心内膜心肌纤维化病的外科治疗

手术治疗心内膜心肌纤维化右室型及左室型各１例，取得良好疗效。本症较少见，易误诊，应提高对其认识和警惕性。根据临床表现，结合超声心动图及心脏造影检查可以作出诊断。无论左室型或右室型，手术经心房切口为宜，对纤维化内膜行谨慎的锐性剥离并行受累房室瓣膜替换术。术中心肌保护和术后心功能维护是取得良好疗效的重要因素。     

Endomyocardial Fibrosis Mimicking Ebstein's Anomaly: A Diagnostic Challenge

Endomyocardial fibrosis is a rare disease that is seen most commonly in tropical countries. It usually presents with characteristics of right-heart failure. Herein, we report the case of a 14-year-old adolescent boy who experienced endomyocardial fibrosis. Upon transthoracic echocardiography, the condition was mistakenly diagnosed as Ebstein''s anomaly of the tricuspid valve. Sixteen months after undergoing tricuspid annuloplasty and receiving a bidirectional Glenn shunt, the patient showed no echocardiographic evidence of valvular regurgitation. We discuss imaging and surgical techniques that enable the diagnosis and treatment of endomyocardial fibrosis.Key words: Adolescent, arteriovenous shunt, surgical, diagnosis, differential, diagnostic imaging/methods, endomyocardial fibrosis/complications/diagnosis/pathology/radiology/surgery, heart ventricles/radiography, prognosis, treatment outcome, tricuspid valve insufficiency/diagnosis/surgery, ventricular dysfunction, right/etiologyEndomyocardial fibrosis (EMF) is a rare disease that is seen most commonly in tropical countries. It usually presents with characteristics of right-heart failure. A 14-year-old adolescent boy''s case of EMF was mistakenly diagnosed upon transthoracic echocardiography as Ebstein''s anomaly of the tricuspid valve. Here, we discuss the case of this patient, along with imaging and surgical techniques for the diagnosis and treatment of EMF.     

Technical aspects in surgery for endomyocardial fibrosis: experience with 37 patients

The surgical technique of endocardiectomy and valve replacement for patients with uni- or biventricular endomyocardial fibrosis is described. Technical details of the operation are outlined, based upon experience with 37 surgical cases.     

M-mode echocardiographic features of endomyocardial fibrosis.

M-mode echocardiographic findings are described in 21 patients with endomyocardial fibrosis. Features associated with right ventricular endomyocardial fibrosis include: (i) exaggerated motion and thickening of the anterior right ventricular wall; (ii) increased right ventricular end-diastolic dimension; and (iii) paradoxical septal motion. Pericardial effusion (viz an echo-free space behind the posterior left ventricular wall) was shown in three patients. The tricuspid valve was easily recorded in all. Six patients with left ventricular endomyocardial fibrosis had diminished left ventricular end-diastolic dimension. Three had echo features of pulmonary hypertension (viz reduced e-f slope, absent a wave in sinus rhythm, and systolic notching of the pulmonary valve echogram). Fine fluttering of the anterior mitral valve and tricuspid valve echo was observed in two patients (one of whom was in sinus rhythm) with biventricular endomyocardial fibrosis, and no angiographic evidence of aortic regurgitation.     

Cardiac biopsy evidence for a cardiomyopathy associated with symptomatic mitral valve prolapse

Right ventricular endomyocardial biopsy was performed in 14 patients with mitral valve prolapse to determine the existence of an associated cardiomyopathic process. All 14 patients had echocardiographic, angiographic or auscultatory evidence of mitral valve prolapse, and all were symptomatic. The group had a high incidence rate of conduction system abnormalities (50 percent) and arrhythmias (64 percent), but only one patient had a significant hemodynamic abnormality. Light microscopy revealed an increase in endocardial and interstitial fibrosis in eight patients (57 percent). Electron microscopy, performed in 11 patients, showed mitochondrial degenerative changes in all 11. Nuclear chromatin clumping, intracell edema and myocyte degeneration were frequently present. It is concluded that endomyocardial and myocardial abnormalities exist in some symptomatic patients with idiopathic mitral valve prolapse.     

Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease.

Eleven patients with a restrictive cardiomyopathy are described. Seven of these had biventricular disease and in them the haemodynamic abnormality resembled that in constrictive pericarditis but the condition was distinguishable because of unequal involvement of the two ventricles, murmurs of atrioventricular valve regurgitation, or characteristic ventricular deformity on angiography. Two of these patients had eosinophilia with the clinical features of Löffer's endocarditis. In 4 patients the disease was apparently confined to the left ventricle; they were investigated on account of atypical chest pain, third heart sound, or abnormal electrocardiogram. Simultaneous measurements of left ventricular pressure and volume throughout diastole were made in 3 patients and showed rapid but abruptly curtailed left ventricular filling. Transvenous endomyocardial biopsy in 2 patients showed fibrous tissue with collagen and irregular elastic fibrils. Surgical biopsy in 3 patients excluded pericardial constriction but was diagnostically unhelpful because of failure to obtain endocardium. Necropsy in one patient showed that the heart had features indistinguishable from tropical endomyocardial fibrosis. It is suggested that the spectrum of ventricular abnormalities in these patients results from endomyocardial fibrosis of varying severity and probably of differing cause. This study has shown that "tropical" endomyocardial fibrosis may occur outside the tropics and suggests that eosinophilia may play a leading role or associated part in the genesis of some cases.     

Successful surgical treatment of two patients with eosinophilic endomyocardial disease.

Cardiac surgery to treat severe heart failure was of benefit to two patients with endomyocardial disease and hypereosinophilia-eosinophilic endomyocardial disease. Both patients had severe biventricular fibrosis with mitral and tricuspid regurgitation. One had predominant right ventricular disease and was treated by right ventricular endocardectomy with tricuspid and mitral xenograft valve replacement. The second patient's main haemodynamic problem was considered to be mitral regurgitation. His mitral valve was replaced by a Starr-Edwards prosthesis; endocardectomy was not performed. Though both patients had toxic confusional states for several weeks postoperatively there was distinct symptomatic and objective evidence of improvement which has been maintained for over 16 months. Previous reports of surgical treatment of 22 patients without eosinophilia (all of whom had endomyocardial resection) and three other patients with eosinophilia have shown equally encouraging results. There has been no evidence of recurrence or progression of heart damage in follow-up periods of up to seven years. It is concluded that cardiac surgery is an important advance in the treatment of endomyocardial disease in patients with or without an eosinophilia.     

Surgical treatment of endomyocardial fibrosis with preservation of mitral valve.

A 48-year-old Kenyan African, who presented with a history of coronary and cerebral embolism, was found to have endomyocardial fibrosis of the left ventricle. It proved possible to remove all diseased tissue at operation, and at the same time to preserve the mitral valve. It is important to inspect the mitral valve from both the atrial and ventricular aspects so that the valve can be spared, if it is not involved in the disease process. The choice of transatrial of transventricular surgical approach for the removal of pathological tissue may depend on whether or not the mitral valve requires replacement.     

Unique presentation of hypereosinophilic syndrome: recurrent mitral prosthetic valve thrombosis without endomyocardial disease

Hypereosinophilic syndrome (HES) is defined as a prolonged, unexplained peripheral eosinophilia in a patient presenting with end-organ damage. The heart is frequently involved, resulting in eosinophilic endomyocardial disease, which is characterized by mural thrombus formation and endocardial fibrosis. Thromboembolic complications in HES are mediated by material released from eosinophilic granules. Herein is reported the case of a patient who presented, 15 years after valve replacement with a mechanical prosthesis, with clinical signs of recurrent prosthetic valve thrombosis that was caused by missed hypereosinophilia. The unique feature of the case was that the mitral prosthetic valve obstruction was the result of an eosinophilic thrombus, though no tissue infiltration or inflammation had been detected by random biopsy of the left ventricular myocardium. After nine years of effective treatment of HES there were no cardiac or extracardiac complications.     

8例心内膜心肌纤维化的诊疗经验

目的总结8例心内膜心肌纤维化(EMF)的诊断和治疗经验。方法对有不明原因的心力衰竭并考虑为EMF的8例患者施行超声心动图(UCG)、核磁共振成象(MRI)、心内膜心肌活检(EMB)等检查,4例予内科治疗,4例行外科手术。结果UCG、MRI及EMB获得EMF诊断的例数分别为3/8,4/4及3/4,同时行以上3种检查诊断的例数为3/3。4例经内科治疗,2周至6个月内全部死亡。2例施行右心室心内膜切除加三尖瓣成形术,术后恢复良好,随访6～9年,心功能2级,能从事轻度工作。2例行右心室心内膜切除加三尖瓣置换术,手术死亡1例,1例存活6年,死于心力衰竭。结论综合应用UCG、MRI及EMB可提高EMF的诊断率,对病变较轻的EMF患者行心内膜切除加瓣膜成形或置换术可获得良好的效果,对病变重的病例应行心脏移植。     

One-and-a-half ventricle repair for right ventricular endomyocardial fibrosis

One-and-a-half ventricle repair, consisting of endocardiectomy with tricuspid valve replacement and bidirectional cavopulmonary shunt, was performed on a patient with right ventricular endomyocardial fibrosis and right ventricular outflow tract obstruction. The patient made a smooth recovery. We believe that this repair provides good palliation for a subset of patients with right ventricular endomyocardial fibrosis.     

Left endomyocardial fibrosis with severe mitral insufficiency; the case for mitral valve repair. A report of 4 cases

Left ventricular endomyocardial fibrosis accompanied by severe mitral insufficiency occurring in 4 adolescent African patients is reported. Mitral valve repair was successfully performed in all 4 cases including annuloplasty in 3 and chordal shortening in one. Short-term follow-up of up to 10 months indicated substantial clinical improvement and decrease of the cardiothoracic ratio in all. It is emphasized that mitral valve repair appears to be the method of choice in treating mitral incompetence in the localized form of endomyocardial fibrosis, where endocardiectomy is not required in the area of the papillary muscles.     

Myocardial and endocardial involvement in tuberculous constrictive pericarditis: difficulty in biopsy distinction from endomyocardial fibrosis as a cause of restrictive heart disease

Differentiation of constrictive pericarditis and endomyocardial fibrosis by the available diagnostic methods is difficult at present. With this in mind, we compared autopsy data in heart specimens from 8 patients of constrictive pericarditis of tuberculous origin and 6 patients with tropical endomyocardial fibrosis. Post-mortem endomyocardial biopsies were also performed from both the ventricles. Pericardial thickening and adhesions were present in all cases of constrictive pericarditis and in none of those with endomyocardial fibrosis. Interestingly, significant endocardial thickening was seen in four, and myonecrosis, lymphohistiocytic cellular infiltration and myofibrosis were observed in seven of the eight cases of constrictive pericarditis. This suggests that the tubercular insult to heart may result in involvement of all three layers with varying severity. The endomyocardial biopsy specimens from the affected areas revealed similar histopathological changes of endocardial thickening and focal myofibrosis with cellular infiltrates in patients with both constrictive pericarditis and endomyocardial fibrosis, making distinction of the two difficult. The hallmark of differentiation between these two clinical entities appears to be a diligent attempt at identification of the thickened pericardium.     

Mitral valve repair for severe mitral regurgitation caused by endomyocardial biopsy

Mitral regurgitation (MR) following endomyocardial biopsy is a rare and severe complication. A 70-year-old man with severe MR due to chordal injury caused by left ventricular endomyocardial biopsy is described. In this patient, a few chordae tendineae of the posterior-median papillary muscle were injured by the biopsy forceps. Due to the chordal rupture, both anterior and posterior leaflets were prolapsed and severe MR developed. MR was successfully treated by artificial chordal replacement using extended polytetrafluoroethylene sutures and ring annuloplasty. This mitral valve repair with artificial chordal replacement was considered suitable to treat MR resulting from iatrogenic chordal injury as the leaflets were not involved in the degenerative process and papillary muscle function was preserved. To avoid MR, the transvenous approach should be used routinely for endomyocardial biopsies; biopsy from the left ventricle is not justified.     

Techniques for right and left ventricular endomyocardial biopsy

Right ventricular endomyocardial biopsy using percutaneous right internal jugular approach proved a safe and easily performed technique in more than 1,300 procedures. Adequate tissue was obtained in more than 98 percent of patients and morbidity rate was remarkably low. Other approaches to the right ventricle may be used, but retrograde left ventricular endomyocardial biopsy appears to be the safest and most reliable alternative to transjugular right ventricular biopsy. The safety and success of the techniques for right and left heart biopsy described depend on meticulous attention to methodologic detail.     

Valve repair for mitral insufficiency secondary to idiopathic juvenile polyarthritis

A female patient in whom idiopathic rheumatoid polyarthritis was diagnosed at the age of 8 years required surgery for severe mitral valve insufficiency 16 years later. Intraoperative analysis revealed a fibrotic endocarditis involving mainly the posterior leaflet. Granulomatous vegetations as well as a large thrombus which filled the left ventricular apex and simulated endomyocardial fibrosis were noted. Valve repair was achieved using an anterior leaflet augmentation with a patch of mitral homograft associated with a prosthetic ring annuloplasty. Postoperatively, a severe pericardial effusion required surgical drainage. Eight years later, the patient had no cardiac symptoms and echocardiography confirmed a normally functioning mitral valve.     

Endomyocardial fibrosis. Presentation of a clinical case]

The authors report a case of right ventricle endomyocardial fibrosis in a 6-year-old boy. Particular attention is paid to the discussion of the new methods for diagnosing and treating these conditions, namely echocardiography, endomyocardial biopsy and surgery.     

Findings on endomyocardial biopsy in infants and children with dilated cardiomyopathy

Fifteen infants and children with dilated cardiomyopathy underwent transvascular endomyocardial biopsy. The light and electron microscopic findings were reviewed to evaluate the presence of lymphocytes as an indicator of active myocarditis. Both ventricles were biopsied in 13 patients, and the right ventricle only was biopsied in 2. None of the endomyocardial specimens obtained by biopsy revealed an inflammatory process. Interstitial fibrosis, myofiber hypertrophy, degeneration and necrosis were found. Ultrastructural abnormalities of the mitochondria, T tubules or Z bands were noted in approximately one-third of patients. Persistent, active myocarditis is an uncommon cause of dilated cardiomyopathy in children. Immunosuppressive therapy, which may be harmful, should be considered only after myocardial inflammation has been documented by endomyocardial biopsy.     

Endomyocardial fibrosis with massive endocardial calcific deposits

Massive endocardial calcific deposits in the left ventricular chamber is such an uncommon lesion that only three cases have been reported previously. This report describes an apparently asymptomatic patient with endomyocardial fibrosis and massive endocardial calcific deposits. The results of electrocardiography, echocardiography, angiography, and endomyocardial biopsy findings are presented, and the pathogenesis of massive endocardial calcification is speculated upon. The interesting point is that the clinical history and manifestation of this patient suggests that endomyocardial fibrosis may occur in a benign form and long-term survival may be expected.     

Cerebral ischemia: a complication of right ventricular endomyocardial biopsy

During percutaneous transvenous endomyocardial biopsy, two cardiac transplant recipients developed symptoms of focal cerebral ischemia. Cerebrovascular injury has not been previously associated with endomyocardial biopsy. This procedure should be recognized as one of the causes of stroke following heart transplantation.