Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight <2.5 kg.

OBJECTIVE: To compare management strategies for neonates <2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR). METHODS: Consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing <2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group. RESULTS: The cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P = .735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P = .214), as well as during short-term (<4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P = .071) and midterm (>4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P = .717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P = .072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group. CONCLUSIONS: In this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight <2.5 kg, mortality and reintervention burden was high and independent of treatment strategy. Other potential advantages were observed with each approach.

Cardiovascular, hematological and neurosensory impact of COVID-19 and variants.

OBJECTIVE: The purpose of this article was to review our clinical experience with COVID-19 patients observed in the Cardiovascular Division of Pompidou Hospital (University of Paris, France) and the Department of Neurology of the Eastern Piedmont University (Novara, Italy), related to the impact on the cardiovascular, hematological, and neurologic systems and sense organs. PATIENTS AND METHODS: We sought to characterize cardiovascular, hematological, and neurosensory manifestations in patients with COVID-19 and variants. Special attention was given to initial signs and symptoms to facilitate early diagnosis and therapy. Indications of ECMO (extracorporeal membrane oxygenation) for cardiorespiratory support were evaluated. RESULTS: Preliminary neurosensorial symptoms, such as anosmia and dysgeusia, are useful for diagnosis, patient isolation, and treatment. Early angiohematological acro-ischemic syndrome includes hand and foot cyanosis, Raynaud digital ischemia phenomenon, skin bullae, and dry gangrene. This was associated with neoangiogenesis, vasculitis, and vessel thrombosis related to immune dysregulation, resulting from "cytokine storm syndrome". The most dangerous complication is disseminated intravascular coagulation, with mortality risks for both children and adults. CONCLUSIONS: COVID-19 is a prothrombotic disease with unique global lethality. A strong inflammatory response to viral infection severely affects cardiovascular and neurological systems, as well as respiratory, immune, and hematological systems. Rapid identification of acro-ischemic syndrome permits the treatment of disseminated intravascular coagulation complications. Early sensorial symptoms, such as gustatory and olfactory loss, are useful for COVID-19 diagnosis. New variants of SARS-CoV-2 are emerging, principally from United Kingdom, South Africa, and Brazil. These variants seem to spread more easily and quickly, which may lead to more cases of COVID.

Assessment of the conjunctival microcirculation in adult patients with cyanotic congenital heart disease compared to healthy controls.

PURPOSE: Congenital heart disease (CHD) is the most common live birth defect and a proportion of these patients have chronic hypoxia. Chronic hypoxia leads to secondary erythrocytosis resulting in microvascular dysfunction and increased thrombosis risk. The conjunctival microcirculation is easily accessible for imaging and quantitative assessment. It has not previously been studied in adult CHD patients with cyanosis (CCHD). METHODS: We assessed the conjunctival microcirculation and compared CCHD patients and matched healthy controls to determine if there were differences in measured microcirculatory parameters. We acquired images using an iPhone 6s and slit-lamp biomicroscope. Parameters measured included diameter, axial velocity, wall shear rate and blood volume flow. The axial velocity was estimated by applying the 1D + T continuous wavelet transform (CWT). Results are for all vessels as they were not sub-classified into arterioles or venules. RESULTS: 11 CCHD patients and 14 healthy controls were recruited to the study. CCHD patients were markedly more hypoxic compared to the healthy controls (84% vs 98%, p = 0.001). A total of 736 vessels (292 vs 444) were suitable for analysis. Mean microvessel diameter (D) did not significantly differ between the CCHD patients and controls (20.4 ± 2.7 µm vs 20.2 ± 2.6 µm, p = 0.86). Axial velocity (Va) was lower in the CCHD patients (0.47 ± 0.06 mm/s vs 0.53 ± 0.05 mm/s, p = 0.03). Blood volume flow (Q) was lower for CCHD patients (121 ± 30pl/s vs 145 ± 50pl/s, p = 0.65) with the greatest differences observed in vessels >22 µm diameter (216 ± 121pl/s vs 258 ± 154pl/s, p = 0.001). Wall shear rate (WSR) was significantly lower for the CCHD group (153 ± 27 s-1 vs 174 ± 22 s-1, p = 0.04). CONCLUSIONS: This iPhone and slit-lamp combination assessment of conjunctival vessels found lower axial velocity, wall shear rate and in the largest vessel group, lower blood volume flow in chronically hypoxic patients with congenital heart disease. With further study this assessment method may have utility in the evaluation of patients with chronic hypoxia.

Cutaneous Manifestations of COVID-19.

Patients with coronavirus disease 2019 (COVID-19) present with multisystem signs and symptoms, including dermatologic manifestations. The recent literature has revealed that dermatologic manifestations of COVID-19 often are early onset and provide helpful cues to a timely diagnosis. We compiled the relevant emerging literature regarding the dermatologic manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) so that physicians can be aware of the various clinical cutaneous presentations in this time of high incidence of COVID-19.

Chronic perinatal hypoxia delays cardiac maturation in a mouse model for cyanotic congenital heart disease.

Compared with acyanotic congenital heart disease (CHD), cyanotic CHD has an increased risk of lifelong mortality and morbidity. These adverse outcomes may be attributed to delayed cardiomyocyte maturation, since the transition from a hypoxic fetal milieu to oxygen-rich postnatal environment is disrupted. We established a rodent model to replicate hypoxic myocardial conditions spanning perinatal development, and tested the hypothesis that chronic hypoxia impairs cardiac development. Pregnant mice were housed in hypoxia beginning at embryonic day 16. Pups stayed in hypoxia until postnatal day (P)8 when cardiac development is nearly complete. Global gene expression was quantified at P8 and at P30, after recovering in normoxia. Phenotypic testing included electrocardiogram, echocardiogram, and ex vivo electrophysiology study. Hypoxic P8 animals were 47% smaller than controls with preserved heart size. Gene expression was grossly altered by hypoxia at P8 (1,427 genes affected), but normalized after recovery (P30). Electrocardiograms revealed bradycardia and slowed conduction velocity in hypoxic animals at P8, with noticeable resolution after recovery (P30). Notable differences that persisted after recovery (P30) included a 65% prolongation in ventricular effective refractory period, sinus node dysfunction, 23% reduction in ejection fraction, and 16% reduction in fractional shortening in animals exposed to hypoxia. We investigated the impact of chronic hypoxia on the developing heart. Perinatal hypoxia was associated with changes in gene expression and cardiac function. Persistent changes to the electrophysiological substrate and contractile function warrant further investigation and may contribute to adverse outcomes observed in the cyanotic CHD population.NEW & NOTEWORTHY We utilized a new mouse model of chronic perinatal hypoxia to simulate the hypoxic myocardial conditions present in cyanotic congenital heart disease. Hypoxia caused numerous abnormalities in cardiomyocyte gene expression, the electrophysiologic substrate of the heart, and contractile function. Taken together, alterations observed in the neonatal period suggest delayed cardiac development immediately following hypoxia.

Systematic Review of Negative Pressure Pulmonary Edema in Otolaryngology Procedures.

OBJECTIVE(S): Negative pressure pulmonary edema (NPPE) is a rare perioperative complication with a potentially fatal outcome. The aim of this study was to perform a systematic review of NPPE in adult otolaryngology procedures with the goal of identifying risk factors, clinical presentation, diagnosis, management and outcomes. METHODS: Systematic review performed using PubMed, Scopus, Web of Science, and Cochrane databases. RESULTS: Sixty-nine studies including data from 87 individual patients were included in this review. Fifty-six (68%) patients were male and the average patient age was 37 years old. Type 1 NPPE occurred in 63 (72%) cases, while type 2 NPPE accounted for 20 (23%) cases. The most common procedures leading to NPPE were septoplasty, rhinoplasty or sinus surgery (n = 22, 25%), directly laryngoscopy or bronchoscopy (n = 13, 15%), and tracheostomy or cricothyroidotomy (n = 11, 13%). The most employed treatment options included diuretics (n = 55, 63%) and mechanical ventilation (n = 54, 62%). Seventy-eight (90%) patients made a full recovery with an average time to NPPE resolution of 33 hours and an average length of hospitalization of 5.6 days. Five (6%) patients had a long-term morbidity and four (5%) patients died, with age and ICU stay increasing risk for death and long-term morbidity (OR 1.044 and 7.42, respectively, P < .05). CONCLUSION: Septoplasty, rhinoplasty and sinus surgery account for the majority of NPPE cases in adult otolaryngology procedures. Young, healthy patients are the most commonly involved with a slight male predominance. The vast majority of patients recover fully, however there is a significant risk for morbidity and mortality.

Pulmonary embolism and acro-ischemia in a lung cancer patient with COVID-19 / Tromboembolismo pulmonar y acro-isquemia en un paciente con cáncer de pulmón y COVID-19

No disponible

Initiation of peritoneal dialysis in a patient with chronic renal failure associated with tetralogy of Fallot: a case report.

BACKGROUND: Tetralogy of Fallot is the most common cyanotic congenital heart disease. Patients with the condition have a high risk of developing chronic kidney disease. Treatment of kidney disease in patients with complex hemodynamics presents unique challenges. However, there are very few reports on the treatment of end-stage renal failure in patients with tetralogy of Fallot. CASE PRESENTATION: We present a rare case of peritoneal dialysis in a 47-year-old man with tetralogy of Fallot who had not undergone intracardiac repair. Peritoneal dialysis successfully removed fluids and solutes without adversely affecting the patient's hemodynamics. Our patient was managed with peritoneal dialysis for 5 years before he succumbed to sepsis secondary to digestive tract perforation. CONCLUSIONS: In this paper, we discuss the importance of monitoring acid-base balance, changes in cyanosis, and hyperviscosity syndrome during peritoneal dialysis in patients with tetralogy of Fallot. Lower leg edema and B-type natriuretic peptide level were useful monitoring parameters in this case. This case illustrates that with attention to the patient's unique requirements, peritoneal dialysis can provide successful renal replacement therapy without compromising hemodynamics in patients with tetralogy of Fallot.

[Position dependent dyspnea and cyanosis of the lips at the daily round - A case of Platypnea-Orthodeoxia Syndrome]. / Lageabhängige Dyspnoe und Lippenzyanose im Rahmen der täglichen Visite ­ Platypnoe-Orthodeoxie Syndrom.

HISTORY AND CLINICIAL FINDINGS: We elaborate a case of a 79-year-old patient who presented with position dependent shortness of breath and cyanosis of the lips at the daily round. INVESTIGATIONS: In supine position the patient's oxygen saturation was normal > 95 %, in upright position we noticed a reproducible decrease to 76-85 %. Echocardiographic examination revealed a patent foramen ovale (PFO) with spontaneous right-left shunt and additionally a thoracic aortic aneurysm. DIAGNOSIS: Due to a typical position dependent cyanosis, dyspnea and decreased oxygen saturation in combination with patent foramen ovale and aortic aneurysm, Platypnea-Orthodeoxia Syndrome was our suspected diagnosis. TREATMENT AND COURSE: After percutaneously occlusion of the foramen ovale the patient presented symptom-free und oxygen saturation remained stable in supine and upright positions. In the follow up examination a significant right-left shunt could no longer be found. CONCLUSION: The combination of position dependent shortness of breath and decreased oxygen saturation should lead to the diagnosis of Platypnea-Orthodeoxia Syndrome. The underlying reasons are diverse and not only of cardiologic origin.

Prenatal Growth in Fetuses with Isolated Cyanotic and Non-Cyanotic Congenital Heart Defects.

BACKGROUND: Fetal growth may vary significantly in different congenital heart defects (CHDs). OBJECTIVES: To investigate prenatal growth of CHD fetuses and its correlation with classifications based upon expected oxygen delivery to the fetal brain or structural findings. METHODS: Seventy-nine euploid fetuses with isolated CHD were recruited prospectively and categorized by the expected oxygen supply to the brain (low, intermediate, and high) or by the expected arterial mixing considering two categories (cyanotic or non-cyanotic). Biometry and Doppler were recorded, and Z-scores (Zs) calculated. Growth changes at different time points were analyzed and compared with 150 controls. RESULTS: A total of 664 exams were performed on 229 fetuses. Median head circumference (HC) Zs were lower in all CHD fetuses from the second trimester onwards and in cyanotic CHD fetuses from the first onwards, with associated smaller abdominal circumference (AC) in the third trimester (first-trimester biparietal diameter Zs cyanotic: -1.3 [-2.36; -0.98], non-cyanotic -0.72 [-1.25; -0.6], p = 0.044, second-trimester HC Zs cyanotic: -1.47 [-2.3; -0.84]; non-cyanotic -0.45 [-0.83; -0.02], p < 0.0001; AC Zs cyanotic 0.0 [-0.44; 0.86]; non-cyanotic 0.65 [0.31; 1], p = 0.0006). Birth-weight centiles were smaller in CHDs (particularly in cyanotic) with no differences between categories of brain oxygen delivery. CONCLUSIONS: Fetuses with cyanotic CHD have fetal growth restriction, impaired head growth, yet normal posterior fossa dimensions and fetal-placental Doppler.

The Kidney in Congenital Cyanotic Heart Disease.

This column is supplied by Benjamin J. Lee, MD, MAS, an assistant professor of clinical medicine at both the Houston Methodist Institute for Academic Medicine and Weill Cornell Medical College. After earning his medical degree at Harvard Medical School, Dr. Lee completed a residency in internal medicine at the University of California, San Francisco (UCSF). He subsequently completed a nephrology fellowship at UCSF while simultaneously obtaining a Master of Advanced Study in clinical research from the UCSF Department of Epidemiology and Biostatistics. Dr. Lee is a Fellow of the American Society of Nephrology, a Certified Hypertension Specialist through the American Hypertension Specialist Certification Program, and a member of the American Society of Transplantation. He maintains his clinical practice with the Houston Kidney Consultants.

Removal of a minimal amount of subdural hematoma is effective and sufficient for term neonates with severe symptomatic spontaneous parenchymal hemorrhage.

INTRODUCTION: Spontaneous parenchymal hemorrhage of term neonates is usually asymptomatic and does not require surgical intervention. However, there is no consensus on the management of cases with severe life-threatening symptoms, including repeated apnea, respiratory failure with severe cyanosis, severe bradycardia, or uncontrolled seizures. CASES: Our medical records of term neonates with intracranial hemorrhage who underwent surgical intervention were retrospectively reviewed. There were two cases with spontaneous parenchymal hemorrhage. Both cases were delivered vaginally without any use of forceps or vacuum devices. Neither of them showed asphyxia, hypoxic-ischemic encephalopathy, hematological abnormalities, congenital vascular anomalies, infection, or birth trauma. Common symptoms included apnea, cyanosis, bradycardia, and decreased consciousness. The original location of bleeding was the parenchyma of the right temporal lobe. The hemorrhage extended to subdural spaces in both cases. Subdural hematoma (SDH) removal was performed without manipulating the parenchymal hematoma. Only a small amount of SDH (approximately 5 ml) was drained spontaneously with irrigation, which was sufficient to decrease the elevated intracranial pressure. The patients' respiratory conditions improved dramatically after the surgery. CONCLUSION: We propose that removing only a small amount of SDH would be effective and sufficient to relieve severe symptoms of increased intracranial pressure in term neonates with massive spontaneous parenchymal hemorrhage.

Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung.

Clinically significant unilateral pulmonary arteriovenous malformations (PAVM) can develop in patients with a Fontan circulation when there is unbalanced distribution of hepatic venous (HV) blood flow to the lungs. There are reported surgical and transcatheter techniques to treat PAVMs by rerouting HV return, with promising short-term results. We report a case of a novel, technically simple transcatheter approach to redirect HV blood flow in an adult Fontan patient with polysplenia syndrome and severe unilateral PAVMs. Our patient had a two-stage procedure, the first to redirect all HV blood flow to the affected lung with a single covered stent, and a second to confirm resolution of PAVMs and to reintroduce HV effluent to the unaffected lung. At 10-month follow-up, her oxygen saturations had increased from 75% to 93% with a marked improvement in her functional status.

Evaluation of Heart Rate Variability in Children With Breath-Holding Episodes.

AIM: We evaluated heart rate variability in children with breath-holding episodes (BHEs). METHODS: Sixty children with BHEs were included in the study; these individuals were further subdivided into children with cyanotic BHEs (n = 42) and children with pallid BHE (n = 18). Sixty healthy children of matched age and sex served as a control group. Twenty-four hour Holter monitoring was applied to all included children. Minimum, mean, maximum heart rate, rhythms, and corrected QT were evaluated. Time domain parameters of heart rate variability such as standard deviation of all R-R intervals, standard deviation of the average of R-R intervals in all five-minute segments of the entire recording, mean of the standard deviations of all N-N (normal-normal RR) intervals for all five-minute segments, root mean squares differences between adjacent R-R intervals, percentage of differences between adjacent R-R intervals that are greater than 50 milliseconds were also assessed. RESULTS: All time domain parameters of heart rate variability were significantly higher in children with pallid BHEs than those with cyanotic BHEs and control group. Minimum, mean, and maximum heart rate were significantly lower in children with pallid BHEs than those with cyanotic BHEs and control group. Asystole was observed in three children with pallid BHEs. Long corrected QT was observed in another two children with pallid BHEs. CONCLUSIONS: Heart rate variability increased significantly in children with pallid BHEs. Evaluation of heart rate variability is crucial for children with BHEs especially those with pallid episodes.

Hemodynamic Impact of Oxygen Desaturation During Tracheal Intubation Among Critically Ill Children With Cyanotic and Noncyanotic Heart Disease.

OBJECTIVES: To determine a level of oxygen desaturation from baseline that is associated with increased risk of tracheal intubation associated events in children with cyanotic and noncyanotic heart disease. DESIGN: Retrospective analysis of prospectively collected data from the National Emergency Airway Registry for Children, an international multicenter quality improvement collaborative for airway management in critically ill children. SETTING: Thirty-eight PICUs from July 2012 to December 2016. PATIENTS: Children with cyanotic and noncyanotic heart disease who underwent tracheal intubation in a pediatric or cardiac ICU. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Our exposure of interest was oxygen desaturation measured by a fall in pulse oximetry from baseline after preoxygenation. Primary outcome was the occurrence of hemodynamic tracheal intubation associated events defined as cardiac arrest, hypotension or dysrhythmia. One-thousand nine-hundred ten children (cyanotic, 999; noncyanotic, 911) were included. Patients with cyanotic heart disease who underwent tracheal intubations were younger (p < 0.001) with higher Pediatric Index of Mortality 2 scores (p < 0.001), more likely to have a cardiac surgical diagnosis (p < 0.001), and less likely to have hemodynamic instability (p = 0.009) or neurologic failure as an indication (p = 0.008). Oxygen desaturation was observed more often in children with cyanotic versus noncyanotic heart disease (desaturation of 15% to < 30%: 23% vs 16%, desaturation ≥ 30%: 23% vs 17%; p < 0.001), with no significant difference in occurrence of hemodynamic tracheal intubation associated events (7.5% vs 6.9%; p = 0.618). After adjusting for confounders, oxygen desaturation by 30% or more is associated with increased odds for adverse hemodynamic events (odds ratio, 4.03; 95% CI, 2.12-7.67) for children with cyanotic heart disease and (odds ratio, 3.80; 95% CI, 1.96-7.37) for children with noncyanotic heart disease. CONCLUSIONS: Oxygen desaturation was more commonly observed during tracheal intubation in children with cyanotic versus noncyanotic heart disease. However, hemodynamic tracheal intubation associated event rates were similar. In both groups, oxygen desaturation greater than or equal to 30% was significantly associated with increased occurrence of hemodynamic tracheal intubation associated events.

Serum Iron Status of Children with Cyanotic Congenital Heart Disease in Lagos, Nigeria.

OBJECTIVES: Cyanotic congenital heart disease (CCHD) predisposes patients to iron deficiency due to compensatory secondary erythrocytosis. This study aimed to determine the serum iron status and prevalence of iron deficiency among children with cyanotic congenital heart disease attending the Paediatric Cardiology outpatient clinic of Lagos State University Teaching Hospital, Lagos, Nigeria. METHODS: This cross-sectional case-control study took place between May and October 2015 at the Lagos State University Teaching Hospital. A total of 75 children with cyanotic congenital heart disease and 75 apparently healthy age-, gender- and socioeconomically-matched controls were analysed to determine serum iron status and the prevalence of iron deficiency as defined by the World Health Organization criteria. RESULTS: The mean age of the children was 47.5 ± 2.9 months (range: 6-144 months old). Iron deficiency was significantly more frequent among CCHD patients compared to control subjects (9.3% versus 0%; P = 0.006). While latent iron deficiency was more prevalent among children in the control group compared to those with CCHD, this difference was not statistically significant (13.3% versus 9.3%; P = 0.303). No cases of iron deficiency anaemia were observed in the studied sample. CONCLUSION: Neither the children in the control group nor those with CCHD had iron deficiency anaemia. However, iron deficiency was significantly more prevalent among children with CCHD in Lagos. Periodic serum iron status screening is therefore recommended for this population.