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negative C-reactive protein and procalcitonin&#44; increased alkaline phosphatase &#40;178 U&#47;l&#41; and gamma-glutamyl transpeptidase &#40;109 U&#47;l&#41;&#44; normal aspartate aminotransferase&#44; alanine aminotransferase and total bilirubin&#44; reduced total protein &#40;4&#46;6 g&#47;dl&#41; and albumin &#40;1&#46;83 g&#47;dl&#41;&#44; normal protein electrophoresis&#44; negative HIV&#44; HBV and HCV&#44; normal anti-neutrophil cytoplasmic antibody&#44; immunoglobulin and complement&#44; and normal thyroid function&#46; Abdominal ultrasound showed an enlarged liver with a diffuse heterogeneous structure&#44; suggesting chronic liver disease&#44; and dilatation of the inferior vena cava and suprahepatic veins&#46; The chest X-ray revealed calcification of the cardiac silhouette and right pleural effusion &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; Echocardiography showed severe left atrial dilatation &#40;area 44 cm<span class="elsevierStyleSup">2</span>&#41; 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dip-and- plateau pattern of the ventricular curve&#59; equalization of ventricular end-diastolic pressures&#59; right curve with prominent y descent&#59; and mild pulmonary hypertension &#40;PASP 45 mmHg&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; Magnetic resonance imaging showed foci of hypointense signal in the region of the inferior pericardium due to residual calcifications and an associated ventricular restrictive component&#44; as shown by myocardial hypokinesis on dynamic sequences &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Following intensive diuretic therapy&#44; salt restriction and fluid therapy&#44; the patient presented a favorable clinical course with resolution of heart failure symptoms&#46; 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and virtually any inflammation can cause constriction&#46; In developed countries the main causes of CP are idiopathic or secondary to surgery or radiation&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Tuberculosis used to be one of the most frequent causes&#44; but the efficacy of antibiotic therapy has reduced the incidence of tuberculous CP in these countries&#46; However&#44; in developing countries it remains one of the most common causes&#44;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> and in Africa there is a frequent association between tuberculous CP and HIV-positive status&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Constriction usually develops over a period of years&#44; ultimately resulting in fibrotic thickening of the pericardium&#44; often calcified&#44; with adhesions between the parietal and visceral layers&#44; forming a non-compliant shell around the heart&#44; which restricts diastolic filling of the cardiac chambers&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> This leads to elevation and equalization of filling pressures in the chambers and systemic and pulmonary veins&#46; Ventricular filling is abnormally rapid at the beginning of diastole due to elevated atrial pressure and elastic recoil&#44; then ceases abruptly during mid- or end-diastole when the intracardiac volume reaches the limit imposed by the thickened pericardium&#59; this means that practically all ventricular filling occurs at the beginning of diastole&#44; resulting in systemic venous congestion&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Clinical presentation of CP consists mainly of signs and symptoms of right heart failure&#46; The early stage is characterized by peripheral edema&#44; abdominal discomfort and some degree of liver congestion&#46; Disease progression is generally accompanied by ascites&#44; anasarca and jaundice&#46; Signs and symptoms of elevated pulmonary venous pressure may appear&#44; including dyspnea&#44; cough and orthopnea&#44; as well as AF and tricuspid regurgitation&#46; Fatigue and cachexia due to low cardiac output may also be present in more advanced stages&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> Occasionally angina occurs due to coronary artery disease or compression of a coronary artery by the thickened pericardium&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Findings on physical examination include marked elevation of jugular venous pressure with a prominent rapidly collapsing y descent&#59; positive Kussmaul&#39;s sign &#40;increased venous pressure on inspiration&#41;&#59; paradoxical pulse &#40;fall of &#62;10 mmHg in systolic arterial pressure during inspiration&#41;&#44; found in up to a third of patients with CP&#59; weak apical pulse&#59; distant heart sounds&#59; pericardial knock &#40;an early diastolic sound over the left sternal border or apex caused by abrupt cessation of ventricular filling&#41;&#59; hepatomegaly&#44; ascites and jaundice&#59; palmar erythema&#59; spider angioma and lower limb edema&#46; As mentioned above&#44; in advanced stages there may be cachexia and muscle wasting&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Differential diagnosis includes pulmonary embolism&#44; chronic obstructive pulmonary disease&#44; liver failure and restrictive cardiomyopathy&#44;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> the latter being the most difficult but also the most important to exclude&#44; since the therapeutic approaches are completely different&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The chest X-ray shows pericardial calcification in a small number of patients&#44; raising the suspicion of tuberculous CP&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> Transthoracic electrocardiography may show pericardial thickening&#44; abrupt posterior motion of the interventricular septum at the beginning of diastole &#40;septal bounce&#41;&#44; dilatation of the inferior vena cava without inspiratory collapse and of the suprahepatic veins&#44; and predominantly early ventricular filling&#46; Doppler study may document increased transmitral flow during expiration &#40;&#62;25&#37; variation&#41;&#59; a restrictive flow pattern&#59; deceleration time &#60;160 ms&#59; increased duration and velocity of pulmonary venous flow with inspiration&#59; or increased diastolic reversal velocity of &#62;25&#37; of forward flow in the suprahepatic veins during expiration&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> Pericardial thickening and calcification may be seen on magnetic resonance imaging&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Cardiac catheterization can document the hemodynamic effects of CP and enable differential diagnosis with restrictive cardiomyopathy&#46; In CP atrial pressures and ventricular diastolic pressure are elevated and equalized &#40;around 20 mmHg&#41;&#46; The right atrial pressure curve shows a typical M or W shape resulting from the existence of a preserved descending x wave in systole&#44; a prominent descending y wave in diastole and small a and v waves of the same amplitude&#46; Ventricular diastolic pressure curves show a fall at the beginning of diastole&#44; followed by a plateau &#40;dip-and-plateau pattern&#41;&#46; PASP is generally less than 45&#8722;50 mmHg&#46; Ejection volume is usually reduced but cardiac output at rest is preserved through compensatory tachycardia until more advanced stages of the disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The European Society of Cardiology guidelines recommend pericardiectomy as the only definitive treatment for CP&#59; this may by anterolateral thoracotomy or median sternotomy<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> and can be complete or partial&#46; Chowdhury et al&#46; retrospectively compared the surgical results between complete and partial pericardiectomy and found that survival and functional outcome were superior with complete pericardiectomy compared with partial pericardiectomy&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Complete pericardiectomy is defined as wide excision of the pericardium anteriorly extending to both phrenic nerves and including the diaphragmatic pericardium&#46; Partial pericardiectomy is defined as any pericardial excision that does not meet criteria for phrenic-to-phrenic pericardiectomy&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Ling et al&#46; studied patients who underwent pericardiectomy at the Mayo Clinic between 1985 and 1995&#44; comparing them with a historic cohort&#44; and found lower perioperative mortality &#40;6&#37; vs&#44; 14&#37;&#59; p&#61;0&#46;011&#41;&#44; higher median age at the time of pericardiectomy &#40;61 vs&#46; 45 years&#41;&#44; and an increased prevalence of CP due to radiation&#44; but late survival was not as good as expected&#46; The principal causes of perioperative mortality were low output state&#44; sepsis&#44; uncontrolled bleeding&#44; renal failure and respiratory insufficiency&#46; Independent predictors of late survival were age&#44; NYHA class and previous radiation&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">A retrospective study by Bertog et al&#46; of 163 patients who underwent pericardiectomy over a 24-year period concluded that the only predictors of late events were post-radiation etiology&#44; age&#44; left ventricular dysfunction&#44; high pulmonary artery pressure&#44; creatinine and serum sodium&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> George et al&#46; concluded that post-radiation CP&#44; hyperbilirubinemia and hypoalbuminemia were significant risk factors for decreased long-term survival after pericardiectomy&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Right-sided heart failure after pericardiectomy can be caused by incomplete pericardiectomy&#44; recurrent constriction due to exuberant scar tissue&#44; diastolic dysfunction or extension of pericardial calcification into the myocardium&#46;<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">7&#44;9</span></a> Other possible etiologies include cardiomyopathy and pulmonary hypertension&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Identification of constriction in patients who have recurrent symptoms after partial pericardiectomy is challenging&#44; since the characteristic Doppler echocardiographic features may not be present if a portion of the ventricles is free from constricting pericardium&#46; Furthermore&#44; many of these patients are under intensive diuretic therapy and&#47;or have underlying cardiac disease&#44; such as coronary artery disease&#44; that may mask the signs and symptoms&#46; Magnetic resonance imaging and cardiac catheterization may be required to establish the diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">One possible approach for recurrent CP following pericardiectomy is repeat pericardiectomy&#44; although the risks and benefits of a second operation are not well established&#46; Cho et al&#46; analyzed the outcomes of second pericardiectomy in 41 patients hospitalized for recurrent CP between 1993 and 2010&#46; They divided the study population into two groups according to the interval between the first and second operations &#40;&#8804;1 year vs&#46; &#62;1 year&#41;&#44; and found that five-year survival was significantly better in the group with a shorter interval between operations &#40;73&#37; vs&#46; 29&#37;&#44; p&#61;0&#46;032&#41;&#46; Multivariate analysis showed that NYHA class was also an important predictor of survival&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> The authors stressed the importance of complete resection at first operation given the significant mortality associated with repeat pericardiectomy&#59; at the same time&#44; the poor clinical outcome of late &#40;more than 1 year&#41; reoperation may be explained by progressive diastolic dysfunction or myocardial involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">In conclusion&#44; CP is a heterogeneous disease&#44; increasingly important causes of which in the current era include radiation and cardiac surgery&#46; Although pericardiectomy is often performed and the results are excellent in some patients&#44; it may not offer a cure or good long-term results in advanced or post-radiation CP&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> It is therefore essential to investigate other treatment options&#44; particularly heart transplantation&#44; which should be considered in selected patients without recurrent tumor and with good pulmonary reserve&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">CP is rare&#44; and recurrent CP rarer still&#44; and there are as yet no diagnostic or treatment guidelines for management of the disease&#46; There is thus a need to establish the best approach in these patients&#44; for which further studies and much research will be required&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Ethical disclosures</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Protection of human and animal subjects</span><p id="par0100" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Confidentiality of data</span><p id="par0105" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Right to privacy and informed consent</span><p id="par0110" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conflicts of interest</span><p id="par0115" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Recurrent right-sided heart failure after pericardiectomy may be caused by incomplete pericardiectomy&#44; recurrent constriction&#44; diastolic dysfunction or myocardial involvement&#46; Identifying recurrent constrictive pericarditis &#40;CP&#41; in patients who have recurring symptoms after pericardiectomy is challenging&#44; since the characteristic Doppler echocardiographic features may not be present if a portion of the ventricles are free of constricting pericardium&#44; and there are no diagnostic or treatment guidelines for management of recurrent CP&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The authors report the case of a 59-year-old man with a history of pericardiectomy for tuberculous CP in 1984&#44; admitted to our hospital with signs and symptoms of right heart failure&#46; After a complete diagnostic workup&#44; recurrent CP was diagnosed&#46; Given the scarcity of cases reported on this disease&#44; three possible therapeutic approaches are discussed&#58; a second pericardiectomy&#44; heart transplantation and medical therapy&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Insufici&#234;ncia card&#237;aca direita recorrente ap&#243;s pericardectomia pode ser causada por pericardectomia incompleta&#44; constri&#231;&#227;o recorrente&#44; disfun&#231;&#227;o diast&#243;lica ou atingimento mioc&#225;rdico&#46; A identifica&#231;&#227;o de pericardite constritiva &#40;PC&#41; recorrente ap&#243;s pericardectomia &#233; desafiante&#44; uma vez que&#44; muitas das caracter&#237;sticas da constri&#231;&#227;o podem estar ausentes e n&#227;o existem ainda <span class="elsevierStyleItalic">guidelines</span> de diagn&#243;stico ou terap&#234;utica para a abordagem desta patologia dada a sua extrema raridade&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Os autores descrevem o caso de um homem&#44; 59 anos de idade&#44; com antecedentes de pericardectomia ap&#243;s PC tuberculosa em 1984&#44; internado para esclarecimento de um quadro cl&#237;nico dominado por sinais e sintomas de insufici&#234;ncia card&#237;aca direita&#46; Ap&#243;s estudo complementar foi diagnosticado PC recorrente&#46; Dada a escassez de casos reportados sobre esta patologia&#44; foram discutidas poss&#237;veis abordagens terap&#234;uticas nomeadamente uma segunda pericardectomia&#44; transplante card&#237;aco e terap&#234;utica m&#233;dica&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Ferreira R&#44; Gonzaga A&#44; Santos L&#44; et al&#46; Pericardite constritiva recorrente &#8211; um desafio diagn&#243;stico e terap&#234;utico&#46; Rev Port Cardiol&#46; 2015&#59;34&#58;421&#46;e1&#8211;421&#46;e5&#46;</p>"
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Case report
Recurrent constrictive pericarditis: A diagnostic and therapeutic challenge
Pericardite constritiva recorrente – um desafio diagnóstico e terapêutico
Raquel Ferreira
Corresponding author
ana_rakel_ferreira@hotmail.com

Corresponding author.
, Anabela Gonzaga, Luís Santos, José António Santos
Serviço de Cardiologia, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal
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            "entidad" => "Servi&#231;o de Cardiologia&#44; Centro Hospitalar do Baixo Vouga&#44; Aveiro&#44; Portugal"
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        "titulo" => "Pericardite constritiva recorrente &#8211; um desafio diagn&#243;stico e terap&#234;utico"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Coronary angiography showing no coronary artery disease and marked calcification of the cardiac silhouette&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Case report</span><p id="par0005" class="elsevierStylePara elsevierViewall">The authors report the case of a 59-year-old man with a history of tuberculosis at age 12&#44; who subsequently developed tuberculous constrictive pericarditis &#40;CP&#41; and underwent pericardiectomy in 1984&#46; The surgical report refers to pericardiectomy by median sternotomy but gives no further details&#46; He also had a history of permanent atrial fibrillation &#40;AF&#41;&#44; type 2 diabetes&#44; hypothyroidism and multiple emergency department &#40;ED&#41; admissions for clinical settings interpreted as liver failure&#59; he was medicated with warfarin&#44; bisoprolol 2&#46;5 mg daily&#44; metformin 1000 mg twice daily and levothyroxine 0&#46;1 mg daily&#46; He was admitted to the ED again in September 2013 with abdominal discomfort&#44; peripheral edema and ascites&#46; On physical examination&#44; he presented jugular distension&#44; weak apical pulse&#44; and audible and arrhythmic S1 and S2&#44; with no cardiac murmurs&#59; diminished breath sounds in the lower half of the right hemothorax&#59; palpable hepatomegaly and hepatojugular reflux&#59; and ascites and lower limb edema&#46; He was admitted to the internal medicine department&#46; During hospitalization&#44; he suffered an episode of tight chest pain radiating to both arms&#44; associated with profuse sweating&#44; and he was accordingly transferred to the cardiology department for diagnostic investigation&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">The electrocardiogram revealed AF with a pattern of right bundle branch block&#44; right axis deviation and increased QTc interval &#40;470 ms&#41;&#46; Laboratory tests showed hemoglobin 13&#46;0 g&#47;dl&#44; normal renal function and electrolytes&#44; negative serial troponin I&#44; pro-brain natriuretic peptide 313 pg&#47;ml&#44; negative C-reactive protein and procalcitonin&#44; increased alkaline phosphatase &#40;178 U&#47;l&#41; and gamma-glutamyl transpeptidase &#40;109 U&#47;l&#41;&#44; normal aspartate aminotransferase&#44; alanine aminotransferase and total bilirubin&#44; reduced total protein &#40;4&#46;6 g&#47;dl&#41; and albumin &#40;1&#46;83 g&#47;dl&#41;&#44; normal protein electrophoresis&#44; negative HIV&#44; HBV and HCV&#44; normal anti-neutrophil cytoplasmic antibody&#44; immunoglobulin and complement&#44; and normal thyroid function&#46; Abdominal ultrasound showed an enlarged liver with a diffuse heterogeneous structure&#44; suggesting chronic liver disease&#44; and dilatation of the inferior vena cava and suprahepatic veins&#46; The chest X-ray revealed calcification of the cardiac silhouette and right pleural effusion &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; Echocardiography showed severe left atrial dilatation &#40;area 44 cm<span class="elsevierStyleSup">2</span>&#41; and mild right atrial dilatation &#40;25 cm<span class="elsevierStyleSup">2</span>&#41;&#44; while the other chambers and the roots of the great vessels were of normal size&#59; normal left ventricular thickness&#59; altered interventricular septal motion&#59; preserved left ventricular systolic function &#40;ejection fraction 61&#37;&#41;&#59; no wall motion abnormalities&#59; diastolic mitral flow compatible with a restrictive pattern &#40;mean E&#47;E&#8242; 19&#41;&#59; preserved right ventricular systolic function &#40;tricuspid annular plane systolic excursion 17 mm&#41;&#59; no significant valve abnormalities&#59; pulmonary artery systolic pressure &#40;PASP&#41; estimated at 45 mmHg&#59; thickening of the diaphragmatic pericardium due to fibrosis&#59; and dilatation of the inferior vena cava and suprahepatic veins&#46; Coronary angiography revealed no coronary artery disease &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#44; but hemodynamic data on catheterization were suggestive of constrictive physiology&#58; dip-and- plateau pattern of the ventricular curve&#59; equalization of ventricular end-diastolic pressures&#59; right curve with prominent y descent&#59; and mild pulmonary hypertension &#40;PASP 45 mmHg&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; Magnetic resonance imaging showed foci of hypointense signal in the region of the inferior pericardium due to residual calcifications and an associated ventricular restrictive component&#44; as shown by myocardial hypokinesis on dynamic sequences &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Following intensive diuretic therapy&#44; salt restriction and fluid therapy&#44; the patient presented a favorable clinical course with resolution of heart failure symptoms&#46; The possibility of redo pericardiectomy was discussed with the cardiac surgery team&#44; but it was decided not to perform a repeat procedure &#40;30 years after the first&#41; after weighing the risks and benefits&#46; Heart transplantation was also discussed and the patient underwent respiratory function testing&#44; which revealed moderate obstruction&#44; but the decision was postponed until such time as the patient presented NYHA class III&#47;IV heart failure refractory to medical therapy&#46; He was discharged and referred for cardiology consultation&#59; diuretic therapy was continued and he has remained in NYHA class I&#47;II&#44; with no rehospitalization for decompensation to date&#46; He has also been referred for cardiopulmonary stress testing for further assessment of his functional capacity&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0020" class="elsevierStylePara elsevierViewall">CP is an uncommon condition and its diagnosis remains a clinical challenge&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> It is defined as the end stage of an inflammatory process involving the pericardium&#44; and virtually any inflammation can cause constriction&#46; In developed countries the main causes of CP are idiopathic or secondary to surgery or radiation&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Tuberculosis used to be one of the most frequent causes&#44; but the efficacy of antibiotic therapy has reduced the incidence of tuberculous CP in these countries&#46; However&#44; in developing countries it remains one of the most common causes&#44;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> and in Africa there is a frequent association between tuberculous CP and HIV-positive status&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Constriction usually develops over a period of years&#44; ultimately resulting in fibrotic thickening of the pericardium&#44; often calcified&#44; with adhesions between the parietal and visceral layers&#44; forming a non-compliant shell around the heart&#44; which restricts diastolic filling of the cardiac chambers&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> This leads to elevation and equalization of filling pressures in the chambers and systemic and pulmonary veins&#46; Ventricular filling is abnormally rapid at the beginning of diastole due to elevated atrial pressure and elastic recoil&#44; then ceases abruptly during mid- or end-diastole when the intracardiac volume reaches the limit imposed by the thickened pericardium&#59; this means that practically all ventricular filling occurs at the beginning of diastole&#44; resulting in systemic venous congestion&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Clinical presentation of CP consists mainly of signs and symptoms of right heart failure&#46; The early stage is characterized by peripheral edema&#44; abdominal discomfort and some degree of liver congestion&#46; Disease progression is generally accompanied by ascites&#44; anasarca and jaundice&#46; Signs and symptoms of elevated pulmonary venous pressure may appear&#44; including dyspnea&#44; cough and orthopnea&#44; as well as AF and tricuspid regurgitation&#46; Fatigue and cachexia due to low cardiac output may also be present in more advanced stages&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> Occasionally angina occurs due to coronary artery disease or compression of a coronary artery by the thickened pericardium&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Findings on physical examination include marked elevation of jugular venous pressure with a prominent rapidly collapsing y descent&#59; positive Kussmaul&#39;s sign &#40;increased venous pressure on inspiration&#41;&#59; paradoxical pulse &#40;fall of &#62;10 mmHg in systolic arterial pressure during inspiration&#41;&#44; found in up to a third of patients with CP&#59; weak apical pulse&#59; distant heart sounds&#59; pericardial knock &#40;an early diastolic sound over the left sternal border or apex caused by abrupt cessation of ventricular filling&#41;&#59; hepatomegaly&#44; ascites and jaundice&#59; palmar erythema&#59; spider angioma and lower limb edema&#46; As mentioned above&#44; in advanced stages there may be cachexia and muscle wasting&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Differential diagnosis includes pulmonary embolism&#44; chronic obstructive pulmonary disease&#44; liver failure and restrictive cardiomyopathy&#44;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> the latter being the most difficult but also the most important to exclude&#44; since the therapeutic approaches are completely different&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The chest X-ray shows pericardial calcification in a small number of patients&#44; raising the suspicion of tuberculous CP&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> Transthoracic electrocardiography may show pericardial thickening&#44; abrupt posterior motion of the interventricular septum at the beginning of diastole &#40;septal bounce&#41;&#44; dilatation of the inferior vena cava without inspiratory collapse and of the suprahepatic veins&#44; and predominantly early ventricular filling&#46; Doppler study may document increased transmitral flow during expiration &#40;&#62;25&#37; variation&#41;&#59; a restrictive flow pattern&#59; deceleration time &#60;160 ms&#59; increased duration and velocity of pulmonary venous flow with inspiration&#59; or increased diastolic reversal velocity of &#62;25&#37; of forward flow in the suprahepatic veins during expiration&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> Pericardial thickening and calcification may be seen on magnetic resonance imaging&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Cardiac catheterization can document the hemodynamic effects of CP and enable differential diagnosis with restrictive cardiomyopathy&#46; In CP atrial pressures and ventricular diastolic pressure are elevated and equalized &#40;around 20 mmHg&#41;&#46; The right atrial pressure curve shows a typical M or W shape resulting from the existence of a preserved descending x wave in systole&#44; a prominent descending y wave in diastole and small a and v waves of the same amplitude&#46; Ventricular diastolic pressure curves show a fall at the beginning of diastole&#44; followed by a plateau &#40;dip-and-plateau pattern&#41;&#46; PASP is generally less than 45&#8722;50 mmHg&#46; Ejection volume is usually reduced but cardiac output at rest is preserved through compensatory tachycardia until more advanced stages of the disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The European Society of Cardiology guidelines recommend pericardiectomy as the only definitive treatment for CP&#59; this may by anterolateral thoracotomy or median sternotomy<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> and can be complete or partial&#46; Chowdhury et al&#46; retrospectively compared the surgical results between complete and partial pericardiectomy and found that survival and functional outcome were superior with complete pericardiectomy compared with partial pericardiectomy&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Complete pericardiectomy is defined as wide excision of the pericardium anteriorly extending to both phrenic nerves and including the diaphragmatic pericardium&#46; Partial pericardiectomy is defined as any pericardial excision that does not meet criteria for phrenic-to-phrenic pericardiectomy&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Ling et al&#46; studied patients who underwent pericardiectomy at the Mayo Clinic between 1985 and 1995&#44; comparing them with a historic cohort&#44; and found lower perioperative mortality &#40;6&#37; vs&#44; 14&#37;&#59; p&#61;0&#46;011&#41;&#44; higher median age at the time of pericardiectomy &#40;61 vs&#46; 45 years&#41;&#44; and an increased prevalence of CP due to radiation&#44; but late survival was not as good as expected&#46; The principal causes of perioperative mortality were low output state&#44; sepsis&#44; uncontrolled bleeding&#44; renal failure and respiratory insufficiency&#46; Independent predictors of late survival were age&#44; NYHA class and previous radiation&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">A retrospective study by Bertog et al&#46; of 163 patients who underwent pericardiectomy over a 24-year period concluded that the only predictors of late events were post-radiation etiology&#44; age&#44; left ventricular dysfunction&#44; high pulmonary artery pressure&#44; creatinine and serum sodium&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> George et al&#46; concluded that post-radiation CP&#44; hyperbilirubinemia and hypoalbuminemia were significant risk factors for decreased long-term survival after pericardiectomy&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Right-sided heart failure after pericardiectomy can be caused by incomplete pericardiectomy&#44; recurrent constriction due to exuberant scar tissue&#44; diastolic dysfunction or extension of pericardial calcification into the myocardium&#46;<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">7&#44;9</span></a> Other possible etiologies include cardiomyopathy and pulmonary hypertension&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Identification of constriction in patients who have recurrent symptoms after partial pericardiectomy is challenging&#44; since the characteristic Doppler echocardiographic features may not be present if a portion of the ventricles is free from constricting pericardium&#46; Furthermore&#44; many of these patients are under intensive diuretic therapy and&#47;or have underlying cardiac disease&#44; such as coronary artery disease&#44; that may mask the signs and symptoms&#46; Magnetic resonance imaging and cardiac catheterization may be required to establish the diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">One possible approach for recurrent CP following pericardiectomy is repeat pericardiectomy&#44; although the risks and benefits of a second operation are not well established&#46; Cho et al&#46; analyzed the outcomes of second pericardiectomy in 41 patients hospitalized for recurrent CP between 1993 and 2010&#46; They divided the study population into two groups according to the interval between the first and second operations &#40;&#8804;1 year vs&#46; &#62;1 year&#41;&#44; and found that five-year survival was significantly better in the group with a shorter interval between operations &#40;73&#37; vs&#46; 29&#37;&#44; p&#61;0&#46;032&#41;&#46; Multivariate analysis showed that NYHA class was also an important predictor of survival&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> The authors stressed the importance of complete resection at first operation given the significant mortality associated with repeat pericardiectomy&#59; at the same time&#44; the poor clinical outcome of late &#40;more than 1 year&#41; reoperation may be explained by progressive diastolic dysfunction or myocardial involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">In conclusion&#44; CP is a heterogeneous disease&#44; increasingly important causes of which in the current era include radiation and cardiac surgery&#46; Although pericardiectomy is often performed and the results are excellent in some patients&#44; it may not offer a cure or good long-term results in advanced or post-radiation CP&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> It is therefore essential to investigate other treatment options&#44; particularly heart transplantation&#44; which should be considered in selected patients without recurrent tumor and with good pulmonary reserve&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">CP is rare&#44; and recurrent CP rarer still&#44; and there are as yet no diagnostic or treatment guidelines for management of the disease&#46; There is thus a need to establish the best approach in these patients&#44; for which further studies and much research will be required&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Ethical disclosures</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Protection of human and animal subjects</span><p id="par0100" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Confidentiality of data</span><p id="par0105" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Right to privacy and informed consent</span><p id="par0110" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conflicts of interest</span><p id="par0115" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Recurrent right-sided heart failure after pericardiectomy may be caused by incomplete pericardiectomy&#44; recurrent constriction&#44; diastolic dysfunction or myocardial involvement&#46; Identifying recurrent constrictive pericarditis &#40;CP&#41; in patients who have recurring symptoms after pericardiectomy is challenging&#44; since the characteristic Doppler echocardiographic features may not be present if a portion of the ventricles are free of constricting pericardium&#44; and there are no diagnostic or treatment guidelines for management of recurrent CP&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The authors report the case of a 59-year-old man with a history of pericardiectomy for tuberculous CP in 1984&#44; admitted to our hospital with signs and symptoms of right heart failure&#46; After a complete diagnostic workup&#44; recurrent CP was diagnosed&#46; Given the scarcity of cases reported on this disease&#44; three possible therapeutic approaches are discussed&#58; a second pericardiectomy&#44; heart transplantation and medical therapy&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Insufici&#234;ncia card&#237;aca direita recorrente ap&#243;s pericardectomia pode ser causada por pericardectomia incompleta&#44; constri&#231;&#227;o recorrente&#44; disfun&#231;&#227;o diast&#243;lica ou atingimento mioc&#225;rdico&#46; A identifica&#231;&#227;o de pericardite constritiva &#40;PC&#41; recorrente ap&#243;s pericardectomia &#233; desafiante&#44; uma vez que&#44; muitas das caracter&#237;sticas da constri&#231;&#227;o podem estar ausentes e n&#227;o existem ainda <span class="elsevierStyleItalic">guidelines</span> de diagn&#243;stico ou terap&#234;utica para a abordagem desta patologia dada a sua extrema raridade&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Os autores descrevem o caso de um homem&#44; 59 anos de idade&#44; com antecedentes de pericardectomia ap&#243;s PC tuberculosa em 1984&#44; internado para esclarecimento de um quadro cl&#237;nico dominado por sinais e sintomas de insufici&#234;ncia card&#237;aca direita&#46; Ap&#243;s estudo complementar foi diagnosticado PC recorrente&#46; Dada a escassez de casos reportados sobre esta patologia&#44; foram discutidas poss&#237;veis abordagens terap&#234;uticas nomeadamente uma segunda pericardectomia&#44; transplante card&#237;aco e terap&#234;utica m&#233;dica&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Ferreira R&#44; Gonzaga A&#44; Santos L&#44; et al&#46; Pericardite constritiva recorrente &#8211; um desafio diagn&#243;stico e terap&#234;utico&#46; Rev Port Cardiol&#46; 2015&#59;34&#58;421&#46;e1&#8211;421&#46;e5&#46;</p>"
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Revista Portuguesa de Cardiologia (English edition)
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