Complex congenital heart disease: complete AV canal, L-TGA, pulmonary atresia with multiple MAPCAs & total anomalous pulmonary venous drainage (TAPVD)

Case contributed by Dr Mohammad A. ElBeialy

Presentation

A baby with cyanosis.

Patient Data

Age: 10 months
Gender: Male

Heart:

  • Situs solitus, apex to the left.
  • Atrio-ventricular discordance: the right atrium opens into right sided small rudimentary ventricle (morphologically left ventricle), while the left atrium opens into a left sided dilated ventricle (morphologically right ventricle with characteristic moderator band).
  • Ventriculo-arterial: Great vessels arise from the dilated left sided morphologically right ventricle; aorta arises anterior and to the left, while the pulmonary artery arises posterior and to the right with total pulmonary atresia, pulmonary circulation is supplied by multiple MAPCAs.
  • Great vessel relationship: transposed.
  • A large ASD is noted.
  • A large inlet VSD is noted.
  • Moderate cardiomegaly is noted.
  • No pericardial effusion.

Aorta:

  • Aorta is seen arising from the left sided morphologically right ventricle. Ascending aorta is measuring 1.67cm (at the level of main pulmonary artery).
  • Right sided aortic arch gives three mirror image vessels (the brachiocephalic, the right common carotid and the right subclavian arteries) with normal distribution of its branches. The aortic isthmus measures 1.21 cm (at the level of the right subclavian artery’s origin)
  • The descending aorta is seen running in front of the vertebral column and of about 0.61cm in diameter (measured at the level of the diaphragm).
  • Single origin of the right and left coronary arteries from the right coronary sinus of Valsalva with normal distribution of both of them.
  • Multiple major aorto-pulmonary collaterals (MAPCAs) were seen:
    • The 1st  MAPCA arises from the right side of the descending thoracic aorta about 1.92cm distal to the right subclavian artery origin, it passes upward and to the right to supply the upper part of the right lung, it measures about 0.42cm in diameter.
    • The 2nd  MAPCA arises from the left side of the descending thoracic aorta about 2.09cm distal to the right subclavian artery origin, it passes downward and to the left to supply the middle and lower parts of the left lung, it measures about 0.37cm in diameter.
    • The 3rd MAPCA arises from the right side of the descending thoracic aorta about 2.63cm distal to the right subclavian artery origin, it passes downward and to the left to supply the upper and middle parts of the left lung, it measures about 0.48cm in diameter.
    • The 4th MAPCA arises from the left side of the descending thoracic aorta about 2.72cm distal to the right subclavian artery origin, it turns downward and to the right to supply the lower lobe of the right lung, it measures about 0.38cm in diameter.
    • The 5th MAPCA arises from the left side of the descending thoracic aorta about 3.51cm distal to the right subclavian artery origin, it passes the left to supply the upper and middle parts of the left lung, it measures about 0.32cm in diameter.
  • No patent ductus arteriosus (PDA) is noted.
  • No evidence of thoracic or abdominal aortic coarctation.

Pulmonary Arteries & Veins

  • Confluent small sized main pulmonary artery and its branches.
  • The main pulmonary artery is very short and uniform in caliber that measures 0.45cm in diameter.
  • The RPA measures about 0.26cm and 0.37cm in diameter proximal and distal respectively.
  • The LPA is uniform in caliber that measures about 0.40cm in diameter.
  • Abnormal drainage of the four pulmonary veins into a confluent chamber draining into the proximal part of the SVC near its opening into the right atrium: Supracardiac TAPVR with characteristic snow-man configuration. 

Venae Cava & Innominate Veins

Lungs:   Congested both lung fields, no pleural sac collections.

 

Case Discussion

A case of complex congenital heart disease showing complete AV canal, L-TGA and pulmonary atresia with:

  • Total anomalous pulmonary venous drainage into a confluent chamber draining directly into the proximal part of the superior vena cava (supracardiac type).
  • Small sized main pulmonary artery and branches; multiple MAPCAs supplying both lungs.
  • Abnormal coronary anatomy.

The case is courtesy of Dr. Sarah Moharem-Elgamal, CBBCT & Dr. Mohammed A. ElBeialy, FRCR, MD. 

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Case information

rID: 32092
Case created: 12th Nov 2014
Last edited: 16th Dec 2015
System: Cardiac
Inclusion in quiz mode: Included

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