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Open Access 20-02-2025 | Heart Beat

Noonan syndrome and parasternal pericardiocentesis

Auteurs: Pitt O. Lim, May H. Ohn

Gepubliceerd in: Netherlands Heart Journal

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A 47-year-old woman with Noonan syndrome and pneumococcal pneumonia presented with cough, breathlessness, and hypotension caused by cardiac tamponade (Fig. 1a). Due to associated abdominal organomegaly, she underwent the recently described two-step parasternal pericardiocentesis approach [1]. The pericardial pressure was 17 mm Hg (normal < 4 mm Hg). About 1 L of serosanguinous fluid was withdrawn, resulting in immediate symptomatic relief and a doubling of the electrocardiographic R‑wave amplitude—an intuitive phenomenon not widely reported in the medical literature. The drain was removed the following day and a chest X‑ray showed pneumopericardium (Fig. 1b). Her CT scan confirmed the diagnosis and additionally revealed right basal lung consolidation and hepatosplenomegaly, which made subcostal pericardiocentesis challenging. Although there are no published cases of Noonan syndrome and percutaneous pericardiocentesis. The mechanism of pneumopericardium remains unclear but may result from pneumonic intrathoracic suction or potentially air leakage caused by the drain slipping out, exposing a side hole which is 7.5 cm from the tip [2]. Her follow-up echocardiogram however showed no tamponade physiology.

Acknowledgements

The authors thank the Department of Cardiology as led by Drs. Rajan Sharma and Manav Sohal, cardiac catheter laboratory managers; Mary Keal and Menene Endaya (nursing), Dinesh Sajnani (radiography); Drs Mark Specterman and Viral A. Sagar who took part in the procedure; and the cardiac catheter laboratory staff. Special thanks to the patient described in this report who consented to allow this case to be described for medical education. Also, the author’s gratitude to coronary care unit and Belgrave ward auxiliary and nursing staff involved in the care of this patient during her stay in the hospital, and last but not least, Farida Ismail who reliably keeps in stock all the equipment the authors use.
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://​creativecommons.​org/​licenses/​by/​4.​0/​.
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Netherlands Heart Journal

Het Netherlands Heart Journal wordt uitgegeven in samenwerking met de Nederlandse Vereniging voor Cardiologie. Het tijdschrift is Engelstalig en wordt gratis beschikbaa ...

Metagegevens
Titel
Noonan syndrome and parasternal pericardiocentesis
Auteurs
Pitt O. Lim
May H. Ohn
Publicatiedatum
20-02-2025
Uitgeverij
BSL Media & Learning
Gepubliceerd in
Netherlands Heart Journal
Print ISSN: 1568-5888
Elektronisch ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-025-01931-w