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Right Ventricular Disorder

Disorder of Right Cardiac Ventricle


Presentation

  • 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]
  • 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]
  • Method: CMRI of a young Asian female who presented with palpitations and history of sudden death in the family, imaged by a 3.0 Tesla MRI scanner with a maximum gradient strength of 33mT/m and a 16 element phased-array coil with electrocardiography gating[omicsonline.org]
  • 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]
Amyloidosis
  • […] that inflame the heart Iron buildup in your heart muscle (hemochromatosis) A condition that causes inflammation and can cause lumps of cells to grow in the heart and other organs (sarcoidosis) A disorder that causes the buildup of abnormal proteins (amyloidosis[mayoclinic.org]
  • Possible causes include viral infections (Coxsackie B - CVB), auto-immune diseases during pregnancy, the build-up of proteins in the heart muscle (amyloidosis), and an excess of iron in the heart (hemochromatosis).[childrenscardiomyopathy.org]
  • Management of extensive ventricular infiltration (eg, in amyloidosis) has improved considerably.[merckmanuals.com]
Respiratory Disorders
  • Get comprehensive, authoritative coverage on today's hot topics, such as interstitial lung disease, respiratory disorders in the newborn, congenital lung disease, swine flu, genetic testing for disease and the human genome, inflammatory cytokines in the[books.google.de]
  • disorders, or thromboembolic disease.[phaonlineuniv.org]
Heart Disease
  • Some people are born with heart disease.[icd10data.com]
  • Genetic changes in heart disease : Our medical team and other researchers at Stanford continue to identify more genetic mutations, or differences, that cause heart disease.[familyheart.stanford.edu]
  • Double outlet right ventricle (DORV) is a heart disease that is present from birth (congenital).[medlineplus.gov]
  • The right heart in adults with congenital heart disease. Rev Esp Cardiol. 2010;63:1070–86. PubMed CrossRef Google Scholar 58. Perloff JK. Congenital pulmonary stenosis. In: Perloff JK, editor. Clinical recognition of congenital heart disease.[link.springer.com]
Family History of Heart Disease
  • 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]
Akinesia
  • Major clinical features and signs identified in ARVD/ARVC affected patients include right ventricular akinesia, dyskinesia or aneurism accompanied with abnormal sizes of parasternal long and short axes and changes in end-systolic volume and right ventricular[centogene.com]

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]
Complete Left Bundle Branch Block
  • A: Electrocardiogram showing sinus rhythm with second-degree atrioventricular block Mobitz I, epsilon wave, inverted T waves from V1 to V4, and isolated ventricular ectopic beats with complete left bundle branch block.[revespcardiol.org]

Treatment

  • Virology: Research and Treatment about the journal submit your research Indexing: DOAJ, EBSCO, PubMed Central (PMC) and more .[insights.sagepub.com]
  • 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]
  • 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]

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]
  • PAH is associated with a poor prognosis.[phaonlineuniv.org]

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 [ 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]
Sex distribution
Age distribution

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

  • […] enhancement of transmural right ventricular free wall, right ventricular trabeculations and mild right ventricular septal enhancement on the post contrast phase was noted Conclusion: ARVC can be diagnosed by CMRI using the revised task force criteria, can prevent[omicsonline.org]
  • 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]

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