Pulmonary Hypertension in Neonates with High-Risk Congenital Diaphragmatic Hernia Does Not Affect Mid-Term Outcome

Abstract

Purpose: Congenital diaphragmatic hernia (CDH) presents with a broad spectrum of severity, depending on the degree of pulmonary hypoplasia and persistent pulmonary hypertension (PPH). It is currently not clear whether pulmonary hypertension may affect late morbidity. Aim of the present study was to evaluate the influence of PPH on mid-term morbidity in high-risk CDH survivors.

Methods: All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 h of life) CDH survivors, treated between 2004 and 2008 in our Department were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary and orthopedic evaluations were done at specific time-points (at 6, 12, and 24 months of age). Patients were grouped depending on the presence/absence of pulmonary hypertension (defined by expert pediatric cardiologists after echocardiography). Paired t-test and Fisher's exact test were used as appropriate. P<0.05 was considered significant.

Results: 70 survivors out of a total of 95 high-risk CDH infants treated in our Department attended our follow-up clinic and were prospectively evaluated. 17 patients were excluded from the present study because no clear data was available regarding the presence/absence of PPH during the perinatal period. Moreover, 9 infants were not enrolled because they did not reach at least 6 months of age. A total of 44 survivors were finally enrolled since they met the inclusion criteria. 26 infants did not present with PPH during the first hospital admission, while 18 had PPH. The 2 groups did not differ with regard to any of the outcomes considered at follow-up (p>0.2).

Conclusion: In our cohort of high-risk CDH survivors, PPH was not found to affect late sequelae at mid-term follow-up. This may indicate that postnatal pulmonary development is not (always) influenced by perinatal PPH. Nevertheless, a longer follow-up and more patients are needed to properly quantify possible late problems in high-risk CDH survivors with associated neonatal PPH.

Key words

congenital diaphragmatic hernia - persistent pulmonary hypertension - mid-term follow-up - auxological evaluation - gastroesophageal evaluation

References

• 1 Chukwu J, Iro C, Donoughue V. et al . Congenital diaphragmatic hernia: neonatal outcomes following referral to a pediatric surgical centre.  Ir Med J. 2009;  102 (8) 260-261
• 2 Vuletin JF, Lim F-Y, Cnota J. et al . Prenatal pulmonary hypertension index: novel prenatal predictor of severe postnatal pulmonary artery hypertension in antenatally diagnosed congenital diaphragmatic hernia.  J Pediatr Surg. 2010;  45 703-708
• 3 Takahashi S, Oishi Y, Ito N. et al . Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices.  J Pediatr Surg. 2009;  44 2101-2106
• 4 Casaccia G, Crescenzi F, Dotta A. Birth weight and McGoon index predict mortality in newborn infants with congenital diaphragmatic hernia.  J Pediatr Surg. 2006;  41 25-28
• 5 Miguet D, Claris O, Lapillonne A. et al . Preoperative stabilization using high-frequency oscillatory ventilation in the management of congenital diaphragmatic hernia.  Crit Care Med. 1995;  22 887-892
• 6 Chiu P, Sauer C, Mihailovic A. et al . The price of success in the management of congenital diaphragmatic hernia: is improved survival accompanied by an increase in long-term morbidity?.  J Pediatr Surg. 2006;  41 888-892
• 7 Section on Surgery and the Committee on Fetus and Newborn . Postdischarge follow-up of infants with congenital diaphragmatic hernia.  Pediatrics. 2008;  121 627-632
• 8 Bagolan P, Morini F. Long-term follow-up of infants with congenital diaphragmatic hernia.  Semin Pediatr Surg. 2007;  16 134-144
• 9 Vanamo K, Rintala R, Lindahl H. Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects.  J Pediatr Surg. 1996;  31 551-554
• 10 Dotta A, Palamides S, Braguglia A. et al . Lung volume and distribution of ventilation in survivors to congenital diaphragmatic hernia (CDH) during infancy.  Pediatric Pulmonology. 2007;  42 600-604
• 11 Vanamo K, Peltonen J, Rintala R. et al . Chest wall and spinal deformities in adults with congenital diaphragmatic defects.  J Pediatr Surg. 1996;  31 851-854
• 12 Noori S, Friedlich P, Wong P. Cardiovascular effects of Sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension.  Neonatology. 2007;  91 92-100
• 13 Stege G, Fenton A, Jaffray B. Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia.  Pediatrics. 2003;  112 532-535
• 14 Jaillard SM, Pierrat V, Dubois A. et al . Outcome at 2 years of infants with congenital diaphragmatic hernia: a population based study.  Ann Thorac Surg. 2003;  75 (1) 250-256
• 15 Nobuhara KK, Lund DP, Mitchell J. et al . Long-term outlook for survivors of congenital diaphragmatic hernia.  Clin Perinatol. 1996;  23 (4) 873-887
• 16 Van Meurs KP, Robbins ST, Reed VL. et al . Congenital diaphragmatic hernia: long term outcome in neonates treated with extracorporeal membrane oxygenetation.  J Pediatr. 1993;  122 (6) 893-899
• 17 Kieffer J, Sapin E, Berg A. et al . Gastroesophageal reflux after repair of congenital diaphragmatic hernia.  J Pediatr Surg. 1995;  30 (9) 1330-1333
• 18 Fitzgerald D, Evans N, Van Asperen P. et al . Subclinical persisting pulmonary hypertension in chronic neonatal lung disease.  Arch Dis Child Fetal Neonatal Ed. 1994;  70 (2) 118-122
• 19 Peetsold MG, Heij HA, Kneepkens CMF. et al . The long-term follow-up of patients with congenital diaphragmatic hernia: a broad spectrum of morbidity.  Pediatr Surg Int. 2009;  25 1-17

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