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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four neonates (three having undergone repair of a congenital diaphragmatic hernia and developing a persistent fetal circulatory pattern and one having severe infant respiratory distress syndrome) have been supported with prolonged extracorporeal membrane oxygenation (ECMO) at Children's Hospital of Pittsburgh between December of 1979 and April of 1980. Three have survived. This encouraging experience indicates that the pattern of persistent fetal circulation in the newborn infant who has undergone repair of a diaphragmatic hernia can be successfully managed with ECMO even when efforts to lower pulmonary hypertension and improve oxygenation with vasodilators (tolazoline, phenothiazine, acetylcholine, or prostaglandin E1) and have been ineffective. The effectiveness and safety of ECMO is convincing enough to warrant its consideration as therapy for congenital diaphragmatic hernia and persistent fetal circulation prior to the use of vasodilators.
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PMID:Extracorporeal membrane oxygenation. Successful treatment of persistent fetal circulation following repair of congenital diaphragmatic hernia. 720 62

We report a case of congenital diaphragmatic hernia in which aneurysmal dilatation of ductus arteriosus developed after lipo-prostaglandin E1 (PGE1) therapy for persistent fetal circulation. After surgery Lipo-PGE1 was used at a dosage of 5 ng/kg per minute from days 2-9 during which aneurysmal dilatation (7 mm in diameter) developed and remained thereafter. Ductus arteriosus ligation was then performed. Lipo-PGE1 may promote ductus arteriosus dilatation in cases of persistent fetal circulation.
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PMID:Aneurysmal dilatation of ductus arteriosus during lipo-prostaglandin E1 therapy for diaphragmatic hernia. 827 14

We investigated Doppler flow patterns through the ductus arteriosus (DA) of 13 newborn infants with congenital diaphragmatic hernia (CDH) using color Doppler ultrasonography (CDUS). Patterns were classified into 3 types: left-to-right (L-R), bidirectional (BD) and right-to-left (R-L) shunting. Among the 13 patients examined, 3 showed L-R shunting, 5 BD shunting and 5 R-L shunting. Patients with L-R shunting showed significantly better levels of PaO2 and AaDO2 than those with other Doppler flow patterns. However, there were no differences in clinical findings between patients with BD and R-L shunting. All patients with L-R shunting survived after CDH repair without pre-operative stabilization including Lipo-PGE1 (LPE) administration. Four of the 5 patients with BD shunting survived, but only one of the 5 patients with R-L shunting survived after CDH repair following administration of LPE. It was suggested that Doppler flow patterns through the DA may be useful for predicting prognoses and selecting suitable treatment for CDH.
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PMID:Doppler flow patterns through the ductus arteriosus in patients with congenital diaphragmatic hernia. 1087 75

To date, uniform standards for congenital diaphragmatic hernia (CDH) management have not existed. The purpose of this study was to evaluate the evolving clinical outcome of the patients with CDH and to present our recent management protocol using echocardiography. Sixty patients treated for CDH at our hospital from 1978 through 2001 were reviewed. Periods of treatments were divided arbitrarily into three periods;1978-1991 (period I, n=26), 1992-1994 (period II, n=6), 1995-2001 (period III, n=28). Immediate repair was performed during period I. We performed preoperative stabilization and delayed repair since the start of period II, and nitric oxide (NO) was introduced in period III. In period III, our management strategy was the use of fentanyl for sedation and analgesia; vasoactive agents such as dopamine, dobutamine, and prostaglandin E1 in selected cases; the use of high-frequency oscillating ventilation (HFOV), inhaled NO; and venovenous extracorporeal membrane oxygenation (ECMO) if indicated. The details of stabilization management and the timing of surgery were determined using echocardiography to evaluate pulmonary hypertension (PH) by measuring dimension and shunt patterns through the ductus arteriosus (DA), right pulmonary artery (rPA) and left pulmonary artery (lPA). Overall, 42 of 60 patients survived (70%). The number of patients surviving in each period was 14 of 26 (54%) in period I, 4 of 6 (67%) in period II, and 24 of 28 (86%) in period III. Seventeen of 28 patients in period III required inhaled NO (group A). Of these 17 patients, 5 required ECMO; of these 5, 3 were long-term survivors. The remaining 11 patients from period III who were managed without NO (group B) survived. In left-sided CDH cases, the dimension of DA at admission in group A (5.07+/-1.79 mm) was significantly larger than in group B (2.99+/-1.68 mm) (P<0.01). The dimension of rPA in group A (3.37+/-0.80 mm) was significantly smaller as compared with group B (4.28+/-0.72 mm) (P<0.01). Although the dimension of lPA was not significantly different between group A (3.03+/-0.74 mm) and group B (3.46+/-0.48 mm), lPA blood flow was noticeably stronger in group B. DA shunt patterns were bi-directional (53%), right-to-left (40%) and left-to-right (7%) in group A, whereas no patients in group B showed a right-to-left shunt pattern. After confirmation of closure of DA or dominant left-to-right shunt, and marked increase of pulmonary arterial blood flow, patients in both group A and B underwent surgery successfully. In four non-survivors, findings of improving PH were not observed. We conclude that echocardiographic examination is useful to manage persistent pulmonary hypertension with recent treatment modalities including NO and HFOV and to determine the proper timing of surgery, which contributes to an improved outcome of CDH.
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PMID:Congenital diaphragmatic hernia: efficacy of ultrasound examination in its management. 1268 43

Congenital diaphragmatic hernia is a rare entity in childhood carrying a high mortality rate of 30% to 50%. Ipsilateral pulmonary hypoplasia, increased pulmonary vascular resistance, and potential cardiac failure complicate early postnatal life. Surgical correction can either be performed on the first day of life or be deferred to a time after stabilization of the infant. Our patient presented with a left-sided Bochdalek's hernia containing large and small bowel. She required intubation and resuscitation on day 1 of life, and surgical repair had to be postponed. Further respiratory deterioration required commencement of inhaled nitric oxide and high-frequency ventilation. Pulmonary artery pressure rose to suprasystemic level. Closure of the ductus arteriosus on day 8 resulted in imminent right-sided heart failure. Commencement of alprostadil (prostaglandin E1) reopened the ductus and stabilized the patient. Surgical repair was successful 3 days later. Alprostadil should be considered as an important component of therapy in severe cases of congenital diaphragmatic hernia, where deterioration of right-sided heart function occurs.
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PMID:Prevention of heart failure in the management of congenital diaphragmatic hernia by maintaining ductal patency. A case report. 1656 66

Pulmonary hypertension can complicate the early clinical course in newborn infants with congenital diaphragmatic hernia. We report the successful use of combination therapy with enteral sildenafil and intravenous prostaglandin E1 in a preterm infant with severe pulmonary hypertension and right heart failure early after congenital diaphragmatic hernia repair.
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PMID:Combination pharmacotherapy for severe neonatal pulmonary hypertension. 1663 Mar 27

Clinically significant pulmonary hypertension (PHTN) is a common finding in newborn infants with congenital diaphragmatic hernia (CDH) resulting in right to left shunting at pre- and postductal level, hypoxemia, and acute right heart failure in those most severely affected. Even in those without clinical manifestations of ductal shunting, cardiac echo studies would suggest that increased pulmonary vascular resistance and right ventricular pressures are almost a universal finding in this disease, and in some instances, may persist well into the postnatal period. The lung is small and structurally abnormal, and the pulmonary vascular bed is not only reduced in size, but responds abnormally to vasodilators. During the last 20 years, "gentle" ventilation, delayed surgery, and improved peri-operative care have made the greatest impact in decreasing mortality in this condition. Use of PGE1 should be considered early if there is hemodynamically significant PHTN, right ventricular dysfunction, and the patent ductus arteriosus (PDA) is becoming restrictive. In individual patients, inhaled nitric oxide (iNO) might be helpful, but the response to iNO should be confirmed using echocardiography. In patients who survive operation and leave the hospital, there are chronic causes of morbidity that need to be looked for and managed in a multi-disciplinary follow-up clinic.
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PMID:Pulmonary hypertension in congenital diaphragmatic hernia. 1746 65

We report a neonate with left congenital diaphragmatic hernia and severe left ventricular dysfunction, in whom the blood flow in the transverse arch and its branches was supported in a retrograde fashion by patent ductus arteriosus. There was only minimal antegrade flow across the aortic valve and hemodynamic physiology resembled critical aortic stenosis, necessitating the immediate use of prostaglandin E1 infusion to maintain the patent ductus arteriosus.
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PMID:Functional critical aortic stenosis with transient retrograde flow in a neonate with left diaphragmatic hernia. 2267 27

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of failed circulatory adaptation at birth, seen in about 2/1000 live born infants. While it is mostly seen in term and near-term infants, it can be recognized in some premature infants with respiratory distress or bronchopulmonary dysplasia. Most commonly, PPHN is secondary to delayed or impaired relaxation of the pulmonary vasculature associated with diverse neonatal pulmonary pathologies, such as meconium aspiration syndrome, congenital diaphragmatic hernia, and respiratory distress syndrome. Gentle ventilation strategies, lung recruitment, inhaled nitric oxide, and surfactant therapy have improved outcome and reduced the need for extracorporeal membrane oxygenation (ECMO) in PPHN. Newer modalities of treatment discussed in this article include systemic and inhaled vasodilators like sildenafil, prostaglandin E1, prostacyclin, and endothelin antagonists. With prompt recognition/treatment and early referral to ECMO centers, the mortality rate for PPHN has significantly decreased. However, the risk of potential neurodevelopmental impairment warrants close follow-up after discharge for infants with PPHN.
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PMID:Update on PPHN: mechanisms and treatment. 2458 Jul 63

Persistent pulmonary hypertension of the newborn (PPHN) is the most common neonatal form and mostly reversible after a few days with improvement of the underlying pulmonary condition. When pulmonary hypertension (PH) persists despite adequate treatment, the severity of parenchymal lung disease should be assessed by chest CT. Pulmonary vein stenosis may need to be ruled out by cardiac catheterisation and lung biopsy, and genetic workup is necessary when alveolar capillary dysplasia is suspected. In PPHN, optimisation of the cardiopulmonary situation including surfactant therapy should aim for preductal SpO2between 91% and 95% and severe cases without post-tricuspid-unrestrictive shunt may receive prostaglandin E1 to maintain ductal patency in right heart failure. Inhaled nitric oxide is indicated in mechanically ventilated infants to reduce the need for extracorporal membrane oxygenation (ECMO), and sildenafil can be considered when this therapy is not available. ECMO may be indicated according to the ELSO guidelines. In older preterm infant, where PH is mainly associated with bronchopulmonary dysplasia (BPD) or in term infants with developmental lung anomalies such as congenital diaphragmatic hernia or cardiac anomalies, left ventricular diastolic dysfunction/left atrial hypertension or pulmonary vein stenosis, can add to the complexity of the disease. Here, oral or intravenous sildenafil should be considered for PH treatment in BPD, the latter for critically ill patients. Furthermore, prostanoids, mineralcorticoid receptor antagonists, and diuretics can be beneficial. Infants with proven or suspected PH should receive close follow-up, including preductal/postductal SpO2measurements, echocardiography and laboratory work-up including NT-proBNP, guided by clinical improvement or lack thereof.
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PMID:Pulmonary hypertension associated with acute or chronic lung diseases in the preterm and term neonate and infant. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. 2705 98


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