Presentation
Because of the progressive nature of the disorder, patients may present with any number of symptoms. [af-ablation.org]
Unfortunately, SCA may also be the presenting symptom; patients with no prior symptoms may present with SCA. [geneticheartdisease.org]
Our case presentation is interesting for 2 reasons: first, because of its rare presentation and second, because of the patient's history of life-threatening arrhythmias. [revespcardiol.org]
Presentation usually occurs in the neonatal and childhood years with clinical aspects of congestive heart failure. [radiopaedia.org]
The topics discussed are relevant to chest physicians, thoracic surgeons, nurses, students, and teachers, and a well-balanced mix of contributions ensures that doctors, clinicians, and institutions from all around the world will find the information presented [books.google.de]
Cardiovascular
- Heart Disease
ICD-10-CM Codes › I00-I99 Diseases of the circulatory system › I30-I52 Other forms of heart disease › I51- Complications and ill-defined descriptions of heart disease › Heart disease, unspecified 2016 2017 2018 2019 Billable/Specific Code I51.9 is a billable [icd10data.com]
Treatments include: We treat a full range of hereditary heart and blood vessel diseases: Clinical trials for inherited heart disease often test new medications. [familyheart.stanford.edu]
Double outlet right ventricle (DORV) is a heart disease that is present from birth (congenital). [medlineplus.gov]
The right ventricle in congenital heart disease. Heart. 2006;92(Suppl1):i27–38. PubMedCentral PubMed CrossRef Google Scholar 4. Haddad F, Hunt SA, Rosenthal DN, Murphy J. Right ventricular function in cardiovascular disease. [link.springer.com]
Sudden death is the first manifestation of the disease in 11% to 22% of patients. 1 We present the case of a 58-year-old man, with no personal or family history of heart disease, who was admitted to our hospital with a 1-month history of dyspnea. [revespcardiol.org]
Workup
These measurements could be included as part of the routine workup in all end-stage renal disease patients on hemodialysis. © 2016 S. [karger.com]
Treatment
Virology: Research and Treatment about the journal submit your research Indexing: DOAJ, EBSCO, PubMed Central (PMC) and more. [insights.sagepub.com]
In an emergency, treatment for ventricular tachycardia may include: CPR electrical defibrillation electric shock antiarrhythmic medication Long-term treatment may include oral antiarrhythmic medication. [healthline.com]
Management and treatment In the presence of two viable ventricles, biventricular repair is the optimal treatment for DORV and it is quite safely achieved in the simple forms (DORV-VSD type, DORV-Fallot type and DORV-TGA type). [orpha.net]
A consensus statement has recently been published for the treatment of ARVD and it includes an algorithm to identify patients who will derive the greatest benefit from ICD implantation. [revespcardiol.org]
Prognosis
Prognosis Without treatment, prognosis is poor due to severe cyanosis, congestive heart failure or pulmonary hypertension. With biventricular repair, patients have, on average, a normal life expectancy. [orpha.net]
Prognosis Prognosis depends strongly on the underlying cause of the acute right heart syndrome. In pulmonary embolism, prognosis is quite good if the patient can be stabilized hemodynamically, but very poor (> 50% mortality) if not. [clinicaladvisor.com]
The overall prognosis in ARVD is not clear. [geneticheartdisease.org]
However, individuals with a family history of ARVC may benefit from genetic testing and counseling before planning for a baby The prognosis for Arrhythmogenic Right Ventricular Cardiomyopathy is favorable, if the condition is diagnosed early and treated [dovemed.com]
Holubarsch CJ, Colucci WS, Meinertz T, Gaus W, Tendera M; Survival and Prognosis: Investigation of Crataegus Extract WS 1442 in CHF (SPICE) trial study group. Eur J Heart Fail. 2008 Dec;10(12):1255-63. 13. [passeportsante.net]
Etiology
Humphrey Abstract We report a case of transient right ventricular dysfunction associated with prolonged cardiac tamponade, an unusual complication of uncertain etiology. [ajol.info]
In the remaining cases, it is believed to be due to an acquired etiology (e.g. virus infection) or to unidentified inheritance. The genes identified to date include the ryanodine receptor 2 (RYR2) and desmoplakin. [bcm.edu]
Etiology DORV results from an error in formation of the cono-truncus, which is the primary outlet of the heart during embryonic development. The cono-truncal anomalies result in neural crest and second heart field dysfunction. [orpha.net]
Acute lung edema was diagnosed in 19 (7.3 %) patients and ischemic etiology as the cause of heart failure was discovered in 198 (76.5 %) patients. [cardiovascularultrasound.biomedcentral.com]
(Etiology) Arrhythmogenic Right Ventricular Cardiomyopathy is caused by mutations in at least 5 genes involved in the formation and function of desmosomes. [dovemed.com]
Epidemiology
Summary Epidemiology DORV accounts for about 2-3% of all congenital heart defects, with a birth prevalence rate of 1/ 10,000 neonates as reported in Germany. [orpha.net]
House AA: Cardio-renal syndrome type 4: epidemiology, pathophysiology and treatment. Semin Nephrol 2012;32:40-48. [karger.com]
Epidemiology The incidence of acute cor pulmonale depends on the underlying cause. [clinicaladvisor.com]
Epidemiology of heart failure: The prevalence of heart failure and ventricular dysfunction in older adults over time. A systematic review. European Journal of Heart Failure, 18(3), 242-252. Von Bibra, H., Ströhle, A., St. [oiiq.org]
Epidemiology [ edit ] The prevalence of ARVD is about 1/10,000 in the general population in the United States, although some studies have suggested that it may be as common as 1/1,000. [en.wikipedia.org]
Pathophysiology
Can J Anaesth: 2012, 59(6);586-603 [PubMed:22528163] [WorldCat.org] [DOI] (I p) François Haddad, Ramona Doyle, Daniel J Murphy, Sharon A Hunt Right ventricular function in cardiovascular disease, part II: pathophysiology, clinical importance, and management [openanesthesia.org]
“Acute right ventricular failure — from pathophysiology to new treatments”. Intensive Care Med. vol. 30. 2004. pp. 185 Thorough review of all aspects of acute cor pulmonale, with an emphasis on pathophysiology and diagnosis. [clinicaladvisor.com]
Right ventricular contractile reserve and pulmonary circulation uncoupling during exercise challenge in heart failure: pathophysiology and clinical phenotypes. JACC Heart Fail. 2016. doi:10.1016/j.jchf.2016.03.007. [thecardiologyadvisor.com]
Pathophysiology Regardless of the mode of inheritance, it appears that the mutant genes code for similar proteins. The mutant proteins are called desmosomes. [geneticheartdisease.org]
Part II pathophysiology, clinical importance and management of right ventricular failure. Circulation. 2008;117:1717–31. PubMed CrossRef Google Scholar 6. Farb A, Burke AP, Virmani R. [link.springer.com]
Prevention
Infants who receive a heart transplant will need to take medicines for the rest of their lives to prevent their body from rejecting the new heart. References Parker SE, Mai CT, Canfield MA, et al; for the National Birth Defects Prevention Network. [cdc.gov]
The treatment of ARVD is focused on controlling abnormal heart rhythms, preventing sudden death, and managing any symptoms of heart failure that may be present. [brighamandwomens.org]
Endocarditis Prevention Antibiotics to prevent endocarditis are needed before certain dental procedures. See the section on Endocarditis for more information. [heart.org]
Preventing right ventricular hypertrophy from getting worse is possible in many cases. Prevention methods for RVH include reducing individual risk factors and promoting a heart and lung-healthy lifestyle. [medicalnewstoday.com]
The goal of treatment is to correct the heart rhythm immediately and to prevent future episodes. [healthline.com]