprasystemic pulmonary arterial hypertension: a case series. Circ Cardiovasc Interv 6:e18–e20

    Article  PubMed  Google Scholar 

  10. Latus H, Apitz C, Moysich A, Kerst G, Jux C, Bauer J, Schranz D (2014) Creation of a functional Potts shunt by stenting the persistent arterial duct in newborns and infants with suprasystemic pulmonary hypertension of various etiologies. J Heart Lung Transplant 33:542–546

    Article  PubMed  Google Scholar 

  11. D’Alto M, Santoro G, Palladino MT, Parisi F, Russo MG (2015) Patent ductus arteriosus stenting for palliation of severe pulmonary arterial hypertension in childhood. Cardiol Young 25:350–354

    Article  PubMed  Google Scholar 

  12. Schubert S, Peters B, Berger F (2017) Interventional re-opening of a PDA for reverse Potts shunt circulation after ADO I implantation in a child. Catheter Cardiovasc Interv 89:E133–E136

    Article  PubMed  Google Scholar 

  13. Levy PT, Sanchez Mejia AA, Machefsky A, Fowler S, Holland MR, Singh GK (2014) Normal ranges of right ventricular systolic and diastolic strain measures in children: a systematic review and meta-analysis. J Am Soc Echocardiogr 27:549–560

    Article  PubMed  PubMed Central  Google Scholar 

  14. Romanowicz J, Ferraro AM, Harrington JK, Sleeper LA, Adar A, Levy PT, Powell AJ, Harrild DM (2023) Pediatric normal values and Z-score equations for left and right ventricular strain by two-dimensional speckle-tracking echocardiography derived from a large cohort of healthy children. J Am Soc Echocardiogr 36(3):310–323

    Article  PubMed  Google Scholar 

  15. Jone PN, Duchateau N, Pan Z, Ivy DD, Moceri P (2021) Right ventricular area strain from 3-dimensional echocardiography: mechanistic insight of right ventricular dysfunction in pediatric pulmonary hypertension. J Heart Lung Transplant 40:138–148

    Article  PubMed  Google Scholar 

  16. Vitarelli A, Mangieri E, Terzano C, Gaudio C, Salsano F, Rosato E, Capotosto L, D’Orazio S, Azzano A, Truscelli G, Cocco N, Ashurov R (2015) Three-dimensional echocardiography and 2D–3D speckle-tracking imaging in chronic pulmonary hypertension: diagnostic accuracy in detecting hemodynamic signs of right ventricular (RV) failure. J Am Heart Assoc 4:e001584

    Article  PubMed  PubMed Central  Google Scholar 

  17. Fine NM, Chen L, Bastiansen PM, Frantz RP, Pellikka PA, Oh JK, Kane GC (2013) Outcome prediction by quantitative right ventricular function assessment in 575 subjects evaluated for pulmonary hypertension. Circ Cardiovasc Imaging 6:711–721

    Article  PubMed  Google Scholar 

  18. Driessen MMP, Meijboom FJ, Hui W, Dragulescu A, Mertens L, Friedberg MK (2017) Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: insights into mechanics of right ventricular dysfunction. Echocardiography 34:888–897

    Article  PubMed  Google Scholar 

  19. Li Y, Xie M, Wang X, Lu Q, Fu M (2013) Right ventricular regional and global systolic function is diminished in patients with pulmonary arterial hypertension: a 2-dimensional ultrasound speckle tracking echocardiography study. Int J Cardiovasc Imaging 29:545–551

    Article  PubMed  Google Scholar 

  20. Hardegree EL, Sachdev A, Villarraga HR, Frantz RP, McGoon MD, Kushwaha SS, Hsiao JF, McCully RB, Oh JK, Pellikka PA, Kane GC (2013) Role of serial quantitative assessment of right ventricular function by strain in pulmonary arterial hypertension. Am J Cardiol 111:143–148

    Article  PubMed  Google Scholar 

  21. Grady RM, Canter MW, Wan F, Shmalts AA, Coleman RD, Beghetti M, Berger RMF, Del CerroMarin MJ, Fletcher SE, Hirsch R, Humpl T, Ivy DD, Kirkpatrick EC, Kulik TJ, Levy M, Moledina S, Yung D, Eghtesady P, Bonnet D (2021) Pulmonary-to-systemic arterial shunt to treat children with severe pulmonary hypertension. J Am Coll Cardiol 78:468–477

    Article  PubMed  PubMed Central  Google Scholar 

  22. Boudjemline Y, Sizarov A, Malekzadeh-Milani S, Mirabile C, Lenoir M, Khraiche D, Levy M, Bonnet D (2017) Safety and feasibility of the transcatheter approach to create a reverse Potts shunt in children with idiopathic pulmonary arterial hypertension. Can J Cardiol 33:1188–1196

    Article  PubMed  Google Scholar 

  23. 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

    Article  PubMed  Google Scholar 

  24. Lawrence KM, Berger K, Herkert L, Franciscovich C, O’Dea CLH, Waqar LN, Partridge E, Hanna BD, Peranteau WH, Avitabile CM, Hopper RK, Rintoul NE, Hedrick HL (2019) Use of prostaglandin E1 to treat pulmonary hypertension in congenital diaphragmatic hernia. J Pediatr Surg 54:55–59

    Article  PubMed  Google Scholar 

  25. Le Duc K, Mur S, Sharma D, Aubry E, Recher M, Rakza T, Storme L, Center for Rare D (2020) Prostaglandin E1 in infants with congenital diaphragmatic hernia (CDH) and life-threatening pulmonary hypertension. J Pediatr Surg 55:1872–1878

    Article  PubMed  Google Scholar 

  26. Shiyanagi S, Okazaki T, Shoji H, Shimizu T, Tanaka T, Takeda S, Kawashima K, Lane GJ, Yamataka A (2008) Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone. Pediatr Surg Int 24:1101–1104

    Article  PubMed  Google Scholar 

  27. Koopman LP, Slorach C, Manlhiot C, McCrindle BW, Jaeggi ET, Mertens L, Friedberg MK (2011) Assessment of myocardial deformation in children using Digital Imaging and Communications in Medicine (DICOM) data and vendor independent speckle tracking software. J Am Soc Echocardiogr 24:37–44

    Article  PubMed  Google Scholar 

  28. Risum N, Ali S, Olsen NT, Jons C, Khouri MG, Lauridsen TK, Samad Z, Velazquez EJ, Sogaard P, Kisslo J (2012) Variability of global left ventricular deformation analysis using vendor dependent and independent two-dimensional speckle-tracking software in adults. J Am Soc Echocardiogr 25:1195–1203

    Article  PubMed  Google Scholar 

  29. Marwick TH (2012) Will standardization make strain a standard measurement? J Am Soc Echocardiogr 25:1204–1206

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

As mentioned in the discussion of this manuscript, 4 of these patients were included in Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension by Grady et al. [21]; a report from the International Potts Registry.

Funding

No funding was received to assist with the preparation of this manuscript.

Author information

Authors and Affiliations

  1. Department of Cardiology, Boston Children’s Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA

    Jason S. Kerstein, Eleonore Valencia, Shane Collins, Alessandra M. Ferraro, David M. Harrild, Kimberlee Gauvreau, Ryan Callahan & Mary P. Mullen

  2. Harvard Medical School, Boston, MA, USA

    Jason S. Kerstein, Eleonore Valencia, David M. Harrild, Kimberlee Gauvreau, Ryan Callahan & Mary P. Mullen

  3. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Ryan Callahan

Authors
  1. Jason S. Kerstein
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Eleonore Valencia
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Shane Collins
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alessandra M. Ferraro
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. David M. Harrild
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Kimberlee Gauvreau
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ryan Callahan
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mary P. Mullen
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by JSK, SC, AMF, and KG. The first draft of the manuscript was written by JSK and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Jason S. Kerstein.

Ethics declarations

Competing interests

The authors have no relevant financial or non-financial interests to disclose.

Ethical Approval

This study was approved by the Boston Children’s Hospital IRB and a waiver of consent was obtained per the retrospective nature of the study (IRB-P00042655).

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 16 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kerstein, J.S., Valencia, E., Collins, S. et al. Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain. Pediatr Cardiol (2023). https://doi.org/10.1007/s00246-023-03233-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00246-023-03233-7

Keywords

Search

Navigation