Patients presenting with complete Uhl anomaly should be considered for cardiac transplantation. [orpha.net]

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]

Presentation usually occurs in the neonatal and childhood years with clinical aspects of congestive heart failure. [radiopaedia.org]

• Atrial Septal Defect

  They made a 4 mm atrial septal fenestration; whereas we completely closed the atrial septal defect. A fenestration would have been made if the right atrial pressure had elevated. [academic.oup.com]

  The treatment is surgical repair frequently with a bidirectional Glenn shunt which is performed by closing the tricuspid valve and creating an atrial septal defect, effectively excluding the right ventricle. [healio.com]

  […] enlargement right ventricular dilation accompanied by paradoxical septal motion associated congenital cardiac anomalies may include: tricuspid valve malformations especially Ebstein's anomaly tricuspid atresia atrial septal defects patent ductus arteriosus [radiopaedia.org]

  septal defect Cardiac output is maintained Restrictive interatrial shunting through the foramen ovale leads to fetal hydrops and demise Celermajer index: the ratio of the area of the right atrium and the atrialized right ventricle to the area of the [ultrasoundregistryreview.com]

• 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]

  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]

  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]

• Hypoxemia

  He presented as a neonate with severe hypoxemia (arterial P o 2 of 29 mm Hg) and functional pulmonary atresia. [ahajournals.org]

  Hypercarbia and hypoxemia should be avoided. Perioperative invasive cardiac monitoring is highly recommended. Prophylactic antibiotics must be given as indicated. [accessanesthesiology.mhmedical.com]

  The patient was admitted to the hospital due to a respiratory infection, presenting with fever, dyspnea and hypoxemia (peripheral oxygen saturation of 55%). [revportcardiol.org]

• Hypothermia

  Adherence to hypothermia guidelines: a French multicenter study of fullterm neonates. PLoS One. 2013;8(12):e83742. 22. Chougrani I, Luton D, Matheron S, Mandelbrot L, Azria E. Safety of protease inhibitors in HIV-infected pregnant women. [xn--epop-inserm-ebb.fr]

• Dyspnea

  The presentation may vary from arrhythmia induced syncope or sudden cardiac death to a syndrome consistent with isolated right-sided heart failure, including 5: hepatomegaly jugular venous distension dyspnea cyanosis ECG sinus rhythm multi-component epsilon [radiopaedia.org]

  In contrast to ARVD where the classic presentation is usually with ventricular arrhythmias, patients with Uhl’s Anomaly generally present with cyanosis, dyspnea and right sided failure, usually in infancy or early childhood. [chdbabies.com]

  Abstract A 1-day-old female Holstein–Friesian calf was presented for severe dyspnea. Physical examination revealed respiratory distress, moderate edema of the ventral neck, and swollen jugular veins. The calf died and was submitted for necropsy. [journals.sagepub.com]

  The patient was admitted to the hospital due to a respiratory infection, presenting with fever, dyspnea and hypoxemia (peripheral oxygen saturation of 55%). [revportcardiol.org]

• Hepatomegaly

  The presentation may vary from arrhythmia induced syncope or sudden cardiac death to a syndrome consistent with isolated right-sided heart failure, including 5: hepatomegaly jugular venous distension dyspnea cyanosis ECG sinus rhythm multi-component epsilon [radiopaedia.org]

  Figure 1: Ultrasonography of the abdomen showing hepatomegaly with coarse echotexture and dilated hepatic vein Click here to view A chest X-ray revealed cardiomegaly [Figure 2]. [heartviews.org]

  The two heart sounds S1 and S2 were heard, but S2 was single with tender hepatomegaly. Anterior fontanel was fused. [nigjcardiol.org]

  The infant underwent semitotal resection of the right ventricular free wall, tricuspid annuloplasty, and a one and one-half ventricular repair. 2 Case report A 51-day-old boy was admitted to our hospital for progressive cyanosis and hepatomegaly. [academic.oup.com]

• Heart Disease

  Mal Cardiovasc 9:121 Google Scholar Sherman FE (1963) An atlas of congenital heart disease, pp 270. [link.springer.com]

  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]

  Repairing the tricuspid valve in congenital heart diseases other than Ebstein’s. Cardiology in the Young, Vol. 24, Issue. 6, p. 1077. [cambridge.org]

  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]

• Tachycardia

  The tachycardia may consist of frequent uniform premature ventricular complexes, transient repetitive bursts of ventricular tachycardia, ventricular tachycardia induced during exercise or manifest only immediately after stopping exercise. [chdbabies.com]

  Figure 3Narrow QRS tachycardia. A: Induction of supraventricular tachycardia (SVT) with 1:2 atrioventricular (AV) response. [heartrhythmcasereports.com]

  435-7 } } Fu-Ping Li, Ying-Bin Xiao, Xuefeng Wang Published in Journal of cardiac surgery 2011 DOI: 10.1111/j.1540-8191.2011.01266.x We report a case of a 23-year-old male with Uhl's anomaly who presented with nonparoxysmal atrioventricular junctional tachycardia [semanticscholar.org]

  In reciprocating a tachycardia the RP distance on surface ECG is prolonged due to delayed atrial conduction in almost all patients. [pedcard.rush.edu]

  Left Ventricular Outflow Tract Obstruction LDS Loeys Dietz Syndrome Long QT Syndrome LGL Lown-Gangong-Levine Syndrome Marfan Syndrome MVP Mitral Valve Prolapse MAPCA Multiple Aorto-Pulmonary Collateral Arterials Noonan Syndrome PAT Paroxysmal Atrial Tachycardia [savinglittlehearts.com]

• Cyanosis

  Initially, other more common causes of poor right ventricular systolic shortening, sluggish pulmonary blood flow and cyanosis were entertained. [annalspc.com]

  The presentation may vary from arrhythmia induced syncope or sudden cardiac death to a syndrome consistent with isolated right-sided heart failure, including 5: hepatomegaly jugular venous distension dyspnea cyanosis ECG sinus rhythm multi-component epsilon [radiopaedia.org]

  However, he did note that he had suffered cyanosis over the past three years, which was never investigated, and had no other symptoms. [revportcardiol.org]

• Hypertension

  Keywords: Absent myocardium and portal hypertension, adult Uhl's anomaly, Uhl's anomaly How to cite this article: Agarwal R, Datta R, Saha M, Sarkar N. Uhl's anomaly: A rare case of portal hypertension. [heartviews.org]

  Despite the previous catheterization data, the diagnostic and therapeutic target was pulmonary hypertension, and thus she underwent an open lung biopsy to rule out pulmonary capillary alveolar dysplasia as a cause of severe pulmonary hypertension. [annalspc.com]

  Uhl's Anomaly: A Rare Case of Portal Hypertension Medicine Heart views : the official journal of the Gulf Heart Association 2016 A case of Uhl's anomaly is described in a 27-year-old young male patient presenting with portal hypertension and esophageal [semanticscholar.org]

• Palpitations

  Case report – A 12-year old girl presented with progressive breathlessness and palpitations. Patient was admitted with right-sided heart failure symptoms. She was treated for cardiac failure. [recentscientific.com]

  A personal history of palpitations, especially in a young person, or a family history of sudden cardiac death or death at an early age should raise the index of suspicion for ARVD. [aafp.org]

  Complaints like palpitation, syncope, ventricular tachycardia, and sudden death, many times induced by physical exercise, are more frequently related to arrhythmogenic dysplasia 6,7. [scielo.br]

  The clinical finding in Uhl's anomaly is heart failure with complaints of palpitation, syncope, tachycardia, and sudden death. [jcecho.org]

  Prior to this event, the patient had been feeling well, without any significant limitations of physical activity or known palpitations. [heartrhythmcasereports.com]

• Skin Ulcer

  Fisher BH: Hyperbaric oxygen for skin ulcers. Roche Med Image Commentary, 1969 13. *Fischer BH: Treatment of ulcers on the legs with Hyperbaric oxygen. J Dermatol Surg; 1(3) pp 55-8, 1975 14. [hbot.al]

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]

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]

Palliative treatment includes the treatment of heart failure using antifailure regimen, treatment of arrhythmias with antiarrhythmic drugs, and drainage of effusion.[10] Surgical palliative measures include closing the tricuspid valve, creating an atrial [nigjcardiol.org]

[…] the overall prognosis of Uhl anomaly. [checkorphan.org]

(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 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. [academic.oup.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]

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]

Further studies investigating risk factors and co-morbid birth defect associations with the etiology of hearing loss are warranted. [aap.confex.com]

KASSAB P, COLOMBIER ML, KAMINSKI M, LELONG N, SIXOU M, NABET C ; EPIPAP (EPIdemiological study on the relation between Periodontitis and Adverse Pregnancy outcomes) group. Impact of periodontitis definition in epidemiological research. [xn--epop-inserm-ebb.fr]

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]

Uhl's anomaly: clinical spectrum and pathophysiology. Int J Cardiol, 209 (2016), pp. 118-121 [9] A.M. Taksande, V. Gautami. Uhl's anomaly with absent tricuspid valve in an infant. J Cardiovasc Echogr, 25 (2015), pp. 90-92 [10] N.M. Ikari, E. [revportcardiol.org]

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]

Female urinary incontinence, from pregnancy to menopause, a review of epidemiologic and pathophysiologic findings. Acta Obstet Gynecol Scand 2012 ; 91 ; 901-910 29. [xn--epop-inserm-ebb.fr]

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]

Prevention - Uhl anomaly Not supplied. Diagnosis - Uhl anomaly The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of Uhl anomaly. [checkorphan.org]

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. [academic.oup.com]