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.

Hepatomegalia como forma de presentación en pericarditis constrictiva: Caso clínico pediátrico / Hepatomegaly as a form of presentation in constrictive pericarditis: A pediatric clinical case

La pericarditis constrictiva es una entidad poco frecuente en pediatría, en la cual existe una limitación para la diástole cardíaca por fibrosis del pericardio. El origen etiopatogénico de esta patología es múltiple, encontrándose en primer lugar, la pericarditis constrictiva idiopática y, en segundo, la infección por Mycobacterium tuberculosis. El diagnóstico constituye un desafío clínico, ya que requiere de un alto grado de sospecha. Suele presentarse de forma oligosintomática. La presencia de edema, ascitis y alteración de la función hepática suele orientar el estudio hacia una enfermedad hepática primaria. Una cuidadosa historia clínica y examen físico, junto con estudios por imágenes adecuados, constituyen las piedras angulares del diagnóstico. El tratamiento quirúrgico realizado de forma oportuna resulta curativo en la gran mayoría de los pacientes. Se presenta el caso de un paciente de 16 años que inicia estudios por hallazgo de hepatomegalia asociada a disnea grado 1-2 en un control de salud habitual.

Constrictive pericarditis is a rare entity in pediatrics in which there is a limitation for cardiac diastole due to fibrosis of the pericardium. The etiopathogenic origin of this pathology is multiple, finding idiopathic constrictive pericarditis firstly and Mycobacterium tuberculosis infection secondly. Diagnosis is a clinical challenge since it requires a high degree of suspicion. It usually presents as oligosymptomatic or with signs and symptoms of low cardiac output. The presence of edema, ascites and impaired liver function usually guides the study towards primary liver disease. A careful clinical history and physical examination together with adequate imaging studies are the cornerstones of the diagnosis. Surgical treatment is curative in the vast majority of patients. We present the case of a 16-year-old patient with hepatomegaly and dyspnea grade 1-2 found in a routine health check-up.

Importance of Clinical and Laboratory Findings in the Diagnosis and Surgical Prognosis of Patients with Constrictive Pericarditis / Importância dos Achados Clínicos e Laboratoriais no Diagnóstico e Prognóstico Cirúrgico de Pacientes com Pericardite Constritiva

Abstract Background: International studies have reported the value of the clinical profile and laboratory findings in the diagnosis of constrictive pericarditis. However, Brazilian population data are scarce. Objective: To assess the clinical characteristics, sensitivity of imaging tests and factors related to the death of patients with constrictive pericarditis undergoing pericardiectomy. Methods: Patients with constrictive pericarditis surgically confirmed were retrospectively assessed regarding their clinical and laboratory variables. Two methods were used: transthoracic echocardiography and cardiac magnetic resonance imaging. Mortality predictors were determined by use of univariate analysis with Cox proportional hazards model and hazard ratio. All tests were two-tailed, and an alpha error ≤ 5% was considered statically significant. Results: We studied 84 patients (mean age, 44 ± 17.9 years; 67% male). Signs and symptoms of predominantly right heart failure were present with jugular venous distention, edema and ascites in 89%, 89% and 62% of the cases, respectively. Idiopathic etiology was present in 69.1%, followed by tuberculosis (21%). Despite the advanced heart failure degree, low BNP levels (median, 157 pg/mL) were found. The diagnostic sensitivities for constriction of echocardiography and magnetic resonance imaging were 53.6% and 95.9%, respectively. There were 9 deaths (10.7%), and the risk factors were: anemia, BNP and C reactive protein levels, pulmonary hypertension >55 mm Hg, and atrial fibrillation. Conclusions: Magnetic resonance imaging had better diagnostic sensitivity. Clinical, laboratory and imaging markers were associated with death.

Resumo Fundamento: Estudos internacionais têm relatado o valor de perfil clínico e exames de imagem no diagnóstico e prognóstico da pericardite constritiva. Entretanto, dados da população brasileira são escassos. Objetivo: Avaliar as características clínicas, sensibilidade de exames de imagem e fatores relacionados ao óbito em uma série de casos de pericardite constritiva submetidos à pericardiectomia. Métodos: Pacientes com pericardite constritiva confirmada por cirurgia foram avaliados retrospectivamente quanto a variáveis clínicas e laboratoriais. Dois métodos diagnósticos foram utilizados: ecocardiograma transtorácico e ressonância cardíaca. Preditores de mortalidade foram determinados por análise univariada usando metodologia das proporções de Cox e hazard ratio. Todos os testes foram considerados bicaudais e um erro alfa ≤ 5% foi considerado como significante. Resultados: Foram estudados 84 pacientes com idade média de 44 ± 17,9 anos, sendo 67% do sexo masculino. Sinais e sintomas de insuficiência cardíaca (IC) predominantemente direita estiveram presentes com estase jugular, edema e ascite em 89%, 89% e 62% dos casos, respectivamente. Etiologia idiopática foi observada em 69% dos casos, seguida por tuberculose em 21%. Apesar do grau de IC, encontramos baixos níveis de BNP (mediana de 157 pg/mL). As sensibilidades diagnósticas para constrição do ecocardiograma e da ressonância foram 53,6% e 95,9%, respectivamente. Durante a evolução clínica, houve 9 óbitos (10,7%) e os fatores de risco foram: anemia, elevações de BNP, PCR, hipertensão pulmonar > 55 mmHg e fibrilação atrial. Conclusões: Pericardite constritiva manifesta-se com sinais e sintomas de IC biventricular com predomínio à direita e baixos níveis de BNP. A ressonância magnética apresenta melhor sensibilidade para diagnóstico. Marcadores clínicos, laboratoriais e de imagem estiveram associados ao óbito.

Pericarditis constrictiva, siempre un desafío diagnóstico / Constrictive pericarditis: always a diagnostic challenge

Reportamos el caso de un hombre de 52 años, con antecedente de larga data de dolor torácico, estudiado el año 2005 con una coronariografía que descarta lesiones en las arterias epicárdicas. Se agrega luego de forma progresiva dolor y distensión abdominal. Sin controles hasta el año 2015, cuando se realiza una endoscopia digestiva alta que no objetiva hallazgos patológicos, encontrándose el paciente en fibrilación auricular e iniciando terapia. A fines del 2016 por persistencia y aumento de las molestias se hospitaliza en nuestro centro para estudio. Se realiza una ecocardiografía que evidencia; severa dilatación biauricular, buena función global y segmentaria bi-ventricular y un movimiento de rebote protodiás-tolico que aumentaba notoriamente en inspiración a nivel del septum interventricular. El pericardio se encontraba engrosado e hiperrefringente de forma difusa, con falta de deslizamiento entre sus hojas y movimiento en bloque de los tejidos blandos adyacentes. Presentaba además comportamiento reverso del anillo mitral al Doppler tisular y la vena cava inferior se encontraba dilatada y sin variación con el ciclo ventilatorio. Un sondeo cardíaco izquierdo y derecho descarta lesiones coronarias y corrobora la fisiología constrictiva. Se amplía el estudio con una tomografía computada de tórax que informa engrosamiento difuso y extensa calcificación del pericardio, además de signos de daño hepático crónico. Se realiza una pericardiotomia exitosa, los hallazgos del intraoperatorio e histopatológicos confirman el diagnóstico de pericarditis constrictiva calcificada. El paciente es dado de alta al quinto día postoperatorio sin incidentes.

We report the case of a 52 y.o. male, with longstanding history of chest pain. Coronariography performed in 2005 showed normal coronary arteries. In the following years he developed progressive abdominal bloating and pain, upper gastrointestinal endoscopy was normal, but atrial fibrillation was diagnosed initiating therapy. During the year 2016 symptoms got worse, being admitted to our hospital. Echocardiography revealed severe biatrial enlargement, preserved bi-ventricular function, and a paradoxical bouncing motion of the interventricular septum which augmented during inspiration (septal bounce). The pericardium was thickened with absence of normal sliding between its layers, and traction of adjacent soft tissues was observed. Mitral ring tissue Doppler showed reversal of lateral and medial e' velocities. Inferior vena cava was dilated with no respiratory variation. Cardiac catheterization was consistent with constrictive pericarditis. Computed tomography confirm extensive pericardial calcification and thickness and found signs of chronic liver disease. Uneventful pericardiectomy was performed, intraoperative and histological findings confirm the diagnostic of calcified pericardial constriction, being discharged on the fifth day post-surgery.

Pericarditis purulenta constrictiva, una complicación grave que puede ser evitada / Constrictive purulent pericarditis, an avoidable serious complication

La pericarditis purulenta se define como la ocupación del saco pericárdico por fluido purulento. Es una enfermedad de curso letal si no se trata con prontitud; la mortalidad varía de 2 a 20 por ciento. La combinación de antibioticoterapia y drenaje pericárdico provee los mejores resultados clínicos, sin embargo, existe controversia en relación con el momento y la vía para realizarlo. Algunos enfermos desarrollan adherencias pericárdicas que producen constricción con repercusión hemodinámica por compromiso del llenado diastólico de las cavidades cardíacas derechas. Se presenta un paciente masculino, de 4 años de edad y 13 kg de peso corporal, con el diagnóstico de pericarditis purulenta de un mes de evolución, con signos de respuesta inflamatoria sistémica y compromiso hemodinámico por pericarditis constrictiva. Se intervino quirúrgicamente de urgencia para realizar pericardiectomía y drenaje del absceso mediastinal. Se comentan la prevención y la conducta ante esta grave complicación

Purulent pericarditis is defined as the occupation of the pericardial sac by the purulent effusion. It is a lethal disease if not treated as early as possible since the mortality rate ranges 2 to 20 percent. The combination of antibiotics and pericardial drainage provides the best clinical results; however, there are controversies about the time and the way of performing these actions. Some patients develop pericardial adhesions that may cause constriction with hemodynamic repercussion due to compromised dyastolic filling of the right heart cavities. Here is a male 4 years-old patient weighing 13 kg, who was diagnosed with purulent pericarditis of one month of evolution and presented signs of systemic inflammation and hemodynamic compromise due to constrictive pericarditis. He was operated on at the emergency service to perform pericardiectomy and mediastinal abscess drainage. The prevention of this problem and the behavior to be followed to manage this serious complication were commented on in this report

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)

Comparison of B-type natriuretic peptide and left ventricular dysfunction in patients with constrictive pericarditis undergoing pericardiectomy.

Chronic constrictive pericarditis (CCP) due to tuberculosis has high morbidity and mortality in the periopeartive period following pericardiectomy because of left ventricular (LV) dysfunction. Brain-type natriuretic peptide (BNP) is considered a marker for both LV systolic and diastolic dysfunction. We undertook this prospective study in 24 patients, to measure the BNP levels and to compare it with transmitral Doppler flow velocities, that is, the E/A ratio (E = initial peak velocity during early diastolic filling and A = late peak flow velocity during atrial systole), as a marker of diastolic function and systolic parameters, pre- and post-pericardiectomy, at the time of discharge. The latter parameters have been taken as a flow velocity across the mitral valve on a transthoracic echo. There was a significant decrease in the mean values of log BNP (6.19 ± 0.33 to 4.65 ± 0.14) (P = 0.001) and E/A ratio (1.81 ± 0.21 to 1.01 ± 0.14) (P = 0.001) post pericardiectomy, with a positive correlation, r = 0.896 and 0.837, respectively, between the two values at both the time periods. There was significant improvement in the systolic parameters of the LV function, that is, stroke volume index, cardiac index, systemic vascular resistance index, and delivered oxygen index. However, no correlation was observed between these values and the BNP levels. We believe that BNP can be used as a marker for LV diastolic dysfunction in place of the E/A ratio in patients with CCP, undergoing pericardiectomy. However, more studies have to be performed for validation of the same.

Pericardiectomy for constrictive pericarditis: is prolonged inotropic support a bad omen?

Sixty-seven patients who underwent pericardiectomy for constrictive pericarditis at JIPMER, Pondicherry between 1987 and 1998 were the subjects of the study. Pre-operatively 70% of cases were in the New York Heart Association classes III and IV categories with clinical signs suggestive of constriction ie, raised jugular venous pressure in 99%, pleural effusion in 77%, pedal oedema in 61% and ascites in 55% of the cases. Seventy-five per cent of the cases underwent pericardiectomy through a median sternotomy and the rest via left anterolateral thoracotomy. Low cardiac output was evidenced in 70% of cases postoperatively which was managed by early institution and prolonged use of inotropes. There was 9% mortality especially in the early part of the experience. Tuberculous pathology was confirmed histologically in 57% cases. Sixty-three per cent of cases are presenting in follow-up in New York Heart Association class I. Prolonged use of inotropes instituted early in postoperative period is recommended to prevent postoperative ventricular dysfunction with adrenaline being the preferred inotrope. It is concluded that postoperative New York Heart Association class and long term survival were not significantly influenced by pre-operative New York Heart Association class, operative approach or peri-operative low cardiac output syndrome requiring prolonged inotropic support.

Traitement chirurgical de la pericardite chronique constrictive : a propos de 60 cas experience de l'Institut de Cardiologie d'Abidjan

La pericardite chronique constrictive est due a une sclerose retractile associee ou naon a une impregnation calcaire des deux feuillets pericardiques aboutissant a une gene au remplissage et a l'expansion diastolique des ventricules. Dans nos regions sa pri;cipale etiologie est la tuberculose. Son seul veritable traitement une fois diagnostiquee est la pericardectomie. Il s'agit d'une intervention delicate et minutieuse qui consiste a enlever tout le pericarde pathologique. La voie d'abord actuellement utilisee est la sternotomie mediane verticale qui offre un meilleur jour permettant de realiser une pericardectomie sub-phrenique bilaterale. Les facteurs que nous avons identifies qui influencent les resultats de cette chirurgie sont : la duree d'evolution superieure a 5 ans; le stade fonctionnel III ou IV de la NYHA; l'existence d'une atteinte myocardique; la presence de calcifications importantes; l'existence d'une fibrillation auricylaire d'ou l'interet d'operer les patients tot avant l'installation de lesions importantes. Mais cela restedifficiel dans nos pays ou le systeme sanitaire reste peu develpope

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.

Pericarditis purulenta: relato de un caso / Purulent pericarditis: report of a case

Se presenta un paciente de 51 años, alcoholista y fumador intenso, que en el curso de una neumopatía aguda, sufre una pericarditis aguda supurada, que evolucionó en una semana al taponamiento cardíaco. A pesar del drenaje quirúrgico efectuado, se desarrolló en un lapso de semanas un cuadro clínico y hemodimámico típico de pericarditis constrictiva. La pericardiectomía total logró la curación del paciente. A propósito del caso, se comentan aspectos clínicos, patológicos y terapéuticos

Pericarditis constrictiva: experiencia quirúrgica en el Hospital México / Constrictive pericarditis: surgical experience in the Hospital México

Se estudiaron trece pacientes tratados quirúrgicamente por pericarditis constrictiva, en el servicio de Cirugía Cardíaca del Hospital México. Fueron especial interés, la etiología en cada caso, siendo la TB la causa mas frecuentemente observada, y los síntomas recopilados de relevancia clínica. Se observó un predominio del sexo femenino en un 61 por ciento (n:8) con una edad promedio de 34 a. La cirugía practicada fue la pericardiectomía parcial por abordaje subxifoideo en el 47 por ciento de los casos, y un abordajetransesternal en un 53 por ciento, este último con mejores resultados en cuanto a mortalidad operatoria que fue del 0 por ciento. La mortalidad global fue del 23 por ciento (n:3), y estuvo relacionada directamente con la enfermedad subyacente. Se revisa la literatura y se comentan los abordajes incluyendo el uso de circulación extracopórea.

Pericardite constrictiva como diagnóstico diferencial de doença hepática / Constrictive pericarditis as differential diagnosis of hepatic disease

Pericardite crônica constrictiva em um paciente masculino de 13 anos, previamente co "hepatite crônica" por sete anos com uso de furosemida e espironolactona. A investigaçäo diagnóstica constou de radiografia de tórax, ecodopplercardiografia, toracocentese com biópsia pleural e tomografia computadorizada de tórax, que mostraram tratar-se de uma síndrome de restriçäo diastólica ventricular por pericardite crônica constrictiva. Após oito semanas de tratamento com tuberculostáticos, foi submetido a estudo hemodinâmico, que confirmou o diagnóstico, sendo o paciente enviado à pericardiectomia. A evoluçäo tardia mostrou regressäo do quadro de restriçäo diastólica com diminuiçäo de hepatoesplenomegalia e da estase jugular, sendo mantido o esquema inicial por mais 12 meses, associado a corticoterapia

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

Surgical treatment for chronic constrictive pericarditis.

One hundred and twelve patients were operated for constrictive pericarditis from December 1963 through May 1987 at J.J. Group of Hospitals, Bombay. All the patients had haemodynamically significant pericardial constriction preoperatively, and pericardial disease was confirmed at operation. Preoperatively, the patients were either in NYHA class III (96 cases), or class IV (16 cases). All the patients had ascites and/or peripheral oedema with an enlarged liver. Pericardiectomy was performed through a left anterior thoracotomy (92 cases), or median sternotomy (20 cases). There was an early mortality (deaths occurring within 30 days of operation) in 21 cases (18.75%). Early mortality was related to preoperative disability of the patients. Post-operatively, NYHA classification of patients at six months after operation showed improvement in functional class with 80 patients in either class I or II and 6 patients in class III.