二尖瓣脱垂合并胸廓骨骼畸形

本文对83例二尖瓣脱垂进行了X线分析,发现有40例至少合并有一种胸廓骨骼畸形,占48％;其中扁胸直背27例,占27％,胸椎侧弯9例,占11％,漏斗胸4例,占5％。指出,胸廓骨骼畸形是二尖瓣脱垂的重要征象之一。胸廓骨骼畸形也可能影响二尖瓣脱垂的X线诊断。     

Mitral valve prolapse

Mitral valve prolapse is a common mitral valve disorder manifested clinically as a midsystolic click and/or a late systolic murmur (the click-murmur syndrome) and pathologically as billowing or prolapsing mitral leaflets (the floppy valve syndrome). Not only is it one of the two most common congenital heart diseases and the most common valve disorder diagnosed in the United States, but it is also prevalent throughout the world. Mitral valve prolapse may be associated with a variety of other conditions or diseases. Diagnosis of mitral valve prolapse should be made on clinical grounds and, if necessary, supported by echocardiography. The majority of patients with mitral valve prolapse suffer no serious sequelae. However, major complications such as disabling angina-like chest pains, progressive mitral regurgitation, infective endocarditis, thromboembolism, serious arrhythmias, and sudden death may occur. Unless these serious complications occur, most of the patients with mitral valve prolapse need no treatment other than reassurance, including those with atypical chest pain or palpitation unconfirmed by objective data. Therapy with a beta-blocker for disabling chest pain and/or arrhythmias and antiplatelet therapy for cerebral embolic events may be indicated. In occasional patients with significant mitral regurgitation surgery may be necessary.     

Current management of mitral valve prolapse

Mitral valve prolapse is a pathologic anatomic and physiologic abnormality of the mitral valve apparatus affecting mitral leaflet motion. "Mitral valve prolapse syndrome" is a term often used to describe a constellation of mitral valve prolapse and associated symptoms or other physical abnormalities such as autonomic dysfunction, palpitations and pectus excavatum. The importance of recognizing that mitral valve prolapse may occur as an isolated disorder or with other coincident findings has led to the use of both terms. Mitral valve prolapse syndrome, which occurs in 3 to 6 percent of Americans, is caused by a systolic billowing of one or both mitral leaflets into the left atrium, with or without mitral regurgitation. It is often discovered during routine cardiac auscultation or when echocardiography is performed for another reason. Most patients with mitral valve prolapse are asymptomatic. Those who have symptoms commonly report chest discomfort, anxiety, fatigue and dyspnea, but whether these are actually due to mitral valve prolapse is not certain. The principal physical finding is a midsystolic click, which frequently is followed by a late systolic murmur. Although echocardiography is the most useful mode for identifying mitral valve prolapse, it is not recommended as a screening tool for mitral valve prolapse in patients who have no systolic click or murmur on careful auscultation. Mitral valve prolapse has a benign prognosis and a complication rate of 2 percent per year. The progression of mitral regurgitation may cause dilation of the left-sided heart chambers. Infective endocarditis is a potential complication. Patients with mitral valve prolapse syndrome who have murmurs and/or thickened redundant leaflets seen on echocardiography should receive antibiotic prophylaxis against endocarditis.     

The treatment of mitral valve prolapse syndrome in a nurse-managed outpatient mitral valve prolapse clinic

Symptoms of mitral valve prolapse syndrome, the most common valvular disorder, include anxiety, fatigue, palpitations, headaches, and chest pain, to name a few. This syndrome is effectively treated in a nurse-managed, outpatient clinic that specializes in the treatment of mitral valve prolapse syndrome.     

Acute coronary syndrome in a young woman with a giant coronary aneurysm and mitral valve prolapse: a case report and literature review

Acute coronary syndrome in the young population is infrequently seen and has a different etiology from that in the elderly population. Giant coronary artery aneurysms are rare and usually asymptomatic, but they can cause acute clinical symptoms such as chest pain or chest tightness. We herein describe a young woman with a history of mitral valve prolapse who developed sudden-onset chest pain. She had mild elevations of her creatine kinase and cardiac troponin levels; however, no ST segment alteration was found on an electrocardiogram, and no abnormal regional wall movement was noted on echocardiography. Cardiac magnetic resonance imaging with late gadolinium enhancement revealed a “mass” at the right coronary artery and linear subendocardial enhancement at the posterior wall. Coronary angiography later confirmed a giant coronary aneurysm with a substantial thrombus. The combined presence of the coronary artery aneurysm and mitral valve prolapse in this patient was likely a sequela of Kawasaki disease.     

The Phenomenon of Dysautonomia and Mitral Valve Prolapse

In recent years research has shown that subsets of patients with mitral valve prolapse also have associated autonomic or neuroendocrine dysfunction that can result in a number of related symptoms, including fatigue, palpitations, chest pain, exercise intolerance, dyspnea, dizziness, headache, sleep disorders, gastrointestinal disturbances, cold extremities, and panic attacks. These patients have been classified as having mitral valve prolapse syndrome. This article discusses the pathogenesis and management of mitral valve prolapse syndrome and serves to make clinicians aware of newer developments in the study of autonomic function and dysfunction.     

超声心动图诊断儿童马凡综合征

目的 观察儿童马凡综合征（MFS）患者的超声心动图表现。方法 回顾性分析临床疑诊MFS的59例患儿的影像学及临床检查资料,观察其超声心动图表现。结果 12例诊断为MFS,6例诊断为潜在MFS,其中11例存在家族史,7例为散发病例。上述18例中,4例存在主动脉窦部增宽,11例存在主动脉窦部扩张,1例合并主动脉瓣轻度反流,2例合并肺动脉窦部扩张;8例二尖瓣受累,5例二尖瓣脱垂并中—重度反流,5例三尖瓣受累;1例同时存在二尖瓣及三尖瓣脱垂而接受二尖瓣成形术;1例合并室间隔缺损,接受室间隔缺损修补术。结论 儿童MFS中,以主动脉窦部增宽或扩张最为常见,可合并二尖瓣脱垂;超声心动图检查有助于早期诊断和及时干预儿童MFS。     

Mitral Valve Prolapse and Pre-Excitation

Two out of 118 patients with mitral valve prolapse were found to have pre-excitaiton. The presence of mitral valve prolapse in one patient with Lown-Ganong-Levine syndrome and the persistence of prolapse in (he presence and absence of pre-excitation in another patient with intermittent type B Wolff-Parkinson-White syndrome suggest that mitral valve prolapse in pre-excitation syndrome may not be secondary to the abnormal activation pattern of the left ventricle, which has been suggested as a possible mechanism by some investigators. (PACE, Vol. 5, September-October, 1982)     

Association of mitral valve leaflet prolapse with cerebral ischaemic events in the young and early middle-aged patient

In order to clarify possible links between mitral valve leaflet prolapse and cerebral ischaemic events we carried out a study prospectively over a period of two years on 66 patients (35 men and 31 women) under the age of 50 who suffered from transient cerebral ischaemic attacks or completed strokes. Twenty-three (34.8 per cent) of the 66 patients were found to have mitral valve leaflet prolapse syndrome. In 16 (24.2 per cent) of those, the only abnormality found was mitral valve leaflet prolapse, suggesting that the mitral valve leaflet prolapse syndrome may be a potential cause of cerebral ischaemic events. Therapeutic approaches for preventing further similar events are discussed.     

A Novel Truncating Variant in FLNC-Encoded Filamin C May Serve as a Proarrhythmic Genetic Substrate for Arrhythmogenic Bileaflet Mitral Valve Prolapse Syndrome

A 51-year-old man with a long-standing history of bileaflet mitral valve prolapse accompanied by mitral annular disjunction and mild mitral regurgitation presented for evaluation of increasingly frequent palpitations. Ambulatory Holter monitoring, cardiac magnetic resonance imaging, serial transthoracic echocardiography, and diagnostic electrophysiology studies were consistent with a diagnosis of arrhythmogenic bileaflet mitral valve prolapse syndrome. Because of the presence of a similar phenotype in the proband’s mother, brother, and maternal aunt, research-based whole exome sequencing was pursued and a novel truncating variant (p.Trp34*-FLNC) in the cardiomyopathy-causative FLNC-encoded filamin C unearthed that cosegregated with disease. Unexpectedly, these observations provide the first evidence that a heritable proarrhythmic genetic substrate (ie, FLNC haploinsufficiency–mediated weakening of cell-cell adhesion) may underlie, at least in part, some cases of arrhythmogenic mitral valve prolapse syndrome.     

Mitral valve prolapse in children

Although it is the most common congenital cardiac problem in children, mitral valve prolapse usually is asymptomatic and requires no treatment. Dr Greenwood believes, however, that if other factors, such as Marfan's syndrome, mitral regurgitation murmur, abnormalities on ECG or other tests, or chest pain or palpitations, are present, the patient should be followed closely. In this article, he groups patients according to initial symptoms and outlines diagnostic and follow-up regimens for each group.     

Follow-up of patients with mitral valve prolapse presenting with rhythm disturbances

The aim of the study was to analyze rhythm disturbances and clinical course in 18 patients aged 20-52 years with mitral valve prolapse. The patients were followed up for a mean of three years (range: 1-11 years). We analyzed clinical signs, resting ECG 24 hour ECG, exercise ECG, echocardiograms and the effects of antiarrhythmic treatment. One patient developed marked mitral incompetence. Supraventricular arrhythmias occurred in three patients and ventricular extrasystoles in all 18 patients (class I in 2, II in 6, III in 4, IV in 3, V in 3, according to Lown). Five patients required combined antiarrhythmic treatment. Course of pregnancy and births in two patients with mitral valve prolapse syndrome and class IV arrhythmias were uncomplicated for both the mothers and their babies. Great clinical variability of mitral valve prolapse syndrome ranging from an asymptomatic form to ventricular fibrillation is emphasized.     

Pain in mitral valve prolapse

Thirty subjects with mitral valve prolapse were carefully observed, in order to investigate whether chest pain, characteristic of angina pectoris, was present. No cases of angina pectoris or of heart pain were observed. A characteristic which did emerge was that 86% of the subjects suffered a typical myofascial pain of the muscles of the chest. There does not appear to be a clear relationship between mitral valve prolapse and myofascial pain.     

Association of Mitral Valve Leaflet Prolapse with Cerebral Ischaemic Events in the Young and Early Middle-aged Patient

In order to clarify possible links between mitral valve leafletprolapse and cerebral ischaemic events we carried out a studyprospectively over a period of two years on 66 patients (35 men and 31 women) under the age of 50 whosuffered from transient cerebral ischaemic attacks or completedstrokes. Twenty three (34.8 per cent) of the 66 patients werefound to have mitral valve leaflet prolapse syndrome. In 16(24.2 per cent) of those, the only abnormality found was mitralvalve leaflet prolapse, suggesting that the mitral valve leafletprolapse syndrome may be a potential cause of cerebral ischaemicevents. Therapeutic approaches for preventing further similarevents are discussed.     

Delayed papillary muscle rupture after radiofrequency catheter ablation: A case report

BACKGROUNDWith an increased number of surgical procedures involving the mitral annular region, the risk of mitral valve prolapse (MVP) has also increased. Previous studies have reported that worsening of MVP occurred early after radiofrequency catheter ablation (RFCA) at papillary muscles in ventricular tachycardia (VT) patients with preoperative MVP. CASE SUMMARYWe report a case where MVP and papillary muscle rupture occurred 2 wk after RFCA in a papillary muscle originated VT patient without mitral valve regurgitation or prolapse before. The patient then underwent mitral valve replacement with no premature ventricular contraction or VT. During the surgery, a papillary muscle rupture was identified. Pathological examination showed necrosis of the papillary muscle. The patient recovered after mitral valve replacement. CONCLUSIONToo many ablation procedures and energy should be avoided.     

Echocardiographically determined mitral valve prolapse in male patients

Mitral valve prolapse syndrome is a common clinical entity that may occur as an isolated condition or in association with other cardiovascular diseases. In this retrospective study, 41% (23/56) of the male patients with echocardiographically diagnosed mitral valve prolapse had an additional form of cardiovascular disease.     

Disorders of cardiac rhythm in combined ventricular pre-excitation syndrome and primary mitral valve prolapse

Primary prolapse of the mitral valve was diagnosed in 27 (11.7%) out of 230 patients with preexcitation syndrome: 10 of them presented with Wolff-Parkinson-White syndrome, 15 with Clerc-Lévy-Critesco syndrome, and two patients with combination of both syndromes. Preexcitation syndrome combined with mitral valve prolapse manifested itself by different disorders of the cardiac rhythm, mainly by the supraventricular forms: by PST in 17, nonparoxysmal tachycardia in 1, atrial fibrillation paroxysms in 7, sinus tachycardia in 2, supraventricular extrasystole in 3, pacemaker migration in the atria in 1 patient. Clerc-Lévy-Critesco syndrome initiated with atrial fibrillation paroxysms in 7 patients with mitral valve prolapse and with the enlargement of the left atrium. The prognostically unfavourable varieties of the preexcitation syndrome and mitral valve prolapse manifested themselves in ventricular tachycardia progressing to ventricular fibrillation, paroxysmal supraventricular tachycardia with a high frequency of ventricular contractions up to 213-230 and atrial tachyarrhythmia up to 250-280 per minute, which were regarded as factor at risk for potential transformation to ventricular tachycardia; as well as in continuously recurrent attacks of arrhythmia in the presence of multiple AV additional tracts or combined with sinoatrial and atrioventricular block.