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PubMed2004

[Expression of the heat shock protein-70 in the myocardial cells of cyanosis congenital heart diseases].

OBJECTIVE: To evaluate the expression and myocardial protection of heat shock protein-70 (HSP70) in the myocardial cells of cyanosis congenital heart diseases. METHODS: The study enrolled 24 patients with congenital heart disease: 12 cases of non-cyanosis (group A), and the other 12 cases of cyanosis (group B). During the operation the samples of myocardium in the right atrium were attained at 0 min ischemia, 20 min ischemia, and 20 min reperfusion. They were used to evaluate the content of HSP70 by immunohistochemistry and activation of SOD and content of MDA by chemochromatometry. RESULTS: The content of HSP70 in myocardial cells during the operation was not significantly different in both groups (P > 0. 05). The content of HSP70 significantly decreased in group B compared with that of group A (P <0.05). The activity of SOD significantly decreased and the content of MDA significantly increased in group B during the operation (P < 0.05). CONCLUSION: Decreased expression of HSP70 is associated with the decreased tolerance to ischemia-reperfusion injury in myocardial cells of cyanosis congenital heart disease.

Double Outlet Right Ventricle↗
PubMed1999

Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device.

A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. Cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999.

Coronary Circulation↗
PubMed1982

Right pulmonary artery to left atrium communication. An unusual cause of cyanosis in the newborn.

A one-day-old newborn infant presented with intense cyanosis, a continuous murmur, and mild congestive heart failure. The chest roentgenogram showed an abnormal right-heart border, and the echocardiogram demonstrated enlargement of the left ventricle and left atrium. Cardiac catheterization and angiography demonstrated a right pulmonary artery to left atrium communication. The infant responded favorably to medical management and is asymptomatic with the exception of mild cyanosis with crying. Right pulmonary artery to left atrium communication is a rare but potentially correctable cause of cyanosis in the newborn.

Adult↗
PubMed1987

Intraoperative cyanosis: a case of dapsone-induced methaemoglobinaemia.

Intraoperative cyanosis is most commonly caused by hypoxaemia. The anaesthetist is required to perform a rapid series of diagnostic manoeuvres and take remedial action. Occasionally methaemoglobin, sulfhaemoglobin, or haemoglobin M, undetected preoperatively, is the cause of the cyanosis. We report a case of methaemoglobinaemia secondary to dapsone ingestion that was diagnosed intraoperatively. Dapsone, a sulfone, is used therapeutically to treat leprosy and dermatitis herpetiformis. The differential diagnosis of cyanosis, and the origin and fate of methaemoglobin are discussed. In addition the diagnostic steps and the laboratory investigations required to make the diagnosis are listed.

Adolescent↗
PubMed1992

Pain and cyanosis associated with alpha 1-proteinase inhibitor.

PURPOSE: The purpose of this study was to investigate adverse reaction reports of pain and/or cyanosis attributed to alpha 1-proteinase inhibitor (A1PI), a plasma alpha-globulin protein used to treat A1PI deficiency. PATIENTS AND METHODS: The Food and Drug Administration's (FDA) Spontaneous Reporting System for the collection and analysis of suspected adverse reactions to drugs and biologics was searched for all reports with dates from January 1, 1988, through October 31, 1989, in which A1PI was named as the suspect biologic. A case of pain and/or cyanosis was defined and characteristics of cases were compared with all other reactions. Information about the production of A1PI and results from animal studies conducted by the manufacturer were also gathered. RESULTS: Fourteen cases of acute chest pain, back pain, and/or cyanosis among patients receiving A1PI infusions were reported to the FDA. The clinical aspects of reported cases were consistent with a rapidly acting, nonallergic mechanism and were easily distinguished from other reactions associated with A1PI. The characteristics of reported cases, the epidemic curve, and lot-specific analyses suggested a point source and strongly implicated two A1PI lots. Information about the production of A1PI and results from animal studies further implicated high-molecular-weight polysaccharides associated with sucrose stabilization of the suspect lots. CONCLUSION: These cases resemble adverse reactions attributed to complexes of protamine and heparin (a mucopolysaccharide). Similar vasoactive mechanisms are suggested. Research is needed to further define the pathophysiology associated with polysaccharide moieties.

Adult↗
PubMed1994

Intermittent digital cyanosis as the sole presenting sign of pulmonary embolus: an unusual case report.

A 77-year-old man who was undergoing comprehensive rehabilitation for a right subcapital femoral neck fracture presented with intermittent digital cyanosis of 3 days duration. He had no other cardiopulmonary signs or symptoms, therefore a diagnosis of Raynaud's Phenomenon was considered but ruled out because of no prior history of similar episodes or associated conditions. A ventilation-perfusion (V/Q) scan was obtained and was highly indicative of pulmonary embolus. Dopplers and impedance plethysmography (IPG) revealed a left lower extremity deep vein thrombosis. Heparinization resulted in cessation of the cyanotic episodes. Intermittent digital cyanosis as the sole presentation of pulmonary embolus has not been previously reported in humans. Pulmonary embolus is notoriously difficult to diagnose based on clinical signs and symptoms. Intermittent peripheral cyanosis should raise the index of suspicion for the possibility of pulmonary embolus.

Aged↗
PubMed2006

Free amino acids in hearts of pediatric patients with congenital heart disease: the effects of cyanosis, age, and pathology.

BACKGROUND: The immature heart has a much greater dependence than the adult heart on amino acid transamination in determining its ischemic tolerance. Compared with adult hearts, experimental models of the immature heart have quantified higher resting concentrations of free amino acids (AA) which are depleted by acute hypoxia. However, we have found no clinical studies that have looked at the free AA profile of the immature human heart or the effects of cyanosis, age, and pathology upon this. METHODS: One hundred eighty-one pediatric patients (37 cyanotic, 144 acyanotic) undergoing open-heart surgery were recruited. Myocardial biopsies were collected prior to ischemia and analyzed for free AAs (eg, glutamate, aspartate) using high-performance liquid chromatography. The effects of cyanosis, age, and pathology on amino acid concentrations were estimated by multiple regression modeling with and without controlling for diagnosis; the effects of age and pathology were estimated only in acyanotic children. RESULTS: Alanine concentrations were about 20% higher in cyanotic than acyanotic patients (p = 0.04). Cyanosis was not associated with any other amino acid levels. In acyanotic patients, after controlling for diagnosis, concentrations of glutamate, aspartate, and alanine decreased from birth to about 8 to 10 years, then started to increase again (p < 0.05 for both linear and quadratic terms); concentrations of taurine and the branched chain AAs decreased steadily with increasing age (p < 0.05). There were significant effects of pathology on glutamate (p = 0.006), glutamine (p = 0.003), and branched chain AA (p = 0.004) levels. CONCLUSIONS: There is no evidence that chronic hypoxia depletes endogenous AAs. Young age is associated with higher resting AA levels.

Aging↗
PubMed2004

Positional cyanosis in infants: an unusual presentation of right-sided cardiac masses.

Positional cyanosis is an uncommon finding in young patients. We report three infants who presented with positional cyanosis due to a pedunculated tumour in the right heart. Arterial desaturation was the result of right-to-left shunting at the level of the oval foramen caused by obstruction and/or insufficiency of the tricuspid valve. The obstruction at the level of the tricuspid valve was variable because of the pedunculated nature of the tumours, which gave them considerable mobility. Hence, the degree of right-to-left shunting was dependent on the position of the patient. In all the patients, surgical resection of the tumours resolved the cyanosis.

Cyanosis↗
PubMed1976

Hemoglobin Beth Israel. A mutant causing clinically apparent cyanosis.

We found that an abnormal hemoglobin with a very low oxygen affinity was responsible for overt cyanosis in an otherwise healthy adolescent. Hemoglobin Beth Israel, in which serine replaces the asparagine residue normally present at position 102 (G4) of the beta-polypeptide chain, was associated with normal blood counts and no apparent exercise intolerance in the heterozygous carrier. Cyanosis resulted from a drastically right-shifted oxygen dissociation curve, whose position and shape could account for the absence of "physiologic" anemia. The whole-blood oxygen tension at 50 per cent oxygen saturation was 88 mm Hg (normally 26 +/- 1 mm Hg), and the arterial blood was only 63 per cent saturated with oxygen despite a normal oxygen tension of 97 mm Hg. The hemolysate showed a low oxygen affinity but normal Bohr effect. Unexplained cyanosis, particularly in association with normal arterial oxygen tension should prompt a search for an abnormal hemoglobin, which may obviate the need for invasive diagnostic procedures.

Adolescent↗
PubMed2000

Roaster breast meat condemned for cyanosis: a dark firm dry-like condition?

A case-control study (n = 68) of roaster chickens condemned for cyanosis was conducted. Color (CIE L*a*b*) and pH were measured at slaughter, and after 24 h aging on ice, at four predetermined sites of the Pectoralis major. Cyanotic carcasses (dark) had a higher pH than controls at the time of slaughter and at 24 h postmortem (P < 0.01). Perimortem pH was significantly correlated with pH at 24 h postmortem (r = 0.64) and also was correlated with lightness (L*) perimortem and postmortem (24 h; r = -0.36 and -0.50, respectively). Perimortem pH was not correlated with meat redness (a*) at slaughter time and after 24 h. Ultimate pH and lightness at 24 h postmortem were also correlated. Tests based on pH, L*, and a* of the P. major were assessed: the sensitivity and specificity at various cut-off points were, respectively, pH(6.3) = 76.47 and 88.24%, L*(41) = 91.18 and 79.41%, and a*(3) = 76.47 and 97.06%. The repeatability (p) of pH and color measurements was excellent and ranged from 0.87 to 0.98. Breast meat from roasters condemned for cyanosis had dark, firm, and dry (DFD)-like traits, and accurate tests based on color and pH could be described as a means of identifying chickens condemned for cyanosis.

Animals↗
PubMed1986

Notifications of industrial chemical cyanosis poisoning in the United Kingdom 1961-80.

In 325 cases of industrial chemical cyanosis notified to Her Majesty's Factory Inspectorate for 1961-80 the incidence of poisonings showed considerable seasonal variation with substantially greater numbers occurring in the summer months. A correlation between the number of poisonings in any one year and the hotness of that summer was also shown. The vast majority of incidents occurred during the manufacture of chemicals or dyestuffs, and two particular workplaces were responsible for 70% of the cases. Poisoning by amino compounds appear to produce early cyanosis whereas poisoning by nitro compounds tended to produce delayed cyanosis. These latter compounds were also more likely to produce anaemia. Methaemoglobin was determined in 45% of cases with results ranging from less than a few per cent to over 50%. Despite concentrations of MetHb over 50%, several workers complained only of headache and their blue appearance.

Adolescent↗
PubMed2006

Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival.

BACKGROUND: Limited data exist with which to stratify risk in adult congenital heart disease (ACHD). An increased ventilatory response to exercise, expressed as ventilation per unit of carbon dioxide production (V(E)/V(CO2) slope), is an established predictor of impaired survival in acquired heart disease. We sought to establish the distribution, relation to cyanosis, and prognostic value of the V(E)/V(CO2) slope across a wide spectrum of ACHD patients. METHODS AND RESULTS: Five hundred sixty ACHD patients of varying diagnoses and 50 healthy controls underwent cardiopulmonary exercise testing at a single laboratory between 2001 and 2004. Patient age was 33.2 +/- 12.9 years (mean +/- SD). Peak oxygen consumption was 23.5 +/- 9.0 mL.kg(-1).min(-1).V(E)/V(CO2) slope for all patients was 36.3 +/-15.3. The slope was raised in all ACHD groups compared with controls and was 73% higher in cyanotic patients. Cyanosis, with or without pulmonary arterial hypertension, was the strongest predictor of abnormal V(E)/V(CO2) slope. The V(E)/V(CO2) slope was the most powerful univariate predictor of mortality in the noncyanotic group and the only independent predictor of mortality among exercise parameters on multivariate analysis. In cyanotic patients, no parameter was predictive of death. CONCLUSIONS: Ventilatory response to exercise is abnormal across the spectrum of ACHD. Cyanosis is a powerful stimulus for such exaggerated ventilatory patterns irrespective of the presence of pulmonary arterial hypertension. Increased V(E)/V(CO2) slope is the strongest exercise predictor of death in noncyanotic ACHD patients.

Adult↗
PubMed1997

Embryologic features of term fetuses and newborns in CL/Fr mice with special reference to cyanosis.

OBJECTIVE: The objective of this study was to clarify the embryologic features of CL/Fr mice in relation to cyanosis. METHOD: Orofacial examinations were conducted on 18-day-old term fetuses and newborns. Except for open eyelids in a few cases, all of the external abnormalities in this strain were cleft lip and/or palate (CLP). RESULTS: The CLP incidence in term fetuses was 28.4%. The body and placental weights were not different between CLP(+) and CLP(-) fetuses. Several types of CLP were found; the most frequent was bilateral complete CLP (60%) followed by left complete (16%). Bilateral complete CL, which is the most severe CL type, was associated with poorly developed secondary palate and wide cleft. In newborns, 39.4 % of CLP(+) mice showed cyanosis, where no CLP(-) mice had such signs. The body weight of cyanotic mice with CLP was significantly lighter than that of noncyanotic mice with CLP. Cyanotic mice had severe types of CLP and unelevated palatal shelves. CONCLUSION: We suggest that mechanical airway obstruction may be one of the causes of cyanosis in newborn CL/Fr mice.

Airway Obstruction↗
PubMed2003

[A case report of the laryngeal edema and peripheral cyanosis after extubation of the tracheal tube].

A 65-year-old male in malnutrition due to advanced colon cancer underwent resection of transverse colon tumor and the invaded abdominal muscles with necrosis and abscess. After epidural catheter insertion between Th 10-11 for 9 cm cephalad, anesthesia was induced with thiopental 200 mg and fentanyl 50 micrograms. Tracheal intubation was done with vecuronium 5 mg, and anesthesia was maintained with sevoflurane with nitrous oxide in oxygen and epidural block. During surgery, systolic blood pressure often went up to 130 to 140 mmHg and down to 50 to 60 mmHg. Dopamine 3-5 micrograms.kg-1.min-1 was administered but occasional ephedrine bolus injection was still necessary. The intestine, including the intact part, was edematous. After the surgery, when systolic blood pressure was stable at about 130 mmHg and his consciousness was clear with regular spontaneous respiration, the tracheal tube was removed. However, soon after the extubation, expiratory stridor and cyanosis of the bilateral hands and feet were observed. Hydrocortisone 200 mg and nicardipine 0.5 mg were administered and room temperature was raised. About 30 minutes later, stridor and cyanosis subsided. In the ward after surgery, only hoarseness was observed. The stridor might have been due to the laryngeal edema, which could be attributed to stimulation by tracheal tube in the patient with malnutrition. The hemodynamic instability during surgery and cyanosis after extubation might have come from changes of the vascular resistance by sepsis.

Aged↗
PubMed2004

[Methemoglobinemia in children as a differential diagnosis of cyanosis].

Methemoglobinemia is characterized by cyanosis with various degrees of severity. Symptoms range from asymptomatic to unconsciousness and death. Although cyanosis caused by methemoglobinemia is well-documented in the literature, it is rare and the consequences can be fatal. The cause of methemoglobinemia can be environmental, acquired, congenital or a combination of the above. Among the potential sources for methemoglobinemia are local anaesthetics that are in common use in hospitals and clinics, some of which can be purchased without prescription. Although these drugs are considered to be safe, they can still induce methemoglobinemia and can be life-threatening. In this review we describe patients admitted to the Pediatric Intensive Care Unit during the year 2000 suffering from cyanosis and diagnosed as having methemoglobinemia.

Child↗
PubMed1990

[Diagnosis of cyanosis and digital clubbing caused by liver cirrhosis].

Six cases of cyanosis and digital clubbing caused by liver cirrhosis were reported. The mean levels of PaO2 and PaCO2 were 6.4 +/- 0.8 kPa (48.3 +/- 6.2 mmHg) and 3.9 +/- 0.8 kPa (29.5 +/- 6.1 mmHg) respectively. The cause of cyanosis and digital clubbing in liver cirrhosis was discussed. Abnormal intrapulmonary shunting (IPS), which was proved in 4 cases by whole-body radionuclide scanning with 99mTc-MAA, is suggested as the major cause of cyanosis in liver cirrhosis. Clinically IPS is usually associated with debilitating conditions characterized by hyperventilation, abnormality of lung diffusion and orthodeoxia or platypnea. The authors suggest that whole-body radionuclide scanning with 99mTc-MAA may be performed for establishing IPS if liver cirrhosis is highly suspected to be the cause of severe hypoxemia.

Adult↗
PubMed1990

[The importance of Doppler echocardiography in the differential diagnosis of causes of neonatal cyanosis].

The authors report 329 dopplerechocardiographic examinations performed on 167 term and preterm newborn babies presenting cyanosis. Seventy-six congenital heart defects were detected among their patients, 12 of whom were operated without heart catheterization, according to the ultrasound finding. The diagnosis of ten inoperable heart diseases established without hemodynamic examination was confirmed by autopsy. Cyanosis due to cardiac and pulmonary abnormalities without congenital heart defect was diagnosed in 38 patients. Persistent ductus arteriosus was found in 45 very lowbirth-weight premature babies. Doppler echocardiography proved to be a major advance in the differential diagnosis of newborn cyanosis and in choosing appropriate therapy.

Cyanosis↗
PubMed1990

["Acro-erythro-cyanosis"--peculiar vasomotor symptoms due to cervical hernial myelopathy].

Two cases of cervical myelopathy, which exhibited peculiar vasomotor symptoms ("acro-erythro-cyanosis") on distal regions of the four limbs are reported. Continuous reddening, swelling and skin temperature increase were observed on both hands and feet in case 1, a-44-year-old man, and on both hands in case 2, a-47-year-old man. Cold stimulation resulted in cyanosis and decrease of skin temperature on the affected regions paroxysmally. The condition of skin at room temperature may be caused by arteriectasia of arterioles due to hypotonia of vasomotor fibers, and this was similar to erythromelalgia. On the other hand, cold stimulation may have led to this condition, where the contraction of skin arterioles due to hypertonia of vasomotor fibers was added to the dilation of venule, and this was similar to acrocyanosis. Consequently, we provisionally named the vasomotor symptoms in the present cases as "acro-erythro-cyanosis". The other neurological signs were as follows. Case 1: mild weakness in right upper and lower limbs and left small hand muscles, mild superficial and deep sensory disturbance on bilateral palms and soles and decrease of vibration on bilateral lumbar regions and thereunder. Case 2: mild weakness of right small hand muscles, superficial sensory disturbance on distal regions of bilateral upper and lower limbs and a decrease of joint position sense on right hand. Myelography and metrizamide CT myelography revealed a high-degree deformity of the spinal cord due to the herniated disks between C4 and C5 in case 1 and between C3 and C4 in case 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗