It was described in 1952 Uhl anomaly is a congenital heart disease characterized by partial or complete absence of myocardial muscle cells (myocardium) in the free wall of the right ventricle.
Conditions disease (A disease is a change in the functions or health of a living organism, animal…) By Uhl, malformation (A malformation is a congenital morphological change of a tissue or organ…) By Uhl, papery right ventricle Where parchment heart refer to the same entity.
Its frequency will be 1 in 100,000 births.
This is an exceptional malformation (83 cases reported since the first description in 1993) that is probably often confused with another more recent individualized right ventricular malformation, arrhythmogenic right ventricular dysplasia.
The reason for this anomaly is not clear. The latest hypothesis would involve a phenomenonapoptosis (We call this process apoptosis (or programmed cell death or cell suicide)…) cellular, normal during fetal development but excessive in this malformation and can selectively destroy myocardial cells of the free wall of the right ventricle. Experimentally, comparable lesions of the right ventricle were observed after infection virus (A virus is a biological entity that requires a host cell to use…) Coxsackie B3.
This malformation is responsible for the large right ventricular failure that appears from the first weeks life (Life is a given name 🙂 (and probably from childhood) is almost always fatal in a short time without any attempt at surgical treatment.
First observation (Observation is the act of carefully watching events without the will to see them…) often in 1905 a. attributed to Osler described at autopsy man (A male is an adult male individual of a species called Modern Man (Homo…).) A 40-50-year-old heart with very thin walls looked like parchment. However, in 1948, a new study of the anatomical piece by Dr. Harold N Segall (held in a Berlin museum) shows that in fact the walls of this heart retain muscle cells, and the thinning covers the entire walls of both the auricles and the ventricles. .
It therefore appears that the princeps observation was made by Uhl at some point autopsy (Autopsy (or post-mortem examination or necropsy) is the medical examination of corpses. The…) It was performed on an 8-year-old child in 1949 month (A month (Lat. mensis from the word “month”, formerly from the word “menstruation” in the plural) is a period of time…) and was published in 1952. The same year (A year is a unit of time that represents the time between two occurrences of a related event…)Castleman and Towne report a observation (Observation is the act of carefully watching events without the will to see them…) similar in adults. Both describe the loss of myocardial tissue responsible for excessive thinning of the right ventricular walls, which is only rarely preserved. fibers (Fibers are plant or animal, filamentous elementary formations, se…) muscle. In both cases, the inflammatory process is not observed as in a myocarditis (Myocarditis is an inflammatory injury of the myocardium due to various causes.)or could explain the destruction of coronary lesions myocardium (Myocardium is the muscle tissue (myo-, muscle) of the heart (-cardium). Muscle…).
In 1979, Fontaine et al. describe another arrhythmogenic right ventricular dysplasia (ARVD) characterized by localized abnormalities of the right ventricular myocardium, which is replaced by fibrous and fatty tissue. DAVD, although rare, is significantly more common than Uhl anomaly, and it is likely that the few cases of Uhl anomaly reported before 1980 were actually observations of arrhythmogenic dysplasia, especially in adults. .
It is from everyone heart problem (Heart failure (HF) or heart failure corresponds to a condition…) and possibly arrhythmia. It is alone, insufficient and only palliative.
Various interventions have been proposed with success variable (Represented in mathematics and logic by the variable symbol. This…). Because this anomaly is rare, these operations are often performed “in isolation” and we have no comparable series. Their purpose:
– relax the right ventricle by diverting the venous circulation by creating either partial or complete cavo-pulmonary diversion;
– or to strengthen the deficient wall of the right ventricle with the advanced cardiomyoplasty process point (graphic) Together with tricuspid valvuloplasty to correct this valve deficiency by Professor Carpentier’s team.
– Finally, heart transplantation was suggested (ref 12).