Acute Mitral Regurgitation Due to Chordae Tendineae Rupture: A Rare Presentation of Cardiac Amyloidosis.

BACKGROUND In cardiac amyloidosis (CA), misfolded proteins deposit in the extracellular space of cardiac tissue. These deposits classically cause restrictive cardiomyopathy with diastolic dysfunction. Although there are at least 30 proteins known to cause amyloid aggregates, 2 main types make up most diagnosed cases: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Since CA is considered a rare condition, it is often underdiagnosed or recognized in the advanced stages. Once amyloid deposits involve the heart tissue, they are associated with a worse outcome and higher mortality rates, especially in patients presenting symptoms of heart failure. CASE REPORT We report a case of a 22-year-old man presenting with acute severe mitral regurgitation, secondary to posterior mitral leaflet chordae tendineae rupture (CTR). Surgical mitral valve replacement with a mechanical prosthesis was performed, and cardiac tissue biopsy samples were obtained. After surgery, the patient improved significantly but suddenly presented with hemodynamic deterioration, until he died due to severe hemodynamic compromise and multiorgan failure. Although the etiology of the CTR was not established before surgical intervention, the histopathological analysis suggested CA. CONCLUSIONS CA diagnosis can be complex, especially in a 22-year-old-man with atypical clinical and imaging manifestations. In this patient, other differential diagnoses were considered, since CA presenting in a young patient is a rare phenomenon and acute mitral regurgitation secondary to CTR presents more frequently in other heart conditions. Furthermore, rapid postoperative deterioration resulted in the patient's death before biopsy samples were available because suspicion of amyloidosis had not been raised until that point.

Echocardiographic evaluation of patients submitted to replacement of ruptured chordae tendineae.

OBJECTIVE: The objective of this study was to evaluate, using echocardiography, the functioning of the mitral valve apparatus in patients submitted to standardized bovine pericardium chordae implantation to substitute ruptured chordae tendineae or elongated chordae with a significant degree of thinning. METHOD: Standardized bovine pericardium chordae were implanted in 23 patients with mitral valve insufficiency due to ruptured or elongated chordae with significant thinning. The ages of the patients varied from 23 to 84 years old (mean 62 years old). The most common cause was fibroelastic degeneration affecting 20 (87.0%) patients. The standardized bovine pericardium chordae were manufactured in sets connected at both ends by two polyester-reinforced rods thereby forming a single block. The bovine pericardium chordae measure 2 mm wide with 3 mm between the chordae. The sets of bovine pericardium chordae are produced in lengths varying from 20 to 35 mm. In 17 (73.9%) patients bovine pericardium chordae were implanted in the posterior cusp and in 6 (26.1%) in the anterior cusp. All the patients were evaluated in the postoperative period by echocardiography after a mean follow-up of six months. RESULTS: The echocardiography in the postoperative period demonstrated an absence of reflux in 11 (47.8%) patients, slight reflux in 8 (34.8%) and slight to moderate reflux in 3 (13.0%). The opening and mobility of the mitral valve was normal in the 22 surviving patients. CONCLUSION: The echocardiography demonstrated good functioning of the mitral valve apparatus in patients submitted to the implantation of standardized bovine pericardium chordae to substitute ruptured chordae tendineae or elongated chordae with a significant degree of thinning.

Rupture of mitral chorda tendinea following blunt chest trauma.

Acute mitral insufficiency, originated from rupture of mitral chordae tendineae secondary to nonpenetrating thoracic trauma, is an unusual condition. This diagnosis is difficult to establish because physical examination, electrocardiogram, and cardiac enzymes are neither sensitive nor specific. The diagnosis of rupture must be quickly established because this disorder may be fatal. This paper reports the case of a patient with acute mitral insufficiency secondary to a mitral valve chord rupture a week after a nonpenetrating thoracic trauma.

[Diagnosis wi transesophageal echocardiography and clinical course of 35 patients with flail mitral valve]. / Diagnóstico pela ecocardiografia transesofágica e evolução de 35 pacientes portadores de flail mitral valve.

PURPOSE: To describe transesophageal echocardiographic findings in patients presenting with flail mitral valve and to obtain their clinical follow-up. METHODS: From January/93 to March/97, 1675 patients were submitted to transesophageal echocardiogram at our institution; 35 of them were diagnosed as having flail mitral valve and their clinical follow-up was obtained. RESULTS: Thirty-five patients with the diagnosis of flail mitral valve were studied. Mean age was 65 +/- 15 years (12-87) and 27 (77%) were male. The posterior leaflet was involved in 25 (71%) patients. Ruptured chrodae tendineae was present in all but one patient in whom flail mitral valve was due to a very elongated and redundant chordae. The etiology was prolapse and/or mixomatous degeneration in 16 patients, degenerative in 10, ischemic in 5, rheumatic in 4 and endocarditis in 3. Mitral regurgitation was severe in 25 (71%) patients and moderate in 10 (29%). The mean follow-up was 375 +/- 395 days (1 to 1380). Nineteen patients were followed clinically and 16 were submitted to surgery (transesophageal echocardiogram findings were confirmed in all 16). Overall mortality was high (34%). Among the survivors, 17 are in NYHA class I and 6 in class II. CONCLUSION: The diagnosis of flail mitral valve by transesophageal echocardiography is accurate, allowing for the definition of its mechanism and etiology, as well as the evaluation of hemodynamic compromise. The observed high mortality at the time of diagnosis may be related to the severity of the disease causing the flail mitral valve. Although non-operated survivors are doing well, the observed low functional class in these patients may be related to the short period of follow-up.

[Clinical and echocardiographic study of mitral valve in children with severe rheumatic carditis. Aspects of prolapse or rupture]. / Estudo clínico e ecocardiográfico das alterações do aparelho mitral em crianças com cardite reumática grave. Aspecto de prolapso ou ruptura.

PURPOSE: To describe cases of rheumatic carditis with echocardiographic aspects of prolapse or rupture of mitral structures. METHODS: We described 16 cases of acute carditis (ages between 5-15 years). In 10 (group 1) there was aspect of mitral prolapse, in 6 (group 2) aspect of "flail". The measurements of anterior chordae and anuli were compared to the ones of 5 cases of chronic rheumatic fever with dilated left ventricle and aortic regurgitation (group 3) and to 20 normal children of same ages. The same measurements were repeated after treatment. RESULTS: The mean of the measurements in group 1 was; 26.48 +/- 11.46mm-anterior chordae; 32.13 +/- 7.35mm anuli; in the group 2 was respectively 29.63 +/- 4.57mm and 35.63 +/- 7 mm. In the group 3 anterior chordae was 34.52 +/- 6.8mm, anuli: 34.8 +/- 4.61 mm. In the normal group, respectively was: 18.19 +/- 4.26mm and 24.66 +/- 2.85mm. After corticosteroid many cases improved. In the statistical analyses, the measurements among patients of group 1 and 2 compared to normal children, the difference was significant but it was not compared to group 3. Comparing the measurements pre and post treatment the decrease was not significant. CONCLUSION: The acute carditis in these cases lead to significant elongation of anterior chordae and dilatation of anuli, but there was not a significant decrease after treatment.