Two cases of bi-ventricular dysplasia associated with ventricular tachycardia and familial occurrence of sudden death.
Two strikingly similar patients with arrhythmogenic right ventricular dysplasia which severely impaired not only the right ventricle but also the left ventricle are described in association with familial occurrence of sudden death. A 49-year-old man experienced syncope which was due to ventricular tachycardia. Electrocardiography revealed a first degree atrioventricular block, incomplete right bundle-branch block, T wave inversions in leads II, III, a VF and V1 to V5, and multiformal ventricular extrasystoles. Echocardiography and ventricular cineangiography showed not only the right ventricular dilatation with an aneurysm in the right ventricular apex, inflow and outflow tracts, but also mild dilatation of the left ventricle with left ventricular apical and posterior aneurysms. Radionuclide angiography also disclosed dysfunction of both ventricles, especially during exercise. His family history revealed that 3 members of his family died of sudden deaths. A 56-year-old woman experienced syncope secondary to ventricular tachycardia, with left bundle-branch block. Electrocardiography showed complete right bundle-branch block, left axis deviation, and T wave inversions in leads V1 to V4. Echocardiography and ventricular cineangiography revealed not only marked right ventricular dilatation with the "triangle of dysplasia", but also a left ventricular aneurysm in the apex and posterior portion. Her elder brother died of a sudden death, and electrocardiograms of 2 members of her family showed ventricular extrasystoles and T wave inversions. These 2 cases may well be termed "familial bi-ventricular dysplasia".
Nishikawa
H
H
Division of Cardiology, Yamada Red Cross Hospital, Mie.
Kasai
A
A
Ono
N
N
Unno
M
M
Kakuta
Y
Y
Nishimura
S
S
Nishiyama
S
S
Nakanishi
S
S
Seki
A
A
Nakano
T
T
D005117:Cardiac Complexes, Premature / Q000150:complications; D002932:Cineangiography; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004452:Echocardiography; D004562:Electrocardiography; D005192:Family Health; D005260:Female; D006321:Heart / Q000000981:diagnostic imaging; D006352:Heart Ventricles / Q000002:abnormalities* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011877:Radionuclide Imaging; D017180:Tachycardia, Ventricular / Q000150:complications* / Q000175:diagnosis / Q000235:genetics