Constrictive pericarditis with extensive calcification and caseous necrosis

Abstract Constrictive pericarditis is a disease where loss of pericardial elasticity and restriction of filling of the cardiac chambers occurs. It is most often seen as an associated symptom of heart failure. Pericardiectomy provides effective treatment for patients with symptomatic constrictive pericarditis, although high rates of morbidity and mortality are related to the procedure. We present a case with extensive calcification, massive caseous necrosis and an important impairment of right ventricular function successfully operated in our institution.

Polisserosite de Concato: uma doença rara ou pouco conhecida? / Concato's disease: a rare or a little known condition?

Polisserosite consiste na inflamação das serosas (pericárdio, pleurae peritônio) com efusão. Na polisserosite de Concato, além das manifestações inflamatórias das serosas, ocorre pericardite com espessamento dos folhetos do pericárdio, podendo levar a um quadro de pericardite constritiva. Justifica-se este estudo ao considerarmos que a etiologia das serosites são muitas; por isso, há necessidade de melhor estudá-las e difundi-las no meio acadêmico. Nesse sentido, propomo-nos a rever a literatura, por meio de levantamento bibliográfico, de pesquisa em rede e literatura atualizada sobre o tema abordado. Deste modo, pretendemos melhor elucidar a etiopatogenia da doença de Concato, dada a importância do diagnóstico precoce, combinado ao tratamento adequado, na prevenção de consequências indesejáveis e fatais. O principal objetivo desta revisão é meramente acadêmico, pois há um desconhecimento muito grande desta forma de apresentação de tal doença no meio médico. Acreditamos que, com este estudo de revisão, poderemos contribuir para a conscientização e efetivação do diagnóstico precoce da patologia estudada, evitando complicações e, assim, promover: saúde, multiplicação e difusão do conhecimento adquirido.

Polyserositis is the inflammation of serous membranes (pericardium, pleura and peritoneum) with effusion. In Concato’s disease, in addition to chronic inflammatory manifestations of serous membranes, there occurs pericarditis with thickening of pericardium leasflets, which may lead to a picture of constrictive pericarditis. This study is justified by the multiple etiological factors that may lead to serosites, and the need to further study and publish study results. In this sense, a review was proposed by making a literature survey using network research and current literature available on the topic for the purpose of elucidating the etiopathogenesis of Concato’s disease, given the importance of an early diagnosis, in association with the appropriate treatment, in the prevention of undesirable or even fatal consequences. The aim of this review is academic, inasmuch as there is lack of medical knowledge on the manifestations of this condition. By doing this, we aim to contribute to developing an awareness of the importance of an early diagnosis of this pathology, thus avoiding complications and promoting health and the propagation of acquired knowledge.

Pericarditis constrictiva como presentación grave e infrecuente de lupus eritematoso sistémico: reporte de un caso y revisión de la literatura / Constrictive pericarditis as a serious and rare presentation of systemic lupus erythematosus: Report of one case

Constrictive Pericarditis (CP) is an unusual disease. Its most common causes are idiopathic or secondary to cardiac surgery. Less frequently it is caused by connective tissue diseases. We report a 30 years old woman hospitalized due to progressive dyspnea, chest pain and signs of right sided heart failure. During her stay, a Systemic Lupus Erythematosus (SLE) was diagnosed. The echocardiogram suggested a CP and the diagnosis was confirmed by cardiac catheterization. Pericardiectomy was successfully performed. The biopsy confirmed a nonspecific chronic pericarditis, with extensive fibrosis and absence of caseating granulomas. The patient had a satisfactory recovery.

Pyopericardium due to infection with Mycobacterium tuberculosis - A rare case report.

Summary: Pyopericardium or purulent pericarditis is a rare entity but usually associated with a high mortality. We report a case of 30-year-old male presenting with pyopericardium due to Mycobacterium tuberculosis. The patient was treated with Anti-tubercular therapy (ATT) alongwith pericardiocentesis and pericardiectomy. The patient responded well to treatment and recovered completely in due course of time.

Revisión y experiencia en cinco casos de pericardiectomía extensa / Review and experience in five cases of extensive pericardiectomy

Objetivo: describir evolución de casos tratados por pericarditis constrictiva. Métodos: se presentan 5 casos tratados por orden cronológico por pericarditis constrictiva en los hospitales Amalia Simoni de Camagüey (4) y Martín Chang Puga de Nuevitas (1), en 5 años (2004-2008), cuya indicación quirúrgica principal fue la evidencia clínica de taponamiento cardiaco. Resultados: la edad promedio fue 49 años (rango 36-68), 4 pacientes del sexo masculino, en 4 el derrame fue serohemático y otro purulento. En un paciente apareció el corazón en coraza con calcificaciones. La etiología fue postraumática, infección aguda y metástasis pericárdica en un caso cada una. En 2 pacientes no se confirmó la tuberculosis pericárdica extrapulmonar, aunque pudo corresponderse. El acceso al tórax mayormente fue por toracotomía anterior izquierda, y el proceder fue la pericardiectomía extensa, previa punción pericárdica. De los 5 casos, 3 pacientes egresaron vivos y 2 fallecidos. Conclusiones: los pacientes con pericarditis constrictiva, derrame y signos de taponamiento cardiaco deben ser tratados con pericardiocentesis, y la pericardiectomía extensa, cuando esté indicada. Se considera una buena opción terapéutica(AU)

Objective: to describe the evolution of the cases treated by constrictive pericarditis. Methods: a retrospective study was performed in five cases treated by chronological order due to constrictive pericarditis in the hospitals Amalia Simoni of Camagüey province (4) and "Martin Chang Puga" of Nuevitas municipality (1) in 5 years (2004-2008) whose main surgical indication was the clinical evidence of cardiac tamponage. Results: mean age was of 49 years (range 36-68), four male patients in four of the leakage was serohematinic and another purulent type. In a patient there was a barrier heart with calcifications. Etiology was post-traumatic, acute infection and pericardial metastasis in a case each. In two patients extrapulmonary pericardial tuberculosis was not confirmed, although it could correspond. The thorax approach was mainly by left anterior thoracotomy and the procedure was an extensive pericardiectomy, previous pericardial puncture. From 5 cases, three patients were discharged alive and two dyed. Conclusions: patients presenting with constrictive pericarditis, leakage and signs of cardiac tamponage must to be treated with pericardiocentesis and the extensive pericardiectomy, when be prescribed. This is a good therapeutic option(AU)

Pericardite constritiva secundária a trauma ocorrido há 20 anos / Constrictive pericarditis secondary to thoracic trauma occuring 20 years previously

Pericardite constritiva resulta de um espessamento fibroso do pericárdio, secundária à inflamação crônica causada por uma variedade de doenças, levando a progressivos sinais e sintomas de falência cardíaca direita e esquerda. Relata-se um caso em que depois de excluídas causas comuns, foi identificado trauma torácico ocorrido 20 anos antes dos sintomas de insuficiência cardíaca, chamando a atenção para o tempo transcorrido e também justificando a imagem da calcificação evidente à radiografia de tórax.

Constrictive pericarditis results from a fibrous thickening of the pericardium secondary to chronic inflammation caused by a variety of diseases, leadingto progressive signs and symptoms of right and left heart failure. This case study reports on thoracic trauma identified, after excluding common causes, as occurring twenty years prior to the symptoms of heart failure, drawing attention to this time lapse and also justifying the chest radiography image with visible calcification.

Clínica y seguimiento de la pericarditis constrictiva crónica / Clinical finding and follow up of chronic constrictive pericarditis

El objetivo de este estudio fue describir la etiología, presentación clínica, métodos diagnósticos, tratamiento, evolución y pronóstico alejado de 35 pacientes con pericarditis constrictiva crónica que fueron evaluados prospectivamente de acuerdo a un protocolo de enfermedad pericárdica que se aplica en nuestra Institución. En 24 (68%) la pericarditis constrictiva fue idiopática y 11 (32%) presentaron etiología definida, realizándose pericardiectomía en 34 (97%). Hubo 4 (12%) muertes intrahospitalarias, todas con etiología definida. La mediana de seguimiento fue de 5.6 años (percentilos 25-75: 2.4-7.4 años). La supervivencia de acuerdo al método de Kaplan-Meier fue de 97% al año (IC 95% 80 - 99%), 83% a los 5 años (IC 95% 65 - 93%), 78% a los 7 años (IC 95% 60 - 90%), y 69% a los 10 años (IC 95% - 50% - 84%). En conclusión, la pericarditis constrictiva crónica es una enfermedad generalmente idiopática, de diagnóstico tardío, cuyo tratamiento es la pericardiectomía. Los pacientes con etiología específica presentaron mayor mortalidad intrahospitalaria. En el seguimiento a 10 años 2/3 de los pacientes están vivos y con mejoría de los síntomas.

The aim of this study was to describe the etiology, clinical findings, diagnostic methods, treatment, outcome and long-term prognosis of 35 patients with chronic constrictive pericarditis (CCP) that were prospectively analyzed according to a pericardial disease protocol performed in our Institution. Etiology of CCP was idiopathic in 24 patients (68%), and specific in 11 (32%). The majority (34 patients, 97%) underwent pericardiectomy. Perioperative mortality was 12% (4/33) no deaths were registered among patients with idiopathic CCP. Median follow-up was 5.6 years (percentile 25-75: 2.4-7.4 years). The cumulative actuarial survival probability was 97% at 1 year (confidence interval [CI] 80% to 99%); 83% at 5 years, (95% CI 65% to 93%); 78% at 7 years, (95% CI 60% to 90%), and 69% at 10 years (95% CI 50% to 84%). In conclusion, nowadays CCP is generally an idiopathic disease with late diagnosis. The clinical course of the disease produces severe symptoms of congestive heart failure. In a 10 years follow-up 2/3 of patients are alive and improved their quality of life. Idiopathic form of pericarditis did not show mortality during early postoperative period.

[Chronic constrictive pericarditis in a child]

The chronic constrictive pericarditis is a very rare disease in child. We report a case of chronic constrictive pericarditis with indeterminate etiology in an 8-year old girl. The child undergone pericardiectomy with well improvement. Through our case and review of the literature, we study the frequency of chronic constrictive pericarditis in child, its etiology and prognosis

Clinical Characteristics of Constrictive Pericarditis Diagnosed by Echo-Doppler Technique in Korea

A retrospective analysis of clinical data of 71 patients with constrictive pericarditis (CP) diagnosed by echo-Doppler technique (mean age, 49 +/- 17) was done. In 27 patients (38%), the etiology was unknown, and the three most frequent identifiable causes were tuberculosis (23/71, 32%), cardiac surgery (8/71, 11%), and mediastinal irradiation (6/71, 9%). Pericardiectomy was performed in 35 patients (49%) with a surgical mortality of 6% (2/35), and 11 patients (15%, 11/ 71) showed complete resolution of constrictive physiology with medical treatment. Patients with transient CP were characterized by absence of pericardial calcification, shorter symptom duration, and higher incidence of fever, weight loss, and tuberculosis. The 5-yr survival rates of patients with transient CP and those undergoing pericardiectomy were 100% and 85 +/- 6%, respectively, which were significantly higher than that of patients without undergoing pericardiectomy (33 +/- 17%, p=0.0083). Mediastinal irradiation, higher functional class, low voltage in ECG, low serum albumin, and old age were the independent variables associated with a higher mortality. Tuberculosis is still the most important etio-logy of CP in Korea, and not infrequently, it may cause transient CP. Early diagnosis and decision-making using follow-up echocardiography are crucial to improve the prognosis of patients with CP.

Pericardite constritiva em lactente / Constrictive pericarditis in a 19-month-old child

We report a case of chronic nonspecific constrictive pericarditis with myocardial involvement in a 19-month-old infant. The patient underwent total pericardiectomy and had irreversible hemodynamic instability. Constrictive pericarditis is rare in childhood. It may follow several processes, most frequently an infectious disease. The natural course of the disease consists of progressive myocardial dysfunction with atrioventricular valvular involvement. When diagnosis is established early in the course of the disease and treatment started immediately, the evolution is favorable in most instances.

Angiosarcoma del corazón y su ruptura espontánea. Rara causa de pericarditis efusivo-constrictiva. Presentación de un caso y revisión de la literatura / Angiosarcoma of the heart and its spontaneous rupture. A rare cause of effusive-constrictive pericarditis. A case report and review of the literature

The authors present the clinical history of a male 44 year old patient who was hospitalized with the diagnosis of pericardial constriction and effusion and operated on as an emergency because of spontaneous cardiac rupture and was found to have a cardiac adenosarcoma. They review the literature in order to discuss a very rare cause of hemopericardium and constrictive pericarditis with epidemiologic, diagnostic, therapeutic and prognostic commentaries and secondly, to try to establish if this type case does not represent a diagnostic problem for the ecocardiographer since an angiosarcoma that occupies the pericardial space can be confused with a hemopericardium. They also mention other imaging studies that are used to better characterize and diagnose these tumors

Pericardite constrictive apres pontageaorto-coronarien a propos d'un cas

We report a case of pericarditis following aorto coronary by-pass surgery which gradually developed into chronic constrictive pericarditis. The disease was a long time ignored because symptoms are not specific. In our case the clinical presentation looked like a cirrhosis especially that our patient developed an hepatitis after blood transfusion. In this occasion criteria of diagnosis are reminded. Hemodynamic investigation is still the best method of diagnosis and the only treatment is surgical and consists of pericardial decortication

Pericardite constritiva: tratamento cirúrgico / Constrictive pericarditis: surgical treatment

A pericardite é caracterizada pela fibrose do pericárdio, o qual é espesso e aderente, restringindo o enchimento diatólico do coraçäo. O tratamento é quadro sempre cirúrgico. Foram estudados 13 pacientes submetidos a pericardiectomia no Instituto do Coraçäo em Juiz de Fora, MG. Foi estabelecida a etiologia em 3 casos de tuberculose, 3 pós cirurgia cardíaca, 1 pós cirurgia renal e em seis näo foi possível estabelecer o fator/etiológico. A dispnéia foi o sintoma mais freqüente. A radiografia revelou aumento da área cardiaca em 6 pacientes, calcificaçäo em 5 e derrame pleural em 6. Seis foram submetidos a ecocardiograma que revelou restriçäo e espessamento pericárdico. O cateterismo cardíaco e a angiografia foram realizados em todos os casos, confirmando o diagnóstico e demonstrando a igualdade das pressöes diastólicas. O tratamento cirúrgico foi através de esternotomia mediana com a máquina de circulaçäo extracorpórea em regime de espera, e usada em 3 ocasiöes. Três paciente faleceram, 2 com miocardiopatia associada a síndrome de baixo débito cardíaco, 1 devido a hemorragia, septicemia e insuficiência respiratória. Os demais apresentaram boa evoluçäo com melhoria significativa da classe funcional

Pancardite com pericardite constritiva pós cirúrgia cardíaca: relato de caso / Pancarditis with constrictive pericarditis after cardiac surgery: a case report

É relatado caso de pancardite com pericardite constritiva, ocorrido no pós-operatório de cirurgía cardíaca. O paciente, submetido à correçäo de estenose pulmonar infundibular, apresentou, no pós-operatório imediato, sinais de insuficiência cardíaca direita, atribuída à ventriculotomia. No pós-operatório tardio, como persiste com o mesmo quadro, foi reavaliado clinicamente e por meio de ECG, RX e ecocardiograma, sendo diagnosticada pericardite constritiva. O paciente foi submetido a pericardiectomia total e evoluiu com quadro de sindrome de baixo débito. Foi feito novo estudo hemodinâmico que evidenciou elevaçäo da pressäo diastólica final e severa hipocinesia dos ventrículos. Com este quadro conclui-se pela presença de um processo de pancardite, com comprometimento também do miocárdio, de etiologia provavelmente auto-imune. Foi, entäo instituído tratamento com corticosteróides e o paciente apresentou melhoria rápida e evoluiu bem desde entäo. Trata-se de uma rara complicaçäo que deve ser lembrada porque em uma fase precose pode apresentar boa resposta aos corticóides e evitar a cirurgia de ressecçäo do pericárdio

Cuarenta años de experiencia en el tratamiento quirúrgico de la pericarditis constructiva / Forty years experience in the treatment of constructive pericarditis

Con el fin de conocer los resultados tempranos y tardíos de la pericardiectomía en el tratamiento de la pericarditis constrictiva, en el Instituo Nacional de Cardiología Ignaci Chávez de México, revisamos los expedientes de 58 enfermos, que se sometieron a esta investigación entre 1947 y 1986. Los factores etiológiocos más frecuentes fueron la tuberculosis y el origen idiopático (en 68.3% y 24.1% respectivamente). Antes de la intervención quirúrgica el 3.4% de los pacientes estaban en clase funcional I de la New York Heart Association, 31% en clase II, 48,3% en clase III y 16.2% en clase IV. La mortalidad operativa global fue del 6.89% y en la última década fue del 0%. La complicación operatoria más frecuente fue el bajo gasto, que ocurrió en el 15.5% de los pacientes. El desgarro accidental de la aurícula derecha ocurrió en el 8.6% de los casos. El tiempo de seguimiento fue de 5.6 ñ 6.3 años (con un máximo de 25.6 años). La mortalidad por paciente y año fue del 2.04%. La supervivencia a los cinco y diez años, excluida la mortalidad operatoria fue de 82% y 71% respectivamente. Después de la pericardiectomía el 76% de los enfermos estaba en clase funcional I de la New York Heart Association (p <0.001), el 16% en clase II (p <0.001), el 8% en clase III (p <0.001) y el 0% en clase IV (p <0.05). No hubo correlación entre la clase IV funcional preoperatoria ni la duración de los síntomas antes del diagnóstico y la mortalidad temprana y tardía, y la clase funcional postoperatoria. Se concluye que la pericardiectomía es el tratamiento de elección en la pericarditis constrictiva sintomática, al producir una importante y duradera mejoría en los síntomas y en la capacidad funcional, con una baja mortalidad operatoria, sobre todo en la última década

Pericardite constritiva como complicaçäo de doença granulomatosa crônica na infância / Constrictive pericarditis as a complication of chronic granulomatous disease in infancy

Apresenta-se um paciente de 11 meses de idade, do sexo masculino, acompanhado na Unidade de Imunopatologia do infstituto da Criança "Prof. Pedro de Alcantara" do Hospital das Clínicas da Faculdade de Medicina da Universidade de Paulo, portador de doença granulomatosa crônica da infância, que evoluiu com pericardite constrictiva, manifestaçäo rara nesta imunodeficiência. Discutem-se alguns aspectos desta entidade, dando ênfase ao diagnóstico precoce e à terapêutica de suporte