Scimitar syndrome is an unusual congenital defect characterized by total or partial anomalous venous drainage of the right lung into the inferior vena cava, partial systemic arterial supply, hypoplasia of the affected lung, dextrocardia, and bronchial and pulmonary lobulation abnormalities. It is called this way because the right anomalous pulmonary venous return (total or partial) resembles a scimitar, a Turkish sword, short and curved.

In this clinical case, a patient with recurrent history of hemoptysis, had a congenital heart disease surgery: scimitar syndrome with partial anomalous venous drainage of the right lung veins to the inferior vena cava with repair at 8 years of age and tunneling to the left atrium with a Gore patch.

The pediatric standard CMR protocol was performed in a 1.5T Optima MR450w GEM https://www.linkedin.com/company/gehealthcare/ , in addition the 4D-Flow sequence (post-processed with CVI42 https://www.linkedin.com/company/circl…ging-inc./ ) was used to analyze the anastomosed area since there was suspicion of occlusion.

CONCLUSIONS:

  1. Partial anomaly of the venous drainage of the right lung veins, with ectasias of them, as well as the suprahepatic veins.
  2. Severe stenosis of the tunneling (of the right pulmonary veins) to the left atrium.
  3. Preserved biventricular function.

Research team:

Dr. Juan Manuel Bonelli https://www.linkedin.com/in/juan-bonel…-6b111665/

BSc. Daniel Fino https://www.linkedin.com/in/danielfino/

Tec. Cecilia Lorca https://www.linkedin.com/in/cecilia-lo…-50b2a6b2/

Paula Di Cesare https://www.linkedin.com/in/paula-di-c…62b477217/

Tec. Cristian Allard https://www.linkedin.com/in/cristian-a…-77893b46/

BSc. Thomas Doring https://www.linkedin.com/in/thomas-dor…-3400964b/

Eng. Mauro Grioni https://www.linkedin.com/in/mauro-grio…-175a0378/

Tec. Lucas Machado https://www.linkedin.com/in/lucas-mach…a2307a160/