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Intravenous sildenafil in the management of pulmonary hypertension associated with congenital diaphragmatic hernia

Authors » Bialkowski A , Moenkemeyer F , Patel N

Journal » Eur J Pediatr Surg

Year » 2015

This article is included in 1 Systematic review Systematic reviews (1 reference)

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Timing of repair of congenital diaphragmatic hernia in patients supported by extracorporeal membrane oxygenation (ECMO).

Authors » Partridge EA , Peranteau WH , Rintoul NE , Herkert LM , Flake AW , Adzick NS , Hedrick HL

Journal » Journal of pediatric surgery

Year » 2015

Links » Pubmed DOI

This article is included in 1 Systematic review Systematic reviews (1 reference)

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PURPOSE: The optimal timing of repair for congenital diaphragmatic hernia (CDH) in patients requiring extracorporeal membrane oxygenation (ECMO) is controversial. Repair during ECMO may improve respiratory function by restoring normal anatomy. However, there is increased risk of complications including surgical bleeding. The purpose of this study was to examine the impact of timing of CDH repair on outcomes in a large cohort of patients treated at a single institution. METHODS: We retrospectively reviewed charts of all CDH patients in our Pulmonary Hypoplasia Program from 2004 to 2013. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p<0.05). RESULTS: A total of 77 CDH patients required ECMO support during the study dates. Of these, 16 patients did not survive to repair, 3 patients were repaired prior to cannulation, 41 patients were repaired during ECMO, and 17 patients were repaired after decannulation. Survival was 67%, 43.9%, and 100% for those repaired prior to, during, or post ECMO, respectively, with statistical significance associated with repair after decannulation (P<0.0001). Operative bleeding requiring transfusion occurred in 12 patients repaired on ECMO, while no significant bleeding occurred in patients repaired after decannulation (P=0.003). CONCLUSION: Outcomes were improved in CDH patients undergoing surgical repair following ECMO with significantly increased survival, lower rates of surgical bleeding, and decreased total duration of ECMO therapy compared to patients repaired on ECMO. In patients who can be successfully weaned from ECMO, our study supports a role for delayed repair off ECMO with reduced operative morbidity and increased survival.

Prolonged extracorporeal membrane oxygenator support among neonates with acute respiratory failure: a review of the Extracorporeal Life Support Organization registry.

Authors » Prodhan P , Stroud M , El-Hassan N , Peeples S , Rycus P , Brogan TV , Tang X

Journal » ASAIO journal (American Society for Artificial Internal Organs : 1992)

Year » 2014

Links » Pubmed DOI

This article is included in 1 Systematic review Systematic reviews (1 reference)

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The objective of this study was to identify types of neonatal diseases associated with prolonged (≥21 days) extracorporeal membrane oxygenation (ECMO), characteristics of survivors and nonsurvivors among those requiring prolonged ECMO, and factors associated with mortality. Data were obtained from the Extracorporeal Life Support Organization registry over the period from January 1, 1998, through December 31, 2011, for all neonates (age <31 days), with respiratory failure as the indication for ECMO. The primary outcome was survival to hospital discharge. Survivors and nonsurvivors were compared for 1) patient demographics, 2) primary diagnosis, 3) pre-ECMO clinical course and therapies, and 4) ECMO course and associated complications. The most common diagnosis associated with prolonged ECMO support in neonates is congenital diaphragmatic hernia (CDH; 69%). Infants with meconium aspiration syndrome had the highest survival rate (71%) compared with other diagnoses analyzed (26.3%; p < 0.001). Nonsurvivors were more likely to experience complications on ECMO, and multivariate analysis showed that the need for inotropes while on ECMO support (odds ratio, 2.2 [95% confidence interval, 1.3-3.7]; p = 0.003) was independently associated with mortality. Neonates requiring prolonged ECMO support have a 24% survival to discharge. Many of these cases involve CDH. Complications are common with prolonged ECMO, but only receipt of inotropes was shown to be independently associated with mortality. This report may help guide clinical decision making and family counseling for neonates requiring prolonged ECMO support.

A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: does timing of operation matter?

Authors » Hollinger LE , Lally PA , Tsao K , Wray CJ , Lally KP , Congenital Diaphragmatic Hernia Study Group

Journal » Surgery

Year » 2014

Links » Pubmed DOI

This article is included in 1 Systematic review Systematic reviews (1 reference)

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BACKGROUND: Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains undefined. We sought to evaluate the influence of surgical timing on patient outcomes and hypothesized that delayed repair does not improve survival in CDH. METHODS: Prospectively collected data from 1,385 CDH Registry infants without preoperative extracorporeal membrane oxygen therapy (ECMO) were evaluated. Patients were stratified by timing of repair: Day of life (DOL) 0-3 (group 1), 4-7 (group 2), or >8 (group 3), and the effect of surgical timing on mortality was determined by logistic regression and risk-adjusted for severity of illness. RESULTS: The unadjusted odds ratio (OR) for mortality increased significantly with delayed repair (group 2, 1.73 [95% CI, 1.00-2.98; group 3, 3.42 [95% CI, 1.97-5.96]). However, when adjusted for severity of illness, delay in repair did not predict increased mortality (group 2, 1.2 [95% CI, 0.7-2.2]; group 3, 1.4 [95% CI, 0.8-2.6]), nor did it portend an increased need for postoperative ECMO (group 2, 1.1 [95% CI, 0.5-2.4]; group 3, 0.5 [95% CI, 0.2-1.4]). CONCLUSION: After adjustment for known risk factors, the timing of CDH repair in low-risk infants does not seem to influence mortality. However, specific clinical parameters guiding timing of elective CDH repair remain unknown.

Extracorporeal life support in patients with congenital diaphragmatic hernia: how long should we treat?

Authors » Kays DW , Islam S , Richards DS , Larson SD , Perkins JM , Talbert JL

Journal » Journal of the American College of Surgeons

Year » 2014

Links » Pubmed DOI PubMed Central

This article is included in 1 Systematic review Systematic reviews (1 reference)

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BACKGROUND: Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and, despite advances, extracorporeal life support (ie, extracorporeal membrane oxygenation [ECMO]) is commonly required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. STUDY DESIGN: We conducted a single-institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO and define how long patients should be supported. RESULTS: Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar score at 1 minute, Apgar score at 5 minutes, and Congenital Diaphragmatic Hernia Study Group predicted survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. Lung-to-head ratio also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. CONCLUSIONS: In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO can take 4 weeks or longer, and might require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations <4 weeks should be avoided.

Intermittent intravenous sildenafil for pulmonary hypertension management in neonates and infants.

Authors » Stultz JS , Puthoff T , Backes C , Nahata MC

Journal » American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists

Year » 2013

Links » Pubmed DOI

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PURPOSE: The use of intermittent i.v. sildenafil dosing in three patients with pulmonary hypertension (PH) and limited venous access is reported. SUMMARY: One preterm infant with PH in addition to bronchopulmonary dysplasia and two full-term neonates with PH after congenital diaphragmatic hernia repairs were successfully treated for PH with adjunctive intermittent i.v. sildenafil. sildenafil dosages ranged from 0.4 to 2 mg/kg every six hours. Infusion periods ranged from one to three hours. The longer infusion periods were used to minimize the risk of hypotension during infusion, with continued efficacy assessment between dosing intervals by monitoring ongoing oxygenation saturation trends and oxygen requirements when the drug was not infusing. Treatment duration ranged from 5 to 50 days. Decreases or fluctuations in systemic blood pressure were noted at the beginning of treatment, but minimal interventions were required to maintain blood pressure, which generally increased during extended treatment. Fraction of inspired oxygen requirements were decreased or remained stable during each patient's first dose, and the need for respiratory support decreased over time, with improvements in oxygenation and prevention of continual life-threatening desaturation episodes. PH eventually resolved in each patient, based on improvements in serial echocardiographic studies with decreased requirements for inhaled nitric oxide, oxygen, and mechanical ventilation. All three patients required weaning from sildenafil treatment, suggesting a potential for rebound respiratory insufficiency with abrupt discontinuation of sildenafil. CONCLUSION: Intermittent i.v. sildenafil dosing provided a well-tolerated, practical, and potentially effective treatment for PH in three patients when enteral intake was undesirable and when there was a need to conserve available venous access.

Thoracoscopic repair of neonatal congenital diaphragmatic hernia (CDH): outcomes after a systematic quality improvement process

Authors » Jancelewicz T , Langer JC , Chiang M , et al

Journal » J Pediatr Surg

Year » 2013

This article is included in 1 Systematic review Systematic reviews (1 reference)

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Impact of our new protocol on the outcome of the neonates with congenital diaphragmatic hernia.

Authors » Kimura O , Furukawa T , Higuchi K , Takeuchi Y , Fumino S , Aoi S , Tajiri T

Journal » Pediatric surgery international

Year » 2013

Links » Pubmed DOI

This article is included in 1 Systematic review Systematic reviews (1 reference)

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BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) remains a defiant challenge for pediatric surgeons. Since 2003, we developed a new protocol aiming for the better outcome. In this study, the usefulness of our new protocol was evaluated. MATERIALS AND METHODS: Forty-six neonates with CDH at the age of less than 24 h were divided into two groups based on the difference of era and treatment protocols. In Group 1, 15 patients were treated between 1997 and 2002 and 31 patients were treated between 2003 and 2011 in Group 2. In the latter group, a new protocol was introduced focusing on the prevention of lung edema as well as lung injury by steroid administration and on the stabilization of cardiopulmonary function using continuous D-mannitol infusion. The survival rate and the postoperative intubation period (POIP) were compared between the two groups. RESULTS: The overall survival rate was significantly increased from 53 % (8/15) to 81 % (25/31) (p < 0.05). In isolated CDH, the survival rate was increased from 58 to 93 %. The average POIP was remarkably shortened from 39.0 to 4.4 days (p < 0.01). CONCLUSION: Our new protocol remarkably improved the survival rate and shortened the period of mechanical ventilation in neonates with CDH.