Atrial arrhythmias and thromboembolic complications in adults post Fontan surgery

ObjectivePatients with Fontan surgery experience late complications in adulthood. We studied the factors associated with the development and maintenance of atrial arrhythmias and thromboembolic complications in an adult population with univentricuar physiology post Fontan surgery.MethodsSingle centre retrospective cohort study of patients ≥18 years of age with Fontan circulation followed at our quaternary care centre for more than 1 year were included. Univariate and multivariate regression models were used where applicable to ascertain clinically significant associations between risk factors and complications.Results93 patients were included (age 30.2±8.8 years, 58% men). 28 (30%) had atriopulmonary Fontan connection, 35 (37.6%) had lateral tunnel Fontan and 29 (31.1%) had extracardiac Fontan pathway. After a mean of 7.27±5.1 years, atrial arrhythmia was noted in 37 patients (39.8%), of which 13 developed had atrial fibrillation (14%). The presence of atrial arrhythmia was associated with the number of prior cardiac surgeries/procedures, increasing age and prior atriopulmonary Fontan operation. Thromboembolic events were present in 31 patients (33%); among them 14 had stroke (45%), 3 had transient ischaemic attack (9.7%), 7 had pulmonary embolism (22.6%) and 5 had atrial thrombus with imaging (16.1%). The presence of thromboembolic events was only associated with age and the presence of cirrhosis in multivariate analysis.ConclusionsAtrial arrhythmias are common in adults with Fontan circulation at an early age, and are associated with prior surgical history and increasing age. Traditional risk factors may not be associated with atrial arrhythmia or thromboembolism in this cohort.

Download Full-text

Non-vitamin K antagonist oral anticoagulants in adult congenital heart disease: a single-center study

European Heart Journal ◽

10.1093/eurheartj/ehab724.1867 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

P Garcia Bras ◽

T Mano ◽

T Rito ◽

A Castelo ◽

V Ferreira ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Septal Defect ◽

Oral Anticoagulants ◽

Fontan Circulation ◽

Bleeding Complications ◽

Atrial Arrhythmias ◽

Thromboembolic Events ◽

Bleeding Events

Abstract Introduction Adults with congenital heart disease (ACHD) are at an increased risk for thromboembolic events and atrial arrhythmias are common in this population. Non-vitamin K anatagonist oral anticoagulants (NOACs) prescription is increasing, however data on efficacy and safety in ACHD is unclear, particularly in patients (P) with complex CHD. The aim of the study was to review the use of NOACs in various types of ACHD and assess its safety and efficacy. Methods Evaluation of consecutive ACHD P started on NOAC therapy from 2014 to 2020. P were followed-up for bleeding or thromboembolic events and mortality. CHA2DS2-VASc and HASBLED scores were calculated and risk factors for bleeding were identified. Results 93 ACHD P were included, mean age 52±15 years, 58% female, 44% with complex CHD (3.2% with Fontan circulation), with diagnosis of: 22.2% atrial septal defect, 20% tetralogy of Fallot, 11.1% transposition of the great arteries, 10% Ebstein's anomaly, 8.9% ventricular septal defect, 7.8% pulmonary stenosis, 5.6% ductus arteriosus, 4.4% AV septal defect, 3.4% univentricular heart, 3.4% coarctation of aorta, 2.2% supra-aortic stenosis and 1% with Uhl disease. Most P were anticoagulated with rivaroxaban (43%), followed by edoxaban (24%), apixaban (20%), and dabigatran (13%). The indications for anticoagulation were: atrial arrhythmias (81%), pulmonary embolism (PE) (6.3%), atrial thrombi (4.3%), thromboprophylaxis in Fontan circulation (3.2%), deep vein thrombosis (3.2%) and stroke (2%). 66% of P had a CHA2DS2-VASc score ≥2 and 82% HASBLED score ≤2. In a mean follow-up of 41±21 months (400.4 patient-years), there were embolic events in 2P (1 splenic infarction and 1 PE) albeit both were in the context of oral anticoagulation interruption. The cardiovascular mortality was 2% and allcause mortality 5%, however with no relation to thrombosis or bleeding events. 6 P (6.5%) suffered a minor and 3 P (3.2%) suffered a major bleeding, a median time of 12 (IQR 15) months after starting NOAC therapy. The annual risk for bleeding was 2.2%/patient/year. P with bleeding events showed no significant difference regarding age (55±16 vs 52±15 years, p=0.587), gender (13% female vs 5.1% male, p=0.295) or CHD type (p=0.582). 8.6% of P required dose reduction, mostly for bleeding (3.2%) or renal impairment (2.2%). Renal disease was a strong risk factor for major bleeding (HR 14.6 [95% CI 1.23–73.6], p=0.033 and multivariate analysis showed that an increased HASBLED score was an independent predictor of minor (adjusted HR 3.44 [95% CI 1.13–10.52], p=0.030) and major (adjusted HR 5.29 [95% CI 1.14–24.45], p=0.033) bleeding complications. Conclusion Anticoagulation with NOACs is a safe and effective option for selected ACHD P, although bleeding complications were not negligible, particularly in P with renal disease. Larger scale research studies are required, especially regarding complex CHD such as P with Fontan circulation. FUNDunding Acknowledgement Type of funding sources: None.

Download Full-text

Abstract TMP111: Risk Factors Associated With Watershed Stroke in Pediatric Patients Undergoing Fontan Operation

Stroke ◽

10.1161/str.51.suppl_1.tmp111 ◽

2020 ◽

Vol 51 (Suppl_1) ◽

Author(s):

Jay Duncan ◽

Jo A Tilley ◽

Mahnoor Javed ◽

Johnbosco Umejiego ◽

Tyler Hamby ◽

...

Keyword(s):

Risk Factors ◽

Ventricular Function ◽

Cardiac Disease ◽

Fontan Operation ◽

Significant Risk ◽

Disease Diagnosis ◽

P Value ◽

Valve Regurgitation ◽

Factors Associated ◽

Fontan Surgery

Introduction: Cardiac disease is one of the leading causes of stroke in children. In an effort to separate pulmonary and systemic circulations, patients with single ventricle physiology undergo the Fontan surgery. Watershed strokes have been reported as a complication of Fontan surgery but literature on its prevalence and associated risk factors is lacking. Methods: Retrospective review of records of patients who underwent Fontan operation at our center from 2007 to 2018. Demographic, clinical, imaging, and outcome data were documented for all patients. Key risk factors evaluated:cardiac diagnosis, presence of significant AV-valve regurgitation, poor ventricular function, cardio-pulmonary bypass (CPB) time, Hematocrits, mean arterial pressure (Map) and significance of post-operative resuscitation measured by cardiac index and vasoactive infusion score. Univariate logistic regression was used for analyses, and a p-value of <0.05 was considered statistically significant. Results: We identified 149 patients;14 (10%) had clinical evidence and radiographic confirmation of a watershed stroke. There were no arterial ischemic strokes.From all the variables analyzed (Table 1), patients with significant A-V valve regurgitation pre- and post-operatively, depressed single ventricular function, and underlying diagnosis of hypoplastic left heart syndrome (HLHS) were at a higher risk for watershed strokes. No other risk factors were identified. (Table 1). All had no significant motor deficits at discharge. Conclusion: At our center, watershed infarcts occurred in 10% of patients undergoing Fontan surgery. Cardiac disease diagnosis and factors associated to cardiac function were significant risk factors for watershed stroke rather than surgical related variables.

Download Full-text

Abstract 17119: Sufficient Pulmonary Vascular Bed Before Fontan Surgery Secures Favorable Fontan Hemodynamics

Circulation ◽

10.1161/circ.138.suppl_1.17119 ◽

2018 ◽

Vol 138 (Suppl_1) ◽

Author(s):

Seiko Kuwata ◽

Hirofumi Saiki ◽

Manabu Takanashi ◽

Kenji Sugamoto ◽

Hideaki Senzaki

Keyword(s):

Organ Dysfunction ◽

Fontan Procedure ◽

Fontan Operation ◽

Fontan Circulation ◽

Cardiovascular Remodeling ◽

Vascular Bed ◽

Pulmonary Arterial ◽

Fontan Surgery ◽

Fontan Patients ◽

Pulmonary Vascular Bed

Introduction: While improvement of perioperative care markedly expanded candidacy of Fontan surgery, increasing number of Fontan patients is currently subjected to advanced heart failure therapies. Low pulmonary flow before Fontan procedure has been considered to be acceptable as it keeps pulmonary resistance low, however, compromised growth of pulmonary vascular bed after Fontan completion may deteriorate potential to preserve better Fontan circulation later. We tested our hypothesis that sufficient growth of pulmonary vascular bed before Fontan surgery chronically preserves favorable Fontan hemodynamics and prevents patients from cardiovascular remodeling. Methods and Results: Consecutive 33 patients who had undergone Fontan operation (median year after the surgery: 5.3years) were enrolled in this study. During cardiac catheterization, pulmonary arterial index (PAI) as the representative of vascular bed both before and after Fontan procedure was measured and hemodynamics as well as blood samples as a marker for end-organ dysfunction were analyzed. Before Fontan procedure, PAI ranged 74-426 and resistance of pulmonary artery (PAR) ranged 0.4-3.1. After median of 5.3 years of Fontan procedure, CVP exhibited modest decline with preserving cardiac index (CI). Interestingly, central venous pressure (CVP) with chronic Fontan circulation was negatively correlated with PAI of pre-Fontan procedure (P=0.02) whereas it was independent of PAR. Consistent with this, higher PAI before Fontan procedure was also significantly associated with higher CI (P<0.01) and lower levels of plasma brain natriuretic peptides (P=0.03). Importantly, patients with low PAI showed markedly elevated activation of renin-angiotensin-aldosterone (RAAS) activation and elevation of serum gamma-gltamyl transferase (P<0.01), a surrogate marker of liver congestion. Multivariate analysis revealed that high PAI was determinant of low CVP in the chronic Fontan patients, independent of body size, postoperative interval and pulmonary arterial resistance. Conclusion: Sufficient pulmonary vascular bed before Fontan procedure independently lowers CVP, may prevent cardiovascular remodeling by RAAS suppression as well as end organ dysfunction in chronic Fontan patients. Our result suggested importance of strategies to accelerate pulmonary vascular growth before Fontan procedure.

Download Full-text

Thromboembolic Events During Chemotherapy for Germ Cell Cancer: A Cohort Study and Review of the Literature

Journal of Clinical Oncology ◽

10.1200/jco.2000.18.10.2169 ◽

2000 ◽

Vol 18 (10) ◽

pp. 2169-2178 ◽

Cited By ~ 154

Author(s):

Nir I. Weijl ◽

Marc F. J. Rutten ◽

Aeilko H. Zwinderman ◽

H. Jan Keizer ◽

Marianne A. Nooy ◽

...

Keyword(s):

Risk Factors ◽

Confidence Interval ◽

Germ Cell ◽

Cancer Patients ◽

Odds Ratio ◽

Cell Cancer ◽

Germ Cell Cancer ◽

Thromboembolic Events ◽

Thromboembolic Complications ◽

High Doses

PURPOSE: To evaluate the risk of major thromboembolic complications in male germ cell cancer patients receiving cisplatin-based chemotherapy and to review the literature on this subject. PATIENTS AND METHODS: One hundred seventy-nine germ cell cancer patients treated between January 1979 and May 1997 in our hospital were analyzed with respect to risk factors for developing thromboembolic events, such as baseline tumor characteristics, prior tumor therapy, administration of cytostatic agents, and the use of antiemetic drugs. The patients were treated with a variety of combination chemotherapy regimens, primarily cisplatin-containing combination regimens. RESULTS: Of the 179 patients, 15 patients (8.4%) were identified who developed a total of 18 major thromboembolic complications in the time period between the start of chemotherapy and 6 weeks after administration of the last cytostatic drug in first-line treatment. Of these 18 events, three (16.7%) were arterial events, including two cerebral ischemic strokes, and 15 (83.3%) were venous thromboembolic events, including 11 pulmonary embolisms. One (5.6%) of the 18 events was fatal. Liver metastases (odds ratio, 4.9; 95% confidence interval, 1.1 to 20.8) and the administration of high doses of corticosteroids (≥ 80 mg dexamethasone per cycle; odds ratio, 3.5; 95% confidence interval, 1.2 to 10.3) as antiemetic therapy were identified as risk factors for the development of major thromboembolic complications. CONCLUSION: Germ cell cancer patients who receive chemotherapy, in particular those who have liver metastases or receive high doses of corticosteroids, are at considerable risk of developing thromboembolic complications.

Download Full-text

Thrombosis as a complication of the Klippel-Trénaunay syndrome and other vascular malformations

Phlebologie ◽

10.1055/s-0037-1622263 ◽

2009 ◽

Vol 38 (03) ◽

pp. 115-121

Author(s):

A. Geyer ◽

F. Benedix ◽

A. Strölin ◽

V. Lichte

Keyword(s):

Risk Factors ◽

Vascular Malformations ◽

Post Partum ◽

Clinical Development ◽

Young Age ◽

Thromboembolic Events ◽

Thromboembolic Complications ◽

Deep Vein ◽

Klippel Trenaunay Syndrome ◽

Deep Vein Thromboses

SummaryThe Klippel-Trénaunay syndrome (KTS) is a congenital disorder of vascular malformations characterized by the clinical trias of naevi flammei, varicosis and hypertrophy of the affected limb. According to its clinical development KTS can be diagnosed directly post partum or becomes apparent in the course of the development particularly regarding incomplete forms. The most described symptoms next to swelling are pain and augmented sweating of the affected extremity. Complications of KTS are bleeding from mechanically traumatised vascular malformations or widened vessels, but also thromboembolic events are reported. We report on two patients with KTS and one patient with angiodysplasia, who developed deep vein thromboses in the affected limb at young age. None of the patients suffered from thrombophilia or other predisposing risk factors for thrombosis. KTS and other angiodysplastic disorders seem to be a risk factor for thromboembolic complications even at young age. A comprehensive counceling of the patients concerning the prevention of complications and especially the symptoms of thromboembolic events is important for early diagnosis and immediate therapy.

Download Full-text

Incidence, risk factors and outcomes of atrial arrhythmias in adult patients with atrioventricular septal defect

EP Europace ◽

10.1093/europace/euab116.497 ◽

2021 ◽

Vol 23 (Supplement_3) ◽

Author(s):

E Jacquemart ◽

F Bessiere ◽

N Combes ◽

M Ladouceur ◽

L Iserin ◽

...

Keyword(s):

Risk Factors ◽

Adult Congenital Heart Disease ◽

Pacemaker Implantation ◽

Atrioventricular Septal Defect ◽

Adult Patients ◽

Atrial Arrhythmia ◽

Public Institution ◽

Right Atrial ◽

Atrial Arrhythmias ◽

Logistic Regression Models

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): French Federation of Cardiology Background The number of adults with atrioventricular septal defects (AVSD) is growing, with however, very few data regarding the natural history of atrial arrhythmias in this specific population. We aimed to assess the incidence, associated factors and outcomes of atrial arrhythmias among adult patients with AVSD. Methods Multicentric retrospective cohort of patients with AVSD from 3 referral centers specialized in adult congenital heart disease. Unbalanced AVSD, univentricular hearts, and Eisenmenger syndromes were excluded. Lifetime cumulative incidences of different types of atrial arrhythmia (>30 seconds) were analyzed (atrial fibrillation [AF] and intra atrial reentrant tachycardia/focal atrial tachycardia [IART/FAT]). Multiple logistic regression models were used to identify risk factors for atrial arrhythmias. Results The cohort comprised of 391 patients (61.6% of women) with a mean age of 36.3 ± 16.3 years and 17.3 ± 14.2 years of follow-up after surgical repair in operated patients, including 333 (85.1%) partial/intermediate and 58 (1.0%) complete AVSD. Overall, atrial arrhythmias were documented in 98 patients (25.1%). The lifetime risks for developing atrial arrhythmia to ages 20, 40, and 60 were 3.7%, 17.6%, and 54.8%. IART/FAT was the leading arrhythmia until the age of 45 then AF surpassed IART/FAT. Age (OR = 1.4, 95%CI = 1.2-1.6 by 5 years increment), the number of cardiac surgeries (OR = 4.1, 95%CI = 2.5-6.9), left atrial dilatation (OR = 3.1, 95%CI = 1.4-6.8), right atrial dilatation (OR = 4.1, 95%CI = 1.7-10.3), and moderate or severe left AV valve regurgitation (OR = 3.7, 95%CI = 1.2-11.7) were independently associated with a higher risk of atrial arrhythmias. Patients with atrial arrhythmias more frequently had pacemaker implantation (41.8% vs. 8.5%, p < 0.001), heart failure (24.5% vs 1.0%, p < 0.001) and cerebrovascular accidents (11.2% vs 3.4%, p = 0.007). Conclusions The lifetime risk of atrial arrhythmias in patients with AVSD is considerable with more than half of patients who will develop an atrial arrhythmia by the age of 60. Atrial arrhythmias are associated with a significant morbidity in this population. Abstract Figure. Central Illustration AVSD

Download Full-text

Abstract 17376: Atrial Arrhythmias in SCAD Patients

Circulation ◽

10.1161/circ.142.suppl_3.17376 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Alex D Tarabochia ◽

Nicholas Y Tan ◽

Sharonne N Hayes ◽

Patricia J Best ◽

Rajiv Gulati ◽

...

Keyword(s):

Risk Factors ◽

Cardiovascular Risk ◽

Coronary Artery ◽

Cardiovascular Risk Factors ◽

Artery Bypass ◽

Atrial Arrhythmia ◽

Data Repository ◽

Artery Dissection ◽

Atrial Arrhythmias ◽

Coronary Syndrome

Introduction: Spontaneous coronary artery dissection (SCAD) is an uncommon but increasingly recognized cause of acute coronary syndrome and sudden cardiac death. We aim to review the occurrence of atrial fibrillation and atrial flutter in the Mayo Clinic SCAD Registry (MCSR) to gain insight into the association of SCAD and atrial arrhythmias. Methods: Atrial arrhythmias were queried amongst patients in the MCSR using diagnostic codes and text contained in both a large data repository known as the Advanced Cohort Explorer as well as a clinical database maintained by nurse abstractors for patients in the MCSR. All positive were manually verified using available medical records. Univariable logistic regression analysis was performed using JMP to investigate associations with classic cardiovascular risk factors. Results: A total of 1,215 patients from the Mayo SCAD registry were included. Six patients had a pre SCAD atrial arrhythmia, 19 had an atrial arrhythmia during a SCAD event, and 11 had a post SCAD atrial arrhythmia. Univariable analysis did not reveal significant associations with diabetes, hypertension, hyperlipidemia, or smoking. Conclusion: Atrial arrhythmias were not uncommonly seen in patients at the time of or after SCAD, especially after cardiac arrest or coronary artery bypass grafting. No significant associations with traditional cardiovascular risk factors were observed. Further investigation into the mechanism of atrial arrhythmia development as well as its management in the setting of SCAD is warranted.

Download Full-text

Cytoreductive treatment in real life: a chart review analysis on 1440 patients with polycythemia vera

Journal of Cancer Research and Clinical Oncology ◽

10.1007/s00432-021-03855-5 ◽

2021 ◽

Author(s):

Carl C. Crodel ◽

Kathleen Jentsch-Ullrich ◽

Marcel Reiser ◽

Lutz Jacobasch ◽

Annette Sauer ◽

...

Keyword(s):

Risk Factors ◽

High Risk ◽

Acetylsalicylic Acid ◽

Chart Review ◽

Real Life ◽

Therapy Resistance ◽

Thromboembolic Events ◽

Thromboembolic Complications ◽

Healthy Individuals ◽

Cytoreductive Treatment

Abstract Purpose Patients with polycythemia vera (PV) show an elevated incidence of thromboembolic complications and decreased survival when compared to age-matched healthy individuals. Hypercellularity as indicated by elevated hematocrit, pathophysiological changes induced by the JAK2 driver mutation and cardiovascular risk factors contribute to the increased incidence of thromboembolic events. Higher age and a history of thromboembolic events define a high-risk population of PV patients. Depending on the individual risk profile, phlebotomy or pharmacologic cytoreduction is recommended in combination with low-dose acetylsalicylic acid. Stringent cytoreduction is required for effective risk reduction. However, in recent reports, the rate of thromboembolic complications in PV patients under cytoreductive therapy appears still elevated compared to healthy individuals. This study reports on a chart review to assess for cytoreductive therapy of 1440 PV patients in real life. Methods Forty-two eligible hematologists/oncologists in private practice treating patients with MPN were recruited to participate in a paper–pencil-based survey conducted between January 2019 and March 2020 in Germany. Physicians were asked to report primary documented data obtained from patient charts. Descriptive analyses were conducted to assess for patient characteristics, treatment modalities, risk factors and thromboembolic complications. Results Data were collected from the patient charts of 1440 individuals diagnosed with PV. The patient population was older than those reported in multicenter trials with a median age of 72.2 years at the time of reporting and 63.5 years at diagnosis. Age was the main factor accounting for high-risk status with 84.7% of patients being above the age of 60 followed by thromboembolic complications reported in 21.3% of patients. The use of pharmacologic cytoreduction was highly variable between participating centers with an average of 60.7% and a range of 10.1–100%. Hydroxyurea was the most frequently used drug followed by ruxolitinib, while interferons were reported for a minority of patients. For 35.4% of patients a persistent need for phlebotomy in addition to cytoreductive treatment was reported. Although presence of high-risk criteria and insufficient disease control were reported as main triggers to initiate pharmacologic cytoreduction, 28.1% had elevated hematocrit values (> 45%) and 38.6% showed persistence of elevated leukocyte count (> 109/l) while on cytoreductive treatment. In contrast, physician-reported symptom burden was lower than published in clinical trials and patient-reported outcomes. The rate of patients experiencing thromboembolic complications was 32.2% at any time and 14.3% after diagnosis with most patients receiving acetylsalicylic acid and 10.8% remaining on oral anticoagulants or heparin. Conclusions Cytoreductive treatment of high-risk PV in real life is highly variable regarding indication for cytoreduction and definition of therapy resistance. This study highlights the need for (i) improved risk stratification for thromboembolic events, (ii) consequent indication of pharmacologic cytoreduction in high-risk PV and (iii) attention to signs of therapy resistance that can trigger an earlier and stringent switch to second line agents.

Download Full-text