Schneider U, Jenni R, Turina J, Turina M, Hess O M
Division of Cardiology, University Hospital, Zurich, Switzerland.
Heart. 1998 Apr;79(4):362-7. doi: 10.1136/hrt.79.4.362.
To determine the long-term outcome of patients with endomyocardial fibrosis and to compare echocardiographic and haemodynamic data before and after ventricular endocardial resection.
Seventeen patients (11 women and six men; mean age 35.5 years) diagnosed with endomyocardial fibrosis at the University Hospital in Zurich, Switzerland from 1971 to 1995. Twelve patients (70%) had partial obliteration of both ventricles and in five patients (30%) the fibrotic lesions were limited to the left ventricle.
Fourteen of the 17 patients had surgical resection: fibrosis was resected from both ventricles in five patients and from the left ventricle only in nine patients. Ten patients had mitral valve replacement and two had tricuspid valve replacement. Left ventricle endocardial resection was done without reconstruction or replacement of the atrioventricular valve in three patients. Preoperative and postoperative echocardiographic data were available for 11 patients and haemodynamic data for six patients. Patients were followed up for 0.4-19 years (mean 8.6).
Preoperatively four patients were NYHA functional class IV and 10 were class III; postoperatively one patient was class III, seven class II, and six class I. Preoperatively, echocardiography showed obliteration of the left ventricular apex and inflow tract in all patients, which decreased or disappeared after surgery. Left ventricular end diastolic pressure decreased from 25 mm Hg before surgery to 14 mm Hg after successful resection of the fibrosis. Left ventricular and diastolic volume (normal 93 (17) ml/m2) increased from 65 ml/m2 to 97 ml/m2 (p < 0.05) after surgery. Ejection fraction was normal preoperatively (57%) and decreased slightly (52%) after surgery. One patient died five months after surgery from heart failure. Four surgically treated patients died during the follow up period: one each from systolic dysfunction, recurrence of endomyocardial fibrosis, pneumonia, and food poisoning. Overall survival was 65% at five years and 59% at 10 years; the survival rates of the operated patients was 72% and 68%, respectively. Only one of the medically treated patients survived longer than three years from diagnosis.
Endomyocardial fibrosis is a rare disease in European countries and is found mainly in women. The clinical picture is characterised by severe congestive heart failure but heart size is only moderately increased. Systolic performance is normal or only slightly depressed despite severe restriction to filling, atrioventricular valve regurgitation or both. Partial obliteration of the right and/or left ventricle may be detected by echocardiography. Endocardial resection with atrioventricular valve replacement is the treatment of choice with appreciable postoperative improvement and 10 year survival of approximately 70%.
确定心内膜心肌纤维化患者的长期预后，并比较心室心内膜切除术前和术后的超声心动图及血流动力学数据。
1971年至1995年在瑞士苏黎世大学医院诊断为心内膜心肌纤维化的17例患者（11例女性和6例男性；平均年龄35.5岁）。12例患者（70%）双侧心室部分闭塞，5例患者（30%）纤维化病变局限于左心室。
17例患者中的14例行手术切除：5例患者双侧心室纤维化均被切除，9例患者仅左心室纤维化被切除。10例患者行二尖瓣置换术，2例患者行三尖瓣置换术。3例患者行左心室心内膜切除术时未重建或置换房室瓣。11例患者有术前和术后超声心动图数据，6例患者有血流动力学数据。对患者随访0.4至19年（平均8.6年）。
术前4例患者为纽约心脏协会（NYHA）心功能IV级，10例为III级；术后1例患者为III级，7例为II级，6例为I级。术前，超声心动图显示所有患者左心室心尖和流入道闭塞，术后这种情况减少或消失。成功切除纤维化后，左心室舒张末期压力从术前的25 mmHg降至术后的14 mmHg。左心室舒张末期容积（正常为93（17）ml/m²）术后从65 ml/m²增加至97 ml/m²（p<0.05）。术前射血分数正常（57%），术后略有下降（52%）。1例患者术后5个月死于心力衰竭。4例接受手术治疗的患者在随访期间死亡：分别死于收缩功能障碍、心内膜心肌纤维化复发、肺炎和食物中毒。5年总生存率为65%，10年为59%；接受手术治疗患者的生存率分别为72%和68%。仅1例接受药物治疗的患者从诊断起存活超过3年。
心内膜心肌纤维化在欧洲国家是一种罕见疾病，主要见于女性。临床表现为严重充血性心力衰竭，但心脏大小仅中度增加。尽管存在严重的充盈受限、房室瓣反流或两者皆有，收缩功能正常或仅轻度降低。超声心动图可检测到右心室和/或左心室部分闭塞。行房室瓣置换的心内膜切除术是首选治疗方法，术后有明显改善，10年生存率约为70%。
