Prostaglandin E1 Infusion Research Articles

Management of newborns with duct-dependent congenital heart disease with prostaglandin E1 – Slovenian guidelines

Background: Early recognition of duct-dependent congenital heart disease in newborns with duct-dependent pulmonary or systemic blood flow or transposition of the great arteries is critical for early introduction of prostaglandin E1 (PGE1), which allows stabilization of the newborn till cardiac catheterization or surgical intervention. Indications for PGE1, management of newborns with duct-dependent congenital heart disease and interventions in case of side effects of PGE1 are reviewed. Conclusions: It is critical to start with PGE1 infusion as soon as we clinically suspect ductdependent congenital heart disease. With this approach, morbidity and mortality of newborns with congenital heart disease is significantly reduced.

Pulse Diode Laser Irradiation (830 nm) of Lumbosacral Spinal Roots Diminished Hyperreflexia-Induced by Acetic Acid or Prostaglandin E2 Infusion in Rat Urinary Bladder.

Low power diode Iaser (830 nm) irradiation is a useful analgesic tool in superficial pain. Pulse laser irradiation allows us to increase the laser power because the non-irradiation time reduces heating effects and/or direct tissue damage at the irradiation area. This new irradiation device using pulse laser was applied to the dorsal skin to investigate the effects on the micturition reflex in the rat by targeting underlying sacral spinal roots. Vesical pressure measurement during the continuous infusion of the urinary bladder with saline, acetic acid (AA, 0.1%) or prostaglandin E2 (PGE2 , 10(-5) M) were performed in un-anesthetized rats. Multi-unit recording from bladder afferent nerves preformed under urethane anesthesia. Laser irradiation, either continuously at 1 W or in 10 W-pulse mode, was delivered at 830 nm from 1.5 cm above the skin at the lumbosacral joint. During continuous saline infusion to the urinary bladder, neither continuous (1 W) nor pulse (10 W) laser irradiation altered the intercontraction interval and nerve firing during distention of the bladder. Pulse laser, but not continuous laser irradiation, increased the intercontraction interval with AA or PGE2 infusion and diminished nerve firing during distention of the bladder with AA or PGE2 infusion. These data indicate that pulse laser could diminish inflammation related nerve firing from the bladder. Since this laser irradiation did not affect the normal bladder distention elicited nerve firing, it appears capable of reducing urgency sensation without loss of the basic micturition reflex.

The role of peripheral vascular resistance in determining the infrainguinal arterial reconstruction patency

To find if there is any correlation between the peripheral vascular resistance, its change following an intragraft prostaglandin infusion and the infrainguinal reconstruction patency. Ninety-seven patients with infrainguinal reconstructions were included in the study: in 48 patients they were compromised (32 with graft thrombosis and 16 with stenosis of the distal anastomoses); 49 patients had their bypasses patent for no less than 12 months. Intraoperative flowmetry was performed on the target artery under the distal anastomosis, after declamping, and after a five-minute intragraft prostaglandin infusion. We measured the peripheral vascular resistance (PVR) by two methods - as a ratio of the invasively measured average pressure to the average blood flow volume (mmHg/ml/min. = peripheral resistance unit [PRU]) and by using the readings by the flowmeter (ohms). The decrease of peripheral resistance was calculated in the functioning and the compromised reconstructions after administration of prostaglandin. We found that if PVR decreases 4.5 times (in ohms) the prognosis is good; we can make the same positive prognosis when the ratio of the mean invasively measured pressure to the mean blood flow volume (Pmean/Qmean) decreases more than four times. Values greater than 1.07 ohms, after peripheral vasodilatation, are indicative of high peripheral vascular resistance, at a level of specificity of 86%, and values greater than 0.57 PRU - at a level of specificity of 87%. Although PVR measurements cannot predict with absolute certainty that bypasses under the inguinal ligament shall stay patent for a long time, it is a valuable indicator showing the immediate outcome of reconstruction work carried out with the patient on the operating table. Finding Any technical errors and dealing with them saves time and money, as well as prevents the stress on the part of patients caused by the required additional revisions and multiple operations.

Necrotizing enterocolitis in neonates undergoing the hybrid approach to complex congenital heart disease*

To investigate the prevalence of necrotizing enterocolitis (NEC) in neonates undergoing the Stage I hybrid procedure for palliation of complex congenital heart disease (CHD). Neonates undergoing the Norwood surgery for hypoplastic left-heart syndrome have the highest risk for NEC of all CHD patients. The hybrid procedure is another palliative option for hypoplastic left-heart syndrome, but NEC in neonates undergoing this procedure has not been reported. Retrospective chart review of 73 neonates who underwent the hybrid procedure for palliation of complex CHD. Demographic, perinatal, perioperative, clinical, and procedural data were collected. NEC was defined as modified Bell's Stage II and above. The cardiothoracic and neonatal intensive care units in a large free-standing children's hospital. All neonates who underwent the hybrid Stage I procedure for the palliation of complex CHD from April 2002 through April 2008. Seventy-three neonates were reviewed and 11.0% (eight of 73) developed NEC. Of the patients with NEC, 37.5% (three of eight) died and two patients required abdominal surgery. Earlier gestational age (< 37 wks), lower maximum dose of prostaglandin infusion, and unexpected readmission to the intensive care unit were statistically associated with NEC (p = .009, 0.02, and 0.04, respectively). No other demographic, perinatal, perioperative, clinical, or procedural variables were associated with the development of NEC in this patient population, including enteral feeding regimens, umbilical artery catheters, inotrope use, and average oxygen saturation and diastolic blood pressure. The prevalence of NEC in patients undergoing the hybrid procedure is comparable to that reported for neonates undergoing the Norwood procedure. Earlier gestational age is a significant risk factor for NEC in patients who undergo the hybrid Stage I procedure. Multidisciplinary approaches to better understand abdominal complications and to develop feeding regimens in neonates undergoing the hybrid approach to complex CHD are needed to improve outcomes and decrease morbidities.

Critical Pulmonary Stenosis

A 20-day-old girl was diagnosed with critical pulmonary valvular stenosis with patent ductus arteriosus (PDA). She underwent surgical pulmonary valvotomy and infundibular resection. A trial snaring of the PDA resulted in significant systemic desaturation, and the PDA was left undivided. A continuous infusion of prostaglandin was used to keep the PDA open for the next 8 days. The PDA acted as a "natural systemic-to-pulmonary shunt" to provide pulmonary blood flow until right ventricular compliance and function improved. The various causes of persistent desaturation following pulmonary valvotomy are discussed.

Do High-Risk Infants Have a Poorer Outcome From Primary Repair of Coarctation? Analysis of 192 Infants Over 20 Years

Balloon angioplasty for infant coarctation is associated with a high recurrence rate, making operative repair the gold standard for low-risk infants. Debate exists as to whether high-risk infants might be better served with primary angioplasty. We compared the outcome in high-risk versus low-risk infants over 20 years, in a center that always used surgical repair as the primary intervention. Of 192 infants from 1986 to 2005, 56 were considered "high-risk," defined as requiring prostaglandin infusion together with either epinephrine infusion for 24 hours preoperatively, or ventilation and milrinone infusion for 24 hours preoperatively. All high-risk patients had a period of ventricular dysfunction prior to surgery, ranging from mild to severe. Outcomes were compared using Bonferroni comparison of means or the Fischer exact test as appropriate. Although the high-risk patients were smaller (3.3 ± 0.1 vs 4.2 ± 0.2 kg, p < 0.01), younger (18 ± 4 vs 57 ± 7 days, p < 0.01), and more often required a concomitant pulmonary artery band (25% vs 15%, p = 0.05), their cross-clamp times were the same as the low-risk patients (18.9 ± 0.9 vs 18.0 ± 0.4 minutes, p = 0.27) and there was no difference in postoperative morbidity (7% vs 3%, p = 0.11). However, there was a trend toward higher perioperative mortality (7% vs 2%, p = 0.07). When compared with the published studies of primary angioplasty in comparable high-risk infants, the mortality rate in our surgically treated high-risk group is much lower. Additionally, only 11% of our high-risk group required reintervention, with two-thirds treated successfully with a single angioplasty at 3.8 ± 2.2 years later, far lower than recurrence rates with primary angioplasty. We propose that primary surgical repair of coarctation in infants who are high risk should be the primary treatment, with angioplasty reserved for recurrent coarctation.

Le shunt circulaire : complication de la maladie d’Ebstein de forme néonatale

We report a severe neonatal presentation of Ebstein anomaly with homodynamic aggravation at birth attributed to patent ductus arteriosus and circular shunt. Prenatal diagnosis of Ebstein anomaly of the tricuspid valve with functional pulmonary atresia was made at 37 weeks gestation by fetal two- and three-dimensional echocardiography. A cesarean section was performed in view of poor fetal tolerance. The newborn was born with hydrops and multivisceral failure. A post-natal echocardiography demonstrated a left-to-right shunt across the patent ductus arteriosus and functional pulmonary atresia with severe pulmonary insufficiency with absent forward flow. This created a circular shunt, where blood flowed through the ductus to the pulmonary arteries, retrograde through the pulmonary artery and Ebstein valve, across the patent foramen ovale and out the aorta. Prostaglandin E1 infusion was stopped, resulting in clinical and echocardiographic improvement. The management of the phenomenon of a circular shunt across a patent ductus arteriosus with Ebstein malformation involves promoting early ductal closure by stopping prostaglandin therapy.

Updated American College of Critical Care Medicine-Pediatric Advanced Life Support Guidelines for Management of Pediatric and Neonatal Septic Shock

Shock is a major preventable cause of morbidity and mortality in children referred to emergency care. The recently updated American College of Critical Care Medicine guidelines for the management of newborns and children with septic shock emphasize the role of emergency care in improving survival and functional outcomes. Implementation of these guidelines of stepwise use of fluids, antibiotics, and, if necessary, inotropes within the first hour of admission to the emergency department can reduce mortality and neurological morbidity risks 2-fold. Therapies should be goal directed to maintain age-specific threshold heart rates and blood pressure as well as a capillary refill of less than 3 seconds or 2 seconds or less. Inotropes should be delivered through peripheral intravenous or intraosseous access when central access is unavailable because delay in inotrope delivery can greatly increase mortality risks. Emergency care systems should be organized to facilitate recognition, triage, and treatment of shock in the first hour. Emergency departments should be stocked with ready access to antibiotics, fluids, and inotrope infusions, and clinicians should be trained in the delivery of goal-directed fluid, antibiotics, and inotrope therapies in the first hour of resuscitation. For newborns, in addition to fluids, antibiotics, and inotropes, a prostaglandin infusion should be available within 10 minutes if duct-dependent congenital heart disease is a possibility.

611 Pattern of Life Threatening Congenital Heart Diseases - Experience From 2 District General Hospitals in Uk

Aim: To look at pattern, clinical presentation and initial management of life threatening congenital heart defects. Methods: Retrospective audit focused on diagnosis and initial management of babies who presented with life threatening congenital heart diseases to 2 district general hospitals in UK from March 2007 to July 2009. Results: 30 babies with life threatening congenital heart defects were identified. 30% were suspected antenatally. 80% presented within few hours after birth with cyanosis. 40% had murmurs. Diagnosis was confirmed in 29 by urgent echocardiogram performed by a paediatrician with expertise in cardiology and in 1 antenatally suspected case at cardiac centre. Most frequent defects were: Transposition of great arteries (including complex) 11, Hypoplastic left heart syndrome 3; Truncus Arteriosis 3; Pulmonary Atresia 2; Total Anomalous Pulmonary Venous Connection 2, Tetralogy of Fallot 2; and Congenitally Corrected TGA with other defects 2; Other defects (n=1) were Double outlet right ventricle with Ventricular Septal Defect (VSD), Coarctation of aorta, Critical pulmonary stenosis, Complete AVSD and Large VSD. 19 of 20 babies with duct dependent lesions were managed immediately with prostaglandin infusion and all 6 cases with cardiac failure treated with diuretics. 2 sick babies underwent life saving balloon septostomy in these hospitals performed by outreach cardiac team. 30 day mortality was 16%. Conclusion: These were complex, critical and most commonly cyanotic heart diseases, majority undetected antenatally. Commencing prostaglandin is crucial in suspected duct dependent lesions. Routine post-natal pulse-oximetry and facility to have echocardiography in district general hospitals help in appropriate management.

Unusual cause of neonatal cyanosis

We present a case of a full-term female neonate who presented at 6 h of age with severe cyanosis and was partially responsive to oxygen supplementation. An echocardiogram showed an isolated congenital severe tricuspid valve insufficiency due to rupture of the papillary muscle of the anterior tricuspid valve leaflet. Magnesium sulfate was infused to lower the pulmonary resistance and thus enhancing the antegrade pulmonary blood flow. Ductal patency was secured by prostaglandin infusion thus providing an additional pulmonary blood flow through the ductus arteriosus. The above measures were adequate to stabilize the patient with no further deterioration or the need for other supportive measures such as Nitric Oxide therapy or extracorporeal membrane oxygenation (ECMO). Therefore, early diagnosis and adequate measures to improve the pulmonary blood flow are mandatory, important pre-operative measures in the management of these patients.

Constant Intravenous Infusions of Prostaglandin E1 in Severe Peripheral Vascular Disease

Constant Intravenous Infusions of Prostaglandin E1 in Severe Peripheral Vascular Disease

Effects of Intravenous Prostaglandin E1 on Pain and Body Temperature in Patients with Post-Herpetic Neuralgia

Bathing, heating, or sympathetic blockade often alleviates pain due to post-herpetic neuralgia (PHN), suggesting that blood flow may affect PHN pain. Here, we examined the effect of prostaglandin E1 (PGE), which improves blood circulation, on pain and body temperature in patients with PHN. Twenty-four patients with PHN were enrolled in a randomized, double-blind, placebo-controlled, crossover study. After 30-minute rest in a temperature-controlled (20 degrees C) and intravenous cannulation, patients were randomized to receive either 60 microg of PGE dissolved in 100 mL of saline, or 100 mL of saline at an infusion rate of 0.03 microg/kg/min. Following a 7-day washout period, patients were crossed over to receive the other treatment. The visual analog scale (VAS) of ongoing pain and tactile allodynia, and skin and the tympanic temperatures were measured before and after infusion. The two solutions significantly decreased the VAS for ongoing pain, and the reduction was greater with PGE than placebo (P < 0.05). Although the temperatures of the forehead, hand, foot, chest, abdomen, and the most painful region were significantly increased in the two solutions, the elevation in the foot and the most painful region was greater with PGE than placebo (P < 0.01). The tympanic temperature did not significantly change following infusion of PGE. Intravenous infusion of PGE produces analgesia associated with elevation of skin temperature in patients with PHN.

Persistent Hypoxemia and Pulmonary Hypertension in a 3-day-old Infant

### History of Present Illness A 3 day-old ex-term caucasian female with persistent hypoxemia and pulmonary hypertension presents from an outside community neonatal intensive care unit (NICU) for possible extracorporeal membrane oxygenation (ECMO) therapy. ### Prenatal History The patient was born to a 27-year old gravida four mother who received prenatal care from early in the first trimester. An initial prenatal ultrasound showed a two-vessel cord and bilateral hydronephrosis; a higher-level ultrasound did not show any evidence of congenital heart disease. ### Family History There is no history of consanguinity in the family. The patient’s aunt has congenital heart disease, and the brother has hydrocephalus and hydronephrosis of unknown etiology. ### Birth History She was a 3500 gram product of an estimated 39 weeks gestation born via repeat cesarean section with rupture of membranes and clear amniotic fluid at the time of delivery. She received routine care at delivery and transitioned well with Apgars of 8 and 9 at 1 and 5 minutes respectively. ### Initial Presentation She was initially noted to be dusky in the newborn nursery 4 hours after delivery and was transferred emergently to the NICU for evaluation. An initial chest radiograph showed a small cardiac silhouette with clear lung fields; the initial CBC did not have findings suggestive of infection. Congenital heart disease was suspected after a hyperoxia test showed a pre-ductal paO2 of 71 mmHg. A prostaglandin E1 infusion was started and she was transferred to a higher level of care for further evaluation. At an outside Level III NICU, her hypoxemia worsened, and she was eventually transitioned to high frequency oscillatory ventilation with a peak mean airway pressure of 17 cm H20 and 100% oxygen. A postnatal echocardiogram …

An Unusual Case of Congenitally Unguarded Tricuspid Valve Orifice Associated with Pulmonary Valve Atresia and Intact Ventricular Septum

We report on a premature neonate who presented with cyanosis soon after birth. She was found on echocardiography to have an unguarded tricuspid valve orifice associated with pulmonary valve atresia and an intact interventricular septum. Owing to the small-sized pulmonary arteries, she was initially managed conservatively with prostaglandin infusion, and at one year of age underwent a successful bidirectional cavo-pulmonary (Glenn) shunt operation.

Reversible prostaglandin-induced cortical hyperostosis in an infant without 3040C→T mutation in COL1A1

Background: A hereditary form of infantile cortical hyperostosis (ICH), known as Caffey disease, was recently found to be caused by a heterozygous 3040C → T mutation in the COL1A1 gene. Objective: To determine whether a similar mutation was also responsible for a sporadic case of ICH. Methods: We identified a Thai male infant who was a sporadic case of ICH. He had symmetric cortical hyperostosis of all of his long bones, clavicles, and ribs occurring after a prolonged infusion of prostaglandin E1 (PGE1) for a cyanotic congenital heart disease. Mutation analysis of COL1A1 was performed in the patient and his parents by restriction enzyme digestion of PCR products. Results: The particular mutation was not found in our case and in his parents. A follow-up after 15 months demonstrated that the child had normal growth and development. Repeated imaging studies revealed markedly decreased cortical thickenings of the affected bones. Conclusion: Our findings confirm that PGE1-induced cortical hyperostosis is reversible and does not associate with the COL1A1 3040C→T mutation.

The Effects of Prostaglandin E1 on Interleukin-6, Pulmonary Function and Postoperative Recovery in Oesophageetomised Patients

The inflammatory reactions and tissue response after oesophagectomy are leading causes of postoperative morbidity and mortality. We evaluated the effects of intraoperative infusion of prostaglandin E1 (PGE1) on interleukin-6 (IL-6) levels, (A-a) DO2, pulmonary function and complications. This randomised double-blind clinical trial study was performed on patients undergoing transthoracic oesophagectomy due to cancer Thirty patients were randomly allocated to two groups: the PGE1 group (infusion of PGE1 20 ng kg(-1) min(-1)) and a placebo group (infusion of normal saline 0.9%). The infusion was started before induction of anaesthesia and continued until the end of the operation. The groups were comparable in basic characteristics and preoperative pulmonary function. Patients in the PGE1 group were discharged significantly earlier from the intensive care unit (72+/-9 vs 83+/-17 hours) and hospital (13+/-4 vs 18+/-8 days) (P=0.04 and 0.03, respectively). The (A-a) DO2 was significantly less in the PGE1 group at 12 and 24 hours after the operation (P=0.001, P=0.003, respectively). Postoperatively, IL-6 levels were significantly higher in the placebo group than in the PGE1 group. There were no differences in the forced expiratory volume in the first second or forced vital capacity. The findings indicate that infusion of PGE1 attenuates the increase in serum levels of IL-6 in patients undergoing esophagectomy and improves the (A-a) DO2. Stays in the intensive care unit and hospital were shorter in the PGE1 group. However, there were no differences in pulmonary complications.

Index of Suspicion in the Nursery

A 1-day-old male infant develops lower extremity cyanosis after feeding. The infant was born at 38 weeks’ gestation and had Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. There were no prenatal complications. Today he has lower extremity saturations of 80% and upper extremity saturations of 97%. Results of limited/preliminary echocardiography are reported as “pulmonary hypertension and possible aortic arch obstruction.” After starting an infusion of prostaglandin E1, the infant is transferred to a tertiary care center for cardiac evaluation. The initial physical examination reveals a warm, pink neonate who is experiencing no significant distress. His length is at the 75th percentile and weight is at the 25th percentile for age. His temperature is 37.0°C, heart rate is 120 beats/min, respiratory rate is 28 breaths/min, and cuff blood pressure in the right upper extremity is 75/45 mm Hg. No blood pressure gradient is evident between the upper and lower extremities. Oxygen saturation in room air is 98% in the left upper extremity and 85% in the left lower extremity. Lungs are clear and no retractions are noted. Cardiovascular examination reveals a fixed, widely split second heart sound (S2) without an appreciable murmur. Pulses are +2 and equal in …

Critical aortic stenosis in combination with an aorto-left ventricular tunnel: a rare congenital malformation

On the first day of live, a female newborn with a three out of six systolic murmur on the right sternal border showed signs of heart failure: dyspnoea, tachypnoe, low blood pressure. On echocardiography, the diagnosis of a critical aortic stenosis with a pin whole ostium was established and a prostaglandin infusion was started. The aortic valve appeared tricommissural with an opening from the right sinus of valsalva to a lateral and anterior extra-aortic tunnel ( Panel A ), the right coronary artery only few millimetre separate. In subcostal echo views, the tunnel could be demonstrated in a more longitudinal …

Transcatheter perforation followed by pulmonary valvuloplasty in neonates with pulmonary atresia and ventricular septal defect

The classic management of neonates with pulmonary atresia with ventricular septal defect (PAVSD) and moderately hypoplastic pulmonary arteries is usually a systemic to pulmonary artery shunt or ductus arteriosus stenting. We report our experience of transcatheter treatment of PAVSD by perforation followed by balloon dilation of the valve, as it is performed in pulmonary atresia - intact ventricular septum. Three patients were treated at a mean age and weight of 7.5 days (range 7-8) and 2.9kg (range 2.5-3.3), respectively. Two newborns were prenatally diagnosed, with micro deletion 22q11 in one case. The three patients had no other pulmonary blood flow support than the ductus arteriosus and were on prostaglandin E1 infusion. The pulmonary atresia was predominantly valvular without significant muscular obstruction. By echocardiography, the mean size of the pulmonary annulus was 6.5mm (range 6-7). In all cases, the valvular perforation was performed with a 0.014 coronary guidewire, followed by balloon dilation when successfully. The procedure succeeded in two cases but failed in the third newborn in whom a long subvalvar muscular stenosis was found at surgery. In the two successful cases, the mean postprocedural transpulmonary doppler gradient was 33.5mmHg (range 17-50). One patient experienced a femoral venous thrombosis that was successfully treated by heparin and a transient right bundle branch block occurred in another one. In the two successful cases, the prostaglandin E1 infusion was weaned and the surgical repair was performed at 4 and 12 months, respectively. In selected cases with PAVSD, perforation followed by balloon dilation of the pulmonary valve offers an interesting alternative to other surgical or transcatheter palliative therapies.

Prenatal identification of the pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia

To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia. In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management. We carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings. Of 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced. The patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.