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Results for “Persistent Fetal Circulation Syndrome”

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PubMed1994

Plasma cysteine concentrations in infants with respiratory distress.

Because factors that predispose infants to persistent pulmonary hypertension of the newborn (PPHN) may cause oxidant stress, which in turn may increase demands for cysteine and glutathione, we investigated the availability of cysteine and its precursors in PPHN and related disorders. Plasma concentrations of four sulfur-containing and two non-sulfur-containing amino acids were measured by gas chromatography-mass spectrometry in blood from infants with PPHN, both those managed conventionally (PPHN group) and those treated with extracorporeal membrane oxygenation, as well as from infants with hyaline membrane disease. Concentrations also were measured in umbilical venous cord blood samples from a healthy control population, in venous plasma from infants receiving only intravenously administered glucose-containing solutions because they had noncardiopulmonary illnesses ("fasted" group), and from otherwise healthy, orally fed infants ("fed" group). The plasma total cyst(e)ine concentration was markedly lower in the three groups (PPHN, PPHN and extracorpeal membrane oxygenation, and hyaline membrane disease) receiving an elevated inspired oxygen concentration (0.6 to 1.0) than in fasted or fed control infants. In contrast, levels of plasma methionine, the other major sulfur amino acid, were low in the three groups receiving an elevated inspired oxygen concentration, as well as in fasted infants. Glycine and serine, two non-sulfur-containing amino acids, had a pattern similar to that of plasma methionine. Thus infants with PPHN and hyaline membrane disease have low plasma total cyst(e)ine levels, an effect that does not appear to result primarily from nutritional deprivation. We speculate that the role of cysteine in bioactivation of nitric oxide and as a precursor of glutathione may be relevant to the pathogenesis and evolution of PPHN and respiratory distress syndrome. Further studies are needed to determine whether increased demands for cysteine exist in these disorders.

Algorithms↗

PubMed1998

Inhaled nitric oxide therapy for pulmonary disease in pediatrics.

Inhaled nitric oxide (iNO) therapy improves gas exchange and lowers pulmonary vascular resistance in neonates and children with diverse cardiac and pulmonary disorders. Recent multicenter studies have shown that iNO reduces the need for rescue therapy with extracorporeal membrane oxygenation in term neonates with severe hypoxemic respiratory failure. In neonates with severe lung disease, responsiveness to iNO therapy can be enhanced by therapies which enhance lung recruitment, especially high-frequency oscillatory ventilation. Although early studies suggest that iNO therapy is not associated with significant clinical toxicity or adverse effects, more data are needed on long-term neurodevelopmental and pulmonary outcomes. Acute iNO treatment can improve oxygenation in nearly 70% of older infants and children with acute respiratory distress syndrome, but multicenter studies have yet to demonstrate that prolonged iNO therapy can improve outcome in these patients. Further studies are needed to better identify the exact role for iNO therapy in cardiopulmonary diseases associated with hypoxemia or pulmonary hypertension.

Administration, Inhalation↗

PubMed1981

Congenital alveolar capillary dysplasia--an unusual cause of respiratory distress in the newborn.

The clinical and anatomical features of a patient with an unusual pulmonary malformation, is reported. The clinical course was consistent with the syndrome of persistent fetal circulation; morphologically, however, the patient was found to have a unique form of pulmonary dysplasia. Failure of formation and ingrowth of alveolar capillaries led to absence of normal air-blood barriers in this term infant. In addition anomalous veins were present in the bronchovascular bundles. Morphometric study indicated that the lungs were otherwise mature. This selective deficiency and dysplasia suggests that distal pulmonary epithelial and vascular development operate under separate control mechanisms.

Abnormalities, Multiple↗

PubMedDate not supplied

Persistent pulmonary hypertension of the newborn: not a honeymoon anymore.

Persistent pulmonary hypertension of the newborn is a disorder of transition to extrauterine life, in which the newly born baby cannot decrease the high pulmonary vascular resistance and low pulmonary blood flow, characteristic of the fetus, to that of a low pulmonary vascular resistance and high pulmonary blood flow necessary for postnatal survival. The syndrome primarily affects the neonate 34 weeks postmenstrual age and greater. The article will summarize the latest understanding of the pathophysiology and review innovations in management strategies that have greatly decreased mortality and morbidity since the advent of neonatal intensive care units.

Antihypertensive Agents↗

PubMed1997

Inhaled nitric oxide as a selective pulmonary vasodilator in clinical anesthesia.

Inhaled nitric oxide (NO) is a selective pulmonary vasodilator in adult and pediatric patients. Inhaled NO diffuses into the pulmonary vascular smooth muscle where it results in vasodilation via stimulation of guanylyl cyclase. Systemic hemodynamics are not altered because inhaled NO is rapidly inactivated by hemoglobin. Oxygenation is also increased in certain patients as inhaled NO only vasodilates those segments of the pulmonary vasculature which are ventilated. There is growing evidence that inhaled NO may be a useful therapeutic agent in the treatment of pulmonary hypertension and hypoxemia from a variety of causes. Areas of greatest interest to anesthesia and critical care personnel may involve treatment of persistent pulmonary hypertension of the newborn (PPHN), adult respiratory distress syndrome (ARDS), and postoperative pulmonary hypertension secondary to cardiac disease. The potential toxicity of inhaled NO, particularly on immature and developing lungs, must be considered. While inhaled NO exerts acute beneficial effects, it is unclear if there are long-term benefits. Multicenter trials are currently underway to determine if inhaled NO decreases mortality from PPHN or decreases morbidity associated with ARDS.

Administration, Inhalation↗

PubMed2005

Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report.

Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a multisystem disorder with classical changing skin lesions. The other systems that are involved include the central nervous system, eye, hair, teeth, musculoskeletal system and, occasionally, the cardiovascular system. We report a neonate with a diagnosis of incontinentia pigmenti who presented at birth with pulmonary hypertension. This presentation has not been described in the literature.

Fatal Outcome↗

PubMed1986

[Prolonged dopmin infusion in severe respiratory insufficiency in newborn infants].

An effective use of continuous infusion of Dopmin in dose of 2-3 mkg/(kg/min) is described. Dopmin was used in 13 newborns with severe respiratory insufficiency due to postasphyxial syndrome, disease of hyaline membranes and congenital diaphragmal hernia. Continuous infusion of microdoses of Dopmin with artificial ventilation of lungs against the background of a decreased respiratory insufficiency according to clinico-laboratory evidence resulted in decreased value of bloodflow shunt from right to left and oxygen concentration in the inhaled mixture to 45%.

Asphyxia Neonatorum↗

PubMed1994

Nitric oxide: a new area in intensive care.

The relaxing effects of nitric oxide on smooth muscles, first identified in 1987, inaugurated a new area of therapeutic efficacity in intensive care. Nitric oxide is synthesized by endothelial cells, macrophages, brain cells and other cells following immunological activation and plays a role in normal blood pressure homeostasis, neuromodulation, cytotoxicity and intracellular message transmission. Since inappropriate vasodilatation or shock may result from cytokine- or endotoxin-induced overproduction of nitric oxide, experiments have been conducted on the effect of nitric oxide synthase inhibitors on shock-induced hypotension. In animal models, results have demonstrated a new method for treating septic shock since infusing nitric oxide inhibitors can rapidly re-establish haemodynamics. Nevertheless, due to possible interference with the immune defense system further studies on the physiological, pharmacological and metabolic effects are required before routine antishock therapy can be used in the intensive care unit. Inversely, in pulmonary hypertension there may be an insufficient production of endogenous nitric oxide. Thus administration via inhalation would represent a promising replacement therapy. In addition, since nitric oxide is rapidly inactivated by haemoglobin, its vasodilatory effect is restricted to the pulmonary vasculature resulting in lowered pulmonary artery pressure without systemic vasodilatation. Effective protocols have been developed for primary pulmonary hypertension of the newborn and acute respiratory distress syndrome. The bronchodilatory effect of nitric oxide is another area suggesting an alternative approach to treating different causes of bronchoconstriction including asthma. The results of early clinical trials are awaited. When used in low concentrations under continuous monitoring, nitric oxide is a safe new therapeutic option for the treatment of pulmonary hypertension and nitric oxide inhibitors may have an important role to play in the management of septic shock.

Administration, Inhalation↗

PubMed1993

Elevated immunoreactive endothelin-1 levels in newborn infants with persistent pulmonary hypertension.

To study the potential role of endothelin-1, a potent endothelium-derived vasoconstrictor peptide, in the pathophysiology of persistent pulmonary hypertension of the newborn (PPHN), we measured arterial concentrations of immunoreactive endothelin-1 (irET-1) in 24 neonates with PPHN. Secondary diagnoses included meconium aspiration syndrome (13 patients), sepsis (2), congenital diaphragmatic hernia (1), asphyxia (1), pulmonary hemorrhage (1), aspiration of blood (1), and respiratory distress syndrome (1). Compared with irET-1 levels in umbilical cord blood in normal infants (15.1 +/- 4.1 pg/ml; mean +/- SEM) and in newborn infants with hyaline membrane disease who were supported by mechanical ventilation (11.8 +/- 1.2 pg/ml), infants with PPHN had markedly elevated circulating irET-1 levels (27.6 +/- 3.6 pg/ml; p < 0.01 vs cord blood, hyaline membrane disease). Infants with severe PPHN requiring extracorporeal membrane oxygenation (ECMO) therapy had higher irET-1 levels than infants with milder disease (31.0 +/- 4.7 for ECMO-treated infants vs 21.2 +/- 2.0 for non-ECMO-treated infants; p < 0.05). In patients treated without ECMO, irET-1 progressively decreased during the following 3 to 5 days, paralleling clinical improvement. In contrast, irET-1 concentrations remained elevated in infants with severe PPHN during ECMO therapy. We conclude that circulating irET-1 levels are elevated in newborn infants with PPHN, are positively correlated with disease severity, and decline with resolution of disease in patients who do not require ECMO therapy. Whether endothelin-1 contributes directly to the pathophysiology of PPHN or is simply a marker of disease activity remains speculative.

Analysis of Variance↗

PubMed2004

Factors affecting the response to inhaled nitric oxide therapy in persistent pulmonary hypertension of the newborn infants.

Persistent pulmonary hypertension of the newborn infant (PPHN), is a clinical syndrome characterized by elevated pulmonary vascular resistance, resulting from reactive vasoconstriction or structural remodeling of the pulmonary vasculature. Although inhaled nitric oxide (iNO) has emerged as a novel selective treatment of PPHN, responses to iNO are variable according to the etiologies or the clinical situation. A retrospective chart review of 51 newborn infants with PPHN and treated with iNO, was undertaken to evaluate the factors affecting response to iNO. Response to iNO was defined as a reduction in the oxygenation index (OI) of more than 20%, or disappearance of the difference in oxygen saturation between preductal and postductal circulation after iNO therapy. The patients were divided into two groups; the responder group and the non- responder group. Respiratory distress syndrome (RDS) was more commonly associated with PPHN in the responder group than in the non-responder group (p < 0.05), while there were many more patients with congenital diaphragmatic hernia (CDH) in the non-responder group than in the responder group (p < 0.05). Infants with meconium aspiration syndrome (MAS) were similar in both of the two groups. Initial OI, initial mean airway pressure (MAP), and initial and peak NO concentration were significantly lower in the responder group compared to the non-responder group (p < 0.05). Rapid response (response to iNO within the first hour) was shown in 74% of the responder group and 33% of the nonresponder group (p < 0.05). There was no significant differences in the initial chest radiographic findings, such as normal, focal or bilateral diffuse infiltration, with the exception of CDH, between each group. Lower initial OI, lower initial MAP and significant response within the first hour were shown to be favourable factors in response to iNO therapy. Patients with RDS associated with PPHN responded much better to iNO than those with other diseases.

Administration, Inhalation↗

PubMed1992

Indication for using extracorporeal membrane oxygenation in congenital diaphragmatic hernias and pulmonary hypoplasia.

Despite the apparent surgical simplicity of the anatomic defect, congenital diaphragmatic hernia continues to be a critical problem in neonatal surgery, so that survival is still uncertain. Therefore, we must realize that the barriers to survival are pulmonary parenchymal and vascular hypoplasia as well as the complex syndrome of persisting fetal circulation. However, new treatment methods, such as extracorporeal membrane oxygenation (ECMO), although controversial, may improve survival. We believe that no infant should be excluded from diaphragmatic repair or consideration for ECMO-support before accurate predictive parameters have been developed that take both pulmonary hypoplasia and pulmonary hypertension into account. ECMO additionally enables us to postpone the operation until stabilization of the newborn (Late Operation Protocol). Apart from this, we can probably improve the long-term results after ECMO by reconstructing the common carotid artery.

Combined Modality Therapy↗

PubMed1986

Persistent pulmonary hypertension in preterm-infants.

Persistent pulmonary hypertension of the newborn (PPHN) is a cyanotic syndrome that occurs primarily in full-term and postmature infants and causes right-to-left shunts at the atrial or ductal levels or both. Term babies with PPHN show structural changes in pulmonary vascular smooth muscle as a result of chronic prenatal distress. It is our opinion that in preterm babies the PPHN syndrome also exists. In this group of patients the potential pathways to the persistence of high pulmonary vascular resistence are only functional vascular changes precipitated by acute perinatal stress. The cyanosis of PPHN is rapidly regredient in preterm infants and clinical resolution occurs promptly if the diagnosis is correct and the treatment is started as early as possible in centers capable of extensive monitoring and neonatal supportive care to minimize the risks of Tolazoline therapy.

Carbon Dioxide↗

PubMed1983

Leukotriene C4 and D4 in neonates with hypoxemia and pulmonary hypertension.

Persistent pulmonary hypertension of the newborn is a syndrome consisting of severe hypoxemia and pulmonary hypertension that appears within hours of birth. Since certain leukotrienes (C4, D4, and E4) are known to produce some of the features of persistent pulmonary hypertension of the newborn, including pulmonary vasoconstriction, bronchoconstriction, decreased lung compliance, and pulmonary edema, we studied five newborns with the syndrome to determine whether these leukotrienes were present in their airways. We found leukotriene C4 and leukotriene D4 in the lung lavage fluids of all five newborns who had the clinical diagnosis of persistent pulmonary hypertension and who required ventilatory assistance. In contrast, leukotrienes were not demonstrated in a control group of 14 infants requiring ventilatory assistance who did not have the clinical diagnosis of persistent pulmonary hypertension. We conclude that leukotrienes may have a role in persistent pulmonary hypertension of the newborn.

Animals↗

PubMed1996

Caution with use of cimetidine in tolazoline induced upper gastrointestinal bleeding.

A 2-day-old newborn was treated with tolazoline for persistent pulmonary hypertension due to meconium aspiration syndrome. Hypoxemia improved during continuous tolazoline infusion, but gastrointestinal bleeding occurred. After treatment with cimetidine, there was a rapid deterioration with decreased oxygen saturation and arterial PO2 values. Tolazoline induces a dilatation of the pulmonary vascular system by stimulating H2 receptors. Cimetidine, an H2 blocker, may partially abolish the effect of tolazoline. However, given the clinically significant result to the interaction between tolazoline and cimetidine we report, the use of cimetidine in tolazoline induced upper gastrointestinal hemorrhage should deserve more attention.

Cimetidine↗

PubMed1995

[Persistent pulmonary hypertension in newborns].

One of the most critical problems of neonatology persistent pulmonary hypertension in the newborn, is discussed. This syndrome is characterized by increased resistance in pulmonary vessels leading to hypertension in pulmonary arteries and a recurrence of R > L shunting at the ductus arteriosus and foramen ovale. This leads to deep hypoxia and hypoxemia. It is most important to rule out a congenital cyanotic heart defect in the differential diagnosis. The aim of the treatment is to establish normal ventilation and intrapulmonary shunting as well as to control the symptoms leading to pulmonary hypertension that persist over time. The goal preceding treatment should be to determine the initial cause of the persistent pulmonary hypertension.

Cyanosis↗

PubMed1986

Extracorporeal membrane oxygenation: radiographic appearance of the neonatal chest.

The chest radiographs of 26 newborns treated with extracorporeal membrane oxygenation (ECMO) for intractable respiratory failure were reviewed. The typical radiographic appearance of the lungs in these patients is that of diffuse pulmonary opacification with variable volume loss. Air bronchograms and patchy basilar atelectasis are also common findings. Generally, decreasing ECMO requirements were reflected in improving chest radiographs with radiographic improvement lagging behind clinical improvement. Of 167 chest radiographs available for evaluation, 105 (62.8%) reflected changes in ECMO flow rates. Radiographs in patients with individual diagnoses of hyaline membrane disease, meconium aspiration syndrome and sepsis showed the best correlation with clinical improvement (95 [69%] of 137 radiographs). Those obtained in patients with congenital diaphragmatic hernia and persistent pulmonary hypertension of the newborn alone showed the poorest correlation (10 [30%] of 30 of radiographs). Neither the absolute degree of radiographic abnormality nor degree of radiographic improvement correlated well with ECMO requirements. Initial radiographs were useful in confirming the position of bypass cannulae and respiratory tubes. Routine daily examinations did not reveal unexpected abnormalities. However, radiographs taken during periods of increased ECMO requirements due to patent ductus arteriosus or volume overload showed worsening lung opacification.

Humans↗

PubMed1998

Intrachromosomal triplication of distal 7p.

A female infant who died at 2 years of age with growth and psychomotor retardation, wide anterior fontanelle, downward slanting palpebral fissures, large, simple ears, joint dislocation/contractures, recurrent infections, and severe pulmonary hypertension was found to have a de novo 7p+ chromosome. The G banding pattern was suggestive of a triplication of 7p21.3 and 7p22; results of fluorescence in situ hybridisation studies using a chromosome 7 specific library, a subtelomeric 7p repeat (109A6), and yeast artificial chromosome clones 786g1 and 850a1, which are respectively associated with the (CA)n repeat markers D7S517 and D7S513, supported the cytogenetic interpretation and showed that the middle repeat was inverted. The patient's phenotype was consistent with the 7p duplication syndrome, allowing for the effects of the extra burden introduced by the partial tetrasomy. The present rearrangement may have resulted from several meiotic events occurring at the four chromatid stage, namely an unequal crossover or interhomologue translocation with points of exchange at 7p22 and 7p15 followed by the inverted insertion of 7p21.3-->p21.2 at the former breakpoint junction; moreover, a further duplication including D7S517 within the terminal 7p22 band is also required.

Abnormalities, Multiple↗

PubMedDate not supplied

[Persistent pulmonary hypertension of the newborn infant (PPHN)].

Persistent pulmonary hypertension of newborn (PPHN) is due to a defect in the adaptation of pulmonary resistance. The primary form occurs idiopathically, with meconium aspiration syndrome and with hypoplasia of the lung and is characterised by excessive muscular hyperplasia of pulmonary arteries. Secondary PPHN following hypoxia, septicaemia and shock is due to pulmonary vasoconstriction whereby thromboxane, leukotrienes and prostaglandins play a decisive role. Diseases of the pulmonary parenchyma and congenital cardiac malformations have to be excluded. The increased pulmonary resistance can be demonstrated by two-dimensional cardiography. The therapy consists of hyperventilation and tolazoline, which can be complicated by severe side-effects. High frequency oscillation and jet-ventilation are employed experimentally, Extracorporeal Membranoxygenation is a promising new method.

Echocardiography↗