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Management of cyanotic congenital heart disease

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
Vinay Marulasiddappa, B S Raghavavendra
Children with uncorrected cyanotic congenital heart diseases can present for non cardiac surgeries. They pose several challenges to the Anaesthesiologist, especially when they are posted for emergency surgery, due to the complex haemodynamic changes secondary to the heart disease. Pentalogy of Fallot (POF) is a rare form of congenital heart disease characterized by the association of Tetralogy of Fallot (TOF) with an atrial septal defect (ASD). TOF is the leading cause of intracardiac right to left shunt and is the commonest type of cyanotic congenital heart disease to cause a brain abscess...
July 2015: Journal of Clinical and Diagnostic Research: JCDR
Santoshi Kurada, Ranjith B Karthekeyan, Mahesh Vakamudi, Periyasamy Thangavelu
Most infants with tracheomalacia do not need specific therapy as it usually resolves spontaneously by the age of 1-2 years. Severe forms of tracheomalacia characterized by recurrent respiratory infections require active treatment which includes chest physiotherapy, long term intubation or tracheostomy. Aortopexy seems to be the treatment of choice for secondary and even primary forms of severe tracheomalacia. Itentails tracking and suturing the anterior wall of the aorta to the posterior surface of the sternum...
April 2015: Indian Journal of Anaesthesia
Hooman Salimipour, Somayeh Mehdizadeh, Reza Nemati, Mohamad Reza Pourbehi, Gholam Reza Pourbehi, Majid Assadi
Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) is a compensatory response to chronic hypoxia which should be managed with caution. CCHD patients, who have compensated erythrocytosis but do not manifest significant neurologic symptoms, may experience secondary life-threatening complications such as stroke in case of inappropriate phlebotomy. This study reports a young man with CCHD who developed frequently repeated transient neurologic deficits with various presentations after one session of phlebotomy...
2015: Case Reports in Neurological Medicine
Chitra Juwarkar, Sidhesh S Bharne
Tetralogy of Fallot is the most common cyanotic congenital heart disease. We report the anesthetic management of a patient with uncorrected Fallot's tetralogy for Cesarean section.
July 2012: Anesthesia, Essays and Researches
Robert A Palermo, Michael C Monge, Joel Charrow, John M Costello, Conrad L Epting
We report the case of a child with both propionic acidemia and cyanotic congenital heart disease. The presence of an underlying inborn error of metabolism confounded the management of this patient in the postoperative period, resulting in therapeutic misdirection until the true etiology of hyperlactemia was recognized.
April 2015: World Journal for Pediatric & Congenital Heart Surgery
Nithima Ratanasit, Khemajira Karaketklang, Decho Jakrapanichakul, Maytinee Kittipovanonth, Kesaree Punlee, Wandee Rochanasiri, Suteera Phrudprisan
BACKGROUND: The number of adult patients with congenital heart disease (CHD) has increased rapidly and represents a major challenge in cardiovascular medicine. Data regarding the prevalence and characteristics of adult CHD in Thai population over the past decade are lacking. MATERIAL AND METHOD: Patients who underwent comprehensive transthoracic echocardiographic study at Siriraj Hospital during January 2003 to May 2013 with the diagnosis of unrepaired CHD and complete data were enrolled in the study Patients with uncertain diagnosis, prior cardiac intervention, bicuspid aortic valve, patent foramen ovale, mitral valve prolapse, isolated persistent left superior vena cava and congenital cardiomyopathies were excluded...
January 2015: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
Rachel M Wald, Anne Marie Valente, Ariane Marelli
Emerging heart failure (HF) concepts in the growing population of adults with congenital heart disease (ACHD) are reviewed in the following article with a focus on individuals with tetralogy of Fallot (TOF), the largest group of adults with repaired cyanotic congenital heart disease (CHD). In the first section, the changing epidemiology of CHD and HF in ACHD patients is described. We demonstrate the challenges health care providers face when caring for this unique population. Emphasis is placed on the importance and difficulty of identifying patients at risk for HF, of which TOF patients comprise a substantial subset, underscoring the benefits of specialized cardiac care...
July 2015: Trends in Cardiovascular Medicine
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