Abstract

Data from catheter angiography indicate that children with Hypoplastic Left Heart Syndrome (HLHS) in Fontan circulation have hypoplastic central pulmonary arteries. We used novel MRI techniques to quantify pulmonary vascular volume and lung perfusion in a cohort of HLHS patients and a group of healthy control subjects.

Highlights

  • Data from catheter angiography indicate that children with Hypoplastic Left Heart Syndrome (HLHS) in Fontan circulation have hypoplastic central pulmonary arteries

  • The indexed pulmonary total lung volume and the relative vascular volume in HLHS was significantly reduced compared to the control group (650±102 vs. 945±274 ml/m2, p=0.049; 6.5±3.7 vs. 9.,5±2.4 %/m2, p=0.03)

  • We found a weak correlation between cardiac index (2.78±0,82ml/m2/min) and lung perfusion (r=0.34, p=0.,062)

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Summary

Open Access

Assessment of pulmonary vascular volume and lung perfusion in patients with hypoplastic left heart syndrome (HLHS) in Fontan-circulation. Carsten Rickers1*, Eileen Pardun, Michael Jerosch-Herold, Dominik Gabbert, Chris Hart, Inga Voges, Andreas Entenmann, Hans Heiner Kramer. From 2011 SCMR/Euro CMR Joint Scientific Sessions Nice, France. From 2011 SCMR/Euro CMR Joint Scientific Sessions Nice, France. 3-6 February 2011

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