Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Polysplenia with pulmonary arteriovenous malformations

J Papagiannis1, R J Kanter, E L Effman

  • 1Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.

Pediatric Cardiology
|March 1, 1993
PubMed
Summary

Polysplenia syndrome can lead to pulmonary arteriovenous malformations (PAVMs), causing cyanosis. This case highlights PAVMs in a patient with complex congenital heart defects and portal vein anomalies.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Optimizing Peri-Operative Pain Management in Children With Long QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia Undergoing Left Cardiac Sympathetic Denervation: A Case Series on Continuous Erector Spinae Plane Block and Serratus Plane Block.

Paediatric anaesthesia·2025
Same author

2015 SPCTPD/ACC/AAP/AHA Training Guidelines for Pediatric Cardiology Fellowship Programs (Revision of the 2005 Training Guidelines for Pediatric Cardiology Fellowship Programs).

Journal of the American College of Cardiology·2015
Same author

Diagnosis and management of pulmonary arteriovenous malformations.

Images in paediatric cardiology·2012
Same author

Cardiac ion channel gene mutations in Greek long QT syndrome patients.

Journal of applied genetics·2010
Same author

Treatment of pernicious anemia with pteroyl glutamic acid (folic acid).

Proceedings [of the] annual meeting. Central Society for Clinical Research (U.S.)·2010
Same author

Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates.

Heart (British Cardiac Society)·2009

Area of Science:

  • Cardiology
  • Radiology
  • Pulmonology

Background:

  • Polysplenia syndrome is a congenital condition characterized by multiple spleens.
  • Dextrocardia and left atrial isomerism are associated cardiac anomalies.
  • Pulmonary arteriovenous malformations (PAVMs) are abnormal connections between pulmonary arteries and veins.

Observation:

  • A patient with polysplenia syndrome, dextrocardia, and left atrial isomerism presented with progressive cyanosis and clubbing.
  • Angiography and lung biopsy confirmed the presence of PAVMs.
  • A superior mesenteric arteriogram showed hypoplastic intrahepatic portal vein branches and a portosystemic shunt.

Findings:

  • The patient's symptoms were attributed to the newly diagnosed PAVMs.
  • The study identified a correlation between polysplenia syndrome, portal vein anomalies, and PAVMs.

Related Experiment Videos

  • The findings suggest a potential link between visceral heterotaxy and the development of PAVMs.

    • Implications:

    • This case underscores the importance of considering PAVMs in patients with polysplenia syndrome and cyanosis.
    • Early diagnosis and management of PAVMs are crucial to prevent complications.
    • Further research is needed to elucidate the exact etiologies of PAVMs in this context.