Valantine H A, Tazelaar H D, Macoviak J, Mullin A V, Hunt S A, Fowler M B, Billingham M E, Schroeder J S
Division of Cardiology, Stanford University School of Medicine, CA 94305.
J Heart Transplant. 1987 Jul-Aug;6(4):244-50.
From 1976 to 1986, six cases of cardiac sarcoidosis have been documented by myocardial biopsy in three of five instances; on examination of the explanted heart after transplantation in two, and at autopsy in one patient. Right ventricular end-diastolic pressure was elevated in all four patients with right ventricular involvement with sarcoidosis. Of three patients treated with steroids, improvement in ventricular function and decrease in arrhythmia occurred in two, whereas failure to respond led to transplantation in the other patient. Two further patients have undergone heart transplantation, one for resistant ventricular arrhythmia and the other for congestive heart failure. No recurrence of sarcoidosis has occurred in the grafts. Because two of five patients had sarcoidosis diagnosed on gross examination, a negative endomyocardial biopsy does not exclude the diagnosis of myocardial sarcoidosis, which should therefore be pursued in the setting of unexplained heart failure, conduction abnormalities, and ventricular arrhythmia, particularly when right ventricular end-diastolic pressure is raised. Steroids may result in improvement in some patients even in the presence of severe morphological damage. Heart transplantation may be performed without increased risk of recurrence of sarcoidosis.
1976年至1986年间，5例患者中有3例经心肌活检证实为心脏结节病；2例在心脏移植后检查切除的心脏时确诊，1例在尸检时确诊。所有4例累及右心室的结节病患者右心室舒张末期压力均升高。3例接受类固醇治疗的患者中，2例心室功能改善，心律失常减少，而另1例无反应则接受了心脏移植。另有2例患者接受了心脏移植，1例因顽固性室性心律失常，另1例因充血性心力衰竭。移植心脏未发生结节病复发。由于5例患者中有2例经大体检查诊断为结节病，因此心内膜活检阴性并不能排除心肌结节病的诊断，对于原因不明的心力衰竭、传导异常和室性心律失常患者，尤其是右心室舒张末期压力升高时，应进行进一步检查以明确诊断。即使存在严重的形态学损害，类固醇治疗仍可能使部分患者病情改善。心脏移植时结节病复发风险不会增加。
