Journal articles: 'Primary heart involvement'

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Allanore, Yannick, and Andre Kahan. "Primary Heart Involvement in Systemic Sclerosis." Current Rheumatology Reviews 2, no. 3 (August 1, 2006): 245–49. http://dx.doi.org/10.2174/157339706778019593.

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Kumar, Susheel. "Skin Involvement in Primary Systemic Amyloidosis." Mediterranean Journal of Hematology and Infectious Diseases 5, no. 1 (January 2, 2013): e2013005. http://dx.doi.org/10.4084/mjhid.2013.005.

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Primary systemic amyloidosis is a rare disease. It primarily involves kidney, heart, peripheral nerves and liver. Intracutaneous hemorrhage manifesting in the form of petechiae, purpura and ecchymoses due to infiltration of blood vessel walls by amyloid deposits are the most common skin lesions. We report a case of primary systemic amyloidosis with multiple, non-itchy, papular lesions in lower eyelids and lower chest wall bilaterally. Diagnosis was confirmed in this case by biopsy of skin lesions using congo red staining. Papular eruptions as seen in index patient are relatively uncommon form of skin manifestations.

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Ross, Laura, David Prior, Susanna Proudman, Alessandra Vacca, Murray Baron, and Mandana Nikpour. "Defining primary systemic sclerosis heart involvement: A scoping literature review." Seminars in Arthritis and Rheumatism 48, no. 5 (April 2019): 874–87. http://dx.doi.org/10.1016/j.semarthrit.2018.07.008.

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Flores-Umanzor, Eduardo Josué, Paula Sánchez-Somonte, Ana García-Álvarez, and Marta Farrero. "Heart failure due to primary cardiac involvement in systemic sclerosis." Cardiocore 53, no. 1 (January 2018): 42–44. http://dx.doi.org/10.1016/j.carcor.2016.10.001.

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Trukhin, Ivan V., Dmitrij Yu Schekochikhin, Nina A. Novikova, Anna S. Akselrod, Abram L. Syrkin, Helena A. Syrkina, Alexander E. Grachev, et al. "Heart involvement in AL-amyloidosis. Current state of the issue." Annals of the Russian academy of medical sciences 74, no. 5 (December 4, 2019): 307–16. http://dx.doi.org/10.15690/vramn1184.

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AL cardiac amyloidosis is a relatively rare disorder that belongs to the group of infiltrative cardiomyopathies. Diagnosis of primary amyloidosis is challenging due to many unspecific symptoms and sings, which often leads to late diagnosis when treatment options are limited. Primary amyloidosis particularly needs to be excluded in patients with heart failure with preserved ejection fraction. Therapy in cardiac amyloidosis has to main vectors: 1) chemotherapy to eliminate amyloidogenic plasmatic cells 2) heart failure treatment. The main challenge for cardiologists is to support hemodynamics until response to chemotherapy occurs. In the article the issue of diagnostics, risk stratification and treatment of primary cardiac amyloidosis is addressed.

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Bruni, Cosimo, Maya H. Buch, Daniel E. Furst, Giacomo De Luca, Aleksandra Djokovic, Raluca B. Dumitru, Alessandro Giollo, et al. "Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition." Journal of Scleroderma and Related Disorders 7, no. 1 (October 26, 2021): 24–32. http://dx.doi.org/10.1177/23971983211053246.

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Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5–300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46–1.00) for the first round and 0.55 (0.44–1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47–1.00)). Criterion validity showed a 78 (73–84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.

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Boldueva, S. A., I. V. Yarmosh, N. S. Shvets, and V. V. Grokhotova. "Primary systemic amyloidosis with involvement of the heart valued like hypertrophic cardiomyopathy." Kreativnaya kardiologiya 12, no. 3 (2018): 275–83. http://dx.doi.org/10.24022/1997-3187-2018-12-2-275-283.

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Kaderli, A. A., I. Baran, O. Aydin, M. Bicer, T. Akpinar, F. Ozkalemkas, D. Yesilbursa, and S. Gullulu. "Diffuse involvement of the heart and great vessels in primary cardiac lymphoma." European Journal of Echocardiography 11, no. 1 (September 15, 2009): 74–76. http://dx.doi.org/10.1093/ejechocard/jep111.

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Rogowitz, Elisa, Hani M. Babiker, Ravitharan Krishnadasan, Clint Jokerst, Thomas P. Miller, and Michael Bookman. "Heart of Lymphoma: Primary Mediastinal Large B-Cell Lymphoma with Endomyocardial Involvement." Case Reports in Oncological Medicine 2013 (2013): 1–4. http://dx.doi.org/10.1155/2013/814291.

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Primary mediastinal B-cell lymphoma (PMBCL) is an uncommon aggressive subset of diffuse large B-cell lymphomas. Although PMBCL frequently spreads locally from the thymus into the pleura or pericardium, it rarely invades directly through the heart. Herein, we report a case of a young Mexican female diagnosed with PMBCL with clear infiltration of lymphoma through the cardiac wall and into the right atrium and tricuspid valve leading to tricuspid regurgitation. This was demonstrated by cardiac MRI and transthoracic echocardiogram. In addition, cardiac MRI and CT scan of the chest revealed the large mediastinal mass completely surrounding and eroding into the superior vena cava (SVC) wall causing a collar of stokes. The cardiac and SVC infiltration created a significant therapeutic challenge as lymphomas are very responsive to chemotherapy, and treatment could potentially lead to vascular wall rupture and hemorrhage. Despite the lack of conclusive data on chemotherapy-induced hemodynamic compromise in such scenarios, her progressive severe SVC syndrome and respiratory distress necessitated urgent intervention. In addition to the unique presentation of this rare lymphoma, our case report highlights the safety of R-CHOP treatment.

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Said, Sameh M., Prabhjot Nijjar, Molly Klein, and Ranjit John. "Left bundle branch block revealing a primary small bowel carcinoid metastasizing to the interventricular septum." Interactive CardioVascular and Thoracic Surgery 31, no. 3 (August 13, 2020): 408–10. http://dx.doi.org/10.1093/icvts/ivaa099.

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Abstract Carcinoid tumours of the heart occur most commonly as a result of metastatic disease and usually affect the right side of the heart. We report a case of a solitary carcinoid metastasis to the interventricular septum without hepatic involvement in a 74-year-old man.

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Ikic, A., E. Chatelus, E. Epailly, H. Kremer, J. E. Gottenberg, J. L. Pasquali, and J. Sibilia. "AB0607 Heart Transplantation in 2 Patients with Systemic Sclerosis and Primary Cardiac Involvement." Annals of the Rheumatic Diseases 73, Suppl 2 (June 2014): 1006.3–1007. http://dx.doi.org/10.1136/annrheumdis-2014-eular.3894.

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Agarwal, Chirag, Sunny Goel, Eric Stern, Richard Warner, Javier Castillo, Lori Croft, Ronald Lavine, and Jerome Zacks. "Carcinoid Heart Disease without Liver Involvement Caused by a Primary Ovarian Carcinoid Tumour." Heart, Lung and Circulation 24, no. 7 (July 2015): e97-e100. http://dx.doi.org/10.1016/j.hlc.2015.02.014.

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Bachour, Kinan, Sagar Shah, Jennifer Chia, David Shabsovich, Allison Reichl, Adrian Castillo, and Geoffrey Weiting Cho. "PRIMARY CARDIAC LYMPHOMA WITH LOCOREGIONAL LYMPH NODE INVOLVEMENT PRESENTING WITH COMPLETE HEART BLOCK." Journal of the American College of Cardiology 81, no. 8 (March 2023): 3888. http://dx.doi.org/10.1016/s0735-1097(23)04332-2.

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Neu, Rachel, Melvin A. Leonard, Michelle L. Dehoorne, Samantha J. Scalia, Pramodini B. Kale-Pradhan, and Christopher A. Giuliano. "Impact of Pharmacist Involvement in Heart Failure Transition of Care." Annals of Pharmacotherapy 54, no. 3 (October 11, 2019): 239–46. http://dx.doi.org/10.1177/1060028019882685.

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Background: Heart failure (HF) transition of care (TOC) programs may improve continuity of care and coordination and decrease hospital readmissions. Objective: This study evaluated the impact of pharmacy-led HF TOC on HF readmission rate. Methods: This was a single-center, pre-post quasi-experimental study. Pharmacy TOC comprised admission and discharge medication reconciliations and patient education. Patients were included if they had a primary HF diagnosis. Patients were excluded if they were admitted for a non-HF diagnosis, admitted for <24 hours, had a stage IV cancer or dementia diagnosis, or were transferred to hospice care. The primary outcome was HF 30-day readmission rate. Results: A total of 663 patients were included in the study: 330 in the control group and 333 in the intervention group. The average age for both groups was 67 ± 16 years; 48.1% were female; 56.9% were African American; and 51.4% of patients had an ejection fraction ≤40%. In the control group, 57 (17.3%) patients had a HF 30-day readmission compared with 35 (10.5%) patients in the intervention group. After adjusting for age, the intervention group continued to show a difference in readmission (odds ratio = 0.578; 95% CI = 0.367-0.911; P = 0.018). The most common interventions were medication addition (11%), dose titration (7.5%), medication discontinuation (6.6%), and duplication avoidance (2.7%). Conclusion and Relevance: Pharmacy-led HF TOC, as a component of a targeted hospital-based initiative, significantly decreased HF 30-day readmission rate. Results from this study warrant further research to explore which interventions in TOC are most effective.

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Dorobantu, Lucian Florin, Ovidiu Stiru, Anca Prodea, Rodica Cioranu, Alina Georgescu, Daniela Filipescu, and Vlad Anton Iliescu. "Unique Case of Primary Malignant Fibrous Histiocytoma of the Right Ventricle with Moderator Band Involvement." Heart Surgery Forum 14, no. 4 (August 22, 2011): 245. http://dx.doi.org/10.1532/hsf98.20101154.

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Malignant primary tumors of the heart are very rare and in most cases are located in the left side of the heart, but involvement of the moderator band of the right heart has not yet been described in the literature. We report the case of a 22-year-old woman who presented after an episode of transient sudden loss of consciousness. The transthoracic echocardiography (TTE) and transesophageal echocardiography evaluations showed an echogenic mass that originated in the right ventricle and protruded into the tricuspid valve during systole. While operating on the patient, we found a sessile tumor that originated exclusively from the moderator band of the right ventricle. The tumor was completely resected through the tricuspid valve. An immunohistochemistry examination of the tumor confirmed the diagnosis of a malignant fibrous histiocytoma (MFH) (undifferentiated pleomorphic sarcoma). To our knowledge, this case is the first of moderator band involvement among the 50 cases of primary MFH of the heart that have been reported in the literature. After a year and a half, TTE, computed tomography (CT), and positron emission tomography CT (PET-CT) showed the absence of cardiac tumor, a normal tricuspid function, and no metastasis. The completeness of the surgical resection and the absence of local recurrence >1 year after surgery are encouraging signs for the patient's outcome.

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Praena-Segovia, J., A. Sanchez-Gastaldo, M. Bernabeu-Wittel, R. Ocete-Pérez, R. Ávila-Polo, and M. L. Martino. "Primary Systemic Amyloidosis and High Levels of Angiotensin-Converting Enzyme: Two Case Reports." Case Reports in Cardiology 2013 (2013): 1–5. http://dx.doi.org/10.1155/2013/976379.

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Infiltrative heart diseases are caused by a heterogeneous group of disorders; amyloidosis and sarcoidosis are two frequent causes of myocardial infiltration, which differ in clinical and biological outcome and treatment issues. The presence of high levels of angiotensin-converting enzyme (ACE) in a patient with infiltrative heart disease may increase suspicion of sarcoidosis. Nevertheless, no mention about increased ACE levels in extracerebral primary systemic amyloidosis is available. We present two cases of primary systemic amyloidosis, which are cardiac involvement and elevated ACE levels.

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Demko, I. V., L. I. Pelinovskaya, V. A. Mosina, A. Y. Kraposhina, N. V. Gordeeva, I. A. Solovyova, and A. E. Ryazanov. "CASE OF PRIMARY AMYLOIDOSIS WITH INVOLVEMENT OF THE HEART AND PERICARDIUM IN CARDIOLOGY PRACTICE." Russian Journal of Cardiology, no. 2 (January 1, 2017): 103–5. http://dx.doi.org/10.15829/1560-4071-2017-2-103-105.

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Kholopov, L. S., N. B. Gubegrits, O. A. Dyadyk, Yu E. Chirkov, and Yu V. Tsohoyeva. "A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement." Russian Journal of Gastroenterology, Hepatology, Coloproctology 31, no. 6 (March 7, 2022): 47–55. http://dx.doi.org/10.22416/1382-4376-2021-31-6-47-55.

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Aim. Systemic amyloidosis caused by the synthesis and deposition of immunoglobulin light chains (AL amyloidosis) is a relatively rare disease that involves heart, kidneys, peripheral nervous system, gastrointestinal tract, and has a large number of various clinical manifestations. We present a clinical case of systemic AL amyloidosis with a predominant involvement of liver, stomach, intestines, and heart in a Caucasian female.Key points. A Caucasian woman presented to clinic with severe general weakness, abdominal pain, diarrhea, sudden weight loss, and palpitation. Initial examination revealed a duodenal bulb ulcer complicated by bleeding and polyps in the retrobulbar part of duodenum. Decreased hemoglobin levels, elevated levels of alkaline phosphatase, gamma-glutamyltransferase, and N-terminal prohormone of brain natriuretic peptide, signs of heart failure with preserved ejection fraction, and hepatomegaly became the basis for a clinical suspicion of AL amyloidosis and puncture liver biopsy. Histochemical and immunohistochemical studies of liver, stomach, and duodenum biopsy specimens confirmed AL amyloidosis. Timely diagnosis made it possible to conduct a specific therapy with melphalan plus dexamethasone, get a satisfactory response and improve the patient’s condition.Conclusion. A thorough examination of patients along with a pathomorphological and immunohistochemical study of the biopsy specimens is the basis for confirming the diagnosis of AL amyloidosis, selecting the proper therapy, improving the condition of patients and their survival.

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Ainscough, S., AM Power, and AN Brown. "Mantle cell lymphoma: primary oral presentation." Annals of The Royal College of Surgeons of England 99, no. 1 (January 2017): e13-e14. http://dx.doi.org/10.1308/rcsann.2016.0267.

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Mantle-cell lymphoma is an uncommon lymphoid malignancy of B-cells. It is often aggressive and prognosis is poor. A 69-year-old gentleman with a history of ischaemic heart disease was referred from primary care with a painless right floor of mouth swelling that had been present for 1 month. He otherwise completely asymptomatic. Incisional biopsy of the lesion was undertaken and marker studies demonstrated mantle cell lymphoma. Positron emission tomography–computed tomography and bone marrow biopsy showed widespread but low volume involvement. The patient was referred to the haematology multidisciplinary team for further assessment and treatment.

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de la Fuente, Pablo, Kapil Rajwani, Iñigo Sistiaga, and Jose Pedro Lavrador. "Surgery on a Primary Cranial Vault Lymphoma." Neuro-Oncology 24, Supplement_4 (October 1, 2022): iv15. http://dx.doi.org/10.1093/neuonc/noac200.065.

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Abstract AIMS Unique cranial involvement in Lymphomas is extraordinary and when it occurs, it is usually accompanied by systemic involvement. The role of the surgery in these cases is still unclear. METHOD Case report of a 72-year-old female with a primary cranial vault lymphoma with primary surgical management at time of recurrence. RESULTS 72-year-old female, with a background of treated breast cancer and ischemic heart, presented with a right parieto-occipital lytic bone lesion with a soft tissue mass with a diagnosis of Non-Hodgkin’s Lymphoma Type B, compatible with follicular lymphoma, with no other symptoms Systemic involvement was ruled out in PET CT bone marrow biopsy. First-line chemotherapy was precluded due to the cardiotoxicity, and she was started on Rituximab-Bendamustine and radiotherapy. PET-CT one year later shows local progression with lytic destruction. The MRI showed an imprint on the brain and a lesion in intimate contact with the superior longitudinal sinus. As an apparently localized lesion and the patient could not receive the optimal chemotherapy treatment, a craniectomy was proposed. Fifteen months after surgery the patient is free of disease on PET CT. CONCLUSION Primary cranial lymphoma is an unusual entity which precludes clear algorithms. The primary treatment is the chemotherapy, nonetheless, If the patient cannot receive the optimal treatment due to his background or in the presence of recurrent disease with limited treatment options, surgery should be considered if technically plausible and adequate to patient’s performance status.

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Buleu, Florina, Elena Sirbu, Alexandru Caraba, and Simona Dragan. "Heart Involvement in Inflammatory Rheumatic Diseases: A Systematic Literature Review." Medicina 55, no. 6 (June 6, 2019): 249. http://dx.doi.org/10.3390/medicina55060249.

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Introduction: Patients with inflammatory rheumatic diseases have an increased risk of developing cardiovascular manifestations. The high risk of cardiovascular pathology in these patients is not only due to traditional cardiovascular risk factors (age, gender, family history, smoking, sedentary lifestyle, cholesterol), but also to chronic inflammation and autoimmunity. Aim: In this review, we present the mechanisms of cardiovascular comorbidities associated with inflammatory rheumatic diseases, as they have recently been reported by different authors, grouped in electrical abnormalities, valvular, myocardial and pericardial modifications and vascular involvement. Methods: We conducted a systematic search of published literature on the following online databases: EBSCO, ScienceDirect, Scopus and PubMed. Searches were limited to full-text English-language journal articles published between 2010 and 2017 using the following key words: heart, systemic inflammation, autoimmunity, rheumatic diseases and disease activity. After the primary analysis we included 50 scientific articles in this review. Results: The results showed that cardiac manifestations of systemic inflammation can occur frequently with different prevalence in rheumatoid arthritis (RA), systemic lupus erythematosus(SLE), systemic sclerosis(SSc) and ankylosing spondylitis(AS). Rheumatologic diseases can affect the myocardium, cardiac valves, pericardium, conduction system and arterial vasculature. Conclusions: Early detection, adequate management and therapy of specific cardiac involvement are essential in rheumatic disease. Electrocardiographic and echocardiographic evaluation should be performed as routine investigations in patients with inflammatory rheumatic diseases.

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Park, Joon Young, Joo-Young Na, Young-Il Park, Seon Jung Jang, and Yong-Han Jung. "Cardiac Amyloidosis in Autopsy Case of Sudden Unexpected Death." Korean Journal of Legal Medicine 47, no. 1 (February 28, 2023): 14–20. http://dx.doi.org/10.7580/kjlm.2023.47.1.14.

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A 78-year-old woman with a medical history of hypertension and diabetes mellitus who underwent surgery for lumbar stenosis died of sudden cardiac arrest two days after the operation. An autopsy was performed; however, the cause of death was not identified macroscopically. Congo red staining detected amyloid deposits in the systemic organs, including the heart, lungs, liver, thyroid, and kidney. Immunohistochemical staining revealed an immunoglobulin lambda light chain, which can cause the primary form of systemic amyloidosis. The prognosis of patients with systemic amyloidosis is directly associated with cardiac involvement. In this case, amyloid formation was noted in the myocardial interstitium and intramyocardial vascular wall, which caused luminal narrowing, subsequently causing arrhythmia and ischemic heart disease in each tissue, respectively. We present a case of primary systemic amyloidosis with severe cardiac involvement that was diagnosed after a comprehensive postmortem examination.

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Michail, Michalis, Efstathios Kastritis, Sossana Delimpassi, Marie Christine Kyrtsonis, Evridiki Michali, Nicolaos Anagnostopoulos, Konstantinos Zervas, et al. "Clinical Characteristics and Outcome of Primary AL Amyloidosis in Greece." Blood 110, no. 11 (November 16, 2007): 4729. http://dx.doi.org/10.1182/blood.v110.11.4729.4729.

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Abstract Introduction: Primary systemic amyloidosis (AL) is a clonal plasma cells disorder characterized by deposition of amyloid fibrils derived from abnormal light chains, leading to multiorgan involvement and failure. There is no information regarding the clinical, laboratory, treatment characteristics and outcome of such patients in Greece. We performed a retrospective analysis in order to clarify these issues. Patients and Methods: Diagnosis of primary AL amyloidosis was based on positive Congo red staining, immunohistochemistry and the presence of typical clinical and laboratory features. Definition of organ involvement and treatment response was based on established criteria (Gertz et al Am J Hematol 2005). Results: between 1995 and 2007, we identified 109 patients with previously untreated systemic AL amyloidosis. Median age was 66.3 years; 51% were males and lambda-light chain was involved in 74% of patients. Bone marrow biopsy stained positive for Congo-red in 56.5%, immunohistochemical staining was performed in 80 cases: 63 (78.75%) stained positive for λ and 17 for κ light chains. A monoclonal protein by immunofixation was found in the serum and/or urine of 97 (87%) patients. More than 10% bone marrow plasma cells were found in 65%. B2microglobulin was elevated in 36% of patients (median value 2.8 mg/l). The most frequent symptoms at presentation were fatigue and weakness (81%). Heart was involved in 66 (59%), kidney in 79 (71%), liver in 21(19%), GI tract in 17 (16%) and soft tissue in 35 (32%) patients respectively. Symptoms of peripheral and/or autonomic neuropathy were present in 38 (35%) patients. More than two organs were involved in 50 patients (45%). Primary treatment with high-dose dexamethasone based regimens (VAD or pulse Dexamethasone) was used in 45% while 37% of patients were treated with melphalan and prednisone. Six patients (5%) were treated upfront with high dose melphalan and ASCT while another 6 patients were transplanted at a later stage of their disease. Hematologic response was achieved in 50 (46%) including 16 (14.5%) patients who achieved a CR. Organ responses were seen in 32 (29%) patients: 4 had cardiac, 21 renal and 7 liver response respectively while 11 patients had subjective improvement of peripheral or autonomic neuropathy. Median survival from initiation of treatment was 61 months and the 5 year-survival was 44%. Patients with heart involvement or with more than 2 affected organs had a worse prognosis. Survival was significantly longer for patients who responded to primary treatment than for those who did not (p=0.018). Conclusions: Greek patients with AL amyloidosis share the same characteristics with that of patients from other reported studies. Hematologic responses were noted in one-half and organ responses in one-third of patients. Prognosis depended primarily on the presence of heart involvement and on the lack of response to treatment.

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Gorkova, N. B., L. M. Starykh, and L. E. Karpova. "A case of early pelvic-brachial progressive muscular dystrophy with severe heart involvement." Kazan medical journal 72, no. 2 (February 15, 1991): 142–44. http://dx.doi.org/10.17816/kazmj106605.

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Progressive muscular dystrophies (PMD) are a group of hereditary diseases characterized by a primary dystrophic process in muscle tissue. The peculiarity and characteristics of cardiac disorders depend both on the nosological form with a characteristic muscle pathology for each of them, and on the stage, severity of the course of the disease and the mass of the affected muscles, and circulatory failure is less pronounced than damage to skeletal muscles.

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Parmar, Manpreet K., Mariam Alikhan, Vivien M. Hsu, and Amanda Borham. "Echocardiogram: The GPS to GPA’s Heart (Granulomatosis with Polyangiitis)." Case Reports in Rheumatology 2019 (January 16, 2019): 1–3. http://dx.doi.org/10.1155/2019/7609386.

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Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated necrotizing granulomatous vasculitis affecting small- to medium-sized vessels. Common manifestations of this disease process affect the ear, nose, throat, upper and lower airways, and kidneys. Cardiac involvement has been reported in 6–44% of patients, primarily as coronary arteritis and pericarditis. A majority of case reports of pericardial effusions in patients with GPA identify patients having constrictive pericarditis secondary to uremia. We are presenting a case of hemorrhagic pericarditis in a patient with GPA in which the underlying inflammatory vasculitis likely played the primary role in the patient’s presentation. Echocardiographic abnormalities have been found in 80% of patients with GPA. Given the high mortality from cardiac involvement in patients with GPA, screening echocardiograms for this patient population may serve as a helpful tool in gauging disease severity, thereby guiding therapy to prevent serious cardiac complications, such as cardiac tamponade as presented in this case report.

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Zemtsovsky, E. V., E. G. Malev, and S. V. Reeva. "SYSTEMIC INVOLVEMENT OF CONNECTIVE TISSUE AND THE HEART AS IMPORTANT CHARACTERISTICS OF PRIMARY MITRAL VALVE PROLAPSE." Russian Journal of Cardiology, no. 9 (January 1, 2014): 54–60. http://dx.doi.org/10.15829/1560-4071-2014-9-54-60.

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Turcotte‐Gosselin, Frédéric, Pierre Yves Turgeon, Alena Ikic, Jonathan Beaudoin, Philippe Joubert, Michelle Dubois, Pierre Voisine, and Mario Sénéchal. "Is heart transplantation a valuable option in patients with diffuse systemic sclerosis and primary cardiac involvement?" Clinical Case Reports 8, no. 1 (December 19, 2019): 137–41. http://dx.doi.org/10.1002/ccr3.2600.

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Melissaropoulos, Konstantinos, Dimitrios Bogdanos, Theodoros Dimitroulas, Lazaros I. Sakkas, George D. Kitas, and Dimitrios Daoussis. "Primary Sjögren’s Syndrome and Cardiovascular Disease." Current Vascular Pharmacology 18, no. 5 (August 10, 2020): 447–54. http://dx.doi.org/10.2174/1570161118666200129125320.

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Sjögren’s syndrome is a rheumatic autoimmune disease that primarily affects middle-aged women and runs a slowly progressing course with sicca symptoms being the prevalent manifestation. Premature atherosclerosis and increased cardiovascular (CV) morbidity and mortality are frequently encountered in rheumatic diseases characterized by significant systemic inflammation, such as the inflammatory arthritides, systemic vasculitides and systemic lupus erythematosus. In the same context, chronic inflammation and immune aberrations underlying Sjögren’s syndrome are also reported to be associated with augmented risk of atherosclerosis. Increased CV disease (CVD) frequency has been found in recent meta-analyses. The involvement of the CV system is not a common feature of Sjögren’s syndrome; however, specific manifestations, such as autoantibody-mediated heart block, pericarditis, pulmonary arterial hypertension and dysautonomia, have been described. This review focuses on studies addressing CV morbidity in Sjögren’s syndrome and presents current data regarding distinct CV features of the disease.

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Kim, Eunsook, Jung-Hoon Cho, Woo Sang Jung, Sanghoon Lee, and Sok Cheon Pak. "Effect of Acupuncture on Heart Rate Variability in Primary Dysmenorrheic Women." American Journal of Chinese Medicine 39, no. 02 (January 2011): 243–49. http://dx.doi.org/10.1142/s0192415x11008786.

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Primary dysmenorrhea is a common gynecological complaint among young women that is related to an autonomic nervous system (ANS) disturbance. Acupuncture is one of several therapeutic approaches for primary dysmenorrhea, since it can modulate ANS function. The heart rate variability (HRV) parameters such as high frequency (HF), low frequency (LF) and LF/HF ratio are generally accepted tools to assess ANS activity. The purpose of this study was to investigate the effects of acupuncture applied at Hegu (LI4) and Sanyinjiao (SP6) points on HRV of women with primary dysmenorrhea during the late luteal phase. The experimental design was a crossover and patient-blinded procedure. All subjects participated in Sham (SA) and Real Acupuncture (RA) procedure, separated by one month, in a crossover sequence. The participants included 38 women (mean age 22.3 years; weight 53.8 kg; height 162.6 cm). HRV measurement was 15 min before and 15 min after an acupuncture procedure. The RA procedure was performed at two bilateral acupoints, but needles were inserted subcutaneously to the acupuncture points for the SA procedure. The RA induced a significant decrease in LF/HF ratio and a significant increase in the HF power, while SA treatment caused a significant increase only in the HF power. Manual acupuncture at bilateral acupoints of LI4 and SP6 may play a role in dysmenorrhea treatment with autonomic nervous system involvement.

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Soulaidopoulos, Stergios, Anastasia-Vasiliki Madenidou, Dimitrios Daoussis, Konstantinos Melissaropoulos, Sophie Mavrogeni, George Kitas, and Theodoros Dimitroulas. "Cardiovascular Disease in the Systemic Vasculitides." Current Vascular Pharmacology 18, no. 5 (August 10, 2020): 463–72. http://dx.doi.org/10.2174/1570161118666200130093432.

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The vasculitides are a heterogeneous group of disorders, characterized by inflammatory cell infiltration and necrosis of blood vessels that cause vascular obstruction or aneurysm formation, affecting various organs such as lungs, kidneys, skin and joints. Cardiac involvement is commonly encountered in primary systemic vasculitis and it is associated with increased morbidity and mortality. Depending on the dominant pathophysiological mechanism, heart complications may manifest in different ways, including myocardial ischemia due to impaired micro- or macrovascular circulation, progressive heart failure following valvular heart disease and myocardial dysfunction, (sub) clinical myocarditis, pericarditis, pulmonary hypertension as well as arteritis of coronary vessels. Beyond cardioprotective regimens, aggressive immunosuppression reduces the inflammatory burden and modulates the progression of cardiovascular complications. Perioperative management of inflammation, when surgical treatment is indicated, improves surgical success rates and postoperative long-term prognosis. We aim to provide an overview of the pathogenetic, diagnostic and therapeutic principles of cardiovascular involvement disease in the various forms of systemic vasculitis.

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Matsumoto, Renato Augusto Eidy Kiota, Juliana Hiraoka Catani, Mirela Liberato Campoy, Arthur Magalhães Oliveira, and Nestor de Barros. "Radiological findings of breast involvement in benign and malignant systemic diseases." Radiologia Brasileira 51, no. 5 (September 21, 2018): 328–33. http://dx.doi.org/10.1590/0100-3984.2016.0125.

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Abstract Although the primary purpose of periodic mammograms in screening programs is to identify lesions suspected of being carcinomas, the findings are often related to systemic (benign or malignant) diseases, rather than breast cancer. Although the involvement of breast structures in systemic diseases is unusual, it can be included in the differential diagnosis of masses, skin changes, calcifications, asymmetry, and axillary lymphadenopathy. The main diagnostic entities that can be associated with such involvement are diabetes, chronic kidney disease, heart diseases, connective tissue diseases, HIV infection, lymphoma, leukemia, and metastases from primary tumors at other sites. In many cases, information related to knowledge and treatment of chronic diseases is not available to the radiologist at the time of evaluation of the mammography findings. The purpose of this essay is to offer relevant pictorial information to the general radiologist about systemic diseases involving the breast, expanding the range of differential diagnoses in order to avoid unnecessary invasive procedures.

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Lalchandani, Arati, Taruni Lalchandani, Lubna Ahmad, and Devarth Lalchandani. "Prophylaxis of Rheumatic Fever/Rheumatic Heart Disease–Penicillin or Azithromycin, Who Wins?" Indian Journal of Cardiovascular Disease in Women - WINCARS 06, no. 01 (January 2021): 042–45. http://dx.doi.org/10.1055/s-0041-1728220.

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AbstractFor eradication of rheumatic fever (RF)/rheumatic heart disease (RHD), we must have a simplified diagnosis, and a treatment which is painless, easily available and safe; prophylaxis must be painless, safe, easily available, readily administered, and comprising a weekly oral single dose.Arati's regime for management of RF/RHD (ARMOR) consists of diagnosis and management of RF/RHD in today's context in a very easy and simplified way.ARMOR criteria: Arthritis or arthralgia with typical features suggestive of RF, carditis or cardiac involvement, typical of RF or RHD, and echocardiographic evidence of rheumatic heart valve involvement should essentially be the criteria to diagnose RF and RHD with high specificity and sensitivity.With regard to treatment of RF/RHD, we need a drug which is highly efficacious against Group A Beta Hemolytic Streptococcus (GABHS), which is the causative agent for primary prevention and treatment and secondary prophylaxis.The best drug discovered, to date, for GABHS is azithromycin.ARMOR for primary prevention, treatment and secondary prophylaxis of RF/RHD is as follows:Azithromycin must be given in a dose of 500 mg 1 tablet daily for 5 days, followed by 1 tablet once a week for 1 year.Penicillin for treatment and prophylaxis must be given up due to its lack of availability, side effects, risk of anaphylaxis, parenteral preparation, hazards of administration, need for sensitivity test each time, etc. and replaced by azithromycin.

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Goyal, Abhishek, Bishav Mohan, Kavita Saggar, and Gurpreet Singh Wander. "Primary haemochromatosis resulting in dilated cardiomyopathy arising out of mutation in HJV gene in Indian patients: a rare scenario." BMJ Case Reports 13, no. 9 (September 2020): e235650. http://dx.doi.org/10.1136/bcr-2020-235650.

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Primary haemochromatosis (PH) is a genetic disorder of iron metabolism with multiorgan involvement due to mutations in HFE or more rarely haemojuvelin (HJV) gene. Cardiac involvement results in dilated cardiomyopathy with reduced ejection fraction and progressive heart failure. PH is rarely reported from India and cardiomyopathy due to PH from HJV mutations is thought to be uncommon. We report two families with cardiomyopathy resulting from PH. Diagnosis was suspected on the basis of skin pigmentation, markedly elevated serum ferritin and transferring saturation. Genetic testing revealed a rare mutation in HJV gene in one family. Being a treatable condition, PH should be suspected and investigated in cardiomyopathy patients in Indian subcontinent. If HFE is negative, analysis of non-HFE mutation should always be considered.

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Humagain, S., R. Gurung, P. Pant, R. Koju, and TRS Bedi. "Pattern of Valvular Involvement and Demographic Features of Patients on Injection Benzathine Penicillin at Dhulikhel Hospital." Nepalese Heart Journal 9, no. 1 (July 21, 2013): 16–19. http://dx.doi.org/10.3126/njh.v9i1.8342.

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Background Rheumatic heart disease (RHD) is the most common cardiovascular disease in children and young adults. Though declined and almost non-existent in developed nations, RHD is still one of the leading cause for premature death and disability in developing countries. Prevalence of RHD is high in both rural as well as urban area of Nepal. Present study is designed to look at the pattern of valvular involvement and demographic features in RHD. Methods 326 Patients indicated for inj. Benzathine penicillin were selected and echocardiograph performed to see the pattern of vavular involvement. Data analysis was done using SPSS 17. Result The most common type of lesion was mixed type with mitral valve involvement. MR was the most common isolated lesion. MS was more commonly seen in females whereas AS was more common in males. Secondary prophylaxis was more common than primary prophylaxis. Conclusion RHD still being a major problem and a preventable disease so extensive screening program is required to identify them early and prevent the complication. DOI: http://dx.doi.org/10.3126/njh.v9i1.8342 Nepalese Heart Journal Vol.9(1) 2012 pp.16-19

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Woudstra, Odilia I., Gerard J. J. Boink, Jacobus A. Winkelman, and Ron van Stralen. "A Rare Case of Primary Meningococcal Myopericarditis in a 71-Year-Old Male." Case Reports in Cardiology 2016 (2016): 1–3. http://dx.doi.org/10.1155/2016/1297869.

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We describe a case of primary meningococcal C pericarditis with myocardial involvement in a 71-year-old male that is thus far the oldest patient with isolated meningococcal pericardial disease and only the third patient with primary meningococcal myopericarditis described in English literature. Our patient was successfully treated by full sternotomy and surgical drainage combined with intravenous ceftriaxone. Mild symptoms unresponsive to anti-inflammatory treatment and leukocytosis may guide clinicians towards the correct diagnosis. It is important to recognize this cause of pericarditis as the relatively mild clinical presentation may rapidly progress into tamponade and right-sided heart failure.

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Mastrobuoni, S., A. M. Dell'Aquila, and J. Herreros. "FatalToxoplasma gondiiDissemination in a Heart Transplant Recipient: Description of a Case." Case Reports in Transplantation 2012 (2012): 1–3. http://dx.doi.org/10.1155/2012/524279.

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A 45-year-old heart transplant recipient presented with fever, anorexia, asthenia, and lethargy. She had received heart transplantation only 5 weeks earlier for primary systemic amyloidosis with severe cardiac involvement. Serum sodium was low, and tacrolimus through level was high. Blood cultures and serology tests for infection were negative, and atypical pneumonia was suspected. Despite broad antibiotic, antiviral, and antifungal treatment, the patient clinical condition rapidly deteriorated and she died within three days of admission. Postmortem examination revealed a disseminatedToxoplasma gondiiinfection as a result of donor (+)/recipient(−) mismatch forToxoplasmaserology. Although very rare, toxoplasmosis in heart transplant recipient should be suspect in case of neurological deficit and respiratory symptoms. Prophylaxis treatment is recommended in case of mismatch.

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Bruni, C., M. H. Buch, P. Seferovic, and M. Matucci-Cerinic. "AB0556 PRIMARY SYSTEMIC SCLEROSIS HEART INVOLVEMENT (PSSCHI): A SYSTEMATIC LITERATURE REVIEW (SLR), CONSENSUS-BASED DEFINITION AND PRELIMINARY VALIDATION." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1574.1–1575. http://dx.doi.org/10.1136/annrheumdis-2020-eular.1964.

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Background:pSScHI may cause tissue, functional and conduction abnormalities with varied clinical manifestations. The absence of a clear definition of pSScHI impairs the significance and ability of focussed research, frequently not allowing the distinction between primary and secondary involvement.Objectives:We aimed to establish an expert consensus definition for pSScHI, to be used in clinical trials and everyday clinical practice, and to start its validation process.Methods:A SLR for cardiac manifestations and alterations in SSc was conducted using PubMed, Web of Science and Embase. Articles published from inception to December 31st, 2018 were identified. Inclusion criteria included papers in English on adult SSc patients, with heart involvement as outcome. We excluded non-human studies, secondary heart involvement (eg PAH, drugs, infections), reviews and case reports. PRISMA recommendations were followed where applicable. Extracted data were categorized into relevant domains (signs, symptoms, anatomical site involved, physiological abnormalities, pathological changes, prognostic outcomes), which informed the consensus definition. Sixteen senior experts (7 rheumatologists, 8 cardiologists, 1 pathologist) discussed the data and, using a nominal group technique, added expert opinion, provided statements to consider and ranked them. Consensus was attained for agreement >70%. Sixteen clinical cases were evaluated in two rounds to test for face validity, feasibility, inter- and intra-rater reliability and criterion validity (gold standard set by agreed evaluation between expert rheumatologist, cardiologist and methodologist).Results:2593 publications were identified and screened, 251 full texts were evaluated,172 met eligibility criteria. Data from the 7 domains were extracted and used to develop the World Scleroderma Foundation – Heart Failure Association (WSF-HFA) consensus-derived definition of pSSc-HI, as follows:“pSScHI comprises cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications*. pSScHI may be sub-clinical and must be confirmed through diagnostic investigation. The pathogenesis of pSScHI comprises one or more of inflammation, fibrosis and vasculopathy. *Non SSc-specific cardiac conditions (e.g. Ischaemic heart disease, arterial hypertension, drug toxicity, other cardiomyopathy, primary valvular disease) and/or SSc non cardiac conditions (e.g. PAH, Renal involvement, ILD).”Face validity was determined by a 100% agreement on credibility; application was feasible, with a median 60 (5-600) seconds taken per case; inter rater agreement was moderate [mKappa (95%CI) 0.56 (0.46-1.00) and 0.55 (0.44-1.00) for the two rounds] and intra rater agreement was good [mKappa (95%CI) 0.77 (0.47-1,00)]. Content validity was reached based on the wide variety of patients in the SLR, criterion validity was reached with 78 (73-84) % correctness.Conclusion:Using a SLR and modified nominal technique, we have developed a preliminary pSScHI consensus-based definition and started a validation process for it to be used in clinical research and clinical practice.Acknowledgments:Aleksandra Djokovic, Giacomo De Luca, Raluca B. Dumitru,Alessandro Giollo, Marija Polovina, Yossra Atef Suliman, Kostantinos Bratis, Alexia Steelandt, Ivan Milinkovic, Anna Baritussio, Ghadeer Hasan, Anastasia Xintarakou, Yohei Isomura, George Markousis-Mavrogenis, Silvia Bellando-Randone, Lorenzo Tofani, Sophie Mavrogeni, Luna Gargani, Alida L.P. Caforio, Carsten Tschoepe, Arsen Ristic, Karin Klingel, Sven Plein, Elijah Behr, Yannick Allanore, Masataka Kuwana, Christopher Denton, Daniel E. Furst, Dinesh Khanna, Thomas Krieg, Renzo Marcolongo.Disclosure of Interests:Cosimo Bruni Speakers bureau: Actelion, Eli Lilly, Maya H Buch Grant/research support from: Pfizer, Roche, and UCB, Consultant of: Pfizer; AbbVie; Eli Lilly; Gilead Sciences, Inc.; Merck-Serono; Sandoz; and Sanofi, Petar Seferovic: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim

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Feige, Katharina, Janine Rubbert, Annika Raupach, Martin Stroethoff, André Heinen, Markus W. Hollmann, Ragnar Huhn, and Carolin Torregroza. "Cardioprotective Properties of Mannitol—Involvement of Mitochondrial Potassium Channels." International Journal of Molecular Sciences 22, no. 5 (February 27, 2021): 2395. http://dx.doi.org/10.3390/ijms22052395.

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Cardiac preconditioning (PC) and postconditioning (PoC) are powerful measures against the consequences of myocardial ischemia and reperfusion (I/R) injury. Mannitol—a hyperosmolar solution—is clinically used for treatment of intracranial and intraocular pressure or promotion of diuresis in renal failure. Next to these clinical indications, different organ-protective properties—e.g., perioperative neuroprotection—are described. However, whether Mannitol also confers cardioprotection via a pre- and/or postconditioning stimulus, possibly reducing consequences of I/R injury, remains to be seen. Therefore, in the present study we investigated whether (1) Mannitol-induced pre- and/or postconditioning induces myocardial infarct size reduction and (2) activation of mitochondrial ATP-sensitive potassium (mKATP) channels is involved in cardioprotection by Mannitol. Experiments were performed on isolated hearts of male Wistar rats via a pressure controlled Langendorff system, randomized into 7 groups. Each heart underwent 33 min of global ischemia and 60 min of reperfusion. Control hearts (Con) received Krebs–Henseleit buffer as vehicle only. Pre- and postconditioning was achieved by administration of 11 mmol/L Mannitol for 10 min before ischemia (Man-PC) or immediately at the onset of reperfusion (Man-PoC), respectively. In further groups, the mKATP channel blocker 5HD, was applied with and without Mannitol, to determine the potential underlying cardioprotective mechanisms. Primary endpoint was infarct size, determined by triphenyltetrazolium chloride staining. Mannitol significantly reduced infarct size both as a pre- (Man-PC) and postconditioning (Man-PoC) stimulus compared to control hearts (Man-PC: 31 ± 4%; Man-PoC: 35 ± 6%, each p < 0.05 vs. Con: 57 ± 9%). The mKATP channel inhibitor completely abrogated the cardioprotective effect of Mannitol-induced pre- (5HD-PC-Man-PC: 59 ± 8%, p < 0.05 vs. Man-PC) and postconditioning (5HD-PoC-Man-PoC: 59 ± 10% vs. p < 0.05 Man-PoC). Infarct size was not influenced by 5HD itself (5HD-PC: 60 ± 14%; 5HD-PoC: 54 ± 14%, each ns vs. Con). This study demonstrates that Mannitol (1) induces myocardial pre- and postconditioning and (2) confers cardioprotection via activation of mKATP channels.

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Stamatelopoulos, Kimon, Marita Lyka, Christos Papamichail, Maria Roussou, Maria Gavriatopoulou, Constantinos Pamboucas, Evangelos Terpos, Meletios A. Dimopoulos, and Efstathios Kastritis. "Hemodynamic, Functional and Structural Markers of Vascular Involvement in Primary Systemic Light Chain (AL) Amyloidosis." Blood 124, no. 21 (December 6, 2014): 2029. http://dx.doi.org/10.1182/blood.v124.21.2029.2029.

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Abstract Light chain (AL) amyloidosis is characterized by the extracellular deposition of clonal light chain-derived amyloid fibrils in various tissues. Kidneys and heart are most commonly affected; clinically non detectable deposits in other tissues may also result in significant dysfunction. Amyloid deposits within the wall of the arteries or direct toxicity of light chains may cause arterial dysfunction in patients with AL amyloidosis, with cardiovascular consequences. Endothelial dysfunction, subclinical atherosclerosis of the carotid arteries and stiffening of the arterial tree are commonly found as deleterious vascular phenotypes and are considered surrogate markers of cardiovascular disease and outcome. Matrix metalloproteases, advanced glycation products, endothelial dysfunction and neuroendocrine signaling are implicated in arterial dysfunction and are affected in AL amyloidosis. However, arterial involvement in AL has not been evaluated thoroughly. In order to evaluate vascular dysfunction in patients with AL amyloidosis we evaluated non-invasively acquired markers which may be indicative of vascular involvement: hemodynamic [reflected waves and aortic blood pressures (BP)], functional [flow-mediated dilatation and carotid-femoral pulse wave velocity (PWV)] and structural [intima-media thickness (IMT) and the presence of plaques in the carotid arteries] markers of vascular damage were measured. Ninety-one (91) consecutive newly diagnosed patients with systemic AL were prospectively studied and compared to 91 controls matched 1:1 for traditional risk factors of cardiovascular disease (age, gender, smoking, hypertension, hyperlipidemia, diabetes) and for the presence of coronary artery disease. The median age of patients with AL was 65 years (range 40-83), 56% were males, 77% had renal and 57% cardiac involvement. Median NTproBNP was 1420 pg/ml, 25%, 49% & 26% were Mayo stage 1, 2 & 3 respectively, median eGFR was 66 ml/min/1.73 m2and median involved FLC (iFLC) level was 189 mg/L. First we compared indices of vascular damage between AL patients and matched controls: arterial stiffness (PWV: 10.4±3.0 vs 8.6±3.7 m/sec, p=0.002) and arterial wall thickness in the internal carotid artery (IMT: 0.76±0.21 vs 0.68±0.21 mm, p=0.017) were significantly higher in AL patients. Thus, subclinical vascular damage, independent from traditional factors of vascular dysfunction, is present in patients with AL. Peripheral systolic and diastolic blood pressure and aortic systolic, diastolic and pulse pressure (p≤0.001) and reflected waves (augmentation index (AI), p=0.045) were significantly lower in AL patients than in controls. After adjustment for NTproBNP levels, as a marker of cardiac dysfunction, the differences in blood pressures (systolic, diastolic and pulse pressure) remained significant (p≤0.008 for all). Thus, despite increased arterial stiffness, dysfunctional vasculature results in paradoxically lower systolic and diastolic blood pressure, a hallmark of AL amyloidosis, independently from the degree of cardiac dysfunction. We then examined possible associations of markers of AL with the indices of vascular dysfunction, after adjustment for risk factors of cardiovascular disease. The level of iFLCs was an independent determinant of lower peripheral systolic (p=0.021) and aortic systolic (p=0.009) and pulse pressure (p=0.013), hsTnT (p=0.016) and NTproBNP (p=0.001) levels were independent determinants of arterial stiffness (PWV). In a multivariate model mean blood pressure and NTproBNP were the only determinants of PWV. NTproBNP also correlated with the presence of plaques in the internal carotid arteries (p=0.006) independently of other risk factors of vascular disease. Thus, markers of cardiac dysfunction and the load of FLCs were associated with vascular dysfunction. Our results suggest vascular involvement in patients with AL amyloidosis, reflected at low aortic blood pressures, increased arterial stiffness and wall thickness in the internal carotid arteries. The association of the amount of light chains with aortic blood pressure suggests a role of the toxic light chain. The observed association of arterial stiffness with markers of heart involvement is indicative of a parallel process of heart and vascular injury in AL amyloidosis. Further research, to assess the clinical utility of markers of vascular damage in AL amyloidosis is needed. Disclosures No relevant conflicts of interest to declare.

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Benfaremo, D., G. Stronati, L. Manfredi, L. Zuliani, A. Ferrarini, C. Fischetti, C. Dichiara, F. Guerra, A. Dello Russo, and A. Gabrielli. "SAT0307 PROGRESSION OF SUBCLINICAL MYOCARDIAL INVOLVEMENT IN PATIENTS WITH SYSTEMIC SCLEROSIS." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1098.1–1099. http://dx.doi.org/10.1136/annrheumdis-2020-eular.2824.

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Background:Systemic sclerosis (SSc) is a progressive autoimmune disease affecting the skin as well as internal organs, including the heart. A few studies have identified a subclinical heart involvement in patients with no pulmonary hypertension. Changes in myocardial deformation are consistent with the idea of SSc-related cardiomyopathy as a primary condition affecting the heart globally through microvascular dysfunction and subsequent myocardial fibrosis.Objectives:The aim of the present study is to describe the progression of myocardial deformation in patients with SSc and no overt cardiac disease.Methods:Prospective longitudinal study enrolling consecutive SSc patients referred to the Clinica Medica, University Hospital ‘Ospedali Riuniti’, Ancona, Italy, from February 2016 to December 2018. All patients fulfilled the 2013 ACR/EULAR classification criteria for SSc. Patients with structural heart disease, heart failure, atrial fibrillation or pulmonary hypertension were excluded. Disease subset, antibodies pattern, cardiovascular risk factors and involvement of other organ systems were recorded for each patient. An echocardiographic exam was performed for all patients at baseline and during their follow-up evaluation. Standard and speckle-tracking derived variables for the systolic and diastolic function of the left ventricle (LV) and right ventricle (RV) were acquired. Speckle tracking analysis software (EchoPAC 13.0; GE Medical Systems, Milwaukee, USA) was used to assess the GLS of the left and right ventricle, excluding the ventricular septum from right ventricular GLS calculations.Results:Seventy-two patients (68 females, age 56.6±15.4 years) were enrolled. Common echocardiographic parameters of left and right systolic function were within normal range at baseline and did not change during follow-up. Mean GLS, however, worsened for both left (from -19.8±3.5% to -18.7±3.5%, p=.034) and right ventricle (from -20.9±6.1% to -18.7±5.4%, p=.013) during a median follow-up of 20 months (1st-3rd quartile 12-24 months). The increased impairment registered in SSc patients was homogenous across endocardial layers (LV from -22.5±-3.9 to -21.4±3.9, p=.041; RV from -24.2±6.2 to -20.6±5.9, p=.001), mesocardial layers (LV -19.7±3.6 to -18.7±3.5, p=.043; RV from -21.3±5.9 to -18.8±5.7, p=.012) and epicardial layers (LV from -17.1±3.0 to -16.4±3.1, p=.112, RV -18.8±6.3 to -16.0±8.4, p=.035), as well as myocardial segments. No difference in progression rate was observed stratifying patients according to disease subset or other clinical parameters.Conclusion:GLS impairment progressed over a 20-month follow-up period in a cohort of SSc patients without clinically overt cardiac involvement. Further studies are needed to assess the significance of subclinical heart involvement and its progression in patients with SSc.Disclosure of Interests:None declared

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Sharma, Poonam, Urmila Shakya, Sunita KC, and Manish Shrestha. "Clinical Profile and Management in Children with Rheumatic Heart Disease in a Tertiary Cardiac Care Center of Nepal." Nepalese Heart Journal 13, no. 2 (August 27, 2016): 33–36. http://dx.doi.org/10.3126/njh.v13i2.15562.

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Backgrounds and Aims: Rheumatic heart disease is the commonest acquired heart disease in children of developing world presenting earlier and with a more severe valvular lesion. This study was conducted to assess the clinical and demographic profile, pattern of valvular involvement and management modalities among children with rheumatic heart disease.Methods: This was a hospital-based observational study, performed at Shahid Gangalal National Heart Center where all children less than 15 years of age with confirmed echocardiographic diagnosis of rheumatic heart disease from June 2015 to May 2016 were evaluated and relevant data were recorded.Results: Two hundred and eleven children were diagnosed with rheumatic heart disease. The mean age was 11.9 ± 2.2 years with (100) 47.4% male and (111) 52.6% female. The median duration of symptoms among new cases was 1 year (interquartile range of 0.5 to 2 years). The most common symptom was dyspnea present in (187) 88.6% children followed by palpitation (34.5%). Mitral valve was most commonly affected in (141) 96.5% while isolated aortic valve involvement was seen in (8) 3.5%. Isolated mitral regurgitation was the most common lesion present in 118 (55.9%) children, while 50 (23.7%) children had combination of mitral and aortic regurgitation. Mitral valve was significantly more commonly affected in females and aortic valve commonly affected in males (p= 0.003).Conclusions: Rheumatic heart disease although declining in developed countries, remains to be the commonest acquired heart disease in developing and underdeveloped countries. Efforts are needed to improve primary and secondary preventive measures.Nepalese Heart Journal 2016; 13(2): 33-36

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Olivas-Chacon, Cristina I., Carola Mullins, Kevan Stewart, Nassim Akle, Jesus E. Calleros, and Luis R. Ramos-Duran. "Magnetic Resonance Imaging of Non-ischemic Cardiomyopathies: A Pictorial Essay." Journal of Clinical Imaging Science 5 (June 30, 2015): 37. http://dx.doi.org/10.4103/2156-7514.159564.

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Non-ischemic cardiomyopathies are defined as either primary or secondary diseases of the myocardium resulting in cardiac dysfunction. While primary cardiomyopathies are confined to the heart and can be genetic or acquired, secondary cardiomyopathies show involvement of the heart as a manifestation of an underlying systemic disease including metabolic, inflammatory, granulomatous, infectious, or autoimmune entities. Non-ischemic cardiomyopathies are currently classified as hypertrophic, dilated, restrictive, or unclassifiable, including left ventricular non-compaction. Cardiovascular Magnetic Resonance Imaging (CMRI) not only has the capability to assess cardiac morphology and function, but also the ability to detect edema, hemorrhage, fibrosis, and intramyocardial deposits, providing a valuable imaging tool in the characterization of non-ischemic cardiomyopathies. This pictorial essay shows some of the most important non-ischemic cardiomyopathies with an emphasis on magnetic resonance imaging features.

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Lipinski, M., D. Eagles, L. M. Fischer, L. Mielneczuk, and I. G. Stiell. "LO02: Heart failure and palliative care in the emergency department." CJEM 19, S1 (May 2017): S27—S28. http://dx.doi.org/10.1017/cem.2017.64.

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Introduction: Heart failure (HF) is a common ED presentation that is associated with significant morbidity and mortality. Despite recent evidence and recommendations for early palliative care (PC) involvement in these patients, they are still significantly under-served by PC services, often resulting in multiple ED visits. We sought to evaluate use of PC services in patients with HF presenting to the ED. Secondary objectives of the study were to investigate: 1) one year mortality, ED visits, and admissions; 2) application of a novel palliative care referral score. Methods: We conducted a health records review of 500 consecutive HF patients who presented to two academic hospital EDs. We included patients aged 65 years or older who were diagnosed as having a HF exacerbation by the emergency physician (ICD-10 code 150.-). Our primary outcome was PC involvement. Secondary outcomes included one year mortality rates, ED visits, admissions to hospital, as well as the application of a novel PC referral score developed by the institutional cardiac Palliative Care Committee. The score consisted of 6 different aspects of the patient’s illness, including laboratory tests, hospital usage, and markers of decompensation. We conducted appropriate univariate analyses. Results: Patients were mean age 80.7 years, women (53.2%), and had significant comorbidities (atrial fibrillation (51.2%), diabetes (40.4%) and COPD (20.8%)). Compared to those with no PC, the 79 (15.8%) patients with PC involvement had a higher one year mortality rate (70.9% vs. 18.8%, p<0.0001), more ED visits/year for HF (0.82 vs. 0.52, p<0.0001), and more hospital admissions/year for HF (1.4 vs. 0.85, p<0.0001). Using the heart failure palliative care score criteria, 60 patients had scores >=2. Compared to those with scores <2, these patients had a higher 1-year mortality rate (50% vs. 24%, p<0.0001) and more ED visits/year for HF (0.83 vs. 0.54, p<0.01). Only 40.0% of these high risk patients had any PC involvement. Conclusion: We found that few HF patients had PC services involved in their care. Using this novel HF palliative care referral score, we were able to identify patients with a significantly greater risk of mortality and morbidity. This study provides evidence that the ED is an appropriate setting to identify and refer high risk HF patients who would likely benefit from earlier PC involvement and may be a future avenue for PC access for these patients.

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Raat, Willem, Miek Smeets, Stefan Janssens, and Bert Vaes. "Impact of primary care involvement and setting on multidisciplinary heart failure management: a systematic review and meta‐analysis." ESC Heart Failure 8, no. 2 (January 6, 2021): 802–18. http://dx.doi.org/10.1002/ehf2.13152.

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Toscano, G., A. Fabozzo, M. Fedrigo, A. Gambino, A. Angelini, and G. Gerosa. "Post-transplant Lymphoproliferative Disorders: A Rare Case of Primary Pericardial Involvement Epstein-Barr Virus-Correlated in Heart Transplantation." Transplantation Proceedings 47, no. 7 (September 2015): 2287–90. http://dx.doi.org/10.1016/j.transproceed.2015.04.085.

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Sampath Kumar, Arkalgud. "Surgical options in rheumatic heart disease: an Indian surgeon’s perspective." Asian Cardiovascular and Thoracic Annals 28, no. 7 (October 16, 2019): 371–73. http://dx.doi.org/10.1177/0218492319884797.

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Rheumatic heart disease is the primary cause of valvular heart disease in India and other Southeast Asian countries. The disease is quite different from that seen in France, USA, and Australia. Poverty, malnutrition, and delayed referral affect the outcomes. Rheumatic heart disease can affect all four heart valves, mitral being the most common, aortic second, tricuspid next, and finally the pulmonary valve. The combinations of mitral and aortic, mitral and tricuspid, and all three valves are the next in frequency. Acute rheumatic fever usually manifests as quadrivalvular involvement. However, chronic rheumatic quadrivalvular heart disease has also been reported. The technical aspects taught and practiced in one institution over the past four decades are described here. Closed mitral valvotomy, mitral valve repair, or replacement with mechanical, bioprosthetic, and autograft valves are the choices. Aortic valve disease is usually treated with valve repair or replacement with mechanical, homograft or pulmonary autograft valves.

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Severino, Paolo, Andrea D’Amato, Silvia Prosperi, Alessandra Dei Cas, Anna Vittoria Mattioli, Antonio Cevese, Giuseppina Novo, et al. "Do the Current Guidelines for Heart Failure Diagnosis and Treatment Fit with Clinical Complexity?" Journal of Clinical Medicine 11, no. 3 (February 6, 2022): 857. http://dx.doi.org/10.3390/jcm11030857.

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Heart failure (HF) is a clinical syndrome defined by specific symptoms and signs due to structural and/or functional heart abnormalities, which lead to inadequate cardiac output and/or increased intraventricular filling pressure. Importantly, HF becomes progressively a multisystemic disease. However, in August 2021, the European Society of Cardiology published the new Guidelines for the diagnosis and treatment of acute and chronic HF, according to which the left ventricular ejection fraction (LVEF) continues to represent the pivotal parameter for HF patients’ evaluation, risk stratification and therapeutic management despite its limitations are well known. Indeed, HF has a complex pathophysiology because it first involves the heart, progressively becoming a multisystemic disease, leading to multiorgan failure and death. In these terms, HF is comparable to cancer. As for cancer, surviving, morbidity and hospitalisation are related not only to the primary neoplastic mass but mainly to the metastatic involvement. In HF, multiorgan involvement has a great impact on prognosis, and multiorgan protective therapies are equally important as conventional cardioprotective therapies. In the light of these considerations, a revision of the HF concept is needed, starting from its definition up to its therapy, to overcome the old and simplistic HF perspective.

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48

Guimarães-Okamoto, Priscilla Tatiana Chalfun, Maria Claudia Lopes Da Silva, Julio Lopes Sequeira, Alessandre Hataka, Fernanda Chicharo Chacar, Silvano Salgueiro Geraldes, José Francisco Antunes Ribeiro, and Alessandra Melchert. "Primary Diﬀuse Large B Cell Lymphoma in Kidney with Involvement of Central Nervous System and Heart in a Siamese Cat." Acta Scientiae Veterinariae 44, no. 1 (January 16, 2016): 4. http://dx.doi.org/10.22456/1679-9216.82945.

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Background: Lymphoma is the most common neoplasia in cats and usually happens in middle-aged males of European breeds, FeLV-positive test is also observed in 25% to 50% of these cases. The diagnostic of renal lymphoma is based on clinical signs, the diagnosis is based on clinical signs associated with additional tests. Histological an immunohistochemical evaluations allows the classifcation which can provide prognostic information. Central nervous system metastasis is frequently reported, but lingual and lung lobe extension were also reported. Here, we report a case of renal lymphoma with systemic involvement of heart and CNS in a cat. Case: A 3-year-old male Siamese cat presented with weight loss, emesis, anorexia, polydipsia and polyuria for at least 3 weeks. The clinical parameters were stable, but presenting pale mucosal membrane and also severe dehydration. Complete blood count and chemistry profle revealed anemia and severe azotemia. On abdominal palpation, bilateral renomegaly and irregular contours of both kidneys were also detected. The kidneys were enlarged and had irregular contours on palpation, confrmed by abdominal ultrasonography, that revealed kidneys with markedly irregular contours, and the presence of ﬂuid with high cellularity between cortical and renal capsule (capsular ﬂuid). An aspiration citology by fne-needle from the capsular ﬂuid guided by ultrasound was collected and sent to cytological analysis, which suggested lymphoma. The aspirate was moderately cellular and consisted of large round cells with distinct, scant, basophilic cytoplasm, round nuclei with dense chromatin, inconspicuous nucleoli, and large amount of naked cells. On the second day of treatment the cat presented a cardiopulmonary arrest not responsive to cardiopulmonary cerebral resuscitation, died, and the owner authorized the necropsy, allowing the renal biopsy showing that the same neoplastic cells presented in kidneys were also seen in heart and central nervous system, suggesting the occurrence of primary renal lymphoma with involvement of to these tissues, and the diagnosis was high grade diffuse large cell lymphoma. And also histopathological exam diagnosed high grade diffuse large cell lymphoma. Discussion: The patient died, and the post-mortem histological analysis confrmed the diagnosis of primary diffuse large B cell lymphoma in kidney with systemic involvement of heart and central nervous system (CNS). Lymphoma is the most common renal tumor in cats, however primary feline renal tumors are rare and few reports are available at this time. Here we report the occurrence of primary renal lymphoma in a Siamese cat, and there´s no previous report of this type of lymphoma especially in this breed. More investigation is needed to evaluate the susceptibility of Siamese cat breed to the development of primary renal lymphoma. It is diffcult to determine whether the renal involvement is primary or it is a consequence of the multicenter type. In the present report the same neoplasic infltration observed in kidney was also found in central nervous system and heart. The cytology is a quickly, non-invasive, and sensitivity method for the diagnostic of lymphoma. However, the cytology do not provides the immune diagnosis of diffuse large B cell, and although it is useful for staging lymphoma, histopathology and immunohistochemistry is always necessary for classifcation and grading. Since the diagnosis, the cat of this report survived only 2 days, which reinforces the bad prognosis of renal lymphoma. The renal lymphoma should be considered as a potential cause of severe azotemia, and should be taken as the mainly differential diagnosis among neoplasias of kidneys in cats.Keywords: oncology, feline, extranodal, lymph nodes, cancer

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49

Rabadão, Tiago, Leonor Naia, Filipa Ferreira, Mariana Teixeira, Marcelo Aveiro, Margarida Eulálio, and Fernando Silva. "Not Always (and Only) Heart Failure—A Case Report of Primary Pleural Lymphoma in an Elderly Patient." Clinics and Practice 11, no. 1 (January 29, 2021): 32–36. http://dx.doi.org/10.3390/clinpract11010006.

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Pleural involvement in Non-Hodgkin Lymphoma (NHL) is well documented, but primary pleural lymphomas are extremely rare, occurring mostly in immunosuppressed patients or associated with chronic pleural inflammation. Nevertheless, the pathogenesis and therapeutic approaches to counteract primary pleural lymphomas are still matter of debate. The authors present the clinical case of an 81-year-old female with respiratory and constitutional symptoms. A valvular heart disease and bilateral pleural effusion were known. The study carried out showed a large right pleural effusion; the fluid analysis was compatible with Diffuse Large B-cell Lymphoma (DLBCL), and two lymphomatous masses with pleural origin were found at the ipsilateral hemithorax. Primary pleural lymphoma was considered and chemotherapy was initiated with a good response and evolution. The authors report this remarkable clinical case because of its rarity, its excellent clinical evolution and the absence of an immunodeficiency context.

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50

Bani Hani, Amjad, Mahmoud Abu-abeeleh, Murad M. Al Kharabsheh, and Lubaba Qabba'ah. "Libman-Sacks Endocarditis with Unusual Large Size Vegetation Involving the Mitral Valve." Heart Surgery Forum 19, no. 6 (December 21, 2016): 294. http://dx.doi.org/10.1532/hsf.1612.

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Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder characterized by thrombophilia, vascular thrombosis, and recurrent abortions associated with persistent antiphospholipid antibodies. APS may exist in its primary form, or more commonly is found to be associated with variety of rheumatic disorders, such as systemic lupus erythematosus. Cardiac involvement is not an uncommon complication in primary antiphospholipid patients. Libman-Sacks lesions are typically small, sessile, and wart-like, varying in size from 1-4 mm. Here we present an unusual case of a 37 year-old pregnant woman who suffered from heart failure associated with primary antiphospholipid syndrome and Libman-Sacks endocarditis, with large vegetations involving the mitral valve. The patient underwent mitral valve replacement with a mechanical prosthesis.

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Journal articles on the topic 'Primary heart involvement'

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