Rupture Of Mitral Chordae Tendineae Research Articles

Acute rupture of chordae tendineae of the mitral valve in infants: a nationwide survey in Japan exploring a new syndrome.

Recently, infant cases of acute heart failure attributable to rupture of the mitral chordae tendineae have been reported. However, little is known about the pathogenesis and clinical course of this condition. Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013. The clinical manifestations, management strategies, and prognosis were investigated. Eighty-one (85%) patients were between 4 and 6 months (median, 5 months) of age. In 63 (66%) patients, rupture occurred during the spring or summer. The underlying conditions before rupture included Kawasaki disease (10 cases), maternally derived anti-SSA antibodies (2 cases), and infective endocarditis (1 case). Surgery was performed in 80 patients (94 operations), and the final operations included plasty of mitral chordae in 52 cases and mechanical valve replacement in 26 cases. The histopathologic examinations of the mitral valves and chordae (n=28) revealed inflammatory reactions with predominant mononuclear cell infiltration in 18 cases (64%) and increased fibrous and myxoid tissue in 11 cases (39%), suggesting that nonbacterial infectious or autoimmune endocarditis and myxoid changes are involved in the pathogenesis. Eight patients (8.4%) died before (n=6) and shortly after (n=2) the operation, and significant neurological complications persisted in 10 cases (11%). Acute heart failure attributable to rupture of the mitral chordae tendineae in infants is a unique disease resulting from diverse causes. This condition should be recognized as a significant cardiovascular disorder that may cause sudden onset of cardiogenic shock and death in infants.

Abstract 8638: Acute Massive Mitral Regurgitation Due to Rapture of Chordae Tendineae Affects Infants with High Rates of Mortality and Morbidity: Results From a Japanese Nationwide Survey

Introduction: Acute massive mitral insufficiency due to rupture of chordae tendineae is a rare but sometimes fatal condition in children. Serious symptoms such as dyspnea or cyanosis suddenly appear in healthy children. Although early diagnosis and appropriate medical and surgical treatments are necessary, this disease has not been well summarized in literatures and has not been well recognized by general pediatricians, either. Since the number of reported cases has been recently increasing in Japan, we have developed a nationwide survey of the disease. Hypothesis: We assessed the hypothesis that a Japanese nationwide survey clarifies etiology, clinical characteristics, treatments, and prognosis of the disease. Method: A questionnaire was sent to 511 pediatric institutes in Japan to unveil clinical and laboratory findings, surgical procedures, and prognosis of the patients during the recent 10 years. Results: A total of 88 children's data were analyzed. Males and females were 49 and 39, respectively. The number of patients was increasing during the recent 5 years (peaked in 2009, 17 cases). The age at onset mainly ranged from 4 to 6 months (68 cases, 77%) with a median at 5 months. The average body weight at birth and at onset was 2.97+/-0.48kg and 6.83+/-1.19kg, respectively. Seven patients had a history of Kawasaki disease. Maternal-derived serum anti-SSA antibody was detected in 2 patients. Average values of WBC, CRP, and cardio-thoracic ratio at diagnosis were 17,040+/-8,210/uL and 2.53+/-2.83mg/dl, and 55.4+/-5.8%, respectively. Forty-five patients (51%) underwent mitral valve repair and 25 patients (28%) underwent mitral valve replacement. Histopathological examinations of the mitral tissue were performed in 21 cases, showing mononuclear cell infiltration in 10 cases (48%) and myxoid change in 7 cases (33%). Six patients (6.8%) died before (4 cases) and after (2 cases) surgical operation. Moderate to severe brain damage remained in 10 patients (11%). Conclusions: Acute rupture of mitral chordae tendineae affects infants with high rates of mortality and morbidity. Since the incidence is recently increasing, precise recognition, early diagnosis, and appropriate medical and surgical treatments are strongly recommended.

A Novel Method of Leaflet Reconstruction After Triangular Resection for Posterior Mitral Valve Prolapse

Triangular leaflet resection to treat posterior mitral valve prolapse is expedient, sufficient, and durable in eliminating redundant myxomatous tissue and restoring normal leaflet coaptation. After triangular resection and suture reconstruction, mitral scallop height is decreased to a degree commensurate with the area of tissue resected. There are instances however, when excessive bulk of the remaining posterior leaflet may persist, predisposing to systolic anterior motion of the anterior mitral leaflet. We propose a novel adjunctive measure to normalize posterior leaflet height during suture reconstruction by ventricularizing what would otherwise have become the new tip of the posterior mitral leaflet. We have found that ventricularization of the leaflet edge during reconstruction obviates the need for excessive mitral leaflet resection, resulting in a symmetric and mobile neo-free edge that are capable of contributing to a robust zone of coaptation.

Rupture of mitral chordae tendineae: adding to the list of hypertension complications

Background:Many patients with chordae tendineae rupture (CTR) of the mitral valve have obscure aetiologies. The association between pre-existing hypertension and idiopathic CTR was investigated.Methods:494 patients with CTR were identified by...

Aortic stenosis and rupture of mitral chordae tendineae

Ten patients with aortic stenosis and ruptured mitral chordae tendineae constituted 8% of 125 consecutive surgical cases of chordal rupture. Their ages ranged from 54 to 87 years (mean 68). Six patients presented with acute onset of congestive heart failure, and eight were in New York Heart Association functional class III or IV at the time of cardiac catheterization. Extensive mitral anulus calcification was observed by fluoroscopy in seven patients. The mean aortic valve area index was 0.4 cm2/m2 and nine patients had moderate to severe mitral regurgitation by angiography. Calcific aortic stenosis affected a tricuspid valve in nine cases and a bicuspid valve in one case. One patient had a rheumatic mitral valve and one a redundant myxomatous mitral valve; the remaining eight had no abnormality of the mitral apparatus commonly regarded as predisposing to chordal rupture. Mitral anulus calcification and ventricular anatomic and hemodynamic alterations in aortic stenosis may contribute to rupture of the mitral chordae tendineae.

Ultrastructural changes in spontaneous rupture of mitral chordae tendineae.

Mitral valves from ten patients with spontaneous rupture of mitral chordae were examined by light, scanning and transmission electron microscopy. Changes identified at rupture sites included oedema and necrosis of collagen, endothelial loss and, in some cases, partial or complete re-endothelialisation of the disrupted ends. With a solitary exception these abnormalities were confined to chordae which had actually ruptured. Some non-ruptured chordae were irregularly thinned and stretched but were normal by light and electron microscopy. Mucoid degeneration was almost invariable in mitral valve leaflets. These results suggest that spontaneous chordal rupture is more likely to be the result of abnormal mechanical strain rather than a diffuse connective tissue abnormality affecting multiple chordae. They may also provide theoretical justification for reconstruction rather than replacement of mitral valves in this condition.

Indications for surgical replacement of the mitral valve: With particular reference to common and uncommon causes of mitral regurgitation

Mitral valve replacement is considered when there is severe mitral stenosis, severe mitral insufficiency or a combination of the two. Ordinarily, surgical replacement is considered only for patients who are in functional classes III or IV and do not respond to medical management. Patients with symptomatic mitral stenosis should be treated with mitral commissurotomy whenever possible. Patients selected for commissurotomy should have a pliable valve, no other major valve dysfunction, sinus rhythm, no systemic embolism and good left ventricular function. Early operation is not ordinarily required. Mitral insufficiency may require mitral valve replacement in six rather common settings: rheumatic disease, rupture of mitral chordae tendineae, postinfarction rupture of a papillary muscle, intractable infective endocarditis, floppy mitral valve and malfunction of a prosthetic valve. Rupture of mitral chordae tendineae can usually be recognized from the history, physical examination, echocardiogram and angiocardiogram. Severe left ventricular papillary muscle dysfunction is usually due to cardiac infarction, and occurs within the first 9 days of infarction. When only a papillary muscle tip is ruptured the patient may survive long enough for a mitral valve replacement. In infective endocarditis, operation is more often needed because of congestive heart failure than because of refractory infection. Evidence of mitral stenosis or insufficiency in a patient with a previously implanted prosthetic valve usually indicates an urgent need for study and early operation. Uncommon causes of mitral incompetence that may require valve replacement are endocardial fibroelastosis, Marfan's syndrome, calcified mitral anulus, osteogenesis imperfecta, methysergide-induced heart disease and carcinoid heart disease.

Congenital aortic valve disease with rupture of mitral chordae tendineae.

A new clinical entity is described in which free aortic regurgitation from congenital aortic valve disease caused rupture of the chordae to the anterior leaflet of the mitral valve in 7 men aged 45 to 63 years (mean 52 years); 2 of the patients also had rupture of chordae to the posterior leaflet. Comparing these patients with those with ruptured mitral chordae in association with rheumatic heart disease and patients with spontaneous chordal rupture, differences were evident. No patient had a history of rheumatic fever and none had active infection. The typical clinical presentation was of acute mitral regurgitation into a small left atrium, with severe pulmonary oedema which was often resistant to medical treatment. The cause of chordal rupture in these patients was in part the result of progressive left ventricular dilatation, of direct trauma to the anterior cusp of the mitral valve, and possibly of a genetic factor. The anatomical features of both aortic and mitral valves are described, and in 3 histology of the mitral valve was available; 2 had myxomatous degeneration similar to that seen in patients with spontaneous chordal rupture, and in 1 there was degeneration of collagen tissue. All patients were treated surgically but the mortality was high (5 out of 7,70%). Early operation with replacement of the aortic and mitral valves is recommended if this high mortality is to be reduced.

Chordae tendineae rupture complicating the systolic click-late systolic murmur syndrome

In a patient with a documented nonejection systolic click and late systolic murmur, acute mitral insufficiency developed as a result of spontaneous rupture of mitral chordae tendineae. There was no evidence of previous endocarditis, rheumatic fever or congenital heart disease. The ballooning mitral valve syndrome has previously been considered relatively benign. Chordal rupture has been predicted, and presumptive cases have been alluded to by several investigators. This well documented report of such a complication casts further doubt on the benignity of the syndrome.

Spontaneous Rupture of Mitral Chordae Tendineae

Seven patients with spontaneous rupture of mitral chordae tendineae confirmed by operation had a usual clinical profile consisting of a relatively abrupt onset of pulmonary congestion in a late middle-aged man, a systolic murmur, a vigorously pulsating heart, a normal rhythm, and a slight enlargement of the left atrium. Hemodynamic studies confirmed pulmonary congestion due to large pulmonary capillary v waves, and elevation of left ventricular diastolic pressure, although ventricular contractility in terms of total volume ejected was good. Mitral cusps showed atypical mucoid degeneration with no rheumatic or bacterial involvement. A relationship of this condition to others previously associated with mucoid degeneration of the mitral value is suggested. The cause of this syndrome is still unknown.

Spontaneous rupture of mitral chordae tendineae

Spontaneous rupture of mitral chordae tendineae

Combined Aortic and Mitral Incompetence.

s1 May 1968Combined Aortic and Mitral Incompetence.Kenneth I. Shine, M.D., Roman W. DeSanctis, M.D., F.A.C.P., Charles A. Sanders, M.D., W. Gerald Austen, M.D.Kenneth I. Shine, M.D.Search for more papers by this author, Roman W. DeSanctis, M.D., F.A.C.P.Search for more papers by this author, Charles A. Sanders, M.D.Search for more papers by this author, W. Gerald Austen, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-68-5-1161_3 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe problem of mitral incompetence complicating aortic regurgitation was analyzed in 39 patients. In each case the diagnosis was confirmed either at cardiac surgery or autopsy.Three groups were defined according to the cause of mitral insufficiency: group I (12 patients)—rupture of mitral chordae tendineae; group II (13 patients)—mitral insufficiency as a consequence of advanced left ventricular failure; and group III (14 patients)—mitral insufficiency due to rheumatic mitral incompetence.The clinical, hemodynamic, and radiographic features of the three groups were compared. Groups I and II showed many common features, including male predominance, preservation of sinus rhythm, and a small left... This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Boston, Massachusetts PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics 1 May 1968Volume 68, Issue 5Page: 1161-1162KeywordsAutopsyCardiac surgeryHemodynamicsMitral insufficiency Issue Published: 1 May 1968 PDF DownloadLoading ...

Rupture of mitral chordae tendineae: Clinical and pathologic observations on seven cases in which there was no bacterial endocarditis

The histories and pathologie observations in seven cases of rupture of the mitral chordae tendineae are reviewed. Patients with bacterial endocarditis were excluded from the series. All showed fibrosis and chronic injury of the mitral valve. In two, the lesions were those of rheumatic heart disease; in the remainder, the changes suggested quiescent rheumatic disease, but were not pathognomonic. The chordae of the two valve cusps were ruptured with equal frequency in this series, and usually more than one was broken. The point of rupture lay close to the papillary muscle. The stumps consisted of hyalinized and partially degenerated connective tissue, with a covering of endothelium. Scarring extended into the subjacent myocardium. The corresponding papillary muscles underwent atrophy if all their chordae were broken, but showed hypertrophy if a number were left attached. It was clear from inspection that rupture of the chordae must have allowed a high degree of mitral regurgitation. All hearts were dilated and hypertrophied, with an average weight of 580 grams. The histories of the patients did not indicate that external violence or vigorous exertion were etiologic factors of primary importance in rupture of the mitral chordae tendineae. The symptoms after rupture of the mitral chordae tendineae are those of congestive heart failure, which may be insidious or abrupt in its onset and progressive or remittent in its course. Months or even years may elapse between rupture and the onset of frank congestive failure. Rupture of the chordae is suggested by the sudden appearance of a loud precordial systolic murmur, maximal at the apex and left sternal border, where it is usually accompanied by a thrill. An apical diastolic murmur may also be present. Auricular fibrillation sometimes occurs. Roentgenograms show cardiac enlargement, and fluoroscopic examination may demonstrate systolic pulsation of the left atrium. The differential diagnosis includes bacterial endocarditis, rupture of a valve cusp, rupture of a papillary muscle, and perforation of an infarcted interventricular septum.