The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the clinical association of antiphospholipid autoantibodies (aPL) with a syndrome of hypercoagulability that can affect any blood vessel, irrespective of type or size. Involvement of larger vessels, such as arteries or veins, manifests in the form of thrombosis or embolism, whereas involvement of smaller vessels, including capillaries, arterioles, and venules, manifests as thrombotic microangiopathy. Virtually any organ in the body, including the kidney, can be affected. Here, we review the basic principles and recent advances in our understanding of APS, and discuss the broad spectrum of renal diseases that have been observed in association with this syndrome. We also discuss the impact that APS may have on pre-existing renal disease as well as current recommendations for treatment of APS.

The antiphospholipid syndrome.

Table 1. Classes of recommendation

Table 2. Levels of evidence

Table 3. Estimated fetal and maternal effective doses for various diagnostic and interventional radiology procedures

Table 4. Predictors of maternal cardiovascular events and risk score from the CARPREG study

Table 5. Predictors of maternal cardiovascular events identified in congential heart diseases in the ZAHARA and Khairy study

Table 6. Modified WHO classification of maternal cardiovascular risk: principles

Table 7. Modified WHO classification of maternal cardiovascular risk: application

Table 8. Maternal predictors of neonatal events in women with heart disease

Table 9. General recommendations

Table 10. Recommendations for the management of congenital heart disease

Table 11. Recommendations for the management of aortic disease

Table 12. Recommendations for the management of valvular heart disease

Table 13. Recommendations for the management of coronary artery disease

Table 14. Recommendations for the management of cardiomyopathies and heart failure

Table 15. Recommendations for the management of arrhythmias

Table 16. Recommendations for the management of hypertension

Table 17. Check list for risk factors for venous thrombo-embolism

Table 18. Prevalence of congenital thrombophilia and the associated risk of venous thrombo-embolism during pregnancy

Table 19. Risk groups according to risk factors: definition and preventive measures

Table 20. Recommendations for the prevention and management of venous thrombo-embolism in pregnancy and puerperium

Table 21. Recommendations for drug use

ABPM
: ambulatory blood pressure monitoring
ACC
: American College of Cardiology
ACE
: angiotensin-converting enzyme
ACS
: acute coronary syndrome
AF
: atrial fibrillation
AHA
: American Heart Association
aPTT
: activated partial thromboplastin time
ARB
: angiotensin receptor blocker
AS
: aortic stenosis
ASD
: atrial septal defect
AV
: atrioventricular
AVSD
: atrioventricular septal defect
BMI
: body mass index
BNP
: B-type natriuretic peptide
BP
: blood pressure
CDC
: Centers for Disease Control
CHADS
: congestive heart failure, hypertension, age (>75 years), diabetes, stroke
CI
: confidence interval
CO
: cardiac output
CoA
: coarction of the aorta
CT
: computed tomography
CVD
: cardiovascular disease
DBP
: diastolic blood pressure
DCM
: dilated cardiomyopathy
DVT
: deep venous thrombosis
ECG
: electrocardiogram
EF
: ejection fraction
ESC
: European Society of Cardiology
ESH
: European Society of Hypertension
ESICM
: European Society of Intensive Care Medicine
FDA
: Food and Drug Administration
HCM
: hypertrophic cardiomyopathy
ICD
: implantable cardioverter-defibrillator
INR
: international normalized ratio
i.v.
: intravenous
LMWH
: low molecular weight heparin
LV
: left ventricular
LVEF
: left ventricular ejection fraction
LVOTO
: left ventricular outflow tract obstruction
MRI
: magnetic resonance imaging
MS
: mitral stenosis
NT-proBNP
: N-terminal pro B-type natriuretic peptide
NYHA
: New York Heart Association
OAC
: oral anticoagulant
PAH
: pulmonary arterial hypertension
PAP
: pulmonary artery pressure
PCI
: percutaneous coronary intervention
PPCM
: peripartum cardiomyopathy
PS
: pulmonary valve stenosis
RV
: right ventricular
SBP
: systolic blood pressure
SVT
: supraventricular tachycardia
TGA
: complete transposition of the great arteries
TR
: tricuspid regurgitation
UFH
: unfractionated heparin
VSD
: ventricular septal defect
VT
: ventricular tachycardia
VTE
: venous thrombo-embolism
WHO
: World Health Organization

Guidelines summarize and evaluate all available evidence, at the time of the writing process, on a particular issue with the aim of assisting physicians in selecting the best management strategies for an individual patient, with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes but are complements for textbooks and cover the European Society of Cardiology (ESC) Core Curriculum topics. Guidelines and recommendations should help the …

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ESC Guidelines on the management of cardiovascular diseases during pregnancy The Task Force on the Management of Cardiovascular Diseases

Until the 1990s, venous thromboembolism (VTE) was viewed primarily as a complication of hospitalization for major surgery (or associated with the late stage of terminal illness). However, recent trials in patients hospitalized with a wide variety of acute medical illnesses have demonstrated a risk of VTE in medical patients comparable with that seen after major general surgery. In addition, epidemiologic studies have shown that between one quarter and one half of all clinically recognized symptomatic VTEs occur in individuals who are neither hospitalized nor recovering from a major illness. This expanding understanding of the population at risk challenges physicians to carefully examine risk factors for VTE to identify high-risk patients who could benefit from prophylaxis. Factors sufficient by themselves to prompt physicians to consider VTE prophylaxis include major surgery, multiple trauma, hip fracture, or lower extremity paralysis because of spinal cord injury. Additional risk factors, such as previous VTE, increasing age, cardiac or respiratory failure, prolonged immobility, presence of central venous lines, estrogens, and a wide variety of inherited and acquired hematological conditions contribute to an increased risk for VTE. These predisposing factors are seldom sufficient by themselves to justify the use of prophylaxis. Nevertheless, individual risk factors, or combinations thereof, can have important implications for the type and duration of appropriate prophylaxis and should be carefully reviewed to assess the overall risk of VTE in each patient.

Risk factors for venous thromboembolism

Most deep vein thromboses (DVTs) start in the calf, and most probably resolve spontaneously. Thrombi that remain confined to the calf rarely cause leg symptoms or symptomatic pulmonary embolism (PE). The probability that calf DVT will extend to involve the proximal veins and subsequently cause PE increases with the severity of the initiating prothrombotic stimulus. Although acute venous thromboembolism (VTE) usually presents with either leg or pulmonary symptoms, most patients have thrombosis at both sites at the time of diagnosis. Proximal DVTs resolve slowly during treatment with anticoagulants, and thrombi remain detectable in half of the patients after a year. Resolution of DVT is less likely in patients with a large initial thrombus or cancer. About 10% of patients with symptomatic DVTs develop severe post-thrombotic syndrome within 5 years, and recurrent ipsilateral DVT increases this risk. About 10% of PEs are rapidly fatal, and an additional 5% cause death later, despite diagnosis and treatment. About 50% of diagnosed PEs are associated with right ventricular dysfunction, which is associated with a approximately 5-fold greater in-hospital mortality. There is approximately 50% resolution of PE after 1 month of treatment, and perfusion eventually returns to normal in two thirds of patients. About 5% of treated patients with PE develop pulmonary hypertension as a result of poor resolution. After a course of treatment, the risk of recurrent thrombosis is higher (ie, approximately 10% per patient-year) in patients without reversible risk factors, in those with cancer, and in those with prothrombotic biochemical abnormalities such as antiphospholipid antibodies and homozygous factor V Leiden.

Natural History of Venous Thromboembolism

Background— The concept that venous thromboembolism (VTE) and atherosclerosis are 2 completely distinct entities has recently been challenged because patients with VTE have more asymptomatic atherosclerosis and more cardiovascular events than control subjects. We performed a meta-analysis to assess the association between cardiovascular risk factors and VTE. Methods and Results— Medline and EMBASE databases were searched to identify studies that evaluated the prevalence of major cardiovascular risk factors in VTE patients and control subjects. Studies were selected using a priori defined criteria, and each study was reviewed by 2 authors who abstracted data on study characteristics, study quality, and outcomes. Odds ratios or weighted means and 95% confidence intervals (CIs) were then calculated and pooled using a random-effects model. Statistical heterogeneity was evaluated through the use of χ2 and I2 statistics. Twenty-one case-control and cohort studies with a total of 63 552 patients met the inclusio...

Cardiovascular risk factors and venous thromboembolism: a meta-analysis.

Much more is known about the APLS than 15 yr ago, but more needs to be done. Clinically, major characteristics of the disorder have been defined, but the course of the disease and its relationship to other disorders, such as systemic lupus erythematosus (SLE), need to be defined. Good progress has been made in standardizing anticardiolipin and, to a lesser extent, LA. However, problems with interlaboratory and inter-assay variation need to be addressed. Doctors are getting a better idea of how to manage these patients with a view to preventing recurrences of thrombosis or pregnancy losses, but prospective studies are still desirable to give more definitive answers. Attention has turned to the pathogenesis of the disorder. It seems likely that antibodies mediate the clinical disorders with which they are associated, but the mechanisms by which this occurs still require further study.

Harris En

Papers

The antiphospholipid syndrome. Diagnosis, management, and pathogenesis.