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

Persistent fetal circulation (PFC) causes severe pulmonary insufficiency in patients who have demonstrated adequate lung function following diaphragmatic hernia repair. Patent ductus arteriosus (PDA) ligation corrects this condition, but carries the risk of sudden right ventricular failure. Pharmacologic reversal of PFC may be attempted, and if unsuccessful, prolonged venoarterial bypass becomes necessary to provide effective pulmonary support. PDA ligation can then be performed safely and maturation of the pulmonary vasculature allowed to occur. Pulmonary artery pressure monitoring is essential.
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PMID:Management of pulmonary insufficiency in diaphragmatic hernia using extracorporeal circulation with a membrane oxygenator (ECMO). 59 70

One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [PCO2] < 50; partial pressure of oxygen [PO2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.
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PMID:Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia. 141 95

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

The ductus venosus (DV) connects the portal venous system to the inferior vena cava. Rarely, the umbilical venous drainage is anomalous, either connecting to the portal sinus within the liver or having an extrahepatic connection, bypassing the liver and draining to one of a variety of sites, including to the heart directly. Prenatally, there is a recognized association of anomalous umbilical venous drainage with aneuploidy and other structural malformations. The fetus may also develop right heart failure because of unregulated volume loading. We report the postnatal development of diaphragmatic hernia in three fetuses with absent DV and umbilical venous drainage to the right atrium directly in two cases and to the coronary sinus in the third. In all fetuses, the abnormality was well-tolerated in pregnancy, with only a modest degree of right heart dilatation. All three neonates underwent repair of the diaphragmatic hernia and made a good recovery.
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PMID:Diaphragmatic hernia: a postnatal complication of anomalous drainage of the umbilical vein. 2328 7

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

Trans-diaphragmatic intercostal hernia is a rare entity. Patient with multiple medical comorbidities, including obstructive sleep apnoea, presents with shortness of breath, leg oedema and a bulging swelling through the right chest wall. CT shows partial herniation of the right lung and liver through intercostal space and an echocardiogram reveals right heart failure. He was treated initially with continuous positive airway pressure with poor response and subsequently treated with adaptive servo ventilation with much better symptomatic relief and treatment tolerance.
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PMID:Non-traumatic trans-diaphragmatic intercostal hernia and cor pulmonale in a patient with poorly controlled obstructive sleep apnoea. 2779 67

Pulmonary arterial hypertension (PAH) is a rare disease in infants and children that is associated with significant morbidity and mortality. The disease is characterized by progressive pulmonary vascular functional and structural changes resulting in increased pulmonary vascular resistance and eventual right heart failure and death. In many pediatric patients, PAH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. PAH associated with developmental lung diseases such as bronchopulmonary dysplasia or congenital diaphragmatic hernia is increasingly more recognized in infants and children. Although treatment of the underlying disease and reversal of advanced structural changes have not yet been achieved with current therapy, quality of life and survival have improved significantly. Targeted pulmonary vasodilator therapies, including endothelin receptor antagonists, prostacyclin analogs, and phosphodiesterase type 5 inhibitors have resulted in hemodynamic and functional improvement in children. The management of pediatric PAH remains challenging as treatment decisions depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts. This article reviews the current drug therapies and their use in the management of PAH in children.
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PMID:Drug Treatment of Pulmonary Hypertension in Children. 3196 Mar 61

Congenital diaphragmatic hernia (CDH) is a rare disease, which affects 1 in 2,500 newborns. Congenital diaphragmatic hernia can interfere with the normal development of the pulmonary parenchyma and vascular bed, and in severe cases, it can lead to the development of severe pulmonary arterial hypertension (PAH) and right ventricular failure. We present a neonate with CDH who developed severe PAH and right ventricular dysfunction and was managed with a unique strategy combining venoarterial extracorporeal membrane oxygenation, prostaglandin E1, and a variety of PAH therapies.
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PMID:Combination of PGE1 and Pulmonary Vasodilator Therapy in Managing a Challenging Case of Severe PAH Secondary to CDH. 3264 88