Neonatal pulmonary hypertension: a physiologic approach to treatment.
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Bronchodilator Agents↗
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Robust Bayesian methods for monitoring clinical trials.
Bayesian methods for the analysis of clinical trials data have received increasing attention recently as they offer an approach for dealing with difficult problems that arise in practice. A major criticism of the Bayesian approach, however, has focused on the need to specify a single, often subjective, prior distribution for the parameters of interest. In an attempt to address this criticism, we describe methods for assessing the robustness of the posterior distribution to the specification of the prior. The robust Bayesian approach to data analysis replaces the prior distribution with a class of prior distributions and investigates how the inferences might change as the prior varies over this class. The purpose of this paper is to illustrate the application of robust Bayesian methods to the analysis of clinical trials data. Using two examples of clinical trials taken from the literature, we illustrate how to use these methods to help a data monitoring committee decide whether or not to stop a trial early.
Bayes Theorem↗
Persistent pulmonary hypertension after lithium intoxication in the newborn.
A case of lithium intoxication in the newborn is presented. Besides displaying extreme hypotonia and a goitre, the infant developed symptoms of congenital heart disease immediately after birth. Cardiac catheterization and angiocardiography revealed an elevated pulmonary vascular resistance and indicated that the cardiopulmonary symptoms were caused by persistent fetal circulation. Previously, four authors have independently reported cardiopulmonary symptoms in association with lithium intoxication without finding cardiac or pulmonary disease. The similarity between the present and the four earlier reported cases in regard to the symptoms and the course of illness, raises the question of the connection between lithium intoxication and persistent fetal circulation being more than coincidental. In view of recent investigations it is speculated that lithium intoxication in utero may result in the pulmonary vascular changes responsible for the persistence of fetal circulation.
Female↗
Parachute-like sinus venosus remnant: echocardiographic and pathological appearance.
A female infant with multiple congenital abnormalities and a right atrial "mass" on echocardiography is reported. Her general condition at birth was poor, and the chest x-ray showed cardiomegaly. Echocardiography demonstrated the usual atrial arrangement and concordant atrioventricular and ventriculoarterial connections with a mobile right atrial mass. Death occurred at 3 days of age and subsequent postmortem examination revealed a hollow, tube-like sinus venosus remnant mobile between right atrium and ventricle with numerous other abnormalities of the heart, including left heart hypoplasia and tubular hypoplasia of the aortic arch. Filling of the sinus venosus remnant with blood had resulted in an echocardiographic appearance suggestive of a right atrial tumor.
Asphyxia Neonatorum↗
Dysphagia lusoria caused by persistent right aortic arch with aberrant left subclavian artery and diverticulum of Kommerell.
It requires a high index of suspicion to make the diagnosis of dysphagia lusoria. Clinically, these adults will present with symptoms of intermittent solid food dysphagia, and a mediastinal abnormality may be seen on chest x-ray. Noninvasive imaging of the chest with either computerized tomography or magnetic resonance scanning are excellent methods for evaluating the mediastinum for solid tumors or vascular anomalies that can cause extrinsic esophageal compression. Dysphagia lusoria caused by a persistence of the right embryologic aortic arch and diverticulum of Kommerell with an aberrant left subclavian artery may be satisfactorily managed by dietary modification when the symptoms are mild.
Adult↗
Hemodynamics and oxygenation changes induced by the discontinuation of low-dose inhalational nitric oxide in newborn infants.
OBJECTIVE: To assess changes associated with nitric oxide (NO) discontinuation in neonates receiving inhalational NO therapy as a treatment for pulmonary hypertension of the neonate (PPHN). DESIGN: Prospective study. SETTING: A pediatric PICU in a university hospital. PATIENTS AND METHODS: Ten neonates were included. NO discontinuation was attempted when the oxygenation index fell below 10. The mean NO concentration was 4.9 +/- 0.8 ppm. Each infant was studied over three successive 5-min periods and was assigned to either group 1 (NO1+, NO2+, NO-) or group 2 (NO1+, NO-, NO2+). MEASUREMENTS AND RESULTS: Postductal transcutaneous PO2 (tcPO2), postductal oxygen saturation with pulse oxymetry (SpO2), systolic and diastolic blood pressure (BP), heart rate (HR), left ventricular shortening fraction (LVSF), cardiac output (CO), and ratio of pulmonary artery time to peak velocity and right ventricular ejection time (TPV/RVET) were similar during the two successive NO+ periods (group 1), thus demonstrating that the measurements were reproducible. NO removal (groups 1 and 2) did not modify systolic or diastolic BP, HR, CO, or LVSF but did induce a significant decline in SpO2, tcPO2 (- 25 +/- 5%) and TPV/RVET ratio (- 25 +/- 3%). No reinstitution reversed the effects of NO withdrawal on tcPO2, SpO2 and TPV/RVET ratio (group 2) without any changes in systemic hemodynamics. CONCLUSION: The shut-off of low-dose NO induced in each patient a decrease in oxygen delivery that may be due to increased pulmonary vascular resistances and/or redistribution of pulmonary blood flow with ventilation-perfusion mismatching. The optimum weaning-off procedure of inhalational NO remains to be determined.
Administration, Inhalation↗
Inhaled nitric oxide for avoidance of extracorporeal membrane oxygenation in the treatment of severe persistent pulmonary hypertension of the newborn.
UNLABELLED: Inhaled nitric oxide (NO) is thought to provide a noninvasive therapeutic alternative to extracorporeal membrane oxygenation (ECMO) in the treatment of persistent pulmonary hypertension of the newborn (PPHN). OBJECTIVE: Since January 1993, we have studied inhalation of NO in PPHN patients meeting the ECMO criteria of our institution. We focused on the questions of whether or not the need for ECMO could be obviated and whether differences could be found between NO responders and nonresponders. DESIGN: NO gas was delivered via conventional IPPV ventilation in incrementally increasing concentrations from 20 to 80 ppm. PATIENTS: NO therapy was attempted in ten ECMO candidates with clinical and echocardiographical evidence of PPHN (mean OI 51.9, SD 10.4). RESULTS: At various NO levels (30-60 ppm), five patients showed a significant increase in mean PaO2 (range 32.9-85.9 mmHg). Improvement was transient in three patients (6-10 h) and prolonged in two others (54-80 h); in the latter cases, ECMO was avoided. Five patients did not respond at all to treatment. Responders and nonresponders differed in their mean respiratory tidal volume (8.9 vs 4.18 ml/kg, P <0.05). CONCLUSIONS: In our study, inhalation of NO obviated the necessity of ECMO therapy in only two out of ten PPHN patients. Thus, we would discourage any overoptimistic expectations about the effectiveness of NO therapy in PPHN until larger clinical trials have been performed.
Administration, Inhalation↗
Basilar arterio-venous pseudoparallelism due to persistence of embryonal venous pattern.
A vascular malformation, consisting of a venous vessel bridgeing the right inferior petrosal sinus and the anterior spinal veins, was found in the posterior fossa. The vessel presented a ring-like course around the right trigeminal root, and it was parallel and dorsal to the basilar artery. The malformation was associated with cutaneous and hepatic angiomas and peri-osteal lipomas. It had been clinically silent for 52 years, when it thrombosed causing death. The authors think that, within a general mesenchymopatic state, this is a result of the persistence of an embryonal cerebral venous pattern.
Autopsy↗
Aneurysmal dilatation of ductus arteriosus during lipo-prostaglandin E1 therapy for diaphragmatic hernia.
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.
Alprostadil↗
High frequency oscillatory ventilation and extracorporeal membrane oxygenation in severe persistent pulmonary hypertension of the newborn.
We report on 50 term and near-term neonates (birth weight greater than 1800 g, gestational age greater than 33 weeks) with severe persistent pulmonary hypertension of the newborn (PPHN), referred to us from January 1987 to July 1991 after failure of maximum conventional treatment. All infants had paO2 less than 45 mm Hg when ventilated with peak inspiratory pressure greater than 38 cm H2O and FiO2 = 1.0, hence meeting entry criteria for extracorporeal membrane oxygenation (ECMO). High frequency oscillatory ventilation (HFOV) was tried in all patients. If sufficient oxygenation could not be achieved (paO2 less than 40 mm Hg for at least 2 h), ECMO therapy was begun, which was the case in 25 children. Neonates responding to HFOV (n = 25) were of a slightly younger gestational age (37.0 weeks vs 38.8 weeks, P less than 0.05), had higher Apgar scores and were less hypoxaemic before HFOV (paO2 36.6 mm Hg vs 28.8 mm Hg, P less than 0.01); during HFOV there was a significant rise in paO2 (greater than 150 mm Hg; P less than 0.001) and a fall in pCO2 to 21.6 mm Hg (P less than 0.001). Due to air leaks, which was the main complication of HFOV (52%), ECMO therapy had to be begun in two additional infants after an initial positive effect. HFOV tended to be successful in cases of primary PPHN, meconium aspiration and sepsis, but not in infants with lung hypoplasia as a result of diaphragmatic hernia or other reasons. Success or failure of HFOV could not be reliably predicted by any parameter.(ABSTRACT TRUNCATED AT 250 WORDS)
Blood Gas Analysis↗
Fatal ureaplasmal pneumonia and sepsis in a newborn infant.
Ureaplasma urealyticum was isolated in pure culture from blood tracheal aspirate and lung tissue in a newborn infant, who died of a severe pneumonia within 48 h after birth. The clinical course was characterized by persistent pulmonary hypertension of the newborn (PPHN). Post-mortem examination revealed extensive hyaline membrane formation combined with signs of inflammation in both lungs. The clinical and histopathological picture resembled that of early onset group B haemolytic streptococcal pneumonia/sepsis.
Humans↗
Persistence of atrioventricular valve regurgitation and electrocardiographic abnormalities following transient myocardial ischemia of the newborn.
To determine the sequelae of transient myocardial ischemia (TMI) in term infants, we reviewed clinical and investigative data in 59 infants (37 male, 22 female) with structurally normal hearts admitted over the 2-year period of 1983-1985. Twenty-three were diagnosed prior to admission as cases of birth asphyxia (5-min Apgar score less than 6), and 36 had signs of persistent fetal circulation with electrocardiographic (ECG) changes of ischemia greater than 24 h after birth. Murmurs of atrioventricular valve regurgitation (AVVR), detected in 28 patients, were confirmed in 23 of the 24 patients investigated. The murmurs resolved over a 2-day to 6-month period (median 6 days). In three patients, AVVR, left ventricular dyskinesia, and ECG anomalies persisted for 2 months (until death), 4 months, and 48 months. Initial ECGs were abnormal in 57 patients, and (of those reviewed) 60% returned to normal over a 6-day to 7-month period (median 2 months). Residual ECG anomalies included second-degree AV block and persistent ST-T wave changes. Ten patients died from noncardiac causes. Neither the presence nor resolution of AVVR correlated significantly with the severity of birth asphyxia using the Apgar score, nor with the severity of the ischemic changes on the ECG. Although the cardiovascular sequelae of myocardial ischemia are usually transient, the data should prompt the need for careful review after the initial admission.
Asphyxia Neonatorum↗
Management of the infant with congenital diaphragmatic hernia (CDH) and persistent pulmonary hypertension (PPHN).
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Animals↗
The use of L-arginine [correction of F-arginine] and phosphodiesterase inhibitor (dipyridamole) to wean from inhaled nitric oxide.
A full-term, female neonate developed acute hypoxemic respiratory failure complicated by persistent pulmonary hypertension of the newborn (PPHN), and responded to high-frequency oscillatory ventilation (HFOV) and inhaled nitric oxide (iNO). Discontinuation of iNO was attempted three times and was followed by severe desaturation due to right-to-left shunt through the patent ductus arteriosus and patent foramen ovale. As a result of iNO dependency state and rebound pulmonary hypertension, the neonate was maintained on iNO therapy for dipyridamole alone was unsuccessful. However, successful discontinuation of iNO therapy was achieved by combination of L-Arginine and dipyridamole. Exogeous NO may lead to down regulation of endogenous NO production, and further lead to rapid hydrolization of cyclic guanosine 3', 5' monophosphate (cGMP), the smooth muscle relaxant, by the enzyme phosphodiesterase. Moreover L-Arginine, the precursor for the formation of endogenous NO, has been found to be deficient in neonates with PPHN, so we speculated that by inhibiting phosphodiesterase and administrating L-Arginine smooth muscle relaxation occurred, and consequent weaning from iNO was achieved.
Arginine↗
Nitric oxide in preterm infant with pulmonary hypoplasia.
Premature infants with hypoplastic lungs may have elevated pulmonary vascular resistance with right to left shunt across ductus arteriosus and/or foramen ovale. Inhaled nitric oxide (NO) being selective pulmonary vasodilator without significant effects on systemic circulation can potentially reverse this shunt. The authors herewith report a case of a premature infant with severe hypoxemic respiratory failure after preterm premature prolonged rupture of membranes leading to oligohydramnios and pulmonary hypoplasia that was treated successfully with NO and describe the neurodevelopmental outcome at 1 year of age.
Administration, Inhalation↗
Treatment of severe persistent pulmonary hypertension of newborn with magnesium sulfate.
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Female↗
Assisted ventilation in neonates: the Manipal experience.
Case records of 68 newborns who required assisted ventilation over a 24 month period were reviewed. Fortyfour (64.7%) received intermittent mandatory ventilation, 10 (14.7%) received nasal CPAP and the remaining 14 (20.58%) received a combination of the above. Some of the indications for ventilation were infections (21), hyaline membrane disease (16), problems related to asphyxia (11), apnea of prematurity (10) and persistent pulmonary hypertension of newborn (5). The overall survival rate was 41.17%. In the CPAP group 90% (9/10) survived, while in the remaining survival was 32.7% (19/58). The best outcome was observed in persistent pulmonary hypertension of newborn (80%) followed by apnea of prematurity (70%) and hyaline membrane disease (43.75). Outcome was poor in conditions related to birth asphyxia (27.2%) and infections (19.05%). Survival rates were higher (44.4%) in babies weighing > 1500g at birth as compared to 40.9% in babies < 1500g. Babies less than 32 weeks gestation had a survival rate of 32% as compared to 46.5% in those over 32 weeks. This difference was not statistically significant. Complications were seen in 12/68 patients (17.6%). Pneumothorax was the commonest followed by sepsis, intraventricular hemorrhage and blocked endotracheal tubes. Babies with hyaline membrane disease had the highest incidence of complications. Analysis of the data with regard to the indications, outcome and complications is presented.
Apnea↗
Nitric oxide: biological role and clinical uses.
Nitric oxide is a product of the conversion of L-arginine by the enzyme nitric oxide synthase. Nitric oxide is involved in a variety of physiological situations and is produced by many different cell types. It is involved in neurotransmission, maintenance of vascular smooth muscle tone, and cytotoxicity. Nitric oxide has been suggested to play an anti-inflammatory role by inhibiting the expression of the genes for inflammatory cytokines. The pathophysiological role of nitric oxide is also evident in a variety of diseases, including septic shock, asthma, reperfusion injury, etc. Nitric oxide, by stimulating the production of cyclic GMP, relaxes smooth muscles of the cardiovascular, respiratory, gastrointestinal, and genito-urinary systems. Recent studies have provided important information on the use of inhaled nitric oxide for the management of several diseases characterized by the presence of abnormal pulmonary vascular tone, such as persistent pulmonary hypertension of the newborn. This review addresses the biology and clinical uses of inhaled nitric oxide.
Child↗