Congenital Diaphragmatic Hernia Infants Research Articles

Postoperative regional distribution of pulmonary ventilation and perfusion in infants with congenital diaphragmatic hernia

Background/Purpose Advances in management of patients with congenital diaphragmatic hernia (CDH) have improved mortality rates but with a risk of increased pulmonary morbidity. The prognosis for CDH survivors remains difficult to predict owing to the lack of adequate methods. We used single photon emission computed tomography (SPECT) to measure the regional distribution of ventilation and perfusion in CDH infants to quantify the degree of lung function impairment and relate it to neonatal clinical disease severity. Methods Single photon emission computed tomography was performed in 12 CDH infants at the mean age of six months. Ventilation and perfusion were traced with 5 MBq Technegas and technetium-labelled albumin macro-aggregates, respectively. Neonatal clinical data collected during the patient's stay in the pediatric intensive care unit was correlated with the SPECT data. Results Single photon emission computed tomography revealed varying degrees of ventilation-perfusion abnormalities which correlated with the presence of pulmonary artery hypertension, days on ventilator and days on extracorporeal membrane oxygenation. Conclusions The grade of clinical disease severity in infants following CDH repair is closely related to the ventilation-perfusion abnormality as seen using SPECT. The persistence of pulmonary artery hypertension into the postoperative neonatal period appears to be an important pathophysiological factor related to ventilation-perfusion abnormalities. Single photon emission computed tomography provides valuable clinical information for patient follow-up.

Impact of Neuromuscular Blockade on Lung Function during the Initial Resuscitation of Infants with Congenital Diaphragmatic Hernia

Background and Aims: Practice differs as to whether a neuromuscular blocking agent should be given routinely to infants with a congenital diaphragmatic hernia (CDH) during resuscitation on the labour ward. The aim of this study was to investigate the effect of neuromuscular blockade on lung function during the initial resuscitation of CDH infants. Methods: A respiratory function monitor (NM3, Respironics) and a computer laptop with recording and analysis software (Spectra, Groove medical, UK) were used to collect and analyse air flow, airway pressure and tidal volume changes during resuscitation. Lung compliance was calculated from pressure- volume loops. Twenty inflations immediately before the administration of pancuronium (neuromuscular blocking agent) and twenty inflations immediately after were analysed. The study was approved by Outer North London ethics committee and parental consent was obtained for analysis of the data. Results: Ten infants with CDH (median gestational age 37 weeks and birth weight 2855 gms) were studied, all the infants were intubated immediately after birth. Mean peak inflation pressures of 25 (± 0.9) cm H2O were used during resuscitation. The median (IQR) tidal volume and lung compliance before paralysis were 5.4 (4.3-6.2)ml/kg and 0.3 (0.2-0.3) ml/cm H2O/kg. There were significant decrease in both the tidal volume 4.5(4.1-5.8) ml/kg (p< 0.01) and lung compliance 0.2(0.1-0.3) ml/cm H2O/kg (p< 0.01) following pancuronium administration. Conclusion: Infants with congenital diaphragmatic hernia have low lung compliance at birth, which is further reduced by administration of a neuromuscular blocking agent.

Prenatal risk stratification for isolated congenital diaphragmatic hernia: results of a Japanese multicenter study

Background/Purpose The aim of this study was to establish a prenatal prognostic classification system for risk-stratified management in fetuses with isolated congenital diaphragmatic hernia (CDH). Methods A multi-institutional retrospective cohort study of isolated CDH, diagnosed prenatally in fetuses delivered during the 2002 to 2007 period at 5 participating institutions in Japan, was conducted. The risk stratification system was formulated based on the odds ratios of prenatal parameters for mortality at 90 days. The clinical severity in CDH infants were compared among the stratified risk groups. Results Patients were classified into the 3 risk groups: group A (n = 48) consisted of infants showing liver-down with contralateral lung–to–thorax transverse area ratio (L/T) ratio ≥0.08; group B of infants showing liver-down with L/T ratio <0.08 or liver-up with L/T ratio ≥0.08 (n = 35), and group C of infants showing liver-up with L/T ratio <0.08 (n = 20). The mortality at 90 days in groups A, B, and C were 0.0%, 20.0%, and 65.0%, respectively. The intact discharge rates were 95.8%, 60.0%, and 5.0%, respectively. This system also accurately reflected the clinical severity in CDH infants. Conclusions Our prenatal risk stratification system, which demonstrated a significant difference in postnatal status and final outcome, would allow for accurate estimation of the severity of disease in fetuses with isolated CDH, although it needs prospective validation in a different population.

Congenital diaphragmatic hernia (CDH) in wales 1989–2010

AimsTo determine the incidence, co-morbidities and outcome of congenital diaphragmatic hernia (CDH) in Wales.MethodsThis retrospective database review used 2 data-sets-CARIS (Congenital Anomaly Register & Information Service, Wales) to determine incidence,...

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

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. 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. 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). 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.

Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair

BackgroundPulmonary hypertension (PH) associated with congenital diaphragmatic hernia (CDH) remains a significant cause of morbidity and mortality. For improved outcomes, preoperative stabilization is commonly practiced to control the PH in infants with CDH. Some CDH infants who have been considered stabilized and ready for surgery have nevertheless developed significant PH after surgical repair. In fact, the markers and consequences of the preoperative stabilization are still unclear. Therefore, we examine the perioperative course of PH to evaluate the impact of preoperative PH severity on mortality and morbidity of infants who underwent surgical repair of CDH. MethodsThe medical charts of all newborns (n = 49) with CDH who were treated at our institution between January 2000 and December 2009 were reviewed. General management and perioperative data were evaluated for all infants. The ratio of estimated pulmonary artery pressure to systemic pressure (P/S ratio or PSR), based on echocardiographic data, was used to assess the PH severity during the perioperative period. ResultsThe overall survival rate in our group of infants with CDH was 71.4%. Of the 49 infants with CDH, 9 (18.4%) died during the preoperative phase. Forty infants underwent CDH repair at a median age of 3.5 days (range, 1-46 days). Five of these infants (12.5%) subsequently deteriorated and died after surgery. Using receiver operating characteristic curve analysis, a PSR cutoff value before surgery of 0.9 predicted mortality in CDH infants with a sensitivity of 100% and specificity of 84% and with an area under the curve of 0.93 (P = .002). Accordingly, 2 groups of infants with distinct outcomes were identified, as follows: a low-PSR cohort (PSR ≤0.9) with a survival rate of 100% and a high-PSR cohort (PSR >0.9) with a survival rate of 50% (P = .001). The rate of pneumothorax and the frequency of use of several inotropic agents after surgery were significantly higher in the high-PSR group (P = .001 and .007, respectively). Compared with low-PSR infants, infants with high PSR were operated on later (P = .03) and were postoperatively ventilated longer (P = .01). During the entire perioperative period, significant differences in the PH severity were noted between the 2 PSR groups. During the first week of life, infants in the high-PSR group had significantly higher PSRs than those in the low-PSR group (P = .001); and similar tendencies continued to be significant between the 2 groups after CDH repair (P = .04). ConclusionsDuring the perioperative period, PH severity monitoring via the serial assessment of PSR is beneficial. Better outcomes were observed with a preoperative PSR less than or equal to 0.9, and this association needs to be confirmed by prospective study.

Timing of Delivery for Pregnancies With Congenital Diaphragmatic Hernia

Recent data published by the Congenital Diaphragmatic Hernia (CDH) Study Group suggested increased survival among infants with prenatally-diagnosed CDH born at early-term (37–38 weeks' gestation) compared with infants born at later term (39–41 weeks). These results contradict other published data. Moreover, the protective effects of early delivery on mortality became statistically significant only after an adjustment for birth weight (which was likely to overestimate the beneficial effects of early delivery). At present, no firm recommendation regarding the optimal timing of delivery of infants with CDH is possible. The aim of this study was to confirm the protective effect of an early-term delivery by comparing the gestational age-specific mortality patterns of term infants with and without isolated CDH delivered following the spontaneous onset of labor. The study population was 2089 singleton live births with isolated CDH born between 37 and 41 weeks of gestation; of these, 928 were born following vaginal delivery and were included in the final cohort. Log-binomial regression analysis assessed the effect of gestational age on mortality among CDH infants and adjusted the data for potentially confounding factors. With advancing gestational age (37 vs. 40 weeks), both neonatal and infant mortality decreased; neonatal mortality from 25.0% to 16.7% and infant mortality from 36.4% to 19.3%. Log-binomial regression analysis predicting risk of neonatal mortality showed a statistically significant trend of decreased risk with advancing gestational age: the relative risk for death was higher at 37 weeks versus 40 weeks (relative risk, 1.50; 95% confidence interval, 1.02–2.21). These findings suggest that there is no benefit of early-term delivery in lowering mortality due to CDH. This and other available evidence suggests that it may be premature to change clinical practice.

Echocardiographic measures of ventricular function and pulmonary artery size: prognostic markers of congenital diaphragmatic hernia?

To compare echocardiographic measures of biventricular function and pulmonary artery size in infants with congenital diaphragmatic hernia (CDH) and normal controls, and examine their correlation, if any, with outcomes in CDH. We included consecutive neonates (<1 month old) with CDH and term controls without structural heart defects. Clinical and outcomes data were recorded and echocardiograms evaluated for right ventricular (RV) and left ventricular (LV) myocardial performance index (MPI), cardiac output index (CI) and McGoon index, among others. Statistical analyses (SPSS version 17, SPSS, Chicago, IL, USA) included between-group comparisons, using analysis of variance and χ(2)-test and binary regression, with significance set at P<0.05. Infants with CDH (n=34) were comparable with controls (n=35) in their age, weight, gestational age and gender. CDH was left sided in 24 (70%) neonates. Extracorporeal membrane oxygenation (ECMO) was required in 15 (45%) neonates; 18 (53%) infants survived. MPIs, CI and eccentricity index in systole were significantly worse in the CDH group, compared with controls and among CDH infants who died, compared with survivors. Infants with CDH who died or needed ECMO had significantly impaired MPIs and CI than survivors. On regression analyses, LV CI and MPIs were independently associated with mortality. Infants with CDH had significantly impaired ventricular function and pulmonary hypertension, compared with controls. In the CDH group, LV dysfunction was associated with death and adverse outcomes. Further studies incorporating echocardiographic indices as prognostic markers of CDH are warranted.

Expression of eNOS in the lungs of neonates with pulmonary hypertension

Background Neonatal hypoxemic respiratory failure (NHRF) is usually associated with reversible persistent pulmonary hypertension (PPHN). Congenital diaphragmatic hernia (CDH), a cause of refractory NHRF, is associated with irreversible pulmonary hypertension. Nitric oxide (NO) generated in the pulmonary vascular endothelium by endothelial nitric oxide synthase (eNOS) plays a pivotal role in perinatal circulatory adaptation. Objective To compare the expression of eNOS using IHC in postmortem lung tissue from newborns diagnosed clinically with PPHN and CDH. Design/methods Formalin-fixed lung tissue from infants who died following treatment for PPHN (n = 12) or CDH (n = 8) and age and gender matched controls who died from non-respiratory causes (Control, n = 14) was evaluated for expression and staining intensity (1–4 scale) of eNOS using IHC. Results Mean gestational and postnatal age was comparable across groups. Histological evidence of chronic lung disease, pulmonary hypoplasia and pulmonary hypertension were seen more frequently in CDH compared to PPHN and control infants. eNOS expression was increased in arteriolar media of PPHN infants compared to Controls (p = 0.027). CDH infants had increased intensity of staining for eNOS in the arteriolar endothelium (p = 0.022) compared to control and PPHN infants and in the alveolar lining (p = 0.002) compared to Controls. Conclusions Upregulation of eNOS was seen both in infants with CDH and PPHN but was more marked in infants with CDH. These findings may have implications for understanding disease pathophysiology in cases with fatal outcome and development of novel therapies for neonatal pulmonary hypertension.

Maternal periconceptional exposure to cigarette smoking and alcohol consumption and congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a major birth defect that occurs when abdominal organs herniate through a diaphragmatic opening into the thoracic cavity and is associated with high mortality (>50%). The etiology of CDH is not well understood. Using data from the National Birth Defects Prevention Study, we examined associations between CDH and maternal periconceptional exposure (1 month before through the third month of pregnancy) to cigarette smoking and alcohol. Interview reports of exposures were provided by mothers of CDH (n = 503) and unaffected control (n = 6703) infants delivered from October 1997 through December 2005. Any exposure (yes/no), as well as quantity (average number of cigarettes or drinks), type (active/passive smoking; beer, wine, distilled spirits), and duration (e.g., number of months exposed) were examined. Adjusted odds ratios (aORs) and 95% confidence intervals (CIs) were calculated for all CDH cases combined, selected subtypes (Bochdalek, Morgagni, not otherwise specified), and phenotypes (infants with/without additional major birth defects). The aOR for any smoking was nonsignificantly elevated for all CDH cases combined. Odds of any smoking was significant for isolated Bochdalek CDH (aOR, 1.9; 95% CI, 1.2-3.0). The aORs associated with all measures of alcohol consumption were near unity for each CDH category examined. Stratification of smoking exposure by alcohol consumption and stratification of alcohol consumption by smoking exposure did not appreciably change the aORs. These findings identified periconceptional smoking exposure as a potential risk factor for CDH. Future studies need to confirm our findings and explore possible pathways accounting for the teratogenic effect of smoking.

Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program

Purpose The purpose of the study was to evaluate the neurodevelopmental outcome in infants with congenital diaphragmatic hernia (CDH). Methods Between June 2004 and September 2007, 41 CDH survivors were prospectively enrolled in an interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development II (prior 2006, n = 9), the Bayley Scales of Infant Development III (after 2006, n = 27), or the Wechsler Preschool and Primary Scale of Intelligence III (children older than 4 years, n = 5). Scores were grouped as average, mildly delayed, and severely delayed by standard deviation intervals (115-85, 71-84, <70), and mixed if average and mildly delayed in either cognitive or language. Results Median age at last assessment was 24 months (range, 6-62). Average, mixed, mildly delayed, and severely delayed scores for neurocognitive and language skills were found in 49%, 19%, 17%, and 15%, respectively. Psychomotor scores were normal, mildly delayed, and severely delayed in 46%, 23%, and 31%, respectively. Autism was present in 7%. Abnormal muscle tonicity was found in 51% (49% hypotonic, 2% hypertonic). Multivariate risk factors for borderline or delayed neurodevelopmental, neurocognitive, and/or psychomotor outcome were intrathoracic liver position ( P = .02), presence of a right-sided CDH ( P = .02), extracorporeal membrane oxygenation need ( P < .001), Gore-Tex patch repair ( P = .02), O 2 requirement at 30 days of life ( P < .01), and hypotonicity ( P < .01). Conclusions The prospective evaluation in an interdisciplinary follow-up program uncovered striking morbidities in neurodevelopmental status in approximately half of the CDH infants. The most common neurologic sequelae are neuromuscular hypotonicity and psychomotor dysfunction. Patient-specific factors are important determinants of adverse neurologic outcome.

Low Pulmonary Expression of Epithelial Na+ Channel and Na+, K+-ATPase in Newborn Infants with Congenital Diaphragmatic Hernia

Background: It has been suggested from several animal studies and clinical observations that congenital diaphragmatic hernia (CDH) with pulmonary hypoplasia is accompanied by a disturbed perinatal ion transport. This could lead to respiratory distress due to slower clearance of fetal lung fluid at birth. Objectives: The purpose of this study was to determine whether CDH is related to changes in the expression of three rate-limiting transporter proteins in lung epithelium at birth. Methods: Tracheal aspirate was collected from 12 newborn infants with CDH and from 8 newborn control patients. Sampling was performed at postnatal age 18 and at 43 h in the CDH group and at 18 h in the control group. The protein abundance of α-, β- and γ-epithelial Na⁺ channel (ENaC), aquaporin 5 and Na⁺, K⁺-ATPase α₁ was analyzed using semiquantitative immunoblotting. Results: The levels of β-ENaC, γ-ENaC and Na⁺, K⁺-ATPase α₁ collected at 18 h postnatally were significantly lower in CDH infants compared to control infants. In the CDH group, no significant difference in the expression of the ENaC subunits, Na⁺, K⁺-ATPase α₁ or aquaporin 5 could be detected between the two sampling time points. Conclusions: This downregulation may result in an abnormal lung fluid absorption which could be an important mechanism behind the respiratory distress seen in newborn CDH patients.

Serum cytokine levels in congenital diaphragmatic hernia: predictive impact for the need for extracorporeal membrane oxygenation?

Background: Congenital diaphragmatic hernia (CDH) is a common cause of neonatal respiratory failure and a malformation associated with high mortality and morbidity. Extracorporeal membrane oxygenation (ECMO) is one of treatment options for CDH. Despite the ultrasound based lung-to-head ratio and the fetal lung volume determination, there is no reliable prenatal parameter for the clinical course and outcome of fetuses with CDH, in particular for the need of ECMO. Aim: The objective of the study was to investigate serum cytokine profiles and immune response from newborns with CDH requiring ECMO compared to CDH infants without ECMO and healthy newborns. Methods: We used multiplexed immunoassays based on Luminex beat technology to measure cytokines (Eotaxin, GCSF, GMCSF, IFNa2, IFNg, IL10, IL12p40, IL12p70, IL13, IL15, IL17, IL1a, IL1b, IL2, IL3, IL4, IL5, IL6, IL7, IL8, IP10, MCP1, MIP1a, MIP1b, TNFa, TNFb) in serum of newborns with CDH receiving ECMO (n=16) and conventional ventilation (n=27). Serum was collected before (baseline) and 24, 48 and 72 hours during ECMO and ventilation. Cord blood cytokines from healthy term newborns served as controls (n=13). Results: From the examined cytokines IFNg, IL12p70, IL13, IL15, IL17, IL1b, IL2, IL4, IL5, and TNFb were found to be under the detection limit of 3.2 pg/ml in CDH and healthy newborns. The concentrations of remaining cytokines at baseline were significantly elavated in newborns with CDH compared to controls. ECMO group showed higher median levels of IL10 and IL8at baseline compared to ventilated CDH infants (97.4 vs. 46.2 pg/ml and 48.3 vs. 30.6 pg/ml, both p&lt;0.05). With cardiopulmory bypass, IL10 concentration showed a significant decreasing pattern. Baseline serum level and change in IL10 were not associated with fetal lung volume, pre-ECMO oxygenation-index and outcome of CDH. Conclusion: These findings suggest that inflammatory reaction and activation is associated with CDH. Thus, the high circulating levels and decreasing pattern of anti-inflammatory IL10 in CDH patients under ECMO treatment indicate that IL10 may be useful as a marker of the severity of CDH and as parameter for the need of ECMO intervention.

Diaphragm electromyogram in infants with abdominal wall defects and congenital diaphragmatic hernia

Measurement of the diaphragm electromyogram (EMGdi) elicited by phrenic nerve stimulation could be useful to assess neonates suffering from respiratory distress due to diaphragm dysfunction, as observed in infants with abdominal wall defects (AWD) or congenital diaphragmatic hernia (CDH). The study aims were to assess the feasibility of recording EMGdi using a multipair oesophageal electrode catheter and examine whether diaphragm muscle and/or phrenic nerve function was compromised in AWD or CDH infants. Diaphragm compound muscle action potentials elicited by magnetic phrenic nerve stimulation were recorded from 18 infants with surgically repaired AWD (n = 13) or CDH (n = 5), median (range) gestational age 36.5 (34-40) weeks. Diaphragm strength was assessed as twitch transdiaphragmatic pressure (TwP(di)). One AWD patient had prolonged phrenic nerve latency (PNL) bilaterally (left 9.31 ms, right 9.49 ms) and two CDH patients had prolonged PNL on the affected side (10.1 ms and 10.08 ms). There was no difference in left and right TwP(di) in either group. PNL correlated significantly with TwP(di) in CDH (r = 0.8; p = 0.009). Oesophageal EMG and magnetic stimulation of the phrenic nerves can be useful to assess phrenic nerve function in infants. Reduced phrenic nerve conduction accompanies the reduced diaphragm force production observed in infants with CDH.

Protocolized approach to the management of congenital diaphragmatic hernia: benefits of reducing variability in care

Purpose Variable approaches to the care of infants with congenital diaphragmatic hernia (CDH) by multiple providers may contribute to inconsistent care. Our institution developed a comprehensive evidence-based protocol to standardize the management of CDH infants. This report reviews patient outcomes before and after the implementation of the protocol. Methods Retrospective chart review of CDH infants managed with individualized care (preprotocol group, January 1997-December 2001, n = 22) or on the protocol (Protocol group, January 2002-July 2009, n = 47). Survival and other categorical variables were compared by χ 2 analysis, and continuous variables were compared using 1-sided analysis of variance analysis, with significance defined as P < .05. Results Survival to discharge was significantly greater in the Protocol group (40/47; 85%) than the preprotocol group (12/22; 52%; P = .006), although mean gestational age, mean birth weight, and expected survival were not statistically different between the 2 groups. The use of supportive therapies, including high-frequency jet ventilation, inhaled nitric oxide, and extracorporeal life support, was similar between groups as well. Conclusions Since the implementation of a management protocol for infants with CDH, survival has improved significantly compared with expected survival and preprotocol controls. Reduction in the variability of care through use of an evidence-based protocol may improve the survival of CDH infants.

Impact of Target Blood Gases on Outcome in Congenital Diaphragmatic Hernia (CDH)

Neonatal intensive care unit (NICU) stabilization strategies which normalize physiology according to predetermined blood gas targets may contribute to observed improved survival rates of patients with CDH. The purpose of our study was to compare risk-adjusted outcomes of CDH patients managed with or without blood gas targets established at NICU admission. Cases were collected from a national CDH network between May 2005 and November 2007. On NICU admission, the responsible neonatologist was asked to establish target ranges for pH, pCO (2), pO (2), and pre/post-ductal O (2) saturation. The outcomes analyzed were mortality, need for ECMO, days of mechanical ventilation/supplemental oxygen, and length of stay. Of 147 CDH infants, 63 had admission blood gas targets. Severity of illness and gestational age in both groups were comparable (SNAP-II score). Infants with blood gas targets had a significantly lower mortality than those without (Hazard ratio 0.27, p=0.006). Blood gas targets for the management of infants with CDH are associated with improved survival. Although the willingness to create and use stabilization targets to guide early NICU care may be a surrogate for other factors (experience, staffing, lack of interest), it is clearly associated with improved survival in CDH.

Management of prenatally diagnosed congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a congenital anomaly that presents with a broad spectrum of severity dependent upon components of pulmonary hypoplasia and pulmonary hypertension. While advances in neonatal care have improved the overall survival of CDH in experienced centers, mortality and morbidity remain high in a subset of CDH infants with severe CDH. The most important prenatal predictor of outcome in left-sided CDH is liver position. More precise and reproducible prenatal predictive parameters need to be developed to allow standardization of results between centers and appropriate design of clinical trials in CDH. Thus far, all randomized trials comparing prenatal intervention to standard postnatal therapy have shown no benefit to prenatal intervention. Although recent non-randomized reports of success with balloon tracheal occlusion (and release) are promising, prenatal therapy should not be widely adopted until a well-designed prospective randomized trial demonstrating efficacy is performed. The increased survival and subsequent morbidity of CDH survivors has resulted in the need to provide resources for the long-term follow-up and support of the CDH population.

The effect of surgery on lung volume and conventional monitoring parameters in ventilated newborn infants

In newborn infants, thoraco-abdominal surgery is a serious intervention with respect to gas exchange and lung mechanics. This prospective clinical study compared surgery-induced changes in functional residual capacity (FRC) and ventilation inhomogeneity (VI) indices with changes in conventional monitoring parameters. Of 29 ventilated newborns (mean weight 2,770+/-864 g at surgery), 13, nine and seven underwent thoracic, abdominal or congenital diaphragmatic hernia (CDH) surgery, respectively. The multiple breath washout (MBWO) technique using heptafluoropropane as tracer gas (Babylog 8000; Dräger, Lübeck, Germany) was performed <6 h before surgery, 22-24 h after surgery and <6 h before extubation. Gas exchange, respiratory mechanics, FRC and VI index data were recorded. Thoraco-abdominal surgery resulted in changes to FRC and VI indices in a procedure-specific manner; however, these changes were not reflected in conventional mechanical or ventilatory monitoring parameters. FRC decreased in non-CDH infants, while FRC increased and VI indices decreased in CDH infants. Despite improvements, the differences in FRC and VI between CDH and non-CDH infants indicated persistent impaired lung function in CHD infants. MBWO can be advantageously used to measure the effect of surgery on the lung. While FRC and VI indices changed following surgery, conventional monitoring parameters did not.

Impaired somatic growth and delayed lung development in infants with congenital diaphragmatic hernia—evidence from a 10-year, single center prospective follow-up study

Purpose In infants with congenital diaphragmatic hernia (CDH), somatic growth and pulmonary development are key issues beyond the time of intensive care treatment. The aim of the study was to investigate the somatic growth and pulmonary function after discharge and to compare CDH patients with a group of matched controls. Methods Anthropometric measurements and lung function tests were performed in 26 infants after surgical repair of CDH and 26 non-CDH intensive care patients, matched for gestational age and birth weight. Spontaneously breathing infants were tested at a mean of 44 weeks postconceptional age (range, 36-58 weeks). Body weight, body length, respiratory rate (RR), tidal volume (V T), functional residual capacity by body plethysmography (FRC pleth), respiratory compliance (C rs), and respiratory resistance (R rs) were measured. Results The mean (SD) weight gain per week in the CDH infants was significantly lower compared to non-CDH infants (89 [39] g vs 141 [49] g; P = .002). The breathing pattern between both groups differed considerably. In CDH infants, V T was significantly lower ( P < .001) and RR significantly higher ( P = .005). The respiratory compliance was also significantly ( P < .001) reduced, whereas R rs did not differ significantly. No statistically significant differences were seen in FRC pleth related to the body weight between CDH and non-CDH infants (20.3 [4.4] mL/kg vs 21.5 [4.9] mL/kg). Conclusion Despite apparently well-inflated lungs after surgery, evidence of early and significantly reduced weight gain and impaired lung function in CHD patients should prompt careful dietary monitoring and regular lung function testing.

Assessment of Right Ventricular Function Using Tissue Doppler Imaging in Infants with Pulmonary Hypertension

Background: In infants with pulmonary hypertension (PHT), right ventricular (RV) function may be altered and contribute to disease severity. Tissue Doppler imaging (TDI) is a new echocardiographic modality which directly measures myocardial velocities and may allow quantitative assessment of systolic and diastolic ventricular function in infants. Objective: To measure and compare RV myocardial velocities in infants with PHT and in normal control infants, using TDI. Methods: This was a prospective case-control study. Twenty-eight control infants and 15 infants with PHT, of whom 11 had congenital diaphragmatic hernia (CDH), were recruited. TDI was used to obtain systolic and diastolic myocardial velocities in the RV and interventricular septum in all infants. Results: There were significant reductions in systolic isovolumic contraction velocity (IVV; 5.3 vs. 6.6 cm/s) and systolic ejection velocity (S; 6.6 vs. 9.2 cm/s) in the PHT group compared to the control group. Early diastolic myocardial velocity, E’, was also significantly reduced in the RV in the PHT infants compared to controls (–4.3 vs. 8.6 cm/s). The same significant reductions in systolic and early diastolic TDI velocities were observed in the subgroup of CDH infants alone. Conclusions: TDI permits non-invasive assessment of RV myocardial velocities in infants. Reduced systolic and diastolic velocities in PHT may represent impaired systolic contraction and early diastolic relaxation. Therapies which target inotropic and lusitropic function may be appropriate in infants with PHT and RV dysfunction. The load-dependency of TDI measures in infants and the effects of specific therapies on RV function in PHT require further investigation.