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Management of cyanotic congenital heart disease

Vandana Bhardwaj, Poonam Malhotra, Suruchi Hasija, Ujjwal Kumar Chowdury, Neha Pangasa
INTRODUCTION: In the last few years, viscoelastic point-of-care (POC) coagulation devices such as thromboelastography (TEG), rotational thromboelastometry (ROTEM), and Sonoclot (SON) analyzer have been increasingly used in major surgeries for timely assessment and management of coagulopathies. The aim of the present study was to evaluate coagulation profile of cyanotic cardiac patients with TEG, ROTEM, and SON analyzer. In addition, we assessed the correlation of standard laboratory coagulation tests and postoperative chest drain output (CDO) with the parameters of POC testing devices...
April 2017: Annals of Cardiac Anaesthesia
Yuyang Zhang, Hadyn Williams, Darko Pucar
Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology...
March 2017: Nuclear Medicine and Molecular Imaging
Nasib Kabbani, Mohamed S Kabbani, Hayan Al Taweel
Cardiac emergencies in children are not infrequent. Early recognition and management are essential to save life and prevent any comorbidity. The presentation of cardiac emergencies and etiologies is variable depending on the age of child at the time of presentation and type of cardiac lesion. Cyanotic and noncyanotic congenital heart diseases are the main causes in neonates and infants. Acquired heart diseases and dysrhythmia are more common causes for cardiac emergencies in toddler and childhood. In this review, we discuss the most common causes for cardiac emergencies in neonates and young infants highlighting important points in the presentation and management that are essential for early recognition and timely management of infants presenting with these conditions...
January 2017: Avicenna Journal of Medicine
Pamela Partana, Jarrod Kah Hwee Tan, Ju Le Tan, Lay Kok Tan
Pentalogy of Fallot is a cyanotic congenital heart disease that has guarded prognosis without surgical intervention in infancy. Women with uncorrected defects rarely survive into childbearing age and pregnancy in this group is associated with a high rate of perinatal loss. Physiological cardiovascular changes in pregnancy can lead to maternal haemodynamic instability with subsequent adverse cardiac sequelae with or without fetal decompensation. Optimum management and pregnancy outcomes in mother with uncorrected Pentalogy of Fallot and twin pregnancy have not been described in the literature...
January 18, 2017: BMJ Case Reports
Anishkumar Nair, Gopalan Nair Rajesh, Chakanalil Govindan Sajeev
Cardiac tumours in newborns are often asymptomatic and can be sporadically detected on routine screening unless they result in intractable arrhythmias or haemodynamically significant obstructions causing heart failure. Their presentation as a cause of congenital cyanosis is never anticipated. We report a rare case of a newborn presenting with congenital cyanosis consequent to suspected cardiac rhabdomyoma causing tricuspid inflow obstruction. Our experience with this patient with two large cardiac masses illustrates the significance of its inclusion in the differential diagnosis of perinatal cyanosis, as early detection and surgical management might be the only lifesaving options, if performed well in time...
January 12, 2017: Cardiology in the Young
Corinne D'Antico, André Hofer, Jens Fassl, Daniel Tobler, Daniel Zumofen, Luzius A Steiner, Nicolai Goettel
We report the case of a 39-year-old male with complex cyanotic congenital heart disease undergoing emergency craniotomy for a cerebral abscess. Maintenance of intraoperative hemodynamic stability and adequate tissue oxygenation during anesthesia may be challenging in patients with cyanotic congenital heart disease. In this case, we decided to perform the surgery as an awake craniotomy after interdisciplinary consensus. We discuss general aspects of anesthetic management during awake craniotomy and specific concerns in the perioperative care of patients with congenital heart disease...
2016: F1000Research
Rubén Kevin Arnold Tapia-Orihuela, Jorge Huaringa-Marcelo, David Loja-Oropeza
Introduction: Situs inversus totalis is an uncommon anomaly which exist a complete transposition of organs and it's occasionally associated with congenital heart diseases, such as tetralogy of fallot. Pheochromocytoma is a rare neuroendocrine tumor with an annual incidence of 2-8 cases per million people and for years has been studied its relationship with the hypoxic pathway. Case Report: A 29 year old male with a history of tetralogy of fallot corrected at 10 years and situs inversus totalis. He was admitted to hospital with a progressive story of four months of constipation, palpitations, headache, dyspnea and sweating...
2016: Journal of Cardiovascular and Thoracic Research
L Talemal, M T Donofrio
D-transposition of the great arteries (D-TGA) is the most commonly diagnosed cyanotic congenital heart disease presenting in the neonatal period. The survival after an arterial switch operation, with freedom from adverse cardiovascular events, has been reported to be as high as 93% at 25 years. However, despite excellent surgical outcomes, there continues to be significant preoperative morbidity and potential mortality due to compromise in the delivery room from foramen ovale closure requiring urgent balloon atrial septostomy for stabilization in the first minutes of life...
September 16, 2016: Journal of Neonatal-perinatal Medicine
Lucy M Safi, Richard R Liberthson, Ami Bhatt
Ebstein's anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein's anomaly. Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery...
September 2016: Current Treatment Options in Cardiovascular Medicine
J Hitzeroth, N Beckett, P Ntuli
Although infective endocarditis (IE) is relatively uncommon, it remains an important clinical entity with a high in-hospital and 1-year mortality. It is most commonly caused by viridans streptococci. Staphylococcus aureus is responsible for a malignant course of IE and often requires early surgery to eradicate. Other rarer causes are various bacilli, including the HACEK (Haemophilus, Actinobacillus,Cardiobacterium, Eikenella and Kingella spp.) group of organisms and fungi. The clinical presentation varies. Patients may present with a nonspecific illness, valve dysfunction, heart failure (HF) and symptoms due to peripheral embolisation...
February 2016: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
Stacie Knutson, Michael S Kelleman, Lazaros Kochilas
OBJECTIVES: To assess awareness and implementation among pediatric primary care providers of the 2012 American Heart Association (AHA) guidelines for the evaluation and management of developmental abnormalities in children with congenital heart disease (CHD). We hypothesized that children with CHD are not being provided neurodevelopmental screening and support according to the AHA guidelines. STUDY DESIGN: An online survey was administered to licensed pediatric primary care providers in Minnesota (pediatricians = 530, family physicians = 1469) to evaluate awareness of the AHA guidelines, current screening practices, and barriers to implementation of these guidelines...
September 2016: Journal of Pediatrics
Arun Sharma, Sanjeev Kumar, Sarv Priya
Aneurysm of a major aortopulmonary collateral artery (MAPCA) is quite rare. Aneurysmally dilated MAPCA may be complicated with rupture and massive hemoptysis leading to sudden death. Possible pathophysiology for aneurysm formation is persistent high pressure state in collateral circulation. High index of suspicion is necessary to avoid catastrophic complications as the amount of hemoptysis does not correlate with disease severity and etiology. We present a case of large ruptured aneurysm of a MAPCA presenting with massive haemoptysis in a patient of cyanotic congenital heart disease which was salvaged by endovascular deployment of vascular plug...
June 2016: Cardiovascular Diagnosis and Therapy
Marcus P Haw
The ductus arteriosus is a fetal vascular connection between the pulmonary and systemic circulations. It fails to close after birth in a small number of term infants, and in a larger number of infants with cyanotic congenital heart disease. In contemporary practice the majority of patients present with a patent ductus arteriosus (PDA) are premature infants before the gestational age of 28 weeks. The surgical management of PDA in preterm infants is critical for optimal outcomes and is discussed in this article...
2016: Current Pediatric Reviews
Allan Hall, Mark A J White, Pasquale Gallo
INTRODUCTION: We present an unusual case where a large intra-cerebral abscess with severe complications was treated successfully with medical management alone in a patient with Eisenmenger syndrome. PRESENTATION OF CASE: A 40 year old patient with Eisenmenger syndrome presented with a seven day history of headache, neck pain and intermittent vomiting and fever. The only neurological examination finding was blurred vision. MRI revealed a large left occipital lobe abscess, which subsequently ruptured into the lateral ventricle with associated ventriculitis and hydrocephalus...
2016: International Journal of Surgery Case Reports
Chikako Terano, Kenji Ishikura, Masaru Miura, Riku Hamada, Ryoko Harada, Tomoyuki Sakai, Yuko Hamasaki, Hiroshi Hataya, Takashi Ando, Masataka Honda
UNLABELLED: No large cohort study has yet determined the incidence of acute kidney injury (AKI) in children with heart failure treated with renin-angiotensin system (RAS) inhibitors. We thus retrospectively analyzed the incidence and risk factors for severe AKI (stages 2-3 according to the Kidney Disease Improving Global Outcomes (KDIGO) guidelines) at our institutions from 2008 to 2011. Among 312 children (162 boys; median age, 7.3 months), 59 cases of AKI occurred in 45 children. The incidence of AKI was 14...
May 2016: European Journal of Pediatrics
Andreas Petropoulos, Doris Ehringer-Schetitska, Peter Fritsch, Eero Jokinen, Robert Dalla Pozza, Renate Oberhoffer
OBJECTIVE: The burden of cardiac disease in childhood is unknown. It will be a sum of 1% of living births in the general population, suffering from Congenital Heart Disease (CHD) + approximately 2.5% of the general population suffering from bicuspid aortic valve diseases + an unknown higher prevalence of acquired diseases. Cardiomyopathies, arrhythmias - sudden cardiac death (SCD), rheumatic heard disease, hypertension and accelerating atherosclerosis are among the most frequent. Adding on, genetic syndromes including cardiac defects, endocarditis and myocarditis we can address a large pediatric population worldwide, suffering from heart disease...
September 2015: Hellenic Journal of Nuclear Medicine
Mohamed O Hafez, Saed M Morsy, Ragab A Mahfoz, Ahmed R Ali
Background. Children with congenital heart diseases (CHDs) may have a risk of developing myocardial injury caused by volume and pressure overload. Objective. To evaluate the incidence of myocardial injury in children with cyanotic and acyanotic CHDs using cTnI assay and to correlate it with different hemodynamic parameters. Methods. This study included 80 children with CHDs (40 acyanotic and 40 cyanotic) as well as 40 healthy children (control group). Serum cTnI levels were measured for patients and control...
2015: Cardiology Research and Practice
Kimberly J Spronk, Anthony D Olivero, Marcus P Haw, Joseph J Vettukattil
The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahydrofolate reductase (MTHFR) gene. The MTHFR C677T gene decreases the bioavailability of folate and increases plasma homocysteine, a risk factor for thrombosis. There have been no reported cases in the literature on the clinical implications of this procoagulable state in the setting of cyanotic heart disease, which itself has prothrombotic predisposition. Two patients with hypoplastic left heart syndrome developed postoperative thrombotic complications, both were homozygous for MTHFR C677T...
October 2015: World Journal for Pediatric & Congenital Heart Surgery
Joanna Rupa-Matysek, Olga Trojnarska, Lidia Gil, Ludwina Szczepaniak-Chicheł, Ewelina Wojtasińska, Andrzej Tykarski, Stefan Grajek, Mieczysław Komarnicki
BACKGROUND: Patients with cyanotic congenital heart disease (CCHD) have an increased risk of bleeding and thrombotic complications. Prolonged conventional coagulation screening parameters, such as activated partial thromboplastin time or prothrombin time, are reported in less than 20% of CCHD patients. METHODS: The aim of this study was to determine the haemostatic abnormalities in 32 adult patients with CCHD by rotation thromboelastometry (ROTEM) with assessment of coagulation dynamic properties, as a guide for perioperative prophylaxis or haemostatic therapy...
January 1, 2016: International Journal of Cardiology
Ricardo A Weis, Patrick A Devaleria, Sarang Koushik, Harish Ramakrishna
INTRODUCTION: Advances in medical and surgical care have made it possible for an increasing number of patients with Congenital Heart disease (CHD) to live into adulthood. Transposition of the great vessels (TGV) is the most common cyanotic congenital cardiac disease where the right ventricle serves as systemic ventricle. It is not uncommon for these patients to have systemic ventricular failure requiring transplantation. STUDY DESIGN: Hemodynamic decompensation in these patients can be swift and difficult to manage...
October 2015: Annals of Cardiac Anaesthesia
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