Rapidly progressive malignant fibrous histiocytoma of right atrium: a rare case report

Abstract We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

Infantile Marfan syndrome in a Korean tertiary referral center / 소아과

PURPOSE: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. METHODS: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. RESULTS: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. CONCLUSION: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

Surgical treatment of flail leaflet of tricuspid valve / 中华外科杂志

<p><b>OBJECTIVE</b>To explore the approach and the result in tricuspid valve insufficiency treatment by a cusp remodeling technique.</p><p><b>METHODS</b>Nine patients with severe tricuspid regurgitation, congenital lack of chordae in 6 cases and traumatic rupture of chordae in 3 cases, underwent surgical repair between April 1997 and March 2006. There were six male and three female. Their ages ranged from 8 years to 57 years. One or two segments of flail leaflets were presented in these patients. Valve repair was performed by suture of the free edge of the affected cusp segment, plication of the segment of annulus devoid of leaflet, and fixation of the neo-annulus with a flexible annuloplasty ring.</p><p><b>RESULTS</b>All patients survived and recovered after the operation. Echocardiography showed good coaptation with no regurgitation of the tricuspid valve in six patients and a mild residual tricuspid regurgitation in three. A remarkable decrease in the diameter of the right ventricle (anterior to posterior) was observed: from mean (43.6 +/- 4.2) mm (range 29 mm to 64 mm) preoperatively reducing to mean (24.0 +/- 1.8) mm (range 16 mm to 32 mm) postoperatively. All patients are doing well in 1 month to 109 months follow up.</p><p><b>CONCLUSION</b>The procedure provided a simple and valuable option for repair of flail leaflet of tricuspid valve caused by congenital lack of chordae or traumatic rupture of chordae.</p>

Endocardite por Staphylococcus aureus no puerpério de portadora de prolapso de valvas mitral e tricúspide / Staphylococcus aureus endocarditis in a puerperal woman with mitral and tricuspid valves prolapse

Mulher de 17 anos com prolapso de valvas mitral e tricúspide com sinais de degeneraçäo mixomatosa apresentou infecçäo puerperal por Staphylococcus aureus, com sepse e múltiplas embolias sépticas (órbita e globo ocular direitos, polegar esquerdo, baço, flegmäo em gastrocnêmio esquerdo), resultando histerectomia total dno 10§ dia pós-parto e enucleaçäo do globo ocular direito no 16§. Foram diagnosticadas endocardite infecciosa e insuficiência mitral aguda e realizada substituiçäo mitral no 13§ dia pós-parto, ocorrendo bloqueio átrio-ventricular total (BAVT) - necessitando de utilizaçäo de estimulaçäo artificial temporária - no 14§ dia pós-parto, bem como endocardite e insuficiência na tricúspide no 46§ dia pós-parto. A paciente recebeu alta no 62§ dia pós-parto em boas condiçöes clínicas, após término da antibioticoterapia

A 17-year-old woman with mitral and tricuspid valve prolapse and mizomatous degeneration presented puerperal infection by Staphylococcus aureus with clinical picture of sepsis and multiple septic embolism (right eye, left thumb, spleen, and left calf). She underwent total hysterectomy on the 10th day postdelivery and right eye enucleation on the 16th. Temporary total AV block occurred on the 14th day with temporary external pacing during the next couple of days. Acute endocarditis with acute mitral regurgitation was diagnosed on the 13th day, demanding immediate valve replacement. On the 46th day she developed moderate tricuspid valve regurgitation due to another episode of endocarditis. Final clinical discharge took place on the 62nd day after antibiotic therapy completion.

Prevalência de prolapso valvar tricúspide e aórtico em portadores de prolapso valvar mitral / Prevalence of tricuspid and aortic valve prolapse in patients with mitral valve prolapse

Foram estudados, através da ecocardiografia bidimensional, 30 pacientes portadores de prolapso da valva mitral, sendo 15 do sexo feminino e 15 masculino, com idade média de 33,3 anos, com o objetivo de observar a prevalência de concomitante prolapso valvar tricúspide e aórtico. O prolapso da valva tricúspide foi obsrvado em 43,3% dos pacientes (13/30 anos, sendo que o folheto anterior e septal estava acometido em 92,3% e o posterior em 15,3% dos casos. A prevalência de prolapso da valva aórtica foi de 10% dos casos (3/30), estando ambos os folhetos avaliados - coronariano direito e näo coronariano - acometidos. Todos os pacientes com prolapso valvar aórtico apresentavam acometimento dos dois folhetos mitrais e de, no mínimo, dois folhetos tricuspídeos. Conclui-se que é comum o acometimento simultâneo das valvas tricúspide e aórtica em pacientes portadores de prolapso da valva mitral

Tricuspid valve perforation in a child with ventriculoatrial shunt

Ventriculoperitoneal and ventriculoatrial shunting methods are used for the surgical treatment of hydrocephalus. However, many years or months after the treatment, complications can develop. In this article, we report on a patient with perforation of the tricuspid valve and tricuspid insufficiency, which developed as a complication of the ventriculoatrial shunt. This is a previously unreported complication in pediatric patients with a ventriculoatrial shunt. Because of fairly common and life-threatening cardiac complications, ventriculoatrial shunted patients should be monitored closely by echocardiography