A congenital disease characterized by the absence of a normal
development of the myocardial layer of the right ventricle (usually replaced
by fibrous or fatty tissue). Because the myocardial layer is dysplastic, the endocardium and
epicardium are in apposition, which results in severe dilatation and early congestive cardiac
failure. Chest radiography demonstrates marked cardiomegaly and right-sided
chamber enlargement. Echocardiography and right-ventricle angiography
demonstrate increased right heart volumes, severe diffuse hypokinesis, and
tricuspid valve regurgitation.
Parchment Right Ventricle.
Congenital disorder first described by H.S. Uhl in 1952.
Very rare; 80 cases described in the literature.
It is inherited as autosomal dominant with reduced penetrance. Generally sporadic cases caused by mutations
or the results of exposure to toxic or infective agents.
Not precisely known; may be the result of primary
nondevelopment of myocytes or may be a result of selective apoptosis.
The diagnosis of right ventricular cardiomyopathy is based on the echocardiographic and
angiographic documentation of localized or widespread structural and dynamic
abnormalities involving mainly or exclusively the right ventricle. The absence of valvular disease, intracardiac
shunts, active myocarditis, and/or coronary disease is essential to establish the diagnosis. The performance of
endomyocardial biopsy is considered very useful in the differential diagnosis. 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. The most important electrocardiographic abnormalities are T-wave
inversion in the right precordial leads and the presence of late potentials in signal averaging
ECG. The association with cardiac failure is rare except for those affected with the cardiomegalic form.
There is a risk of sudden death and syncopal attacks due to ventricular fibrillation; however,
most of the time the arrhythmias are well tolerated. Affected individuals often have good exercise
tolerance and do not have a history of previous myocarditis. Congestive cardiac failure is the main sign; it
may be associated with massive peripheral edema or massive pleural effusion leading to cardiac tamponade. Pulmonary
atresia can be associated.
Evaluate the cardiac function (clinical,
ECG, echocardiography, chest radiographs), and the extent of the pulmonary involvement
(clinical, radiographs, arterial blood gas analysis). Also assess the hepatic
function (clinical, laboratory investigation including coagulation test,
ultrasound) because of the potential hepatic disturbance secondary to cardiac
Elective surgical procedures must be postponed until
the cardiovascular function has been corrected. Patients presenting with congestive heart
failure should not receive anesthesia for nonurgent procedures. The presence of pericardial
and/or pleural effusions should be treated before the administration of anesthesia. Anesthetic
agents should be primarily chosen on the basis of their effects on the pulmonary resistance
(e.g., nitrous oxide, ketamine, etc.). The pulmonary vascular resistance should be maintained at the
lowest possible level. Hypercarbia and hypoxemia should be avoided. Perioperative invasive
cardiac monitoring is ...