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Am J Cardiol 2019 Feb 23. Epub 2019 Feb 23.

Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, Technical University Munich, Munich, Germany. Electronic address:

Adults with cyanotic congenital heart diseases (CCHD) have a higher risk for bleeding, but also for thrombosis. Rotational thromboelastometry (RT), using tissue factor (EXTEM), a contact activator (INTEM) or cytochalasin (FIBTEM), assesses coagulation by determining the time to initiation of clotting (CT) and clot firmness (MCF) including platelet-fibrin-interaction. The aim of this study was to evaluate RT and whole blood impedance aggregometry (IA) in CCHD compared with a control group without chronic cyanosis (NCCHD). Read More

Childs Nerv Syst 2019 Mar 16. Epub 2019 Mar 16.

Division of Pediatric Neurosurgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito, Ibaraki, 311-4145, Japan.

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. Read More

Turk Kardiyol Dern Ars 2019 Mar;47(2):154

Department of Pediatric Cardiology, Istanbul Medipol University, İstanbul, Turkey.

Turk Kardiyol Dern Ars 2019 Mar;47(2):140-143

Department of Cardiology, Bursa Yüksek İhtisas Training and Research Hospital, Bursa, Turkey.

A pulmonary arteriovenous malformation (PAVM) is a rare anomaly that may have significant clinical complications. PAVMs are commonly seen in patients with hereditary hemorrhagic telangiectasia, while some 10% of PAVMs may be idiopathic. PAVMs can cause cyanosis, fatigue, polycythemia, and paradoxical thromboembolic complications. Read More

BJOG 2019 Mar 14. Epub 2019 Mar 14.

Anaesthesiology& Critical Care, Jawaharlal Institute of Post-graduate Medical Education & Research (JIPMER), Puducherry, India, 605006.

Objective: To study maternal complications and pregnancy outcome in women with pulmonary hypertension, attending a tertiary centre in south India.

Study Design: Retrospective Observational study.

Setting: Tertiary centre in south India. Read More

Am J Forensic Med Pathol 2019 Mar 9. Epub 2019 Mar 9.

Department of Forensic Medicine, ESIC Medical College, Faridabad, Haryana, India.

Application of color on the external body surface before, during, and after death, such as during a festivity, cultural occasion, or after death ritual, can present as an artifact at forensic autopsy. The present study is a retrospective review of body color artifacts collected from postmortem reports, inquest papers and photographs of each individual case autopsied at our institutes during a 12 year period from 2004 to 2015. The reason for body colorations were various festivities, after death rituals and beautification products, among others. Read More

Asian Cardiovasc Thorac Ann 2019 Mar 12:218492319836899. Epub 2019 Mar 12.

1 Cardiothoracic Surgery Division, Department of Surgery, Srinagarind Hospital and Queen Sirikit Heart Center of the Northeast, Faculty of Medicine, Khon Kaen University, Thailand.

Cardiac hemangiomas are extremely rare tumors, especially in infants. We present the case of a 4-month-old girl who developed cyanosis due to a large right atrial tumor causing right ventricular inflow obstruction. The patient underwent emergency surgery for tumor resection and recovered uneventfully. Read More

Evid Based Complement Alternat Med 2019 3;2019:2543413. Epub 2019 Feb 3.

Beijing University of Chinese Medicine (Institute of Traditional Chinese Medicine; Beijing Key Laboratory of TCM Syndrome and Formula; Ministry of Education Key Laboratory of TCM Syndrome and Formula), Bei San Huan Dong Lu, Chao Yang District, Beijing 100029, China.

Objectives: This study investigated the distribution of characteristics of traditional Chinese medicine syndromes and their association with symptoms in 1027 patients with chronic heart failure (CHF).

Methods: An observational study was performed by researchers, collecting data from 1036 patients with CHF from 24 Chinese medicine hospitals from May 2009 to December 2014. Due to incomplete information from nine patients, 1027 patients with CHF were analysed. Read More

Catheter Cardiovasc Interv 2019 Mar 6. Epub 2019 Mar 6.

Bluhm Cardiovascular Institute and the Division of Cardiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.

A 60-year-old woman with progressive dyspnea and cyanosis, O2-dependent pulmonary hypertension despite optimal medical therapy and remote atrial septostomy presented with worsening cyanosis and right-to-left shunting. The creation of a "fenestrated" ASD closure device with the insertion of a peripheral stent through an AMPLATZER™ ASD closure device was deployed to minimize right to left shunting and allow for enlargement of the shunt if needed. This case demonstrates the benefit of diminishing a right to left shunt with a self-fabricated fenestrated AMPLATZER device to improve symptoms in pulmonary hypertension patients with a pre-existing ASD. Read More

Pediatr Cardiol 2019 Mar 4. Epub 2019 Mar 4.

Universidad Pontificia Bolivariana, Medellín, Colombia.

The aim of this study was to describe pediatric patients who underwent early postoperative cardiac catheterization after congenital heart surgery, their clinical indications, findings, interventions, and complications in a cardiovascular center. A descriptive retrospective study was performed. All catheterizations performed within 6 weeks after congenital heart surgery between January 2004 and December 2014 were reviewed. Read More

Sudan J Paediatr 2018 ;18(2):56-60

Cardiac Sciences Department, King Abdulaziz Cardiac Center, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia.

Pulmonary arteriovenous malformations (PAVMs) are congenital defects in the form of an anomalous bridging between a pulmonary arterial and venous system that sidesteps the normal pulmonary capillary. This anomaly is usually associated with hereditary hemorrhagic telangiectasia, leftover small group are sporadic cases but may occur as an isolated anomaly or as multiple lesions. Rarely, such abnormalities can be acquired. Read More

World J Pediatr Congenit Heart Surg 2019 01;10(1):20-27

4 Sibley Heart Center, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA.

Objectives: Current technology advances in virtual surgery modeling and computational flow dynamics allow preoperative individualized computer-based design of Fontan operation. To determine potential role of those innovations in patients undergoing hepatic vein incorporation (HVI) following Kawashima operation, we retrospectively examined historic cohort of patients who underwent HVI following Kawashima with focus on regression of pulmonary arteriovenous malformations (PAVMs).

Methods: Twenty-two children with single ventricle and interrupted inferior vena cava underwent Kawashima operation (2002-12). Read More

BMJ Case Rep 2019 Feb 22;12(2). Epub 2019 Feb 22.

Department of Internal Medicine, New York-Presbyterian/Queens, Flushing, New York, USA.

A 62-year-old man with no pertinent medical history presented with lower extremity weakness and worsening distal fingertips and toe cyanosis/gangrene. In the outpatient setting, he was initially being treated for Raynaud's phenomenon with a calcium channel blocker. On presentation, the patient had elevated inflammatory markers and white blood cell count. Read More

J Vet Cardiol 2019 Feb 6;21:10-17. Epub 2018 Dec 6.

Royal (Dick) School of Veterinary Studies, University of Edinburgh, Roslin, Midlothian, EH25 9RG, UK.

A 2-year 10-month, male neutered, crossbreed dog presented for evaluation of cyanosis and exercise intolerance. Doppler echocardiography revealed severe dilation of the right atrium and right ventricle with moderate pulmonary hypertension. Right-to-left shunting across a large ostium secundum atrial septal defect was confirmed by contrast echocardiography. Read More

BMC Pulm Med 2019 Feb 22;19(1):49. Epub 2019 Feb 22.

College of Medicine and Health Sciences - UAE University, Alain City, United Arab Emirates.

Background: The term hepatopulmonary syndrome typically applies to cyanosis that results from "intrapulmonary vascular dilatation" due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. An adverse consequence of such shunts is intrapulmonary vascular dilatation, which affects the microvascular gas exchange units for oxygen. Read More

Circ J 2019 Feb 19. Epub 2019 Feb 19.

Department of Cardiovascular Surgery, Second Xiangya Hospital, Central South University.

Forensic Sci Int 2019 Feb 7. Epub 2019 Feb 7.

Department of Experimental Medicine, Forensic Toxicology Unit, University of Campania "L. Vanvitelli", Via L. Armanni, 5, 80138, Naples, Italy.

The study reports the environmental, toxicological and histopathological forensic investigations applied on three victims of accidental death (father, mother and son), due to the fall in a volcanic pothole, during the touristic visit of the "Solfatara park", near Naples (Italy). At autopsy greenish skin discolouration was observed and all bodies showed the classical signs of asphyxial deaths, such as cyanosis and hemorrhagic pulmonary edema. Focal micro-hemorrhages were found in the brain at intracranial and subpial levels. Read More

J Vet Intern Med 2019 Feb 14. Epub 2019 Feb 14.

School of Animal and Veterinary Sciences, The University of Adelaide, Roseworthy, South Australia, Australia.

Little is known about genetic causes of congenital methemoglobinemia in dogs. Here, we report a CYB R mutation in a Pomeranian dog with congenital methemoglobinemia. A 6-year-old neutered female Pomeranian dog was investigated for cyanosis noticed during anesthesia for an orthopedic procedure. Read More

Sci Rep 2019 Feb 14;9(1):2034. Epub 2019 Feb 14.

Department of Congenital Heart Disease and Pediatric Cardiology, DZHK (German Center for Cardiovascular Research), partner site Hamburg/Kiel/Lübeck, University Hospital Schleswig-Holstein, Arnold-Heller-Str. 3, Kiel, Germany.

The Fontan procedure provides relief from cyanosis in patients with univentricular hearts. A major clinical unmet need is to understand whether the venous flow patterns of the Fontan circulation lead to the development of congestive hepatopathy and other life-threatening complications. Currently, there is no consensus on whether heart beat or respiration is the main driving force of venous return and which one affects the periodic flow changes for the most (i. Read More

Medicine (Baltimore) 2019 Feb;98(7):e14559

Department of Pediatrics.

Rationale: Pneumocystis jirovecii causes severe pneumonia in immunocompromised hosts. Human immunodeficiency virus infection, malignancy, solid organ or hematopoietic cell transplantation, and primary immune deficiency compose the risk factors for Pneumocystis pneumonia (PCP) in children, and PCP can be an initial clinical manifestation of primary immune deficiency.

Patient Concerns: A 5-month-old infant presented with cyanosis and tachypnea. Read More

Ann Pediatr Cardiol 2019 Jan-Apr;12(1):60-63

University of Mississippi Medical Center, Jackson, Mississippi, USA.

Fontan completion in patients with complex cardiac anatomy, and specifically heterotaxy syndrome, can present unique physiologic considerations. For example, existing venous connections may be "unmasked" after a cavopulmonary anastomosis operation. We present the case of a child with heterotaxy, dextrocardia, single-ventricle physiology, and anomalous hepatic venous drainage that resulted in profound shunting and cyanosis. Read More

BMC Pediatr 2019 Feb 11;19(1):55. Epub 2019 Feb 11.

Department of Cardiology, Shanghai Children's Hospital, Shanghai Jiaotong University, No. 355 Luding Road, Shanghai, 200062, China.

Background: Abernethy malformation is an extremely rare congenital malformation characterised by an extrahepatic portosystemic shunt. Children with Abernathy malformation can develop hepatopulmonary syndrome (HPS) with pulmonary arteriovenous fistulas (PAVF) or pulmonary hypertension. PAVF manifests as central cyanosis with effort intolerance. Read More

Expert Rev Vaccines 2019 Feb 8:1-4. Epub 2019 Feb 8.

a Department of Pediatrics , David Geffen School of Medicine at UCLA , Los Angeles , CA , USA.

Introduction: Today, in the developed world, virtually all deaths due to Bordetella pertussis illnesses occur in young infants. Areas Covered: Pertussis in young infants is characterized by an afebrile cough illness with coryza, apnea, seizures, cyanosis, and emesis. Severe illness is associated with high leukocyte and lymphocyte counts, rapid respiratory and cardiac rates and pneumonia. Read More

Case Reports Hepatol 2019 13;2019:6586478. Epub 2019 Jan 13.

Department of Medicine, Salmaniya Medical Complex, Manama, Bahrain.

Background: There are multiple aetiologies for dyspnea in patients with liver disease, including pneumonia, pulmonary embolism, hepatic hydrothorax, portopulmonary syndrome, and hepatopulmonary syndrome. The aim of this paper is to emphasize the importance of early diagnosis and management of hepatopulmonary syndrome.

Case Presentation: We report a case of a 65-year-old male who was known to have chronic hepatitis C presented with one-year history of shortness of breath and cyanosis. Read More

Pediatrics 2019 Mar 7;143(3). Epub 2019 Feb 7.

Departments of Neonatology and.

Congenital methemoglobinemia is a rare condition caused by cytochrome b5 reductase deficiency, cytochrome b5 deficiency, or hemoglobin M disease. Newborn pulse oximetry screening was developed for the early detection of critical congenital heart disease; however, it also enables the early identification of other hypoxemic conditions. We present the case of a term neonate who was admitted to the neonatal unit after a failed pulse oximetry screening at 3 hours of age. Read More

Curr Opin Pediatr 2019 Apr;31(2):274-283

Division of Pediatric Cardiology, Cohen Children's Medical Center of New York - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York, USA.

Purpose Of Review: We aim to improve diagnosis of congenital heart disease (CHD) with cyanosis by physiology for general practitioners to reduce time to appropriate treatment.

Recent Findings: New implementation of the critical congenital heart disease (CCHD) pulse oximetry screen has improved rate of diagnosis of CHD in recent years. However, many infants with cyanotic heart lesions often decompensate before screening in the newborn nursery, or have lesions that are not amenable to pulse oximetry screening and that present later in the emergency room. Read More

Cardiol Young 2019 Feb 6:1-5. Epub 2019 Feb 6.

1Cardio-Thoracic,Abdominal and Transplantation Department,IRCCS Istituto Giannina Gaslini,Genoa,Italy.

IntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Read More

Int J Cardiol 2019 Jan 26. Epub 2019 Jan 26.

Electrophysiology Service and Adult Congenital Heart Centre, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada. Electronic address:

A convergence of epidemiological and clinical features has prompted a precipitous rise in the prevalence of atrial fibrillation (AF) in the expanding population of adults with congenital heart disease. Herein, we synthesize the current state of knowledge on epidemiological features, associated morbidities, and pathophysiological insights regarding AF in adults with congenital heart disease. Management issues are examined including surgical, pharmacological, and catheter-based therapies. Read More

Ann Vasc Surg 2019 Jan 31. Epub 2019 Jan 31.

Department of Physical Therapy and Rehabilitation of University of Health Sciences, Ankara Health Application and Research Center, Ankara, Turkey.

Background: Hypothenar hammer syndrome (HHS) is an uncommon vascular syndrome of upper extremity. HHS should be considered in patients who are presented with digital ischemia. Distal ulnar artery compression at the level of Guyon's canal with trauma results in thrombus or aneurysm. Read More

Am J Perinatol 2019 Feb 1. Epub 2019 Feb 1.

Neonatal Intensive Care Unit, AOUP "P. Giaccone," Department of Sciences for Health Promotion and Mother and Child Care "G. D'Alessandro," University of Palermo, Palermo, Italy.

Objective:  Methemoglobinemia (MetHb) is a rare congenital or acquired cause of infantile cyanosis. We examined the role of MetHb in a neonatal intensive care unit (NICU).

Study Design:  A retrospective observational study was conducted reviewing blood gas analyses of hospitalized newborns over a 2-year period. Read More

Ter Arkh 2018 Aug;90(8):74-80

B.V. Petrovsky Russian Scientific Center of Surgery, Moscow, Russia.

Ascites and hydrothorax may be the symptoms of congestive heart failure and do not always reflects presense of the decompensated liver cirrhosis. Clinical examination of patient with chronic hepatitis C which cyanosis of the lips, cervival veins pulsation, a triple heart rhythm indicated on pathology of the heart (constrictive pericarditis), which was confirmed by instrumental methods. Congestive heart failure has lead to the congestive liver in a young female patient. Read More

Oxf Med Case Reports 2019 Jan 24;2019(1):omy111. Epub 2019 Jan 24.

Dermatology Unit, Department of Internal Medicine, Sarawak General Hospital, Sarawak, Malaysia.

Limited data regarding methemoglobinemia in pregnancy, particularly secondary to dapsone is available up to date. We report a case of dapsone-induced methemoglobinemia in a pregnant mother with multibacillary leprosy who presented with fever, productive cough and cyanosis of 2 days duration 2 weeks after multidrug therapy was commenced. On examination, she had central cyanosis with low oxygen saturation (SpO = 84-88%). Read More

Respir Res 2019 Jan 28;20(1):19. Epub 2019 Jan 28.

Department of Occupational and Environmental Health, School of Public Health, Tongji Medical College, Huazhong University of Science and Technology, 13 Hangkong Road, Wuhan, 430030, Hubei, China.

Background: Soluble CD14 (sCD14) shedding from CD14 could regulate T lymphocyte activation and function, which has implicated in the pathogenesis of bronchial asthma. The level of sCD14 expression is obviously increased in asthmatic patients during acute asthma attacks. The objective of this study was to investigate the association between plasma sCD14 level and asthma severity in adults. Read More

Ann Thorac Surg 2019 Jan 23. Epub 2019 Jan 23.

Division of Cardiovascular Surgery, University of Tsukuba, 2-1-1 Tennodai, Tsukuba, Ibaraki, Japan 305-8576.

We report a case of a newborn infant that experienced circulatory collapse caused by a calcified lesion occluding the main pulmonary artery (PA). The baby was born via full-term birth at normal birth weight. Cyanosis was noted immediately after birth. Read More

Medicine (Baltimore) 2019 Jan;98(4):e14201

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Rationale: Concomitant cryoglobulinemic vasculitis and cold agglutinin disease (CAD) is an extremely uncommon clinical scenario. The role of bortezomib in the treatment of cryoglobulinemic vasculitis needs further investigation.

Patient Concerns: A 72-year-old Chinese woman presented with a 25-year history of cyanosis of the extremities after cold exposure, which worsened and was accompanied with purpuric skin lesions and proteinuria in recent years. Read More

Hautarzt 2019 Feb;70(2):131-141

Klinik für Hautkrankheiten, Allgemeine Dermatologie und Venerologie, Universitätsklinikum Münster, Von-Esmarch Str. 58, 48149, Münster, Deutschland.

Raynaud's phenomenon (RP) is a painful vasospasm of small arteries, localised in fingers and toes. Typically these body parts turn white (ischemia), then blue (deoxygenation) and then red (reperfusion). Two different types of RP exist: the common primary RP without underlying disease and the rare secondary RP, mostly associated with rheumatoid diseases such as systemic sclerosis. Read More

Acta Cardiol 2019 Jan 16:1-2. Epub 2019 Jan 16.

b Division of Paediatric Radiology , Hôpital Universitaire des Enfants Reine Fabiola (Université Libre de Bruxelles ULB) , Brussels , Belgium.

PLoS Negl Trop Dis 2019 01 14;13(1):e0007084. Epub 2019 Jan 14.

Centers for Disease Control and Prevention, Dengue Branch, San Juan, Puerto Rico.

Background: Hospitalization of patients during outbreaks of chikungunya virus has been reported to be uncommon (0.5-8.7%), but more frequent among infants and the elderly. Read More

A A Pract 2019 Jan 10. Epub 2019 Jan 10.

From the Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland.

Gene mutations that affect the synthesis or structure of hemoglobin are present in 7% of the world's population. These mutations lead to variant hemoglobinopathies with phenotypes that range from clinically insignificant to fatal. Because monitoring oxygen saturation is standard of care and critical to detecting hypoxemia, it is crucial to recognize factors that might interfere with the measurement of oxygen saturation or decrease oxygen delivery. Read More

Dig Liver Dis 2019 Jan 3. Epub 2019 Jan 3.

Department of Internal Medicine, Toyama Prefectural Central Hospital, Toyama, Japan.

Medicine (Baltimore) 2019 Jan;98(2):e13922

Department of Cardiology, West China Hospital, Sichuan University, Chengdu.

Rationale: Complex pulmonary arteriovenous fistula (PAVF) is unusual, and even rarer in 2 members of a family. PAVF may not appear on chest X-ray or computed tomography imaging, especially in asymptomatic patients, and therapy is limited. Herein, PAVFs occurring in a mother and daughter are described, with the current standard methods of diagnosis and treatment of PAVF. Read More

J Ayub Med Coll Abbottabad 2018 Oct-Dec;30(4):511-555

Iqbal Memorial Teaching Hospital/Khawaja Muhammad Safdar Medical College Sialkot, Pakistan.

Background: Nasal continuous positive pressure has been used for management of respiratory distress in neonates in various conditions as a primary modality. Objective of the study is to evaluate the frequency of improved outcome and complications of use of nasal CPAP through infant flow drivers in neonates with respiratory distress. The study was conducted from 2nd April 2017 to 2nd October 2017 in neonatal intensive care unit of Allama Iqbal Memorial Teaching Hospital Sialkot. Read More

Echocardiography 2019 02 9;36(2):422-423. Epub 2019 Jan 9.

Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.

A child with cyanosis and heart failure was noted to have two different vascular arches of similar caliber on echocardiography. A systematic analysis of the rhythm and flow patterns revealed the mysterious "double" arch. Read More

Pediatr Crit Care Med 2019 Jan;20(1):82-84

Cardiac Intensive Care, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.

Neuroradiol J 2019 Apr 7;32(2):143-147. Epub 2019 Jan 7.

Department of Radiology, King Faisal Specialist Hospital and Research Centre, Saudi Arabia.

Recessive congenital methemoglobinemia type II is a very rare autosomal recessive hematologic disorder due to NADH-cytochrome b5 reductase deficiency, usually caused by full-stop mutations or deletions. This disease classically presents with mild neonatal cyanosis, early onset severe progressive developmental delay, movement disorders, and progressive microcephaly. We report two siblings with recessive congenital methemoglobinemia type II whose evaluation revealed a novel p. Read More

J Family Med Prim Care 2018 Nov-Dec;7(6):1573-1575

Department of Medicine, Tata Main Hospital, Jamshedpur, Jharkhand, India.

Clofazimine is commonly used for the treatment of leprosy and chronic use of it can lead to methemoglobinemia, which is a rare but major concern. Iron of hemoglobin remains in the form of ferric (Fe3+) in methemoglobinemia as compared with ferrous form (Fe2+) in normal situation. This transformation prevents oxygen carriage and results in higher level of MetHb in blood which could be dangerous to life. Read More

Cir Cir 2019 ;87(1):113-122

Instituto Nacional de Ciencias Médicas y Nutrición Dr. Salvador Zubirán, Ciudad de México, México.

Acute respiratory distress syndrome was first described by Ashbaugh, Petty, Levine y Bigellow in 1967 writing in The Lancet. Their study was based on a case series of 12 patients with acute respiratory distress, cyanosis refractory to oxygen therapy, decreased lung compliance and diffuse infiltrates evident on the chest radiograph. Mortality was 58% with greater survival in five patients managed with mechanical ventilation and positive end expiratory pressure. Read More