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    Case of the Day
    Fallot tetralogy with additional pulmonary artery loop and isolated upper lobe bronchus

    Modality: MRI Modality: MRI Modality: MRI Modality: MRI Modality: MRI Modality: MRI Modality: Pathology

    View here all images of this case in different sizes !



    Go to the top of the page ID: 20021024095545
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     Fallot tetralogy with additional pulmonary artery loop and isolated upper lobe bronchus   
     Available images: There are MRI images available for this case. [ MRI ] There are MRI images available for this case. [ Pathology ]   
     Author/s:

    N. Abolmaali (Frankfurt am Main)  

     
     Email Address:

    Viewable for logged on visitors (Log on)  

     
     Age:

    5 Months  

     
     Gender:

    N/A  

     
     History:

    5 month-old infant with cyanosis (O2-saturation 85-90%). Moderate difficulties in drinking, normal weight.  

     
     Pathomorphology or Pathophysiology of this disease :

    Fallot Tetralogy

    Most common "Fallot-variants" are composed of:
    1. Stenosis of the pulmonal artery.
    2. Interventricular septum defect. (VSD)
    3. Dextroposition of the aorta (caveat! No transposition!) In 25% of cases with a left-sided aortic arch.
    4. Hypertrophy of the right ventricle.

    Fallot-Pentalogy
    As in the Fallot-Tetralogy, but also
    5. Intraatrial septum defect (ASD)

    Fallot-Trilogy
    1. Pulmonary artery stenosis
    2. Hypertrophy of the right ventricle.
    3. Intraatrial septum defect (ASD) (caveat! No VSD!)

    Pulmonary artery sling (see also the image in Pathology 1):
    Normally the branching of the pulmonary trunk is ventral to the trachea. In a pulmonary artery sling, the branching is shifted dorsally, and crosses the tracheo-bronchial system dorsally. Depending on the area of crossing, the types are categorized in IA, IB, IIA and IIB.  

     
     Radiological findings:


    MRI 1 <- view MRI 1

    MRI 1: T1-transversal: Depiction of the VSD as well as the dextropositioned aortic root (arrow), the right ventricular hypertrophy is also seen.


    MRI 2 <- view MRI 2

    MRI 2: Phase-contrast image (VENC 130 cm/s) measured vertical to the stenotic pulmonary trunk. Flow accelleration in the pulmonary trunk is seen in aliasing. In adjusted VENC (300 cm/s), a flow speed of 2.4 m/s was measured.


    MRI 3 <- view MRI 3

    MRI 3: T1-sagittal: Pulmonary sling with severe compression of the trachia and carina. Broncioscopic tracheomalacia. The ventrally located vessel is the pulmonary trunk, dorsally, the left pulmonary artery can be seen.


    MRI 4 <- view MRI 4

    MRI 4: T1-Short axis slice through the ventricle. Obvious right ventricular hypertrophy (arrow)


    MRI 5 <- view MRI 5

    MRI 5: T1-transversal. Pulmonary sling (long arrow) with severe compression of the trachea (short arrow) and carina (bronchioscopic tracheomalacia). Stenosis of the pulmonary trunk (also seen in the Fallot-Tetralogy symptom of the pulmonary stenosis) - within this stenosis, the phase-contrast flow measurement was made (see 2nd image).


    MRI 6 <- view MRI 6

    MRI 6: T1-transversal : Isolated upper lobe bronchus (right, arrow). This isolated bronchus, as well as the further course of the trachea and carina defined in this case of a Type IB (See classification in the picture "Pathology 1").


    Pathology 1 <- view Pathology 1

    Pathology 1: Sling-Classification of chages of the tracheobroncial system in pulmonary slings in Types IA through IIB

     

     
     Diagnosis confirmation:

    Imaging including endoscopy  

     
     Which DD would be also possible with the radiological findings:

    N/A  

     
     Course / Prognosis / Frequency / Other :

    The Fallot-Tetrology makes up about 10% of all congenital heart defects, and that makes it relatively frequent. An isolated upper lobe bronchus is relative frequent. Statistic proof does not occur in literature, as far as we know. The pulmonal loop is a relatively rare defect.  

     
     Comments of the author about the case:

    N/A  

     
     First description / History:

    Potts WJ, Holinger PH, Rosenblum AH
    Anomalous left pulmonary artery causing obstruction to right main bronchus: report of a case.
    JAMA 1954;155:1409–11.  

     
     Literature:

    Hodina M, Wicky S, Payot M, Sekarski N, Gudinchet F
    Non-invasive imaging of the ring-sling complex in children
    Pediatr Cardiol. 2001 Jul-Aug;22(4):333-7

    Lee KH, Yoon CS, Choe KO, Kim MJ, Lee HM, Yoon HK, Kim B
    Use of imaging for assessing anatomical relationships of tracheobronchial anomalies associated with left pulmonary artery sling
    Pediatr Radiol. 2001 Apr;31(4):269-78

    Berdon WE
    Rings, slings, and other things: vascular compression of the infant trachea updated from the midcentury to the millennium--the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD
    Radiology. 2000 Sep;216(3):624-32

    Lorenz CH
    The range of normal values of cardiovascular structures in infants, children, and adolescents measured by magnetic resonance imaging
    Pediatr Cardiol. 2000 Jan-Feb;21(1):37-46  

     
     Region-Organ:

    Thorax-Other and unknown  

     
     Most likely etiology:

    congenital  

     
     Available images: There are MRI images available for this case. [ MRI ] There are MRI images available for this case. [ Pathology ]   
    More cases from these authors: Search N. Abolmaali in Medline N. Abolmaali (2)     




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