Persistent Pulmonary Hypertension of the Newborn With Transposition of the Great Arteries

Marcus T.R. Roofthooft*, Klasina A. Bergman, Tjalling W. Waterbolk, Tjark Ebels, Beatrijs Bartelds, Rolf M.F. Berger

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

54 Citations (Scopus)


Background: Persistent pulmonary hypertension of the newborn (PPHN) in patients with transposition of the great arteries (TGA) is reported to be a high-risk and often therapy-resistant condition, associated with a high mortality. However, data on its incidence and prognosis are scarce and originate mostly from the era before introduction of inhaled nitric oxide (iNO) therapy for PPHN. Methods: This is a retrospective study of consecutive newborns with TGA, admitted to a tertiary cardiac and neonatal intensive unit over a 10-year period. In this period, iNO therapy was available. Results: Fourteen out of 112 patients with TGA (12.5%) presented with associated PPHN. The PPHN occurred more frequently in patients with TGA and intact ventricular septum (IVS) compared with those with TGA and ventricular septal defect (13 out of 83 patients versus one out of 29 patients, respectively; p = 0.06, Fisher exact test). Of those newborns, six presented with severe PPHN, whereas eight presented with mild-to-moderate PPHN. Despite currently available treatment modalities, including iNO, four out of 14 patients died before corrective surgical procedures were considered to be an option (TGA/PPHN preoperative mortality 28.6%). These included three out of six patients (50%) with severe PPHN and one out of eight (12.5%) with mild-to-moderate PPHN. Conclusions: The combination of TGA with PPHN is a serious and often fatal condition. It may jeopardize the usually favorable outcome of newborns with TGA. Despite the introduction of iNO therapy, the combination of TGA and PPHN remains a condition with unacceptable high mortality (in our series). Additional treatment strategies need to be investigated.

Original languageEnglish
Pages (from-to)1446-1450
Number of pages5
JournalAnnals of Thoracic Surgery
Issue number4
Publication statusPublished - Apr 2007
Externally publishedYes


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