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Pediatric Cardiology

, Volume 31, Issue 6, pp 917–918 | Cite as

Echocardiographic Diagnosis of Bilateral Ductus With Discontinuous Branch Pulmonary Arteries and Heterotaxia Syndrome

  • Connie Kwan-Wong
  • Larry Kramer
  • Monesha Gupta-MalhotraEmail author
Images in Pediatric Cardiology

Congenital discontinuous central branch pulmonary arteries, a conotruncal cardiac anomaly, can be an isolated finding [1] or can be associated with other congenital heart defects [2, 3]. The anomaly can occur with or without pulmonary valve atresia and with a single or dual patent ductus arteriosus (PDA). The single PDA in most cases supplies the left pulmonary artery [4], whereas the right pulmonary artery normally is connected to the main pulmonary artery. The bilateral PDA or ductlike collateral vessels connect directly to the central branch pulmonary arteries, unlike major aortopulmonary collateral arteries, which connect indirectly.

We describe one such case with a diagnosis of complex congenital cardiac malformation in the setting of heterotaxia syndrome and univentricular physiology. A term Hispanic female infant of a diabetic mother with a two-vessel cord and a single ventricle heart seen on obstetric ultrasound was found to be desaturated with a soft ejection systolic murmur...

Keywords

Patent Ductus Arteriosus Branch Pulmonary Artery Persistent Left Superior Vena Total Anomalous Pulmonary Venous Return Common Pulmonary Vein 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Conflict of Interest

There is no conflict of interest to disclose by any of the authors in the sense of the ICMJE requirements for manuscripts.

Supplementary material

Movie clip 1 Bilateral patent ductus arteriosus (PDA). Transthoracic echocardiography in parasternal short axis view shows an anteriorly malposed aorta and posteriorly placed discontinuous branch pulmonary arteries with an antegrade laminar flow in systole. The left pulmonary artery is seen to be fed by a curvilinear left patent ductus arteriosus. Note the four pulmonary veins entering the pulmonary venous confluence (AVI 6630 kb)

Movie clip 2 Bilateral superior vena cava (SVC) and vertical vein to the left superior vena cava (LSVC). Transthoracic echocardiography in subcostal coronal view shows a bilateral superior SVC with an inferiorly directed flow (red flow). Note a large common pulmonary vein or vertical vein arising from the pulmonary venous confluence and coursing superiorly with a laminar antegrade flow (blue flow) to enter the LSVC. Note also the common atrium (AVI 5318 kb)

Movie clip 3 Right superior vena cava (SVC) and vertical vein. Transthoracic echocardiography in subcostal sagittal view shows the common pulmonary vein or vertical vein with a superiorly directed laminar flow (blue flow) and the right-sided superior vena cava with an inferiorly directed flow into the right-sided atrium (red flow) (AVI 4520 kb)

Movie clip 4 Cor triatriatum with flow into the left atrium. Transthoracic echocardiography in parasternal long axis view shows the pulmonary venous confluence behind the left atrium. There is an orifice between the confluence and the left atrium with the antegrade laminar flow (AVI 6513 kb)

References

  1. 1.
    Garcia JA, Bengur AR, Scott WA, Weinstein E, Ring WS (1995) Echocardiographic diagnosis of discontinuous left pulmonary artery as an isolated lesion. J Am Soc Echocardiogr 8:93–96CrossRefPubMedGoogle Scholar
  2. 2.
    Dipchand AI, Giuffre M, Freedom RM (1999) Tetralogy of Fallot with nonconfluent pulmonary arteries and aortopulmonary septal defect. Cardiol Young 9:75–77CrossRefPubMedGoogle Scholar
  3. 3.
    Kutsche LM, Van Mierop LH (1988) Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis. Am J Cardiol 61:850–856CrossRefPubMedGoogle Scholar
  4. 4.
    Elzenga NJ, von Suylen RJ, Frohn-Mulder I, Essed CE, Bos E, Quaegebeur JM (1990) Juxtaductal pulmonary artery coarctation: an underestimated cause of branch pulmonary artery stenosis in patients with pulmonary atresia or stenosis and a ventricular septal defect. J Thorac Cardiovasc Surg 100:416–424PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Connie Kwan-Wong
    • 1
  • Larry Kramer
    • 2
  • Monesha Gupta-Malhotra
    • 1
    Email author
  1. 1.Division of Pediatric CardiologyChildren’s Memorial Hermann Hospital, University of TexasHoustonUSA
  2. 2.Department of RadiologyMemorial Hermann Hospital, University of TexasHoustonUSA

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