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Eisenmenger syndrome

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https://www.readbyqxmd.com/read/29776964/arrhythmias-in-adult-patients-with-congenital-heart-disease-and-pulmonary-arterial-hypertension
#1
Maria Drakopoulou, Heba Nashat, Aleksander Kempny, Rafael Alonso-Gonzalez, Lorna Swan, Stephen J Wort, Laura C Price, Colm McCabe, Tom Wong, Michael A Gatzoulis, Sabine Ernst, Konstantinos Dimopoulos
OBJECTIVES: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain. METHODS: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis...
May 18, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29776810/congenital-heart-disease-in-adults-assessmentof-functional-capacity-using-cardiopulmonary-exercise-testing
#2
Sílvia Aguiar Rosa, Ana Agapito, Rui M Soares, Lídia Sousa, José Alberto Oliveira, Ana Abreu, Ana Sofia Silva, Sandra Alves, Helena Aidos, Fátima F Pinto, Rui Cruz Ferreira
AIM: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET). METHODS: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction...
May 15, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29720395/pulmonary-arterial-hypertension-in-adult-congenital-heart-disease
#3
REVIEW
Margarita Brida, Michael A Gatzoulis
Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care...
May 2, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29708913/case-report-of-an-awake-craniotomy-in-a-patient-with-eisenmenger-syndrome
#4
Boris D Heifets, Erin Crawford, Ethan Jackson, Jessica Brodt, Richard A Jaffe, Mark A Burbridge
We present a detailed report of an awake craniotomy for recurrent third ventricular colloid cyst in a patient with severe pulmonary arterial hypertension in the setting of Eisenmenger syndrome, performed 6 weeks after we managed the same patient for a more conservative procedure. This patient has a high risk of perioperative mortality and may be particularly susceptible to perioperative hemodynamic changes or fluid shifts. The risks of general anesthesia induction and emergence must be balanced against the risks inherent in an awake craniotomy on a per case basis...
May 1, 2018: A&A practice
https://www.readbyqxmd.com/read/29664959/contemporary-survival-of-patients-with-pulmonary-arterial-hypertension-and-congenital-systemic-to-pulmonary-shunts
#5
Chodchanok Vijarnsorn, Kritvikrom Durongpisitkul, Paweena Chungsomprasong, Densiri Bositthipichet, Salisa Ketsara, Yuttapon Titaram, Prakul Chanthong, Supaluck Kanjanauthai, Jarupim Soongswang
OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded...
2018: PloS One
https://www.readbyqxmd.com/read/29625509/adult-congenital-heart-disease-with-pregnancy
#6
REVIEW
Koichiro Niwa
The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk...
April 2018: Korean Circulation Journal
https://www.readbyqxmd.com/read/29521655/selexipag-in-congenital-heart-disease-associated-pulmonary-arterial-hypertension-and-eisenmenger-syndrome-first-report
#7
Karim El-Kersh, Sally Suliman, J Shaun Smith
No abstract text is available yet for this article.
January 23, 2018: American Journal of Therapeutics
https://www.readbyqxmd.com/read/29224925/incidence-and-clinical-characteristics-of-sudden-cardiac-death-in-adult-congenital-heart-disease
#8
Benjamin Moore, Christopher Yu, Irina Kotchetkova, Rachael Cordina, David S Celermajer
BACKGROUND: The life expectancy of adults with congenital heart disease (CHD) has significantly improved in recent decades, with non-cardiovascular causes of death now competing with traditional cardiovascular causes. The risk of sudden cardiac death (SCD), a devastating event, still remains elevated above that of the general population. METHODS: We reviewed 2935 patients in our adult CHD database (age≥16years, seen at least once in our centre) and documented all cases of SCD between 2000-2015...
March 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29201477/a-case-of-advanced-glaucoma-with-increased-episcleral-venous-pressure-in-a-17-year-old-with-eisenmenger-syndrome
#9
Leanne Grech, Adrian Mifsud, Maryanne Caruana, Francis Carbonaro
Eisenmenger syndrome refers to reversal of shunt and central cyanosis due to pulmonary hypertension induced by congenital heart disease with a large systemic-to-pulmonary shunt. We report a case of a 17-year-old man with Eisenmenger syndrome who presented with gradual deterioration in visual acuity and was diagnosed with advanced secondary open angle glaucoma. There have been reports of patients suffering from thrombosis due to hyperviscosity associated with this syndrome; however, to our knowledge, the association of secondary open angle glaucoma with Eisenmenger syndrome has not yet been documented...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/29187101/eisenmenger-syndrome-revisited
#10
Gaetano Thiene
No abstract text is available yet for this article.
November 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29135526/case-report-of-an-awake-craniotomy-in-a-patient-with-eisenmenger-syndrome
#11
Boris D Heifets, Erin Crawford, Ethan Jackson, Jessica Brodt, Richard A Jaffe, Mark A Burbridge
We present a detailed report of an awake craniotomy for recurrent third ventricular colloid cyst in a patient with severe pulmonary arterial hypertension in the setting of Eisenmenger syndrome, performed 6 weeks after we managed the same patient for a more conservative procedure. This patient has a high risk of perioperative mortality and may be particularly susceptible to perioperative hemodynamic changes or fluid shifts. The risks of general anesthesia induction and emergence must be balanced against the risks inherent in an awake craniotomy on a per case basis...
November 9, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/29121718/non-invasive-assessment-of-liver-changes-in-eisenmenger-patients
#12
Siegrun Mebus, Nicole Nagdyman, Johanna Kügel, Reinhart Zachoval, Siegmund Lorenz Braun, Guido Haverkämper, Bernd Opgen-Rhein, Felix Berger, Sophia Horster, Jörg Schoetzau, Claudia Pujol Salvador, Ulrike Bauer, John Hess, Peter Ewert, Harald Kaemmerer
BACKGROUND: Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. METHODS AND RESULTS: 10 adults with Eisenmenger syndrome (six female; median age 44...
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29061688/managing-the-eisenmenger-syndrome-a-call-to-arms
#13
EDITORIAL
Edgar L W Tay
No abstract text is available yet for this article.
October 23, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29021475/successful-pulmonary-artery-embolization-for-the-management-of-hemoptysis-in-a-patient-with-eisenmenger-syndrome-caused-by-patent-ductus-arteriosus
#14
Naoki Tamada, Kazuhiko Nakayama, Yuto Shinkura, Kenichi Yanaka, Naoto Katayama, Takuya Okada, Masato Yamaguchi, Kensuke Matsumoto, Hidekazu Tanaka, Toshiro Shinke, Noriaki Emoto, Ken-Ichi Hirata
The patient was a 19-year-old woman who was diagnosed with patent ductus arteriosus complicating Eisenmenger syndrome at a previous medical institution. She was referred to our hospital and arranged for lung transplantation. She developed hemoptysis after the introduction of i.v. epoprostenol, which was administered as a bridging treatment while the patient awaited lung transplantation. She continued to suffer from recurrent hemoptysis, even after switching from i.v. epoprostenol to i.v. treprostinil. Angiography of the systemic and pulmonary arteries revealed the vessel responsible for the recurrent hemoptysis and pulmonary artery embolization was successfully performed...
December 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29016730/microrna-424-322-as-a-new-marker-of-disease-progression-in-pulmonary-arterial-hypertension-and-its-role-in-right-ventricular-hypertrophy-by-targeting-smurf1
#15
Rui Baptista, Carla Marques, Steve Catarino, Francisco J Enguita, Marina C Costa, Paulo Matafome, Mónica Zuzarte, Graça Castro, Abílio Reis, Pedro Monteiro, Mariano Pêgo, Paulo Pereira, Henrique Girão
Aims: MicroRNAs (miRNAs) have been implicated in the pathogenesis of pulmonary hypertension (PH), a multifactorial and progressive condition associated with an increased afterload of the right ventricle leading to heart failure and death. The main aim of this study was to correlate the levels of miR-424(322) with the severity and prognosis of PH and with right ventricle hypertrophy progression. Additionally, we intended to evaluate the mechanisms and signalling pathways whereby miR-424(322) secreted by pulmonary arterial endothelial cells (PAECs) impacts cardiomyocytes...
January 1, 2018: Cardiovascular Research
https://www.readbyqxmd.com/read/28970634/severe-pulmonary-hypertension-and-right-ventricular-failure
#16
REVIEW
Ajay Kumar, Praveen Kumar Neema
Perioperative management of severe pulmonary hypertension (PH) is challenging. Anaesthesiologists come across perioperative management of such cases during incidental surgeries, surgery for various congenital heart diseases and valvular heart diseases and for caesarean section or painless labour in pregnant patient with Eisenmenger syndrome. Knowledge of pathophysiology of PH and novel drugs acting through different mechanisms is paramount in managing such patients. This review will help understanding pathophysiology of PH, anaesthetising patients with PH, use of novel drugs for PH and use of new mechanical devices for rescue of failing right ventricle...
September 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28966315/prognostic-value-of-pulmonary-artery-compliance-in-patients-with-pulmonary-arterial-hypertension-associated-with-adult-congenital-heart-disease
#17
Xiao-Ling Cheng, Zhi-Hong Liu, Qing Gu, Xin-Hai Ni, Qin Luo, Zhi-Hui Zhao, Jian-Guo He, Chang-Ming Xiong
In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart disease (APAH-CHD). One-hundred and seventy-five patients of APAH-CHD who underwent a comprehensive clinical evaluation were included in this study. All patients were followed up in a 6-month interval and the primary end point was all cause of death...
October 21, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28957530/rv-pressure-overload-from-hypertrophy-to-failure
#18
Cathelijne E E van der Bruggen, Ryan J Tedford, Martin Louis Handoko, Jolanda van der Velden, Frances S de Man
In pulmonary arterial hypertension (PAH), right ventricular (RV) adaptation is essential to overcome the chronic increases in RV pressure overload. Ultimately, RV compensatory mechanisms are not sufficient and patients succumb to RV failure. The processes underlying the transition of RV adaptation to RV failure are not well understood. In this review, we propose that important insights in RV adaptation processes can be obtained by comparing different etiologies of PAH, namely patients with PAH secondary to Eisenmenger syndrome, patients with PAH secondary to systemic sclerosis and patients where no cause is identified: idiopathic PAH...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28882496/the-echocardiographic-characteristics-and-prognostic-significance-of-pericardial-effusions-in-eisenmenger-syndrome
#19
Clare Arnott, Rachael Cordina, David S Celermajer
BACKGROUND AND OBJECTIVES: Pericardial effusion (PE) confers a poor prognosis in non-congenital pulmonary arterial hypertension (PAH). The clinical significance and prognostic implications of PE in Eisenmenger syndrome (ES) is much less well characterised. Accordingly, we aimed to define the frequency, echocardiographic characteristics, natural history and clinical sequelae of PE in those with ES. PATIENTS AND METHODS: We retrospectively studied 55 consecutive individuals presenting since 2000, age >18 years, with ES...
March 2018: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28874283/mortality-in-adults-with-congenital-heart-disease
#20
Pavithra Naidu, Leeanne Grigg, Dominica Zentner
AIM: Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH). METHODS: Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken. Age, gender of deceased individuals, and frequency and cause of death in different congenital diagnosis groups was explored. RESULTS: Between 1991 and 2015, death occurred in 3...
October 15, 2017: International Journal of Cardiology
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