Multimodality imaging in delineation of complex sinus venosus defects and treatment outcomes over the last decade

Li Y Ng, Lars Nolke, Adam James, Brian Grant, Orla Franklin, J Mark Redmond, Jonathan McGuinness, Kevin Walsh, Colin J McMahon*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

BACKGROUND: Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging.

METHODS: Retrospective review of all patients from February 2008 to January 2019.

RESULTS: Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5-15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6-15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter.

CONCLUSION: Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

Original languageEnglish
Article number1047951121003851
Pages (from-to)1112-1120
Number of pages9
JournalCardiology in the Young
Volume32
Issue number7
Early online date15 Sept 2021
DOIs
Publication statusPublished - Jul 2022

Keywords

  • ATRIAL-SEPTAL-DEFECT
  • CLOSURE
  • DIAGNOSIS
  • DRAINAGE
  • Echocardiography
  • FOLLOW-UP
  • PULMONARY VENOUS CONNECTION
  • SINGLE-PATCH
  • SUPERIOR VENA-CAVA
  • SURGICAL REPAIR
  • TRANSCATHETER CORRECTION
  • anomalous pulmonary venous drainage
  • imaging
  • sinus venosus defect
  • surgery
  • warden procedure

Cite this