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.
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.
Authors: Alejandro Avila-Alvarez; Maria Carmen Bravo-Laguna; Lucía Deiros Bronte; Maria Jesus Del Cerro Journal: Pediatr Cardiol Date: 2012-11-11 Impact factor: 1.655
Authors: Victor Bautista-Hernandez; Ravi R Thiagarajan; Francis Fynn-Thompson; Satish K Rajagopal; Daniel E Nento; Vamsi Yarlagadda; Sarah A Teele; Catherine K Allan; Sitaram M Emani; Peter C Laussen; Frank A Pigula; Emile A Bacha Journal: Ann Thorac Surg Date: 2009-10 Impact factor: 4.330
Authors: Prashob Porayette; Joshua F P van Amerom; Shi-Joon Yoo; Edgar Jaeggi; Christopher K Macgowan; Mike Seed Journal: Cardiol Young Date: 2014-06-16 Impact factor: 1.093