Detrimental effects of standard medical therapy in congenital diaphragmatic hernia.

OBJECTIVE To evaluate the impact of a nonstandard ventilation strategy on survival in congenital diaphragmatic hernia (CDH). BACKGROUND Despite recent advances, including nitric oxide, CDH remains an unsolved problem with a mortality rate of 35% to 50%. Hyperventilation and alkalization remain common therapies. METHODS In 1992, the authors prospectively abandoned hyperventilation and alkalization. Patients are lightly sedated and ventilated with the lowest pressure providing adequate chest movement, and the rate is set to patient comfort. Nitric oxide and extracorporeal membrane oxygenation (ECMO) are reserved for life-threatening instability. Surgical repair is delayed 1 to 5 days. Sixty consecutive patients are compared with 29 previous patients treated with hyperventilation and alkalization, 13 before and 16 after the availability of ECMO. RESULTS Overall, 47 of 60 patients (78%) in study era 3 survived compared with 2 of 13 (15%) in the hyperventilation era and 7 of 16 (44%) in the hyperventilation/ECMO era (p < 0.0001). The disease severity and the incidence of associated anomalies did not differ between groups. To compare management strategies, patients who had treatment withheld because of lethal associated conditions were then removed from analysis. Peak inspiratory pressure and arterial pH were lower (p < 0.0001) and Paco2 was higher (p < 0.05) in era 3 than in the previous eras. The rate of pneumothorax (1.9%) decreased (p < 0.0001). In era 3, survival was 47 of 53 (89%) treated patients, and 23 of 25 inborn patients with isolated CDH survived (92%). CONCLUSIONS Nonstandard ventilatory support of patients with CDH has led to significantly improved survival rates. This study sets a survival benchmark and strongly suggests the negative effects of hyperventilation and alkalization.

[1]  T. Jaksic,et al.  Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. , 1998, Journal of pediatric surgery.

[2]  R. Filly,et al.  Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion. , 1998, Journal of pediatric surgery.

[3]  C. Stolar,et al.  Congenital diaphragmatic hernia survival and use of extracorporeal life support at selected level III nurseries with multimodality support. , 1998, Surgery.

[4]  T. Tracy Effect of very delayed repair of congenital diaphragmatic hernia on survival and extracorporeal life support use , 1997 .

[5]  K. Palmér,et al.  Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery. , 1997, Journal of pediatric surgery.

[6]  J. Vacanti,et al.  Congenital diaphragmatic hernia--a tale of two cities: the Boston experience. , 1997, Journal of pediatric surgery.

[7]  J. N. Matthews,et al.  Is delayed surgery really better for congenital diaphragmatic hernia?: a prospective randomized clinical trial. , 1996, Journal of pediatric surgery.

[8]  E. Skarsgard,et al.  Fetal endoscopic tracheal occlusion ('Fetendo-PLUG') for congenital diaphragmatic hernia. , 1996, Journal of pediatric surgery.

[9]  S. Henneberg,et al.  Inhalation of nitric oxide as a treatment of pulmonary hypertension in congenital diaphragmatic hernia. , 1995, Journal of pediatric surgery.

[10]  T. Krummel,et al.  Nitric oxide reversal of recurrent pulmonary hypertension and respiratory failure in an infant with CDH after successful ECMO therapy. , 1995, Journal of pediatric surgery.

[11]  C. Stolar,et al.  Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. , 1995, Journal of pediatric surgery.

[12]  K. Meurs,et al.  Lobar lung transplantation as a treatment for congenital diaphragmatic hernia. , 1994, Journal of pediatric surgery.

[13]  M. Hedrick,et al.  Plug the lung until it grows (PLUG): a new method to treat congenital diaphragmatic hernia in utero. , 1994, Journal of pediatric surgery.

[14]  J. Wladimiroff,et al.  Prenatal diagnosis of congenital diaphragmatic hernia: A retrospective analysis of 28 cases , 1994, Prenatal diagnosis.

[15]  R. Goldstein,et al.  Correction of congenital diaphragmatic hernia in utero: VI. Hard-earned lessons. , 1993, Journal of pediatric surgery.

[16]  P. Lönnqvist,et al.  Near fatal pulmonary hypertension after surgical repair of congenital diaphragmatic hernia , 1993, Anaesthesia.

[17]  P. Donahoe,et al.  Biochemical immaturity of lungs in congenital diaphragmatic hernia. , 1993, Journal of pediatric surgery.

[18]  W. Engle,et al.  Delayed Surgical Repair and ECMO Improves Survival in Congenital Diaphragmatic Hernia , 1992, Annals of surgery.

[19]  J. Vacanti,et al.  Delayed repair and preoperative ECMO does not improve survival in high-risk congenital diaphragmatic hernia. , 1992, Journal of pediatric surgery.

[20]  K. Georgeson,et al.  Improvement in survival of patients with congenital diaphragmatic hernia utilizing a strategy of delayed repair after medical and/or extracorporeal membrane oxygenation stabilization. , 1991, Journal of pediatric surgery.

[21]  T. Kolobow,et al.  Acute lung injury from mechanical ventilation at moderately high airway pressures. , 1990, Journal of applied physiology.

[22]  W P Kanto,et al.  Hidden mortality rate associated with extracorporeal membrane oxygenation. , 1990, The Journal of pediatrics.

[23]  P. B. Mansfield,et al.  Effect of pH and PCO2 on pulmonary and systemic hemodynamics after surgery in children with congenital heart disease and pulmonary hypertension. , 1988, The Journal of pediatrics.

[24]  G. Farr,et al.  A correlation of pulmonary hypoplasia, mean airway pressure, and survival in congenital diaphragmatic hernia treated with extracorporeal membrane oxygenation. , 1987, Journal of pediatric surgery.

[25]  L. Greenfield,et al.  Mortality with extracorporeal membrane oxygenation following repair of congenital diaphragmatic hernia in 93 infants. , 1987, Journal of pediatric surgery.

[26]  George Dk,et al.  Hypoplasia and Immaturity of the Terminal Lung Unit (Acinus) in Congenital Diaphragmatic Hernia , 1987 .

[27]  R. Fumagalli,et al.  Severe impairment in lung function induced by high peak airway pressure during mechanical ventilation. An experimental study. , 1987, The American review of respiratory disease.

[28]  R. Bartlett,et al.  Extracorporeal Membrane Oxygenation (ECMO) in Neonatal Respiratory Failure , 1986 .

[29]  M. Heymann,et al.  Increased Arterial pH, Not Decreased PaCO2, Attenuates Hypoxia-Induced Pulmonary Vasoconstriction in Newborn Lambs , 1986, Pediatric Research.

[30]  L. Reid,et al.  Structure and function in pulmonary hypertension. New perceptions. , 1986, Chest.

[31]  L. S. James,et al.  Management of infants with severe respiratory failure and persistence of the fetal circulation, without hyperventilation. , 1985, Pediatrics.

[32]  J. Vacanti,et al.  Congenital diaphragmatic hernia: arterial structural changes and persistent pulmonary hypertension after surgical repair. , 1985, The Journal of pediatrics.

[33]  R. Filly,et al.  Prenatal diagnosis and natural history of the fetus with a congenital diaphragmatic hernia: initial clinical experience. , 1985, Journal of pediatric surgery.

[34]  M. Heymann,et al.  The independent effects of hyperventilation, tolazoline, and dopamine on infants with persistent pulmonary hypertension. , 1981, The Journal of pediatrics.

[35]  M. Woolley,et al.  Congenital diaphragmatic hernias: eleven years' experience. , 1979, Archives of surgery.

[36]  M. J. Maisels,et al.  Congenital Diaphragmatic Hernia: New Concept in Management , 1979, Annals of surgery.

[37]  M. Kitagawa,et al.  Lung hypoplasia in congenital diaphragmatic hernia a quantitative study of airway, artery, and alveolar development , 1971, The British journal of surgery.

[38]  Robert G. Moore,et al.  Initial clinical experience , 1997 .

[39]  R. L. Anderson,et al.  Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience. , 1990, Journal of pediatric surgery.

[40]  Salzberg Am,et al.  Pathophysiology and management of congenital posterolateral diaphragmatic hernias. , 1978 .