Background: The main etiology of constrictive pericarditis (CP) has changed from tuberculosis to therapeutic mediastinal radiation and cardiac surgery. Occult constrictive pericardial disease (OCPD) is a covert disease in which CP is manifested in a condition of volume overload. Case presentation: A 60-year-old patient with a history of thoracic radiation therapy for non-Hodgkin’s lymphoma (40 years earlier) was transferred to our hospital for treatment of repeated congestive heart failure. For a preoperative hemodynamic study, pre-hydration with intravenous normal saline (50 mL/hour) was used to manifest the pericardial disease and prevent contrast-induced nephropathy. The hemodynamic study showed a right ventricular dip-plateau pattern and discordance of right and left ventricular systolic pressures during inspiration, which was not seen in the volume-controlled state. These responses were concordant with OCPD. A pericardiectomy, aortic valve replacement, and mitral and tricuspid valve repair were performed. Postoperatively, the heart failure was controlled with standard medication. Conclusions: This case revealed a volume-induced change in hemodynamics in OCPD with severe combined valvular heart disease, which suggests the importance of considering OCPD in patients who had undergone radiation therapy 40 years before. Keywords: Occult constrictive pericardial disease, Valvular disease, Radiation therapy Background Radiation therapy induces microcirculation injury with Occult constrictive pericardial disease (OCPD) is a rare endothelial damage, neovascularization, and athero- disease characterized by a change in hemodynamics to a sclerosis, which lead to fibrosis and calcification of typical constrictive pericarditis (CP) pattern after volume valves and pericardium. Here, we report a patient with loading . The main causes of CP are idiopathic (46%), heart failure and OCPD complicated by severe valvular previous thoracic surgery (37%), and radiation therapy disease 40 years after thoracic radiation therapy for (9%) [2, 3]. The incidence of complications after thoracic non-Hodgkin’s lymphoma. radiation therapy for tumors has increased with the use of therapy for thoracic tumors. About 7–20% of patients Case presentation develop chronic pericarditis 10 or more years after radi- A 60-year-old woman presented to another hospital with ation treatment . The average onset time of valvular shortness of breath on exertion. Her family history was disease with or without symptoms after thoracic radi- unremarkable. She had undergone chemo-radiation for ation therapy is 11.5 and 16.5 years, respectively . non-Hodgkin’s lymphoma 40 years earlier and achieved complete remission. CHOP (cyclophosphamide, doxo- * Correspondence: firstname.lastname@example.org rubicin, vincristine, and prednisone) chemotherapy did Department of System Biology, Graduate School of Medical Science, not induce cardiac toxicity. Twenty years earlier, she Kanazawa University, 13-1 Takara-machi, Kanazawa, Ishikawa 920-8641, Japan Full list of author information is available at the end of the article had an anterior myocardial infarction and underwent © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Goten et al. BMC Cardiovascular Disorders (2018) 18:107 Page 2 of 5 percutaneous coronary intervention. Moderate com- effusion was seen. Although moderate mitral regurgita- bined valvular diseases (aortic regurgitation, mitral re- tion, and tricuspid insufficiency were seen at the end of gurgitation, and tricuspid insufficiency) were also a previous hospitalization (Fig. 1a), severe mitral and followed.She hadreceivedstandardtreatment for tricuspid regurgitation were detected at our hospital chronic heart failure. However, she had suffered from admission (Fig. 1b). The septal leaflet of the tricuspid acute exacerbation of congestive heart failure several valve showed decreased mobility. The tricuspid regurgi- times beginning 2 years before and was referred to our tation pressure gradient was 45 mmHg. Systolic flow hospital because of refractory repeated congestive reversal in the hepatic veins was observed, suggesting heart failure. severe tricuspid regurgitation. Pulse doppler flow Her vital signs were blood pressure 106/43 mmHg, showed that the ratio of mitral peak velocity of early heart rate 72/min, and SPO 97% on room air, with a filling (E: 1.45 m/s) to late filling (A: 0.98 m/s) was 1.5, body mass index of 15.8 kg/m . The conjunctiva showed and the deceleration time was 211 ms. The ratio of mi- no signs of anemia or jaundice. The first and second tral peak velocity of early filling (E) to early diastolic heart sounds were normal, but the third one was in- mitral annular velocity (E’: 6.9 cm/s) was 21. The early creased. There was a pan-systolic murmur at the cardiac diastolic left ventricular filling velocity was reduced by apex (Levine III/VI). There were no rales in the lungs. 27% during inspiration. These findings indicate the pos- The abdomen was flat and soft and the liver was pal- sibility of CP and/or restrictive cardiomyopathy. The pated two finger widths below the ribs. There was no inferior vena cava was enlarged to 22 mm and the re- edema, ascites or coldness of the limbs. She had hepato- spiratory fluctuation was decreased. We diagnosed megaly and jugular vein distention remarkable at inspir- acutely developed congestive heart failure due to severe ation, also known as “Kussmaul’s sign”. mitral and tricuspid regurgitation, which was not ob- Laboratory tests revealed mild renal and liver dysfunc- served at the end of hospitalization at the previous hos- tion, and the brain natriuretic peptide (BNP) level was pital. The patient was prescribed additional diuretics to 492 pg/mL. There were no signs of an inflammatory re- reduce volume overload. sponse, anemia, or thyroid dysfunction. Chest X-ray Computed tomography revealed a mild pericardial showed a cardiothoracic ratio of 47% and no evidence of thickening of 4 mm in front of the right ventricle (Fig. 2). pleural effusion, pulmonary congestion, or pericardial Considering the history of radiation therapy, it was calcification. The electrocardiogram showed a pulmon- likely that the patient was suffering from concealed ary P wave, high left-side voltages, poor R progression, pericardial disease. and ST changes in V and V . Prior to the cardiac catheter examination, normal 5 6 Echocardiography showed decreased motion of the left saline was infused at a rate of 50 mL/h for 12 h to anterior septum and the left ventricular ejection frac- manifest the pericardial disease and to protect renal tion (LVEF) was 42%. There was bilateral atrial enlarge- function from the contrast medium. Coronary angiog- ment, but no left ventricular dilatation. No pericardial raphy showed that the coronary arteries were intact and Fig. 1 Color-Doppler Echocardiography of the mitral and tricuspid valves. There was a worsening of both mitral and tricuspid regurgitation in the last hospital admission (b) compared to at the end of previous hospitalization (a). RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle Goten et al. BMC Cardiovascular Disorders (2018) 18:107 Page 3 of 5 after loading of the patient with fluids, we revealed the diagnosis of OCPD. Pericardiectomy, aortic valve replacement, and mitral and tricuspid valvuloplasty were performed. At surgery, there was a hard, thickened pericardium, and very mild pericar- dial adhesion. Histological examination of the resected peri- cardium revealed hyaline-like fibrous hypertrophy. Twelve months postoperatively, there was no acute ex- acerbation of the congestive heart failure. The New York Heart Association functional class had also improved from III to II. On echocardiography, although mild tricuspid and mitral regurgitation remained, the size of both the left and right atria decreased. The percent difference in E-velocity between expiration and inspiration was improved to 12% Fig. 2 Computed tomography showed moderate pericardial after pericardiectomy, compared to 27% before. thickening. (yellow arrow) Discussion and conclusions there was a stent in the left anterior descending artery. To the best of our knowledge, this is the first report of The right atrial, end diastolic right ventricular, diastolic OCPD emerging in a patient who had undergone radi- pulmonary artery, and pulmonary capillary wedge pres- ation therapy 40 years ago. The patient was considered sures were 15 mmHg. This finding was not seen in a right to be suffering from radiation-induced pericardial dis- heart pressure study performed previously in hospital ease as well as valvular heart disease. Veinot et al. re- about 6 months ago (Fig. 3a). In the right atrial pressure ported that the frequency of pericardial disease was as waveform there were prominent x and y descents. The high as 70% when thoracic surgery was performed in pa- right ventricular systolic pressure exceeded 40 mmHg and tients with radiotherapy . This implies that some showed a dip-plateau pattern (Fig. 3b). In the simultaneous cases of CP might not be diagnosed in routine practice. pressure measurement of the left and right ventricle, the Bush et al. reported that intravenous drips were useful difference in end-diastolic pressure was within 5 mmHg. for detecting OCPD . They rapidly injected 1000 mL At maximum inspiration, the systolic pressure of both ven- of warmed normal saline intravenously over 6 to 8 min tricles was dissociated, and showed mirror-image discord- and performed right heart catheterization. In our case, ance (Fig. 4). Based on these changes in hemodynamics we applied non-rapid volume overload because of the Fig. 3 Right heart catheterization showed a normal pressure pattern in a previous pressure study (a). In the current study, the right ventricular systolic pressure exceeded 40 mmHg and there was a dip and plateau pattern (b). RA, right atrium; RV, right ventricle Goten et al. BMC Cardiovascular Disorders (2018) 18:107 Page 4 of 5 Fig. 4 The systolic pressure was dissociated between the two ventricles and showed mirror-image discordance consistent with constrictive pericarditis. RV, right ventricle; LV, left ventricle adverse effects on heart failure. However, a constrictive left ventricular enlargement . In our case, increased physiology pattern was manifested, even using this slow mitral regurgitation was not accompanied by an increase volume injection. Our findings suggest that the volume in left ventricular end diastolic diameter. It may be also of injection should be different among patients with important point to suspect OCPD if left ventricular congestive heart failure who suspected OCPD. dilatation is not observed in a patient with worsening A previous report demonstrated that valvular disease valvular disease. is seen in about 15% of patients within 40 years after Although there are no recommended therapies to date radiation therapy for Hodgkin’s lymphoma. Valvular for OCPD, the treatment generally follows the guidelines diseaseismorefrequentwithradiation doses ≥30 Gy, for CP . In our case, a pericardiectomy was done be- and the frequency of valvular disease increases with cause it was necessary to perform surgery for severe time and radiation dose . Although the underlying combined valvular heart disease. The European Society mechanisms are unclear, aortic and mitral valve dis- for Cardiology guidelines state that the mortality rate of eases are common, while tricuspid and pulmonary valve pericardiectomy for CP is as high as 6–12% per year. disease are infrequent. The major complications of pericardiectomy are acute Right ventricular pressure waveform is important to de- heart failure and heart rupture . Busch et al. reported termine the accurate diagnosis for heart failure. However, that reduced LVEF and right ventricular dilatation were in some cases of heart failure, the right ventricular wave- independent predictors of early mortality, whereas cor- form often shows a dip-plateau pattern on the right heart onary artery disease, chronic obstructive pulmonary dis- catheterization. Both right and left ventricular pressures ease and renal insufficiency were risk factors for late should be measured simultaneously to distinguish be- mortality . Our case showed reduced LVEF (42%) tween heart failure and CP. Hurrell et al. demonstrated and myocardial infarction, which may be related to the that the right and left ventricular pressures in heart failure mortality. Our patient had a satisfactory clinical course decrease concordantly during inspiration, while the left with no heart failure at 12 months postoperatively. ventricular pressure decreases and the right ventricular We experienced a rare case of OCPD with severe pressure increases during inspiration in CP . Therefore, combined valvular heart disease 40 years after thoracic simultaneous both ventricular pressure measurement is radiation therapy for non-Hodgkin’s lymphoma. In needed in patients with refractory heart failure with a his- this case, OCPD emerged due to volume overload. tory of chest surgery or radiotherapy. Therefore, we should consider OCPD in patients who In decompensated heart failure, chronic mitral regur- have undergone mediastinal radiation therapy, even gitation generally deteriorates in response to prominent decades later. Goten et al. BMC Cardiovascular Disorders (2018) 18:107 Page 5 of 5 Abbreviations 9. William HG, Theo EM. Left ventricular response to mitral regurgitation. CP: Constrictive pericarditis; LA: Left atrium; LV: Left ventricle; OCPD: Occult Circulation. 2008;118:2298–303. constrictive pericardial disease; PISA: Proximal isovelocity surface area; 10. Maisch B, Seferović PM, Ristić AD, Erbel R, Rienmüller R, Adler Y, et al. RA: Right atrium; RV: Right ventricle; TRPG: Tricuspid regurgitation pressure Guidelines on the Diagnosis and Management of Pericardial Diseases. Eur gradient Heart J. 2004;25:587–610. 11. Busch C, Penov K, Amorim PA, Garbade J, Davierwala P, Schuler GC, et al. Risk factors for mortality after pericardiectomy for chronicconstrictivepericarditis Availability of data and materials in a large single-centre cohort. Eur J Cardiothorac Surg. 2015;48:110–6. All of the data supporting the conclusions are contained within the manuscript. Authors’ contributions CG cared for the patient, analyzed the data, and drafted the manuscript. HM analyzed and interpreted the data, and drafted and made critical revisions to the manuscript with respect to intellectual content. ST, TK, SU, HF, TS, SS, HT, SK and MT critically revised the manuscript with respect to intellectual content. All authors read and approved the final manuscript. Ethics approval and consent to participate Not applicable. Consent for publication Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent form is available for review by the Editor of this journal. Competing interests The authors declare that they have no competing interests. Publisher’sNote Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Author details Department of System Biology, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa, Ishikawa 920-8641, Japan. Department of Cardiology, National Hospital Organization, Kanazawa Medical Center, 1-1 Shimoishibiki-machi, Kanazawa, Ishikawa 920-8650, Japan. Department of Thoracic, Cardiovascular and General Surgery, Kanazawa University, 13-1 Takara-machi, Kanazawa, Ishikawa 920-8641, Japan. Received: 3 March 2018 Accepted: 24 May 2018 References 1. Bush CA, Stang JM, Wooley CF, Kilman JW. Occult constrictive pericardial disease. Diagnosis by rapid volume expansion and correction by pericardiectomy. Circulation. 1977;56:924–30. 2. Ling LH, Oh JK, Schaff HV, Danielson GK, Mahoney DW, Seward JB, et al. Constrictive pericarditis in the modern era. Evolving clinical Spectrum and impact on outcome after Pericardiectomy. Circulation. 1999;100:1380–6. 3. Bertog SC, Thambidorai SK, Parakh K, Schoenhagen P, Ozduran V, Houghtaling PL, et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol. 2004;43:1445–52. 4. Groarke JD, Nguyen PL, Nohria A, Ferrari R, Cheng S, Moslehi J. Cardiovascular complications of radiation therapy for thoracic malignancies: the role for non-invasive imaging for detection of cardiovascular disease. Eur Heart J. 2014;35:612–23. 5. Carlson RG, Mayfield WR, Normann S, Alexander JA. Radiation-associated valvular disease. Chest. 1991;99:538–45. 6. Veinot JP, Edwards WD. Pathology of radiation-induced heart disease: a surgical and autopsy study of 27 cases. Hum Pathol. 1996;27:766–73. 7. Cutter DJ, Schaapveld M, Darby SC, Hauptmann M, van Nimwegen FA, Krol AD, et al. Risk for valvular heart disease after treatment for Hodgkin lymphoma. J Natl Cancer Inst. 2015;23:107. 8. Hurrell DG, Nishimura RA, Higano ST, Appleton CP, Danielson GK, Holmes DR Jr, et al. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circulation. 1996;93:2007–13.
BMC Cardiovascular Disorders – Springer Journals
Published: May 31, 2018
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