scholarly journals How exercise can deteriorate the clinical course of an ARVC patient: a case report

2021 ◽  
Author(s):  
Enzo Lüsebrink ◽  
Leonhard Binzenhöfer ◽  
Stefan Brunner ◽  
Jörg Hausleiter ◽  
Steffen Massberg ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Disease Onset ◽  
Left Ventricular ◽  
Rapid Decline ◽  
Heart Catheterization ◽  
Term Care ◽  
Patient Reported

Abstract Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic disease characterized by fibro-adipose degeneration of ventricular myocardium. Initial clinical presentation is variable and ranges from asymptomatic cases to chronic heart failure and sudden cardiac death due to malignant arrhythmias. Case summary Here, a 67-year-old male patient who started extensive physical training upon retirement and presented with ventricular tachycardia and progressive heart failure as a first sign of his disease. ARVC/D diagnosis was established according to the 2010 modified Task Force Criteria and supported by HRS/EHRA Consensus based genotyping. After initial discharge on optimal medical therayp and prophylactic ICD-implantation according to his individual ARVC risk score, the patient reported rapid decline in physical capacity on a regular follow-up four months later. To better understand the etiology of his clinical deterioration, we performed stress echocardiography, coronary angiogram and exercise right heart catheterization, which conclusively suggest impaired left ventricular (LV) filling secondary to RV failure as a main cause of global circulatory failure. Discussion The present case report focuses on relation of physical activity to disease onset and the concomitant advent of symptoms during exercise as well as a structured and guideline-aided diagnostic work-up in ARVC/D and staged treatment options. Continuous ARVC/D-center-oriented re-assessment and treatment planning including lifestyle intervention, psychological support, medical, surgical and interventional options are key elements of sustained long-term care for ARVC/D patients.

scholarly journals AB0858 SUCCESSFUL TERM PREGNANCY IN A PATIENT WITH GRANULOMATOSIS WITH POLYANGIITIS AFTER UNDERGOING RITUXIMAB THERAPY - CASE REPORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1452.2-1453
Author(s):  
O. Sirenko ◽  
O. Kuryata ◽  
T. Lysunets ◽  
A. Legkobyt ◽  
H. Mostova
Keyword(s):  
Case Report ◽  
Granulomatosis With Polyangiitis ◽  
Disease Onset ◽  
Biological Agents ◽  
Term Pregnancy ◽  
Aseptic Necrosis ◽  
Azathioprine Therapy ◽  
Disease Remission ◽  
Obstetric Medicine ◽  
Patient Reported

Background:Granulomatosis with polyangiitis (GWP) is a relatively rare disease with the lack of experience in the management of pregnancy currently. Potentially negative factors of pregnancy prognosis are both disease-related and teratogenic effects of specific vasculitis therapy [1].Objectives:To describe a clinical case of Successful term pregnancy in a patient with Granulomatosis with polyangiitis after undergoing rituximab therapy.Methods:19 years-old woman was admitted to the rheumatology department of Clinical Regional Hospital After Mechnicov in 2013 with the debut of Granulomatosis with polyangiitis. Her disease course included recurrent rhinitis and sinusitis, Granulomatosis of the eye orbits. Positive ANCA titers were present in the disease onset. In the preceding 7 years she had been treated with corticosteroids, cyclophospan (discontinuated in 2017 because of lack of efficiency), than azathioprine. Aseptic necrosis of the both femoral head was estimated on fifth disease year. In 2018 azathioprine therapy had been discontinued owing to the disease progression and biological agents were prescribed – rituximab with positive effect. The disease remission was achived by rituximab therapy, the patient was warned about the need for contraception. The last dose of rituximab was introduced in February 2020. However, in March 2020, the patient reported pregnancy and therapy was discontinued. At the onset of pregnancy she was treated with 6 mg oral methylprednisolone. Daily and this dose was stable till all pregnancy period. Fetal growth assessment, congenital abnormalities screening test, and laboratory tests for gestational diabetes and preeclampsia were unremarkable during all gestational trimesters. The ANCA titers remained negative and renal function was normal and there was no flare during all pregnancy period.Results:At 38 weeks’ gestation a spontataneous labor started without induction. A 3270-g healthy boy delivered with APGAR score of 9 at 1 minute and 5 minutes. Postpartum the disease remained in remission, and the patient was maintained on corticosteroids. The infant was healthy, with normal development.Conclusion:Thus, the use of biological agents therapy in patients with GWP shows possible ways to safe reproductive potential with disease remission achievement.References:[1]Daher A, Sauvetre G, Girszyn N, Verspyck E, Levesque H, Le Besnerais M. Granulomatosis with polyangiitis and pregnancy: A case report and review of the literature. Obstetric Medicine. 2020;13(2):76-82. doi:10.1177/1753495X18822581Disclosure of Interests:None declared

scholarly journals A case report: percutaneous management of high-output heart failure from iatrogenic aortocoronary venous grafting to the coronary sinus

2021 ◽  
Vol 73 (1) ◽  
Author(s):  
Akarsh Parekh ◽  
Vivek Sengupta ◽  
Ryan Malek ◽  
Mark Zainea
Keyword(s):  
Heart Failure ◽  
Coronary Sinus ◽  
Saphenous Vein Graft ◽  
Left Ventricular ◽  
Heart Catheterization ◽  
Covered Stents ◽  
Bypass Grafting ◽  
Output State ◽  
State Management ◽  
High Output

Abstract Background Aortocoronary arteriovenous fistula (ACAVF) due to iatrogenic bypass grafting to a cardiac vein is an exceedingly rare complication resulting from coronary artery bypass grafting (CABG) surgery. If not identified in a timely fashion, ACAVF has known significant clinical consequences related to left to right shunting and possible residual myocardial ischemia. Case presentation An 82-year-old male with a history of CABG, presented with dyspnea. Over the span of 2 years following CABG, the patient experienced progressive exertional dyspnea and peripheral edema. The patient was found to have a new cardiomyopathy with a severely reduced ejection fraction at 30–35%. The patient underwent diagnostic left heart catheterization, and an ACAVF was discovered between a saphenous vein graft and the coronary sinus. The patient underwent successful percutaneous coiling of the ACAVF with no residual flow. Follow-up echocardiography at 3 months revealed restoration of left ventricular systolic function to 50% and significant improvement in heart failure symptoms. Conclusions ACAVF is an exceedingly rare iatrogenic complication of CABG that may result in residual ischemia from the non-grafted myocardial territory and other sequelae relating to left to right shunting and a high-output state. Management for this pathology includes but is not limited to the use of percutaneous coiling, implantation of covered stents, graft removal and regrafting, and ligation.

Abstract 225: Frequency and Assessment of Poor Global Outcome in Patients with Left Ventricular Assist Devices

2015 ◽  
Vol 8 (suppl_2) ◽  
Author(s):  
Timothy J Fendler ◽  
Michael E Nassif ◽  
Kevin F Kennedy ◽  
John A Spertus ◽  
Shane J LaRue ◽  
...  
Keyword(s):  
Heart Failure ◽  
Nyha Class ◽  
Left Ventricular ◽  
Advanced Heart Failure ◽  
Ventricular Assist Devices ◽  
Left Ventricular Assist Devices ◽  
Ventricular Assist ◽  
Bridge To Transplant ◽  
Patient Reported ◽  
Left Ventricular Assist

Background: Left ventricular assist device (LVAD) therapy can improve survival and quality of life in advanced heart failure (HF), but some patients may still do poorly after LVAD. Understanding the likelihood of experiencing poorer outcomes after LVAD can better inform patients and calibrate their expectations. Methods: We analyzed patients receiving LVAD therapy from January 2012 to October 2013 at a single, high-volume, high-acuity center. We defined a poor global outcome at 1 year after LVAD as the occurrence of death, disabling stroke (precluding transplant), poor patient-reported health status (most recent KCCQ at 3, 6, or 12 months < 45, corresponding to NYHA class IV), or recurrent HF (≥2 HF readmissions post-implant). We compared characteristics of those with and without poor global outcome. Results: Among 164 LVAD recipients who had 1-year outcomes data, mean age was 56, 76.7% were white, 20.9% were female, and 85.9% were INTERMACS Profile 1 or 2 (cardiogenic shock or declining despite inotropes). Poor global outcome occurred in 58 (35.4%) patients at 1 year, of whom 37 (63.8%) died, 17 (29.3%) had a most recent KCCQ score < 45, 3 (5.2%) had ≥2 HF readmissions, and 1 (1.7%) had a disabling stroke (Figure). Eight of the patients who died also experienced one of the three other poor outcomes prior to death. Patients who experienced a poor global outcome were more likely to be designated for destination therapy (46.4% vs. 23.6%, p=0.01) than bridge to transplant, have longer index admissions (median [IQR]: 39 [24, 57] days vs. 25 [18, 35] days, p=0.003), and have major GI bleeding (44.2% vs. 27.7%, p=0.056), and were less likely to undergo LVAD exchange (0% vs. 12.3%, p=0.004). Conclusion: In this large, single-center study assessing global outcome after LVAD implantation, we found that about a third of all patients had experienced a poor global outcome at 1 year. While LVAD therapy remains life-saving and the standard of care for many patients with advanced heart failure, these findings could help guide discussions with eligible patients and families. Future work should compare patients’ pre-LVAD expectations with likely outcomes and create risk models to estimate the probability of poorer outcomes for individual patients using pre-procedural factors.

Abstract 15314: Left Ventricular Dysfunction in Children and Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Paweena Chungsomprasong ◽  
Robert Hamilton ◽  
Wietske Luining ◽  
Shi-Joon Yoo ◽  
Meena Fatah ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Myocardial Dysfunction ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Myocardial Strain ◽  
Young Patients ◽  
Left Ventricular ◽  
Lv Function ◽  
Ventricular Cardiomyopathy ◽  
End Diastolic Volume

Background: Involvement of the left ventricle (LV) is increasingly recognized in adults with arrhythmogenic right ventricular cardiomyopathy (ARVC) but it is unclear whether LV function is compromised in children with this condition. The aim of this study was examine myocardial contractility in pediatric patients with suspected ARVC. Methods: For this retrospective study, patients with a work-up for ARVC were classified into ‘no’, ‘possible’, ‘borderline’ or ‘definite’ ARVC according to the revised Task Force Criteria (rTFC). Ventricular size and function as well as LV myocardial strain and torsion were measured by cardiac magnetic resonance (CMR). Results: A total of 142 patients were enrolled, of whom 58 (41%) had no, 32 (23%) possible, 29 (20%) borderline and 23 (16%) definite ARVC. The groups were similar in age at CMR. With higher rTFC score, z scores (Z) of right ventricular (RV) ejection fraction (EF) were lower (p<0.001) while z-RV end diastolic volume (EDV) and z-LV EDV were larger (p=0.002 and 0.013, respectively). LV EF did not differ between rTFC categories. Global circumferential strain (GCS) of the LV was lower in patients in higher rTFC categories (p=0.018). Z-LVEDV correlated with z-RVEDV (r2 = 0.69, p<0.001) and z- LVEF correlated with z-RVEF (r2 = 0.55, p <0.001). Z-LVEF and z-RVEF correlated with LV GCS (r2 = 0.48, p<0.001 and r2 = 0.46, p<0.001, respectively) and torsion (r2 = 0.21, p=0.032 for both). Forty-two patients had a follow-up CMR, after a median interval of 2.6 years (0.4- 8.4). The rate of deterioration of LV or RV EF or EDV did not differ between rTFC categories. A more rapid increase of z-RVEDV was associated with a faster decline in z-RVEF (r2 = -0.383, p=0.004) and z-LVEF (r2 = -0.45, p=0.001). A decline of z-LVEF over time correlated with that of z-RVEF (r2 = 0.60, p<0.001) and z-LVEDV increase correlated with z-RVEDV increase (r2 = 0.84, p<0.001). Conclusion: LV myocardial dysfunction is present in young patients with suspected or confirmed ARVC. Quantification of myocardial mechanics with CMR may be a useful tool to detect early LV involvement in ARVC. Progressive LV dysfunction and enlargement appear to parallel those of the RV.

Abstract 17072: Discordance Between Lactic Acid Elevation and Invasive Hemodynamics in Decompensated Heart Failure

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Nikhil Narang ◽  
Bow Chung ◽  
Ann Nguyen ◽  
Teruhiko Imamura ◽  
Sara Kalantari ◽  
...  
Keyword(s):  
Heart Failure ◽  
Lactic Acid ◽  
Inverse Correlation ◽  
Decompensated Heart Failure ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Heart Catheterization ◽  
Ventricular Ejection Fraction ◽  
Significant Difference ◽  
Invasive Hemodynamics

Introduction: Elevated lactic acid (LA) levels carry a poor prognosis in patients admitted with shock. Data is lacking on the association of LA level with the severity of decompensated heart failure (HF). This study assesses the relationship between LA levels, invasive hemodynamics and clinical outcomes. Methods: Patients presenting to the cardiac care unit with decompensated HF between 2015-18 were prospectively enrolled into an invasive hemodynamics study. LA (normal 0.7-2.1 mmol/L) levels were obtained within 12 hours prior to right heart catheterization (RHC). No significant changes in therapy were made in the time between LA level collection & RHC. Patients were divided into 4 groups: 1) normal pulmonary capillary wedge pressure (PCWP) (< 18 mmHg)/ normal Fick cardiac index (CI) (≥ 2.2 L/min/m 2 ), 2) normal PCWP/ low CI (< 2.2 L/min/m 2 ) 3) elevated PCWP (≥ 18 mmHg )/ normal CI, 4) elevated PCWP / low CI. Results: 80 patients were enrolled. Mean age 58±14 years; 78% male, left ventricular ejection fraction was 24±4%. Prior to RHC, 55% patients were on vasoactives and/or inotropes. Mean (SD) PCWP was 26 ± 8.8 mmHg and mean CI was 2.06 ± 0.61 L/min/m 2 . Overall 48 (60%) of the patients had high PCWP and low CI (group 4). 81% had normal LA (≤2.1 mmol/L) prior to RHC. There was no correlation between LA level and PCWP (R=0.12; p=0.30); there was a moderate inverse correlation between LA level and CI (R=-0.40; p<0.01). Only 25% of patients with the highest risk hemodynamic profile (elevated PCWP/low CI) had an elevated LA level (Figure A). 90- day all cause mortality was 33% When LA was stratified by tertile, there was no significant difference in mortality between the tertiles (Figure B). Conclusion: In patients with decompensated HF, normal LA levels do not exclude the presence of cardiogenic shock with profoundly impaired cardiac output. Invasive assessment of hemodynamics should not be delayed based on LA level alone.

scholarly journals Characteristics of Patients With Arrhythmogenic Left Ventricular Cardiomyopathy

2020 ◽  
Vol 13 (12) ◽  
Author(s):  
Michela Casella ◽  
Alessio Gasperetti ◽  
Rita Sicuso ◽  
Edoardo Conte ◽  
Valentina Catto ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Genetic Variants ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Voltage Mapping ◽  
Fatty Replacement

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce. Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Results: Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients. Conclusions: ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

scholarly journals Assessment of right ventricular sympathetic dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy: An 123I-metaiodobenzylguanidine SPECT/CT study

2018 ◽  
Vol 27 (6) ◽  
pp. 2402-2409 ◽  
Author(s):  
Andrei Todica ◽  
Johannes Siebermair ◽  
Julia Schiller ◽  
Mathias J. Zacherl ◽  
Wolfgang P. Fendler ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hybrid Imaging ◽  
State Of The Art ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Sympathetic Innervation ◽  
Left Ventricular ◽  
Sympathetic Dysfunction ◽  
Novel Approach ◽  
Significant Difference

Abstract Purpose The purpose of the study was to evaluate a novel approach for the quantification of right ventricular sympathetic dysfunction in patients diagnosed with ARVC/D through state-of-the-art functional SPECT/CT hybrid imaging. Methods Sympathetic innervation of the heart was assessed using 123I-MIBG-SPECT/CT in 17 patients diagnosed with ARVC according to the modified task force criteria, and in 10 patients diagnosed with idiopathic ventricular fibrillation (IVF). The 123I-MIBG-uptake in the left (LV) and right ventricle (RV) was evaluated separately based on anatomic information derived from the CT scan, and compared to the uptake in the mediastinum (M). Results There was a significant difference in the LV/M ratio between the ARVC/D and the IVF groups (3.2 ± 0.5 vs. 3.9 ± 0.8, P = 0.014), with a cut-off value of 3.41 (77% sensitivity, 80% specificity, AUC 0.78). There was a highly significant difference in the mean RV/M ratios between both groups (1.6 ± 0.3 vs. 2.0 ± 0.2, P = 0.001), with optimal cut-off for discrimination at 1.86 (88% sensitivity, 90% specificity, AUC 0.93). Conclusion Employing state-of-the-art functional SPECT/CT hybrid imaging, we could reliably assess and quantify right and left ventricular sympathetic innervation. The RV/M ratio was significantly lower in patients diagnosed with ARVC/D and provided sensitive and specific discrimination between patients with ARVC/D and IVF patients.

scholarly journals Decitabine Induced Transient Cardiomyopathy: A Case Report

2012 ◽  
Vol 6 ◽  
pp. CMO.S8598 ◽  
Author(s):  
Chitradeep De ◽  
Jaya Phookan ◽  
Valay Parikh ◽  
Tarun Nagrani ◽  
Mayur Lakhani ◽  
...  
Keyword(s):  
Case Report ◽  
Chest Pain ◽  
Troponin I ◽  
Acute Myocarditis ◽  
Coronary Vessels ◽  
Left Ventricular ◽  
Ventricular Rate ◽  
Heart Catheterization ◽  
Exertional Dyspnea ◽  
Coronary Syndrome

Case Report A 75-yr-old gentleman, with a past medical history of diabetes mellitus and Acute Myeloid Leukemia presented to our emergency department with a chief complaint of exertional dyspnea and chest pain. A week prior to this visit, he had recieved a cycle of decitabine chemotherapy at 20 mg/metered square for ten days. This was his second cycle of decitabine. His out patient medications included megesterol, omeprazole, morphine sulfate and insulin glargine. The patient was admitted to the Coronary Care Unit for Acute Coronary Syndrome. His cardiac enzymes were elevated (peak troponin 30 ng/mL, CKMB 67.4 ng/mL). His 12 lead EKG revealed sinus tachycardia with a ventricular rate of 113, but without acute ST–T wave changes. The BNP was 259 pg/mL. A 2D echo revealed moderate diffuse hypokinesis with an EF of 35%. He subsequently underwent a left heart catheterization, which showed non-obstructive CAD. In our patient, the elevated troponins (peak troponin 30 ng/mL) and BNP were seen concomitant with the onset of cardiogenic shock. Two months ago, his 2 D echocardiogram revealed an ejection fraction of about 55%–65% with slightly increased left ventricular (LV) wall thickness. Discussion The most common adverse effects of decitabine include cytopenia, nausea, pain and erythema/nodules at the injection site. To date, there has been only one reported case of a hypomethylating agent inducing acute myocarditis. We a present a case of reversible, non-ischemic cardiomyopathy secondary to decitabine chemotherapy, which resolved after the drug was discontinued. Trials involving decitabine for the treatment of MDS reported no myocarditis. In our case, the diagnosis of transient cardiomyopathy was highly probable since the patient's troponins and echocardiogram returned to baseline after discontinuation of treatment. Also, the patient never had any further chest pain at his 6 month follow up. In this case, we believe that the elevated Troponin I levels, along with a cardiac catheterization revealing patent coronary vessels, favor our hypothesis that our patient suffered from acute myocarditis as a result of direct toxicity from decitabine chemotherapy. We doubt that there was an underlying infectious etiology, since the patient had three negative blood cultures, two negative urine cultures and a negative viral serology. Our case demonstrates that chest pains in a patient treated with hypomethylating agents should be further explored in order to rule out acute myocarditis.

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