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Uhl Anomaly


Presentation

  • Patients presenting with complete Uhl anomaly should be considered for cardiac transplantation.[orpha.net]
  • Patients with Uhl’s Anomaly and ARVD have different clinical modes of presentation.[chdbabies.com]
  • At present, only about 84 cases have been reported in over a century.[nigjcardiol.org]
  • However, adult Uhl's has also been rarely described. [4] Case presentations have varied from prenatal diagnoses [5], [6] to nonparoxysmal atrioventricular junctional tachycardia with Mobitz I block in adults [7] to asymptomatic presentations. [4] The[heartviews.org]
Fatigue
  • Symptoms and Signs UHL symptoms include dyspnoea, fatigue, chest pain, syncope on exertion, and non-specific systolic murmur. The latter can be a cause of cardiac failure and sudden death in neonates and young adults.[dianamossop.com]
  • Symptoms are dyspnoea, fatigue, chest pain and syncope on exertion, non-specific systolic murmur. It is a cause of cardiac failure and sudden death in neonates and young adults. We thank Martin Lehn, Germany, for information submitted.[whonamedit.com]
  • A 12 year old boy was admitted with the complaint of fatigue with effort. A holosystolic murmur was heard on left lower sternal border. Electrocardiography showed right atrial dilation and low voltage in all leads.[heart.bmj.com]
Heart Disease
  • Diagnosis and Management of Adult Congenital Heart Disease, by Drs. Gatzoulis, Webb, and Daubeney, is a practical, one-stop resource designed to help you manage the unique challenges of treating long-term adult survivors of congenital heart disease.[books.google.com]
  • Sherman FE (1963) An atlas of congenital heart disease, pp 270. Lea and Febiger, Philadelphia Google Scholar 21.[link.springer.com]
  • He was suffering from a rare heart disease, found in only one among 200,000 children. Mohammad Bilal was suffering from Uhl's anomaly.[thequint.com]
  • Definition First described in 1952, UHL anomaly is a rare congenital heart disease characterized by a partial or total loss of the myocard muscle in the right ventricle. This results in decreased output of the right side of the heart.[dianamossop.com]
  • Henry Uhl (1921 – 2009) upon examining one of his patients. [1] It is a very rare congenital heart disease (less than 100 cases 1900–1993) with a partial or total loss of the myocardial muscle in the right ventricle .[medbox.iiab.me]
Pansystolic Murmur
  • Cardiac examination revealed a laterally shifted diffuse apical impulse with soft first heart sound and pansystolic murmur of tricuspid regurgitation.[heartviews.org]

Workup

  • The patient was treated with optimal medical therapy while awaiting cardiac transplantation evaluation, but sadly, he died of progressive heart failure before workup was completed. There were no reported ventricular arrhythmias.[scmr.org]

Treatment

  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • Treatment Treatment relies on palliative care but the only hope of extended survival is surgery, which includes cardiac transplantation.[medigest.uk]
  • The response to medical treatment is poor and there is no known ideal treatment. Isolated cases of different surgical options have been described that include cardiac transplant, but there is still a poor prognosis and a high mortality rate.[pesquisa.bvsalud.org]
  • Treatment strategies of chronic right heart failure including drugs and mechanical devices are also discussed. 著者について (2014) Norbert F. Voelkel, MD Virginia Commonwealth University Department of Medicine Richmond, VA, US.[books.google.com]

Prognosis

  • (Outcomes/Resolutions) The prognosis of Uhl Anomaly is dependent upon the severity of the signs and symptoms and associated complications, if any Individuals with mild conditions have better prognosis than those with severe symptoms and complications[dovemed.com]
  • The prognosis is related to the degree of right atrial dilatation and the cardiac compromise. Some cases have been reported to survive into adulthood. Benson CB, Brown DL, Roberts DJ. Uhl's anomaly of the heart mimicking Ebsteins anomaly in utero.[fetalultrasound.com]
  • Isolated cases of different surgical options have been described that include cardiac transplant, but there is still a poor prognosis and a high mortality rate.[pesquisa.bvsalud.org]
  • The prognosis varies greatly from being lethal in the neonatal period to long-term survival in other individuals. The major clinical feature is the presentation of different types of arrhythmias with a left bundle branch block pattern.[accessanesthesiology.mhmedical.com]
  • The prognosis in the case of an infant is uniformly poor; though cases surviving surgery have been reported recently [3–6]. We report a 2-month-old infant undergoing surgical treatment for Uhl's anomaly with critical ventricular arrhythmia.[ejcts.oxfordjournals.org]

Etiology

  • Abstract Uhl's anomaly is a rare cardiac disease of unknown etiology characterized by a markedly distended right ventricle because of partial or complete absence of right ventricular myocardium, which consequently develops a parchment-like appearance.[jstage.jst.go.jp]
  • Several points, however, still require clarification, such as the exclusive involvement of the right ventricle and the existence of a possible etiologic agent in the process.[scielo.br]
  • (Etiology) Presently, the exact cause of Uhl Anomaly is not known.[dovemed.com]
  • Schuster Springer Science & Business Media , 6 dic 2012 - 318 pagine Primary myocardial disease, nowadays referred to as congestive or, more re cently, dilating cardiomyopathy, comprises disorders of varied etiology.[books.google.it]

Pathophysiology

  • Pathophysiology of shock, sepsis, and organ failure, Berlin, itd: Springer Verlag, str. 502-517 [ CR ] [ PM ] [ EČ ][ GS ] Idell, S., James, K.K., Coalson, J.J. (1992) Fibrinolytic activity in bronchoalveolar lavage of baboons with diffuse alveolar damage[scindeks.ceon.rs]
  • Pathophysiology ARVD is characterized by progressive replacement of the right ventricular myocardium by fibrofatty tissue ( Figure 1 ). 8, 9 The most common location for this tissue transformation is between the anterior infundibulum, right ventricular[aafp.org]

Prevention

  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Uhl Anomaly may not be preventable, since it is a congenital disorder Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders Regular medical screening at periodic[dovemed.com]
  • Complete exclusion of the free wall of the right ventricle would be necessary to prevent recurrence of ventricular arrhythmia; and the total exclusion of the right ventricle means the creation of a single ventricle.[ejcts.oxfordjournals.org]
  • Louis, Missouri, USA (3) Department of Clinical Radiology and Imaging Diagnostics, 4th Military Hospital, Wrocław, Poland (4) Center for Diagnosis Prevention and Telemedicine, John Paul II Hospital, Kraków, Poland Abstract Uhl anomaly is a very rare condition[radiologykey.com]

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