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.

Lesión postraumática de la válvula tricúspide / Post-traumatic injury of tricuspid valve

Resumen Se presenta el caso de un adulto con insuficiencia de la válvula tricúspide de origen traumático, debido a ruptura del músculo papilar de la valva anterior en relación a trauma de tórax cerrado, se realizó el diagnóstico mediante ecocardiografía transesofágica. La válvula se reemplazó mediante bioprótesis con resultados clínicos favorables. Este caso enfatiza la necesidad de una alta sospecha de lesión valvular por parte del clínico en los pacientes con trauma torácico cerrado. (Acta Med Colomb 2016; 41: 202-205).

Abstract The case of an adult with traumatic tricuspid valve insufficiency due to papillary muscle rupture of the anterior leaflet in relation to closed chest trauma is presented. Diagnosis was made by trans-esophageal echocardiography. The valve was replaceded by bioprosthesis with favorable clinical outcome. This case emphasizes the need for a high suspicion of valvular lesion by the clinician in patients with blunt chest trauma. (Acta Med Colomb 2016; 41: 202-205).

The use of a high-power laser on swine mitral valve chordae tendineae.

Worldwide, rheumatic fever remains a significant cause of mitral valve insufficiency. It is responsible for approximately 90 % of early childhood valvular surgeries in Brazil. Elongated or flail chordae are frequently responsible and require surgical correction. The purpose of this study was to analyze and compare the histological tissues of the mitral valve chordae and the mechanical resistance generated by the chordae, both with and without the application of a high-power laser. Twenty normal porcine mitral valve chordae were measured and divided randomly into the following two groups: control group (not subjected to a high-power laser) and laser group (subjected to photonic irradiation). Laser surgery was performed under controlled conditions, using following parameters: λ = 980-nm wavelength, power = 3 W, and energy = 60 J. A mechanical test machine was used in combination with a subsequent histological study to measure chordae tensile properties. A histological analysis demonstrated a typical collagen bundle arrangement in the control group; however, under a particular reached temperature range (48), the collagen bundles assumed different arrangements in the laser group. Significant reductions in the chordae tendineae lengths and changes in their resistance in the laser group were observed, as these chordae exhibited less rigid fibers. The chordae tendineae of normal porcine valves subjected to a high-power laser exhibited its length reduction and less stiffness compared to the control group. A histological analysis of the laser treatment specimens demonstrated differences in collagen bundle spatial organization, following slight changes into tissue temperature.

Arcada mitral: causa silente de sibilancias recurrentes en la infancia. Caso clínico / Anomalous mitral arcade: a disguised and silent lesion leading to a recurrent wheezing in children. A case report

El síndrome bronquial obstructivo recurrente o sibilante recurrente constituye uno de los motivos de consulta más frecuentes en pediatría. Entre los diagnósticos etiológicos diferenciales, se encuentran las cardiopatías congénitas. En esta presentación, se describe el caso de un niño de 4 años derivado a nuestra Institución para valoración cardiológica por presentar sibilancias recurrentes. Se trató de un caso de estenosis mitral congénita grave secundaria a arcada mitral. La arcada mitral es una cardiopatía congénita infrecuente. Constituye una variante de obstrucción al tracto de entrada del ventrículo izquierdo, que afecta el aparato subvalvular mitral y genera hipertensión pulmonar retrograda poscapilar y edema intersticial. El diagnóstico se realizó mediante la evaluación clínica, electrocardiográfica, radiológica y, fundamentalmente, ecocardiográfica. Se efectuó una corrección quirúrgica exitosa con remoción completa de la obstrucción mitral.

Recurrent wheezing is a very common clinical ailment throughout infancy and childhood. The most common diagnosis in children with wheezing is asthma. However, some other causes should be considered in the differential diagnosis such as a congenital cardiac defect. We present a case of a four year old boy presenting with recurrent wheezing who was referred to our institution for cardiac evaluation. Severe mitral stenosis secondary to an anomalous mitral arcade was diagnosed by physical examination, chest X-ray, electrocardiogram and mainly transthoracic and transesophageal echocardiography. Anomalous mitral arcade is a rare congenital malformation of the mitral tensor apparatus which comprises the chordae tendineae and papillary muscles. This abnormal anatomy leads to increased filling pressure of the left ventricle, a retrograde post capillary pulmonary hypertension and interstitial aedema. The patient was referred to cardiac surgery and underwent a successful procedure with complete removal of the obstructive mitral lesion.

[Anomalous mitral arcade: a disguised and silent lesion leading to a recurrent wheezing in children. A case report]. / Arcada mitral: causa silente de sibilancias recurrentes en la infancia. Caso clínico.

Recurrent wheezing is a very common clinical ailment throughout infancy and childhood. The most common diagnosis in children with wheezing is asthma. However, some other causes should be considered in the differential diagnosis such as a congenital cardiac defect. We present a case of a four year old boy presenting with recurrent wheezing who was referred to our institution for cardiac evaluation. Severe mitral stenosis secondary to an anomalous mitral arcade was diagnosed by physical examination, chest X-ray, electrocardiogram and mainly transthoracic and transesophageal echocardiography. Anomalous mitral arcade is a rare congenital malformation of the mitral tensor apparatus which comprises the chordae tendineae and papillary muscles. This abnormal anatomy leads to increased filling pressure of the left ventricle, a retrograde post capillary pulmonary hypertension and interstitial aedema. The patient was referred to cardiac surgery and underwent a successful procedure with complete removal of the obstructive mitral lesion.

Análise do efeito do laser de alta potência em valvas mitrais cardíacas de suínos: estudo experimental / Analysis of high power laser effect in swine cardiac mitral valves: experimental study

Introdução e Objetivo: No Brasil, procedimentos valvares representam no mínimo 30% das cirurgias cardíacas realizadas no país. Em nosso meio ainda predominam as lesões valvares decorrentes da febre reumática e doenças degenerativas. Nos pacientes reumáticos, a valva mitral é a mais acometida, predominando a estenose devido à fusão comissural e lesões no plano subvalvar; porém espessamento e retração de cúspides também são comuns, determinando dupla lesão ou insuficiência desta valva. Em processos degenerativos, as cordas tendíneas podem se apresentar alongadas. Na tentativa de utilização futura do laser como método auxiliar na correção cirúrgica dessas valvopatias, iniciamos estudo experimental com o objetivo de comparar histologicamente o tecido valvar mitral de suínos (cordas tendíneas e cúspides) submetido com e sem o laser de alta potência (LAP). Métodos: Foram estudadas cordas tendíneas e cúspides mitrais de suínos jovens e saudáveis, divididos em 6 grupos: G1-Aplicação do laser em cordas tendíneas com o tempo de 15 s; G2- Aplicação do laser em cordas tendíneas com o tempo de 20 s; G3- Aplicação do laser em cordas tendíneas com o tempo de 30 s; G4- Grupo controle relacionado a G1, G2 e G3; G5- Grupo cúspide submetido a corte com lâmina de bisturi; G6- Grupo cúspide submetido a corte com laser. As cordas tendíneas dos grupos G1, G2 e G3 receberam tratamento com laser de diodo (=980 nm, P=3,0 W) e o grupo G6 com laser de diodo em outros parâmetros (=980 nm, P=9,0 W, T=12s). Termografia e tomografia por coerência óptica foram utilizadas durante o experimento. Parte da amostra foi preservada em formaldeído e glutaraldeído para análise microscópica e a outra parte foi preservada em solução fisiológica 0,9% para análise mecânica. Resultados: Houve um decréscimo significante no comprimento das cordas tendíneas após aplicação do laser quando comparado ao comprimento inicial (p=0,005 em G1 e G2 e p=0,004 em G3). À microscopia óptica, G1, G2 e G3 apresentaram...

Introduction and Objective: In Brazil, valvar procedures represent at least 30% of cardiac surgeries performed in the country. It still predominate valvar lesions resulting from rheumatic fever and degenerative diseases. In rheumatic patients, the mitral valve is the most affected, predominantly stenosis due to commissural fusion and lesions in the subvalvar plane, however, thickening and retraction of the leaflets are also common, causing double lesion or mitral insufficiency. In degenerative process, the chordae tendineae may present elongated. In an attempt to further use of the laser as an auxiliary method in the surgical correction of valvar heart disease, we started experimental study with the purpose of comparing the histological swine tissue mitral valve (chordae tendineae and cusps) with and without high-power laser (HPL) application. Methods: Normal porcine mitral valve chordae and cups were studied and divided randomly into the following 6 groups: G1- Laser in the chordae tendineae with time of 15s; G2- Laser in the chordae tendineae with time of 20s; G3- Laser in the chordae tendineae with time of 30s; G4- Control Group; G5- Cusp group cut with a scalpel blade; G6- Cusp group cut with laser. Chordae tendineae from G1, G2 e G3 received diode laser (=980 nm, P=3,0 W) and G6 diode laser with other parameters (=980 nm, P=9,0 W, T=12s). Termography and optical coherence tomography were included during the procedure. Part of the sample was preserved in formaldehyde and glutaraldehyde for microscopic analysis and another part was stored in 0.9% saline for mechanical analysis. Results: There was a significant decrease in chordae length after laser application when compared to the initial length (p=0,005 to G1 and G2; p=0,004 to G3). In laser groups, histological analysis revealed a typical hyaline areas occupying almost the entire thickness of the chordae, when stained with Hematoxylin-Eosin (HE); dark red central areas when stained with Masson ...

Ruptura de cordoalha mitral associada a crise tireotóxica: uma avaliação por ecocardiograma 3D / Mitral chordae rupture associated with thyrotoxic crisis: an assessment by 3D echocardiography

Relatamos o caso de ruptura de cordoalha mitral em um paciente adulto jovem, com apresentação clínica de febre, novo sopro cardíaco e disfunção respiratória, condição em que a hipótese de endocardite infecciosa é mandatória. Entretanto, o curso clínico, com ausência de critérios maiores para endocardite, a presença concomitante de crise tireotóxica e uma válvula mitral sem outras alterações estrututurais ecocardiográficas, reforça a hipótese alternativa de rutura de cordoalha associada ao estado hiperdinânimo. Os achados foram apropriadamente documentados e confirmados por ecocardiograma 3D. A revisão da literatura existente descreve que, embora a presença de regurgitação mitral leve a moderada seja altamente prevalente na tireotoxicose, a ruptura mitral na ausência de outras anormalidades estruturais é um achado raro e inesperado.

We report a case of a mitral chordaea rupture in a young adult patient presenting with fever, new cardiac murmur and respiratory distress, where the hypothesis of infective endocarditis was primarily selected as the main clinical concern. However, the clinical course in the absence of major criteria for endocarditis, the concomitant presence of a thyrotoxic state and no other echocardiographic detected structural alterations, strengthened the alternative hypothesis of primary rupture due to the hyperkinetic state. These findings were thoroughly documented and confirmed by 3D-echocardiography. The literature review describes that, although mild to moderate mitral regurgitation is highly prevalent in thyrotoxicosis, mitral rupture, in the absence of a previous structural abnormality, is a rare unexpected finding.

Surgical repair of chordae tendineae rupture after degenerative valvular regurgitation using standardized bovine pericardium.

OBJECTIVE: To evaluate clinically and by Doppler Echocardiography 22 patients submitted to mitral valve repair after valvular regurgitation using standardized bovine pericardium chordae. METHODS: The patients had degenerative mitral regurgitation. Fourteen (63.6%) patients were male and the age ranged from 19 to 76 years (mean 56.8 ± 13.8 years). The strings of bovine pericardium treated with glutaraldehyde were reinforced in its transverse ends forming a trapezoid. RESULTS: One patient (4.5%) died in the immediate postoperative period with in low cardiac output syndrome and three (13.6%) in the late postoperative period. One patient (4.5%) was reoperated. The actuarial curves for survival free of death from cardiovascular causes and free from reoperation for patients who left the hospital (21), showed rates of 82.0 ± 9.8% and 83.9 ± 10.4% at 70 months postoperatively, respectively. Seventeen patients (77.3%) are alive with native valves. Of the 17 patients alive with native valves 16 (94.1%) were in functional class I. The Doppler Echocardiography postoperatively (mean 41 months, 4-70 months), showed no mitral regurgitation in 11 (64.7%) patients and mild regurgitation in five (29.4%). CONCLUSION: The technique of standard cords of bovine pericardium implantation to replace chordae tendineae of the mitral valve in patients with degenerative mitral regurgitation showed satisfactory results.

Correção da ruptura de cordas tendíneas na insuficiência mitral degenerativa pelo emprego de cordas padronizadas de pericárdio bovino / Surgical repair of chordae tendineae rupture after degenerative valvular regurgitation using standardized bovine pericardium

OBJETIVO: Avaliar, clinicamente e pelo ecodopplercardiograma, o funcionamento da valva mitral em 22 pacientes submetidos à correção do refluxo valvar com substituição das cordas tendíneas nativas por cordas padronizadas de pericárdio bovino. MÉTODOS: Os pacientes apresentavam insuficiência mitral degenerativa. Quatorze (63,6%) pacientes eram do gênero masculino e a idade variou de 19 a 76 anos (média 56,8±13,8 anos). As cordas de pericárdio bovino foram tratadas com glutaraldeído, com reforço de suas extremidades transversais formando um trapézio. RESULTADOS: Um (4,5%) paciente faleceu no pós-operatório imediato em síndrome de baixo débito cardíaco e três (13,6%) no pós-operatório tardio. Uma (4,5%) paciente foi reoperada. As curvas atuariais de sobrevivência livre de óbitos por causa cardiovascular e livres de reoperações para os pacientes que deixaram o hospital (21) demonstraram taxas de 82,0±9,8% e 83,9±10,4%, aos 70 meses de pós-operatório, respectivamente. Dezessete (77,3%) pacientes estão vivos com a própria valva. Dos 17 pacientes vivos com a própria valva 16 (94,1%) estão em classe funcional I. O ecodoppler pós-operatório (média de 41 meses; 4 a 70 meses) demonstrou ausência de regurgitação mitral em 11 (64,7%) pacientes e regurgitação discreta em cinco (29,4%). CONCLUSÃO: A técnica de implante de cordas padronizadas de pericárdio bovino para substituição de cordas tendíneas da valva mitral em pacientes com insuficiência mitral degenerativa demonstrou resultados bastante satisfatórios.

OBJECTIVE: To evaluate clinically and by Doppler Echocardiography 22 patients submitted to mitral valve repair after valvular regurgitation using standardized bovine pericardium chordae. METHODS: The patients had degenerative mitral regurgitation. Fourteen (63.6%) patients were male and the age ranged from 19 to 76 years (mean 56.8 ± 13.8 years). The strings of bovine pericardium treated with glutaraldehyde were reinforced in its transverse ends forming a trapezoid. RESULTS: One patient (4.5%) died in the immediate postoperative period with in low cardiac output syndrome and three (13.6%) in the late postoperative period. One patient (4.5%) was reoperated. The actuarial curves for survival free of death from cardiovascular causes and free from reoperation for patients who left the hospital (21), showed rates of 82.0 ± 9.8% and 83.9 ± 10.4% at 70 months postoperatively, respectively. Seventeen patients (77.3%) are alive with native valves. Of the 17 patients alive with native valves 16 (94.1%) were in functional class I. The Doppler Echocardiography postoperatively (mean 41 months, 4-70 months), showed no mitral regurgitation in 11 (64.7%) patients and mild regurgitation in five (29.4%). CONCLUSION: The technique of standard cords of bovine pericardium implantation to replace chordae tendineae of the mitral valve in patients with degenerative mitral regurgitation showed satisfactory results.

Complete versus partial preservation of mitral valve apparatus during mitral valve replacement: meta-analysis and meta-regression of 1535 patients.

OBJECTIVES: To determine if there is any real difference between complete preservation (CP) and partial preservation (PP) of the mitral valve apparatus during mitral valve replacement (MVR) in terms of hard outcomes. METHODS: MEDLINE, EMBASE, CENTRAL/CCTR, SciELO, LILACS, Google Scholar and reference lists of relevant articles were searched for clinical studies that compared outcomes [30-day mortality, postoperative low cardiac output syndrome (LCOS), 5-year mortality or left ventricle ejection fraction (LVEF) before and after surgery] between MVR-CP vs MVR-PP during MVR until July 2012. The principal summary measures were odds ratios (ORs) with 95% confidence interval (CI)--for categorical variables (30-day mortality, postoperative LCOS, 5-year mortality); difference means and standard error (SE)--for continuous variables (LVEF before and after surgery) and P values (that will be considered statistically significant when <0.05). The ORs were combined across studies using DerSimonian-Laird random effects weighted model. The same procedure was executed for continuous variables, taking into consideration the difference in means. RESULTS: Eight studies (2 randomized and 6 non-randomized) were identified and included a total of 1535 patients (597 for MVR-CP and 938 for MVR-PP). There was no significant difference between MVR-CP or MVR-PP groups in the risk for 30-day mortality (OR 0.870; 95% CI 0.50-1.52; P = 0.63) or postoperative LCOS (OR 0.35; 95% CI 0.11-1.08 and P = 0.07) or 5-year mortality (OR 0.70; 95% CI 0.43-1.14; P = 0.15). Taking into consideration LVEF, neither MVR-CP nor MVR-CP demonstrated a statistically significant improvement in LVEF before and after surgery, and both strategies were not different from each other. No publication bias was observed. CONCLUSIONS: We found evidence that argues against any superiority between both techniques of preservation (complete or partial) of mitral valve apparatus during MVR.

Anomalous chordae tendineae associated with mitral valve dysplasia in a cat

A rare case of anomalous chordae tendineae associated with mitral valve dysplasia was observed during necropsy in a 5- year-old male Persian cat with a history of sudden death. Grossly, there was thickening of the mitral valve, which was connected to numerous, short and thickened chordae tendineae that were ectopically inserted into the myocardium. In addition, marked left atrial dilation and pulmonary congestion and edema were present.(AU)

Anomalous chordae tendineae associated with mitral valve dysplasia in a cat

A rare case of anomalous chordae tendineae associated with mitral valve dysplasia was observed during necropsy in a 5- year-old male Persian cat with a history of sudden death. Grossly, there was thickening of the mitral valve, which was connected to numerous, short and thickened chordae tendineae that were ectopically inserted into the myocardium. In addition, marked left atrial dilation and pulmonary congestion and edema were present.

Conservative surgical management of mitral insufficiency: an alternative approach.

Mitral valve insufficiency is frequently the result of elongated or ruptured chordae tendineae. Several techniques have been described for its correction. However, when there is a severe elongation or rupture of the chordae, the most widely accepted treatment option has been valve replacement. The best long-term outcomes observed in conservative surgeries led us to choose this procedure rather than the correction of mitral valve insufficiency. We described three techniques for correction of mitral prolapse due to elongated and/or ruptured chordae tendineae. In addition, we developed mold pre built bovine pericardial chords (Braile-Gregori prosthesis) for chordae replacement. Finally, since 1987, the rigid prosthetic semicircular ring (Gregori-Braile ring) has been consistently used in our centre for correction of the posterior dilation of mitral annulus preferably in its portion close to the posteromedial commissure.

Conservative surgical management of mitral insufficiency: an alternative approach / Tratamento cirúrgico conservador da insuficiência mitral: uma abordagem alternativa

Mitral valve insufficiency is frequently the result of elongated or ruptured chordae tendineae. Several techniques have been described for its correction. However, when there is a severe elongation or rupture of the chordae, the most widely accepted treatment option has been valve replacement. The best long-term outcomes observed in conservative surgeries led us to choose this procedure rather than the correction of mitral valve insufficiency. We described three techniques for correction of mitral prolapse due to elongated and/or ruptured chordae tendineae. In addition, we developed mold pre built bovine pericardial chords (Braile-Gregori prosthesis) for chordae replacement. Finally, since 1987, the rigid prosthetic semicircular ring (Gregori-Braile ring) has been consistently used in our centre for correction of the posterior dilation of mitral annulus preferably in its portion close to the posteromedial commissure.

A insuficiência mitral é frequentemente resultado do alongamento ou ruptura das cordas tendíneas. Várias técnicas foram descritas para sua correção. Entretanto, quando o comprometimento das cordas é mais intenso, a substituição valvar tem sido mais usualmente empregada. Os bons resultados a longo prazo observados na cirurgia conservadora têm nos levado a eleger esse procedimento como prioridade. Três técnicas foram por nós desenvolvidas para correção de alongamento e/ou ruptura de cordas tendíneas. Além disso, desenvolvemos uma prótese de pericárdio bovino para a substituição de cordas (prótese Braile-Gregori). Finalmente, desde 1987 empregamos o anel rígido semicircular (anel Gregori-Braile) para a correção do alongamento posterior do anel mitral, com ênfase na sua porção junto à comissura póstero-medial.

Typification the tendinous cords of the left valve complex of sheep hearts

Forty Santa Ines sheep (Ovis aries Lin.) hearts were examined with the purpose of studying the present types of chordae  tendineae in the left valve complex. The study was performed at the State University of North Fluminense Darcy Ribeiro, Morphology sector of the Agricultural Science Animal Health Center Laboratory. Hearts were initially kept in 10% formalin solution and later dissected to undergo removal and plannifcation of left atrioventricular complex. With the removal of the left atrioventricular complex began the observation and characterization of the cord. After the analysis we observed the presence of eight types of cuspidal chordae tendineae present in the left valve complex, they were classifed as type I, II, III, IV, V, VI, VII and commissural kind.

Study of the cardiac left atrioventricular valvar complex in water buffaloes (Bubalus bubalis) of the Jafarabadi breed / Estudo do complexo valvar atrioventricular cardíaco esquerdo em búfalos (Bubalus bubalis) da raça Jafarabadi

Atrioventricular valve complex of 30 Jafarabadi water buffaloes, adult males were studied in this research with no heart diseases. The animals were obtained from a slaughterhouse in Brazilian State of Parana. The hearts were opened at the third portion affording access to the valve complex. The complexes had its area, number and type of tendinous cords submitted to analysis. The results showed that the complex is composed by two cusps and four accessory cusps, two or three papillary muscles in which 10-25 tendinous cords fix on the cusps that face the ventricle wall. The total area of the complex was on average 38.56cm², with a minimum of 24.96cm² and a maximum of 55.54cm². Statistically, no relation between the number of cords and the cusps' area where they are inserted or with the number of papillary muscle where they originated from was observed.(AU)

Foram estudados os complexos valvares atrioventricular esquerdo de 30 búfalos da raça Jafarabadi, machos e adultos, sem alterações cardíacas, provenientes de abatedouros do Estado do Paraná. Os corações foram examinados em seu terço médio para acesso ao complexo valvar, que foi submetido a estudos de área, número e tipificação de cordas tendíneas. Os resultados demonstram que este complexo é formado por duas cúspides principais e quatro cúspides acessórias, apresentam em sua formação de 2-3 músculos papilares, nos quais se inserem de 10-25 cordas tendíneas, que se fixam em cúspides voltadas para a parede do ventrículo. A área total deste complexo apresenta uma média de 38,56cm² com um mínimo de 24,96cm² e um máximo de 55,54cm². Estatisticamente não há relação entre número de cordas e a área da cúspide onde estas estão inseridas, nem com o número de músculos papilares dos quais elas provem.(AU)