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[A case report of a patient who developed cyanosis and significant decrease in PtcO2 caused by a contraction of the ductus arteriosus].

A 2-day-old male baby with patent ductus arteriosus and pulmonary atresia had an emergency anoplasty because of anal atresia. During the operation, anesthesia was maintained satisfactorily with 1-2% halothane and 70% nitrous oxide in oxygen, accompanied with continuous infusion of prostaglandin E1 (PGE1). However, after the inspired oxygen concentration was changed to 100% from 30% at the end of the operation, transcutaneous partial pressure of oxygen (PtcO2) began to decrease progressively, developing cyanosis and sinus bradycardia. Immediately the inspiratory concentration of oxygen was returned to 30% from 100% and the infusion rate of PGE1 was increased from 2 micrograms. kg-1.min-1. Atropine 0.2mg was also administered intravenously. A few minutes after this therapy, a gradual increase in PtcO2 was observed, and he recovered from cyanosis and respiratory arrest. In this case, we suspect that the cyanosis developed because of the contraction of PDA. In summary, anesthetic management of a patient with pulmonary atresia and PDA is described. The PtcO2 should be monitored continuously and the inspiration of pure oxygen should be avoided. Continuous infusion of PGE1 may be one of the effective measures to dilate PDA.

Cyanosis↗

Cyanosis in uncomplicated atrial septal defect.

An unusual case of secundum type atrial septal defect with central cyanosis, without any associated conditions to account for cyanosis is presented. The possible mechanism of cyanosis is discussed.

Child↗

[Pulmonary arteriovenous fistula: a rare cause of progressive asymptomatic cyanosis in neonates].

A six month old girl with no significant medical history was admitted to hospital for progressive cyanosis of recent onset refractory to oxygen therapy. There were no detectable cardiac murmurs. Chest X-ray showed an irregular left posterior parahilar infiltration. Echocardiography showed dilatation of the left atrium and ventricle but no cardiac malformation. The diagnosis of pulmonary arteriovenous fistula was suspected. Chest CT scan, magnetic resonance imaging and pulmonary angiography demonstrated the arteriovenous fistula in the lower lobe of the left lung. It was much bigger than the appearances of the chest X-ray suggested. After the left lower lobectomy, the cyanosis completely disappeared. Progressive cyanosis refractory to oxygen therapy without any apparent cardiac or pulmonary disease is strongly suggestive of pulmonary arteriovenous fistula. Surgical treatment is curative whereas the spontaneous outcome of this condition may be lethal.

Angiocardiography↗

Hemoglobin F-Cincinnati, alpha 2G gamma 2 41(C7) Phe-->Ser in a newborn with cyanosis.

A term infant presented with mild cyanosis without evidence of hypoxia. Cardiopulmonary disease, polycythemia, and methemoglobinemia were excluded. Standard hemoglobin electrophoresis, including isoelectric focusing, were normal. However, by reverse-phase C4 HPLC, an abnormal globin chain was detected. Analysis of tryptic peptides and amino acid sequence showed that the patient had an amino acid substitution Phe-->Ser at residue 41(C7) in the G gamma chain. This was confirmed by DNA sequencing that demonstrated a point mutation at the expected site in exon 2 of the G gamma gene, accounting for the appropriate change in the codon. This substitution, hemoglobin F-Cincinnati, alpha 2 gamma 2 41(C7) Phe-->Ser, not previously described, presumably decreased oxygen affinity of the hemoglobin. This substitution is very near the heme group and the alpha 1 beta 2 interface and, hence, in a crucial area of the globin chain. Abnormalities of gamma globin chains tend to be overlooked due to their transient presence and trivial clinical symptomatology, or due to "in utero" selection when physiologically abnormal. Mutant hemoglobins with altered oxygen affinity should be included in the differential diagnosis of newborns presenting with cyanosis, in whom all common causes have been excluded.

Amino Acid Sequence↗

Cyanosis of the hands following the use of alphaprodine in dental anaesthesia.

A case is presented of asymptomatic cyanosis of the hands, seen four hours after dental surgery under intravenous, local and inhalational anaesthesia. Alphaprodine hydrochloride, a synthetic opiate and one of the agents used, may produce dilatation of the venous bed with passive venous pooling. Such a mechanism is thought to have occurred in this case, manifesting as cyanosis. A weakly positive intradermal skin test to alphaprodine suggests immediate type hypersensitivity as a possible underlying cause.

Adolescent↗

Transient neonatal cyanosis: unusual presentation of right-sided cardiac masses.

We report a case of a neonate with multiple cardiac masses, cyanosis, and a heart murmur. Arterial desaturation was the result of right-to-left shunting at the foramen ovale level caused by tricuspid regurgitation. Three right-sided cardiac masses were detected by echocardiography. By 2 weeks of age the patient had complete resolution of his cyanosis and improved tricuspid regurgitation following the normal decrease in pulmonary vascular resistance. At 2 years of age, he has no cardiovascular symptoms and the masses are calcified and have no hemodynamic consequences.

Calcinosis↗

Maturational and hemodynamic factors predictive of increased cyanosis after bidirectional cavopulmonary anastomosis.

Bidirectional cavopulmonary anastomosis (BCA) is thought to be beneficial in the palliation of patients with univentricular congenital heart disease considered at high risk for Fontan repair. Experience with patients undergoing BCA suggested that those who were older or larger at the time of surgery tended to be more cyanotic postoperatively than their younger and smaller counterparts. This study was designed to identify correlates of systemic arterial oxygen saturation after BCA. Specifically, it was postulated that maturational changes in blood flow distribution might be associated with decreasing arterial oxygen saturation. Database records of all 110 patients undergoing BCA at our institution from June 1988 until the end of 1991 were reviewed. Postoperative catheterization data were available for 66 patients. Twenty-one patients were excluded because they had potentially reversible causes of cyanosis yielding inestimable degrees of error in hemodynamic calculations. In the remaining 45 patients, univariate and multivariate regression analyses were used to identify correlates of systemic arterial oxygen saturation. Growth and maturation as represented by body surface area exhibited a highly significant inverse correlation with arterial oxygen saturation (p = 0.005), as did pulmonary vascular resistance (p = 0.003). Patients who underwent BCA when > 3.9 years of age or with body surface area > 0.65 m2 were at significantly increased risk for excessive postoperative cyanosis, defined as systemic arterial oxygen saturation < or = 75% (p < 0.005). The interval between surgery and catheterization correlated directly with arterial oxygen saturation (p = 0.002), indicating a tendency toward earlier study of more cyanotic patients. None of the other variables examined correlated significantly with arterial oxygen saturation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Central cyanosis due to prominence of the eustachian and thebesian valves.

A 9-year-old female child with serious central cyanosis was found to have right to left shunt across a large secundum atrial septal defect despite normal right-sided pressures. Preoperative cross-sectional echocardiography suggested the presence of large sinus venosus eustachian and thebesian valves as the mechanism responsible for diversion of the inferior caval and coronary sinus venous return to the left atrium across the interatrial secundum defect. Surgical excision of the unduly prominent sinus venosus valve and patch closure of the atrial septal defect resulted in complete disappearance of the cyanosis and physiological and clinical cure.

Arteriovenous Malformations↗

Atrial septal defect with cyanosis without pulmonary hypertension or anomalous systemic venous drainage to the left atrium.

A case of a 29-year-old patient with cyanosis due to a right-to-left shunt is reported. This patient presented an atrial septal defect within the oval fossa and an anomalous left superior caval vein. The direction of this shunt is explained by the anatomical relationship between the oval fossa and the dilated coronary sinus, draining venous blood into the left atrium across the atrial septal defect. Cyanosis disappeared after surgical correction of the defect.

Adult↗

Phasic sleep components in infants with cyanosis during feeding.

Although brainstem immaturity has been postulated as one of the pathogenesis underlying cyanosis during feeding (CDF), there has been no widely accepted physiologic parameter that reflects brainstem function. We recently proposed that the dissociation index (DI), one of the phasic sleep parameters, is a reliable and quantitative sleep parameter for assessing brainstem maturation during early infancy. In the present study, we evaluated brainstem impairment in infants with CDF using phasic sleep components. Polysomnographies were obtained for 12 infants with CDF who were equally divided into 2 groups: one had or subsequently experienced apparent life-threatening events or sudden infant death syndrome (ALTE-SIDS group) and the other did not (CDF group). Rapid eye movement density and the number of gross movements (body movements, including the trunk, lasting greater than or equal to 2 sec) in the patients were identical to those in the controls. In the CDF group, the decrease of the average DI value from the controls was significantly less than the decrease in the ALTE-SIDS group. CDF may be a mild expression of brainstem immaturity. DI appears to be useful when evaluating infants with cyanosis during feeding.

Cyanosis↗

Transcatheter closure of dilated left superior vena cava for resolution of late cyanosis following fontan palliation.

We report two adolescents with late cyanosis post-Fontan palliation arising from a persistent and dilated left superior vena cava (LSVC) draining into the pulmonary venous circulation. One has undergone successful transcatheter closure of the left superior vena cava while the other is waiting closure. There is a need for long-term follow-up of Fontan patients including adequate clarification of increasing cyanosis which may be correctable.

Adolescent↗

Methemoglobinemia: an unusual cause of postoperative cyanosis.

Methemoglobinemia, although rare, must be considered in surgical patients presenting with acute respiratory distress and cyanosis. We report two cases of methemoglobinemia in patients undergoing aortic reconstruction. The first patient developed methemoglobinemia while on a nitroglycerin infusion, and the second after receiving benzocaine spray before intubation. Both patients were treated with methylene blue and ascorbic acid, with resolution of their hypoxia and cyanosis. The pathophysiology, etiology, diagnosis, and treatment of methemoglobinemia are reviewed.

Aged↗

An unusual cause of severe cyanosis in infancy.

An infant presented with cyanosis due to a diaphragmatic Morgagni hernia compromising right ventricular diastolic filling and resulting in right-to-left atrial-level shunting as demonstrated by contrast echocardiography. There was complete resolution of cyanosis after repair of the hernia.

Cyanosis↗

Cyanosis in atrial septal defect due to persistent eustachian valve.

Two cyanotic patients had venoarterial shunting from the inferior vena cava to the left atrium in an uncomplicated atrial septal defect with normal right ventricular pressures. Cyanosis was due to a large, anomalous inferior vena caval valve, the eustachian valve. The mechanism of cyanosis and technical problems for surgical repair are discussed.

Adolescent↗

Hemoglobin Denver [alpha 2 beta 2(41) (C7) Phe-->Ser]: a low-O2-affinity variant associated with chronic cyanosis and anemia.

OBJECTIVE: To report a previously undescribed low-O2-affinity hemoglobin variant that is associated with chronic cyanosis. DESIGN: Pertinent laboratory and historical data for the index case (from Denver, Colorado) and certain family members were recorded, and the hemoglobin variant was characterized. MATERIAL AND METHODS: Electrophoresis, high-performance liquid chromatography (HPLC), and isoelectric focusing were used to examine blood specimens for the presence of hemoglobin variants, and the O2 affinity of whole blood was determined. The abnormal peptide detected on reverse-phase HPLC of separated globin chains was analyzed for its amino acid composition and sequence. RESULTS: Although no abnormal hemoglobin band separated from hemoglobin A on electrophoresis, HPLC, and isoelectric focusing, a heat test showed hemoglobin instability, and O2 affinity studies disclosed an appreciably right-shifted dissociation curve. On chromatography, the new variant--hemoglobin Denver--was found to be due to a substitution of serine for phenylalanine at position 41 (C7) in the beta chain. In addition to substantial reduction in O2 affinity, hemoglobin Denver is accompanied by moderate reticulocytosis and mild anemia. CONCLUSION: Hemoglobin Denver causes no clinical symptoms other than cyanosis, which is attributable to the low O2 affinity.

Adolescent↗

Methemoglobinemia as an uncommon cause of cyanosis.

Cyanosis is usually caused by decreased arterial oxygen saturation due to pulmonary or cardiac diseases. Methemoglobinemia is a rare cause, sometimes with lethal outcome. Two patients are described, both with an unremarkable cardiopulmonary history, presented with severe cyanosis due to aniline-induced methemoglobinemia that developed at work. The symptoms and the treatment of methemoglobinemia are discussed.

Adult↗

Pulse oximeter analysis of peripheral cyanosis distal to an AV fistula.

Arteriovenous (AV) fistulas are routinely constructed for hemodialysis in patients with renal failure. The case of a hemodialysis patient with cyanosis of the hand distal to an AV fistula is described. Pulse oximeter analysis found a saturation of 72% in the cyanotic hand with a normal saturation of 97% in the opposing hand. Evaluation of 11 additional patients with AV fistulas and no cyanosis or symptoms found no difference in oxygen saturation between extremities. Pulse oximetry was found to be useful for evaluating the oxygenation status distal to an AV fistula, and in asymptomatic patients with an AV fistula, the oxygenation status distal to the fistula should be no different from that of the opposing extremity.

Aged↗