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  • 32 year old female
    • dyspnea since a child
    • hx of Swyer James syndrome
      • absent perfusion to her RUL
    • 20 years ago diagnosed with PAH on cardiac catheterization.
    • Rx  oral therapies which a
    • controlled her symptoms.

CXR

CXR shows cardiomegaly, with enlarged main pulmonary artery and enlarged bilateral enlarged pulmonary arteries.

The right upper lung field is lucent consistent with the patients known Swyer James Syndrome

Additionally, the right and left hilum are prominent, which again may be secondary to pulmonary hypertension

CXR shows cardiomegaly, with enlarged main pulmonary artery and enlarged bilateral enlarged pulmonary arteries.
The right upper lung field is lucent consistent with the patients known Swyer James Syndrome
Ashley Davidoff MS TheCommonVein.net LPA-from-Aorta-001

Left Ventricular Hypertrophy

Lateral CXR shows cardiomegaly, with left ventricular configuration with an enlarged right pulmonary artery no definite right ventricular enlargement 
Ashley Davidoff MS TheCommonVein.net pulmonary artery off aorta 002

Echo

  • The left atrium is severely enlarged.
  • RV systolic function
    •  mild RV dysfunction.
    • RV wall thickness is
      • increased (8 mm).
      • Mild ventricular septal flattenning
      • mild to moderate tricuspid regurgitation.
      •  estimated PA pressure of
        • ~105/45 mmHg.
      • Mild to moderate pulmonic valvular regurgitation.
  • inferior vena cava is < 2.1 cm and exhibits reduced respiratory
    variability consistent with a
  • right atrial pressure of ~ 8 mm Hg.
  • consistent with severe pulmonary hypertension.
  • Chest CT showed
    • possible PDA
    • physical exam not consistent
      • no differential cyanosis,
      • no erythrocytosis and
      • normal LV dimensions.
      • ? additional shunting

Aorta to LPA

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery
Ashley Davidoff MD TheCommonVein.net

LAE 

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery The left atrium was 42 mm while the right atrium measured upper  normal limits 
Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003

Enlarged MPA and Pulmonary Hypertension

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery
CT shows an enlarged  main pulmonary artery Ashley Davidoff MD TheCommonVein.net
32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery
Coronal CT shows an enlarged  main pulmonary artery Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003b

RAE and LVE

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery.  The right atrium was upper limits normal  (48mm), right ventricle  was mildly enlarged (42mm) and LV was probably enlarged but could not be definitely measured
Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003

Right Aortic Arch

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery  CT shows a right aortic arch
Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003
44 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery
Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003b

PDA Aorta to Right Pulmonary Artery

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery Non contrast CT suggests a PDA from the right aortic arch to the pulmonary artery
Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003

? PDA Aorta to Right Pulmonary Artery

32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery. CT in sagittal suggests a PDA from the right aortic arch to the pulmonary artery
Ashley Davidoff MD TheCommonVein.net LPA-from-Aorta-003a
32 year old female Anomalous Origin of the LPA from Aorta Right Aortic arch Pulmonary Artery hypertension Pruning Swyer James PDA from Aorta to Left Pulmonary Artery
CT in sagittal projection suggests a PDA from the right aortic arch to the pulmonary artery Ashley Davidoff MD TheCommonVein.net CT LPA-from-Aorta-003a01

Swyer James
Hyperlucent Anterior Segment of the RUL

32 year old female with anomalous origin of the LPA from aorta pulmonary hypertension and Swyer-James syndrome 
CT in axial projection suggests shows a hyperlucent anterior segment of the right upper lobe, narrowing of the segmental airway,  and mosaic attenuation in the posterior segments        Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-012
32 year old female with anomalous origin of the LPA from aorta pulmonary hypertension and Swyer-James syndrome 
CT in coronal projection shows a hyperlucent anterior segment of the right upper lobe                       Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-013

Do you see a bull made of the soft tissues looking at the lucent lung?
Keep your eye on the eye of the bull as it will get progressively smaller as the bull goes to sleep and the airway narrows

32 year old female with anomalous origin of the LPA from aorta pulmonary hypertension and Swyer-James syndrome 
CT in coronal projection shows a hyperlucent anterior segment of the right upper lobe   Note the patent segmental airway subtending the upper lobe If you look with an artistic eye you can see a bull made of soft tissues looking at the hyperlucent lung The patent segmental airway is the “eye of the bull”)                                                                                           Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-014
32 year old female with anomalous origin of the LPA from aorta pulmonary hypertension and Swyer-James syndrome 
CT in coronal projection shows a hyperlucent anterior segment of the right upper lobe   Note that the “eye of the bull” has become smaller as the anterior segmental airway becomes progressively narrowed                                                                Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-014b
32 year old female with anomalous origin of the LPA from aorta pulmonary hypertension and Swyer-James syndrome 
CT in coronal projection shows a hyperlucent anterior segment of the right upper lobe   Note that the “eye of the bull” has become smaller as the anterior segmental airway becomes progressively narrowed                                                                                                        Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-015
32 year old female with anomalous origin of the LPA from aorta pulmonary hypertension and Swyer-James syndrome 
CT in coronal projection shows a hyperlucent anterior segment of the right upper lobe extending to the apex.  This finding helps us understand why the ventilation in the VQ scan is deficient in the upper lung extends to the apex despite normal aeration of the superior segment                            Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-016

AP Ventilation View NM Shows Non Ventilation  of the Right Upper Lung Field  and Absent Ventilation  of the Left Lung

32 year old female with anomalous origin of the LPA from aorta, pulmonary hypertension and Swyer-James syndrome 
AP of the ventilation component of the VQ scan shows non ventilation  of the right upper lung field and absent ventilation  of the left lung.  The absent ventilation of the right upper lung field is explained by the expanded hyperlucent anterior segment of the right upper lobe which reaches to the right apex (see image above).  The non ventilation of the left lung is explained by the absent pulmonary blood flow and the QP QS mismatch – just like a large occlusive PE to the left lung                                                                                                                                                                              Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-016

Perfusion Scan – No Perfusion of the Left Lung

32 year old female with anomalous origin of the LPA from aorta, pulmonary hypertension and Swyer-James syndrome 
AP of the perfusion component of the VQ scan shows non perfusion of the  left lung.  The absent perfusion of the left lung is explained by the absent pulmonary blood flow since the blood to the left pulmonary artery is arising from the aorta  – just like a large occlusive PE to the left lung            Ashley  Davidoff MD TheCommonVein.net artery-off-aorta-017
  • Anomalous origin of the right or left pulmonary artery
  • from the aorta
  • rare  prevalence of 0.33%
  • 30% mortality rate within the first 3 months after birth (
  • Anomalous origin of the right pulmonary artery
    • most common subtype,
    • accounting for more than 80% o
    • >70% of patients with anomalous origin of the right pulmonary artery also have a
      • PDA,
    • anomalous origin of the left pulmonary artery
      • less commonly associated with PDA
  • first description by Fraentzel in
    1868
  • proximal subtype has been reported to be
    • secondary to the disappearance of the fifth and sixth arches on the left,
  • distal subtype occurs with a persistent
    • fifth and absent sixth aortic arch
  • PDA and
  • atrial septal defect to be the most commonly associated congenital anomalies 

Links and References

See TCV 5896