- CLINICAL VIGNETTE
Catheter-directed therapy for treatment of acute pulmonary embolism in a teenage patient: The role of close cooperation between the Pulmonary Embolism Response Team and pediatric physicians
Jakub Stępniewski1–3, Wojciech Magoń1–3, Marcin Waligóra1–3, Kamil Jonas1–3, Łukasz Wilczek2, Beata Zauska-Pitak4, Sebastian Góreczny4, Grzegorz Kopeć1, 2
1Pulmonary Circulation Centre, Institute od Cardiology, Jagiellonian University Medical College, Kraków, Poland
2Department of Cardiac and Vascular Diseases, John Paul II Hospital, Kraków, Poland
3Department of Medical Education, Jagiellonian University Medical College, Kraków, Poland
4Department of Pediatric Cardiology, University Children’s Hospital, Jagiellonian University Medical College, Kraków, Poland
Correspondence to:
Grzegorz Kopeć MD, PhD,
Department of Cardiac and Vascular Diseases,
John Paul II Hospital,
Prądnicka 80, 31–202 Kraków, Poland,
phone: +48 500 099 734,
fax: +48 12 614 33 32,
e-mail: grzegorzkrakow1@gmail.com
Copyright by the Author(s), 2023
DOI: 10.33963/v.kp.97954
Received: March 29, 2023
Accepted: October 24, 2023
Early publication date: November 15, 2023
Acute pulmonary embolism (PE) occurs relatively rarely in adolescence. According to the 2018 American Society of Hematology guidelines, pulmonary arteries (PA) reperfusion treatment should be considered in children when hemodynamic compromise is present despite anticoagulation [1]. Catheter-directed therapies (CDT) have emerged as valuable reperfusion modalities [2, 3]; however, evidence of their utility in the pediatric population is scarce. Pulmonary Embolism Response Teams (PERT) have been created to support decision-making in severe and complex PE scenarios [4, 5].
We report a case of a 16-year-old girl referred to our PERT from a pediatric cardiology department due to intermediate-high-risk PE. She had a history of combined (etonogestrel and ethinylestradiol) oral contraceptives and antipsychotic use. Her symptoms occurred suddenly with syncope, dyspnea, and chest discomfort. Once computed angiotomography showed bilateral, proximal PA emboli, intravenous unfractionated heparin was started and continued at our tertiary pediatric cardiology and intensive care unit. Despite anticoagulation with activated partial thromboplastin time maintained between 46–70 s, symptoms and signs of cardiorespiratory compromise persisted over 24 hours. Her systolic blood pressure was 95 mm Hg, and her heart rate (HR) was 120/min. She required an oxygen supply of 4 l/min to maintain arterial oxygen saturation (SatO2) over 90%. Echocardiography revealed persistent dilation and impairment of the right ventricle (RV). No signs of chronic pulmonary hypertension were present. Troponin levels rose from 0.02 to 0.064 ng/ml (reference range <0.014 ng/ml) and the N-terminal pro-B-type natriuretic peptide from 2224 to 4480 pg/ml (reference range <206 pg/ml).
As no significant improvement could be observed, the PERT considered her condition potentially life-threatening and decided to pursue CDT in an off-label fashion for the pediatric population. After receiving her and her parents’ informed consent, urgent percutaneous embolectomy with the Penumbra Lightning 12 system was performed, which evacuated a substantial thrombus and improved the mean PA pressure (mPAP) measured invasively from 34 to 32 mm Hg and the cardiac index (CI) from 1.48 to 1.58 l/min/m2. To optimize the effect of embolectomy, we decided to supplement it with bilateral low-dose-local-thrombolysis with a cumulative alteplase dose of 20 mg delivered over 10 hours. The rationale for such an approach stemmed from reported significant distress experienced by the patient (chest discomfort) during manipulations with the embolectomy catheter, which precluded complete thrombus removal and achievement of intended clinical and hemodynamic efficacy. This strategy resulted in a further mPAP reduction to 21 mm Hg, CI increase to 2.38 l/min/m2, improvement in symptoms and vital signs, and reduction in N-terminal pro-B-type natriuretic peptide levels to 1608 pg/ml.
Figure 1. A. Angio CT of a 16-year-old girl with sudden syncope, dyspnea, and chest discomfort confirming the diagnosis of pulmonary embolism with massive proximal clots in both PAs (arrows). B. Angio CT showing dilation of the RV and the RV-to-LV ratio of 1.7; no thickening of the RV wall is present. C. Clots evacuated from PAs with the PENUMBRA Lightning 12 system during catheter-directed embolectomy procedure based on the Pulmonary Embolism Response Team’s decision as no significant clinical improvement could be reached despite therapeutic anticoagulation, which led to a mPAP reduction from 34 to 32 mm Hg and CI improvement from 1.48 to 1.58 l/min/m2 as assessed by right heart catheterization. D. Fluoroscopy showing infusion catheters placed within remaining clots in both PAs for low-dose catheter-directed thrombolysis, with a total dose of 20 mg delivered during 10 hours and resulting in a further mPAP reduction to 21 mm Hg and CI increase to 2.38 l/min/m2. E. Angio CT presenting treatment results with a reduction of clot burden in both PAs (arrows) and improvement in the RV/LV ratio to 0.78 (F)
Abbreviations: Angio CT, computed angiotomography; CI, cardiac index; LA, left atrium; LV, left ventricle; mPAP, mean pulmonary artery pressure; PA, pulmonary artery; RA, right atrium; RV, right ventricle
The patient was ambulated the next day and transferred back to the pediatric cardiology clinic. Rivaroxaban was started on the 1st post-procedural day. Her subsequent hospital stay was uneventful, and she was discharged home without exercise limitation.
This case showed that CDT use in a teenage girl was safe and resulted in a rapid clinical improvement. It was, to the best of our knowledge, the first successful use of the PENUMBRA Lightning 12 system in a pediatric patient. Promoting partnerships between pediatric physicians and PERTs may bring benefit to PE management in this population.
Article information
Conflict of interest: None declared.
Funding: This article was supported by the science fund of the Saint John Paul II Hospital, Kraków, Poland (no. FN/17/2020 to JS)
Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, which allows downloading and sharing articles with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at kardiologiapolska@ptkardio.pl.
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