Gastrostomy Button Placement in Infants With Cyanotic Versus Acyanotic Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) may exhibit increased metabolic demands, and many will undergo placement of a gastrostomy to achieve adequate nutritional intake. There is a paucity of data, however, comparing the operative risks and overall complications of gastrostomy placement in cyanotic versus acyanotic infants with CHD. We hypothesized that patients with cyanotic CHD would have a higher rate of gastrostomy-associated complications than infants with acyanotic CHD. METHODS: We retrospectively reviewed patients who underwent gastrostomy button placement after cardiac surgery for CHD between 2013 and 2018. Patients were stratified into cyanotic CHD and acyanotic CHD cohorts. Patient data were extracted from the Society of Thoracic Surgeons database and merged with clinical data related to gastrostomy placement and complications from chart review. Unadjusted analyses were used to find covariates associated with cyanotic CHD and acyanotic CHD, using a t-test or Wilcoxon rank-sum test for continuous data, depending on normalcy, and χ2 or Fisher's exact tests for categorical data depending on the distribution. RESULTS: There were 257 infants with CHD who underwent gastrostomy placement during the study period, of which 86 had cyanotic CHD. There were no significant differences in baseline weight or preoperative albumin levels between the two groups. Patients with cyanotic CHD had a lower incidence of comorbid syndromes (P = 0.0001), higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery scores (P < 0.0001), and higher postoperative mortality rate (P = 0.0189). There was a higher rate of granulation tissue formation in patients with acyanotic CHD (48.5% versus 22.1%, P < 0.0001). There were no differences in other gastrostomy button-related complications, including leakage, wound infection, or dislodgement. CONCLUSIONS: Patients with acyanotic CHD demonstrated a higher incidence of granulation tissue. We found no difference in gastrostomy-specific complication rates between the two groups, with the notable exception of granulation tissue formation. Based on this study, the diagnosis of cyanotic CHD does not increase the risk of gastrostomy-related complications.

Early or late fresh frozen plasma administration in newborns and small infants undergoing cardiac surgery: the APPEAR randomized trial.

BACKGROUND: In newborns and small infants undergoing cardiac surgery with cardiopulmonary bypass (CPB) and blood priming, it is unclear whether there is reduced blood loss if fresh frozen plasma (FFP) is added to the CPB priming volume. This single-centre, randomized trial tested the hypothesis that the administration of FFP after CPB (late FFP group) is superior to FFP priming (early FFP group) in terms of postoperative bleeding and overall red blood cell (RBC) transfusion. METHODS: Seventy-three infants weighing <10 kg were randomly allocated to receive FFP to supplement RBCs in the CPB priming solution ( n =36) or immediately after CPB ( n =37). The primary endpoint was a difference in postoperative blood loss; secondary endpoints included the amount of RBCs and FFP transfused through the first 48 postoperative hours. RESULTS: All patients were included in the analysis. Patients in the late FFP arm had greater postoperative mean blood loss than patients in the early FFP arm [33.1 ( sd 20.6) vs 24.1 (12.9) ml kg -1 ; P =0.028], but no differences in transfusions were found. The subgroup of cyanotic heart disease patients had comparable results, but with greater use of RBCs in the late FFP group. CONCLUSIONS: In infants undergoing cardiac surgery, FFP in the priming solution appears slightly superior to late administration in terms of postoperative bleeding. CLINICAL TRIAL REGISTRATION: www.ClinicalTrials.gov , NCT02738190.

Brief resolved unexplained event: New diagnosis in infants.

QUESTION: For many years, the term apparent life-threatening event (ALTE) was associated with sudden infant death syndrome, and parents who described an acute event in their infants were sent to the hospital for admission. I understand that for infants new terminology is recommended. What is the current approach to a near-death experience of an infant? ANSWER: A recent clinical practice guideline revised the name and definition of an ALTE to a brief resolved unexplained event (BRUE). The diagnosis of BRUE in infants younger than 1 year of age is made when infants experience 1 of the following BRUE symptoms: a brief episode (ie, less than 1 minute and usually less than 20 to 30 seconds) that is entirely resolved (infant is at baseline), which remains unexplained after the history and physical examination are completed, and includes an event characterized by cyanosis or pallor; absent, decreased, or irregular breathing; hypertonia or hypotonia; or altered responsiveness. Low-risk infants should not be admitted to the hospital and overtesting is discouraged.

An Uncommon Complication With Use of Topical Local Anesthetic Agents: Methemoglobinemia.

Although the use of topical local anesthetics is generally safe, several potentially fatal complications have been reported. Methemoglobinemia is a rare but potentially fatal complication. Methemoglobin is a naturally occurring oxidized metabolite of hemoglobin, and physiologic levels (<1%) are normal. Methemoglobinemia can be congenital or acquired. Several drugs including topical anesthetic agents like benzocaine can induce this condition. Sudden appearance of cyanosis, with a disproportionately better oxygen saturation of 85% after use of local anesthetics can be a helpful for diagnosis.

Red blood cell transfusion decision making in critically ill children.

PURPOSE OF REVIEW: To discuss the tradeoff between permissive anemia and administering red blood cell transfusion to children in pediatric ICUs. RECENT FINDINGS: Postsurgical mortality in adults increases abruptly if their nadir hemoglobin level falls below 5 g/dl. Patients with sepsis, even those in septic shock, and patients with upper gastrointestinal bleeding do not require red blood cell (RBC) transfusion if their hemoglobin level is above 7 g/dl. SUMMARY: Anemia is common in critically ill children and is well tolerated most of the time. RBC transfusion is required in cases of hemorrhagic shock and in children with a hemoglobin level below 5 g/dl. Children with sepsis, including septic shock, those with a severe upper gastrointestinal bleeding and all stable critically ill children, including noncyanotic cardiac children older than 28 days, do not require an RBC transfusion if their hemoglobin level is above 7 g/dl. Transfusion threshold in children with univentricular physiology and in critically ill children with a hemoglobin level between 5 and 7 g/dl remains to be determined.

[Hospital Admissions due to cyanosis episodes in newborns with gestational age of 34 weeks or more]. / Hospitalización por episodios de cianosis en recién nacidos de edad gestacional igual o mayor a 34 semanas.

OBJECTIVES: A retrospective study was performed between January 2007 and December 2012 to assess the admission rates of newborns due to episodes of cyanosis PATIENTS AND METHOD: Retrospective study that included all the newborns hospitalized with episodes of cyanosis between January 2007 and December 2012. In them were employed two study protocols that considered first and second line tests, the latter in view of recurrence of events. The first line protocol considered general biochemical tests, chest x-ray and echocardiography in selected cases, while the second line protocol included electroencephalogram, electrocardiogram, nuclear magnetic resonance of the brain, expanded metabolic screening, pyruvic acid, lactic acid, and in case of seizures, cytochemical, and culture of cerebrospinal fluid (CSF) and PCR (polymerase chain reaction) for herpes. RESULTS: A total of 98 (2.8%) out of 3,454 newborns were admitted due to episodes of cyanosis. Gestational age: 37.8+1.4 weeks, birth weight: 3,145+477 g. Maternal age: 32+4.8 years. Disease was present in 19.4% of mothers; gestational diabetes (8.1%), pregnancy induced hypertension (5.1%), intrahepatic cholestasis (3.1%), and intrauterine growth retardation (3.1%). Gender: 48.8% male, 51.2% female (NS). Birth: caesarean section, 68.4%, and vaginal delivery, 31.6%. Age on admission 1.9+1.4 days. Hospital stay: 4.2+4.2 days. First line tests were performed in 100% of patients with 39.8% fulfilling the criteria for second line study. A condition was detected in 21.4%, with convulsive syndrome was the most frequent (33%). Newborns with an identified condition had 3.8+2.7 episodes versus 1.5+2,4 in those without diagnosis (NS). A home oxygen monitor was given to 15.4%. There were no re-admissions. CONCLUSIONS: Most newborns admitted due to cyanosis are discharged with a condition of unknown origin. In this study, convulsive syndrome was the most frequent cause.

Ventricular fibrillation in loop recorder memories in a patient with early repolarization syndrome.

We report the first documentation of spontaneous ventricular fibrillation by a loop recorder in a patient with an ECG pattern of early repolarization (ER) in the inferior leads and presenting with syncope.

Acquired methemoglobinemia in children presenting to Italian pediatric emergency departments: a multicenter report.

INTRODUCTION: Methemoglobinemia (MetHb) is a rare inherited or acquired cause of cyanosis in children. Owing to its rarity, case reports and case series are mostly available in the current literature. This study reports data on a large sample of children with acquired MetHb. METHODS: Data on patients admitted for acquired MetHb between January 2007 to December 2020 was extracted from the databases of five Italian pediatric emergency departments (EDs). Demographical and clinical characteristics was reported and discussed. RESULTS: Nineteen cases of acquired MetHb were evaluated. Ten patients (52.6%) were male and 9 (47.4%) were female. The median age was 8.23 months. The median time from trigger to symptom onset was 6 hours. Mostly, the intoxication presumptively occurred by home ingestion of contaminated food, mainly badly preserved vegetable broth. All patients were cyanotic at admission, most patients also presented tachycardia and/or tachypnea, and two patients were comatose. Antidotal therapy with methylene blue was given in 14 patients (73.7%). The median hospital stay was 2 days. All patients survived. DISCUSSION: As MetHb leads to the pathognomonic brown blood discoloration, blood gas analysis is mandatory immediately upon hospital arrival of a cyanotic patient. A correct medical history is crucial to identify the trigger and remove it. In our sample, the age onset was much lower than in the previous literature, and MetHb mainly due to ingestion of contaminated vegetable broth. Methylene blue led to a rapid recovery in all patients. Oxygen therapy may well lead to complete recovery when methemoglobin levels do not exceed 30% in asymptomatic and 20% in mildly symptomatic patients. CONCLUSIONS: The diagnosis and management of acquired MetHb in the emergency setting requires acknowledgment of this condition as a cause of cyanosis in the weaning child. Indeed, when promptly recognized and treated, this severe condition rapidly resolves with no significant acute sequelae.

Emergency manual systemic-to-pulmonary artery autotransfusion for severe cyanotic spell.

Cyanotic spell is an important complication of tetralogy of Fallot. We report a simple, innovative method that bailed out a patient with life-threatening, refractory cyanotic spell in the catheterization laboratory. Manual autotransfusion of blood from aorta into the pulmonary arteries resulted in significant clinical improvement.

Acrocyanosis: the Flying Dutchman.

Acrocyanosis is symmetric, painless, discoloration of different shades of blue in the distal parts of the body that is marked by symmetry, relative persistence of the skin color changes with aggravation by cold exposure, and frequent association with local hyperhidrosis of hands and feet. Described over a century ago and despite seeming familiarity, it remains a poorly understood condition that shares much in clinical presentation with other conditions characterized by skin color changes in the distal parts of the body. The diagnosis remains mostly clinical, and pathological mechanisms vary suggesting that acrocyanosis may not be a single entity. We performed an extensive literature review to summarize existing knowledge about the demographics, pathology, diagnosis, and treatment of this condition.

Outcomes after rigid bronchoscopy in children with suspected or confirmed foreign body aspiration: a retrospective study.

OBJECTIVE: To identify the determinants of immediate outcome after rigid bronchoscopy for suspected or confirmed foreign body (FB) aspiration. The outcome may be affected by the duration of bronchoscopy, the type of FB, the time between inhalation and removal of the FB, and the type of anesthetic induction. Arterial desaturation, bronco-laryngospasm, and the need for tracheal reintubation as complications were investigated. DESIGN: A retrospective study. SETTING: A single tertiary care center. PARTICIPANTS: One hundred seventy-five children who underwent rigid bronchoscopy. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Age, duration after suspected or witnessed inhalation before bronchoscopy, and the type of FB had no relationship to the occurrence of complications. The prolongation of bronchoscopy beyond 30 minutes was associated with a significant increase in complications as was the use of intravenous rather than inhalation induction of anesthesia. CONCLUSIONS: Reducing the bronchoscopy time may not be an option, but an awareness of the risk of complications may prompt a more intense postanesthesia monitoring strategy.

[Management of cardiac high risk pregnancy. Caesarean section in a primagravida with cyanotic cardiac defect]. / Management bei kardialer Hochrisikoschwangerschaft. Sectio bei einer I. Gravida mit zyanotischem Vitium.

A 37-year-old gravida I with cyanotic heart disease presented for caesarean section in the 31st week of gestation. Caesarean section was performed uneventfully with the patient under epidural anaesthesia accompanied by invasive monitoring. Postoperative echocardiography showed no change in the shunt fraction, volumes or the ventricular function. Every patient with complex comorbidities has to be managed according to individual prerequisites and the experiences and preferences of the team. For such high risk pregnancies regional anaesthesia seems to be a possible option although no clear evidence can be found in the literature.

[Stent implantation into a totally occluded Blalock-Taussig shunt: a case report]. / Tam kapali Blalock-Taussig santa stent uygulamasi: Olgu sunumu.

Stent implantation into a ductus arteriosus or systemic-pulmonary shunt is a relatively new but infrequent approach for palliation. A 22-month-old boy with a modified Blalock-Taussig shunt for tetralogy of Fallot was admitted with severe cyanosis. Echocardiographic examination showed complete occlusion of the shunt. Initial balloon angioplasty resulted in reocclusion of the shunt by acute thrombosis. Finally, recanalization was achieved by stent implantation. Oxygen saturation of the patient increased from 32% to 92% following stenting. He was discharged on aspirin therapy.

Overexpression of beta(2)AR improves contractile function and cellular survival in rabbit cardiomyocytes under chronic hypoxia.

Chronic hypoxia usually evokes sustained release of endogenous neurohormones, leading to beta(2)-adrenergic receptor (beta(2)AR) desensitization and downregulation of expression, which impacts cellular contractility. We investigated whether exogenous beta(2)AR could compensate for the functional deficiency of beta(2)AR in rabbit cardiomyocytes under chronic hypoxia, and whether this led to improved contractility and cellular survival. A surgical experimental model of cyanotic heart disease was established in rabbits. Adv.hbeta(2)AR was transfected into cardiomyocytes isolated from animals subjected to 6-week systemic hypoxia. The levels of cellular contractile function, protein expression of hbeta(2)AR, p-Akt, p-Erk, and caspase-3, and cellular survival pre- and post-Adv.hbeta(2)AR delivery were determined. In the cyanotic cells, decreased shortening and lengthening of TPC and R50 were evident. Cellular diastolic functioning showed greater deterioration compared to the systolic function (P<0.05). In cyanotic cells, the positive inotropic response to isoproterenol was decreased (P<0.01), low levels of cellular survival were found, protein levels of beta(2)AR, p-Akt, and p-Erk were downregulated, and protein levels of caspase-3 were upregulated. After Adv.hbeta(2)AR delivery, enhanced contractile function was achieved (P<0.01), TPC and R50 levels recovered up to 99% and 81.7% of the normal control levels, respectively (P<0.05), and cellular survival improved (P<0.01). Our results demonstrate that overexpression of the beta(2)AR gene in cardiomyocytes exposed to chronic hypoxia provides significant catecholamine-dependent inotropic support and cellular protection.

Trans-septal closure of a right pulmonary artery to left atrial communication.

Right pulmonary artery-left atrial communication is a rare anomaly and is characterized by significant cyanosis and normal auscultation of the heart. Interventional closure of the fistula using occluder devices and coils have been rarely reported. We report an easy method of closure of the communication through trans-septal approach using an Amplatzer duct occluder.

Bronchopulmonary dysplasia in a premature infant--case report and literature review.

BACKGROUND: Bronchopulmonary dysplasia is an important cause of morbidity and mortality in premature infants. The aim of this study is to present a premature, extremely low birth weight infant with bronchopulmonary dysplasia. METHOD: A review of the case records of a child with recurrent respiratory distress and the relevant literature. RESULTS: A preterm, extremely low birth weight baby (birth weight was 0.8 Kg), delivered by emergency caesarian section for previous caesarian section and prolonged rupture of fetal membranes at 27 weeks gestational age. She had spontaneous breathing at birth (APGAR scores were 8 in one minute and 10 in 5 minutes). She developed respiratory distress with cyanosis and became oxygen dependent from the second week of life. Examination revealed severe dyspnoea with grunting respiration, tachypnoea, cyanosis and crackles in the lung fields. Chest X-ray showed hyperinflation, right lower zone patchy consolidation with obliteration of the costophrenic angle. Echocardiography was however normal. She was successively managed with intermittent oxygen, dexamethasone, salbutamol and antibiotics (ceftriaxone). She was nursed in the incubator for 3 months. There was no episode of apneic attack throughout admission. She responded to treatment and was discharged home on intermittent oxygen therapy and nebulisation. The weight on discharge was 1.6 kg. At 6 months of age, she is still having recurrent respiratory distress andsupplemental oxygen at home. She is regular to follow up with recurrent episodes of wheeze requiring admissions. CONCLUSION: Bronchopulmonary dysplasia should be suspected in a premature extremely low birth weight infant with early recurrent respiratory distress.

[Cyanosis in adult with congenital heart disease]. / Trattamento del paziente cianotico.

Cyanosis is negative predictor of survival in adult patients with congenital heart disease. When cyanosis is secondary to heart or lung disease, chronic hypoxiemia result in hematologic, neurologic, renal and reumatic complications . Is important,for the optimization of therapeutics procedure, the follow up of heart disease and of oxygen saturation and blood work. For the care of this patients are required specialized centers with a multidisciplinary team of experts.

[Acquired methemoglobinemia--case report]. / Nabyta methemoglobinemia--opis przypadku.

Methemoglobinemia; an increased concentration of methemoglobin in the blood, is an altered state of hemoglobin whereby the ferrous form of iron is oxidized to the ferric state, rendering the heme moiety incapable of carrying oxygen. The authors present a case of 49-year-old man who was admitted to the department of chest medicine with dyspnea, weakness and cyanosis in whom differential diagnosis excluded acute and chronic pulmonary and cardiovascular disease. Clinical cyanosis and low measured oxygen saturation in the presence of normal arterial oxygen tension was highly suggestive of methemoglobinemia ("saturation gap"). Methemoglobin level, measured at the acute phase of disease was elevated at 16%. Episode resolved spontaneously. Causes of methemoglobinemia was not established.

Guidelines for Thrombolytic Therapy for Frostbite.

The data are insufficient to support standardized treatment of all patients with frostbite with thrombolytic therapy. The following guidelines, however, should be applied to all patients with cyanosis persisting proximal to the distal phalanx (Grade 3 or 4 frostbite injury) and demonstrated loss of perfusion at or proximal to the middle phalanx immediately after rewarming.