A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI.

This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the possibility of a diffuse thrombotic process.

Percutaneous renal denervation and the second generation EnligHTN System.

Hypertension remains a major public health burden despite the plethora of therapeutic agents available for this disorder, compelling innovation of alternate therapies including interventional approaches where necessary. The kidney is a major player in the pathophysiology of this disease with increased sympathetic activity being the key factor in the initiation and maintenance of drug resistant hypertension in many patients. Thus renal denervation targeted at decreasing sympathetic drive is becoming the apparent choice in carefully selected patients with resistant hypertension who have exhausted all medical options. The Symplicity and EnligHTN trials using first and second generation catheters respectively have demonstrated that renal sympathetic denervation results in significant blood pressure reduction. The initial renal denervation catheter used in the Symplicity trial was a single electrode system. Refinement of this process has led to the EnligHTN catheter's design. This is a multielectrode self-expanding nitinol basket that allows the positioning of the thermal injury pattern to be pre-specified and in theory lead to better positioning of the lesions. We present a review of the premise behind renal artery denervation, discuss the data and early technologies focusing on the characteristics and utility of the first multielectrode renal denervation device, the EnligHTN renal denervation catheter.

The role and management of sympathetic overactivity in cardiovascular and renal complications of diabetes.

Feedback activation of neurohormonal pathways in the setting of kidney or heart failure contributes to the development and progression of dysfunction in the other. Diabetes and its management independently activate these same pathogenic pathways, feeding into this vicious cycle and contributing to a poor prognosis. One of the most important of these neurohormonal pathways is the sympathetic nervous system (SNS). The activity of the SNS in increased in patients with chronic kidney disease, even in the absence of renal impairment or heart failure. There is a strong relationship between SNS overactivity and prognosis, and evidence that blockade of SNS reduces morbidity and mortality in patients with diabetes. However, modulation of SNS is underutilised as a strategy to protect both the diabetic kidney and the heart. This is partly because of the historically poor tolerability, adverse haemodynamic and metabolic effects, lack of selectivity of ß-blockers and the lack of specificity of other interventions that might modify SNS activation. The advent of "vasodilating ß-blockers" with better tolerability as well as more favourable effects on renal function and metabolic profiles opens the door for their more widespread utility in patients with diabetes. Radiofrequency renal sympathectomy and baroreflex activation technologies also offer exciting new ways to tackle the challenge of sympathetic overactivity.

Cardio-renal syndrome: new perspective in diagnostics.

Chronic heart failure and chronic renal failure are at epidemic proportions. These patients have significantly altered cardiac, renal, and all-cause outcomes. Much of the current research has focused on treating these individual organs in isolation. Although there are positive data on outcomes with neurohormonal modulation, they, however, remain underused. At present, data lacks for novel treatment options, while evidence continues to point at significantly worsened prognosis. Current diagnostic tools that detect acute changes in renal function or renal injury appear retrospective, which often hinder meaningful diagnostic and therapeutic decisions. This review is aimed at exploring the importance of accurate assessment of renal function for the heart failure patient by providing a synopsis on cardio-renal physiology and establishing the possibility of novel approaches in bridging the divide.

Minimally invasive late free flap salvage: indications, efficacy and implications for reconstructive microsurgeons.

One of the most common causes of free flap compromise is microvascular thrombosis. Acland et al describe two described zones of injury: zone 1 the anastomotic site and zone 2 downstream. Factors contributing to zone 1 thromboses include anastomotic irregularities, suture material and platelet adhesion. This often presents in the early postoperative period. Zone 2 however, is less well described and is associated with diffuse microvascular ischaemia. Often, these cases are associated with the use of vein grafts in a delayed reconstructive setting, and present relatively late in their postoperative follow up. There are sporadic reports in the literature of late free flap salvage managed via anastomotic revision, thrombectomy, and the use of thrombolytic agents. We describe the successful use of catheter-directed endovascular urokinase in revascularizing two free flaps which presented in the late postoperative setting. This report demonstrates the safety and efficacy of this technique in free flap salvage. Although late presentation of free flap compromise is uncommon, this report reiterates the importance of long-term surveillance of these patients. It should be remembered, however, that long-term anticoagulation is required, and may not be feasible in certain patient populations. Given that free tissue transfer is often employed when other forms of reconstruction are unavailable, endovascular thrombolysis is a valuable tool for the reconstructive microsurgeon, and its role in early free flap salvage warrants exploration.

Cytomegalovirus cholangitis presenting with recurrent hemobilia.

Cytomegalovirus (CMV) cholangitis is a rare manifestation of CMV infection that has previously been described only in HIV-infected patients and solid-organ-transplant recipients. Reported here is a case of CMV cholangitis that occurred in a patient on chronic corticosteroid therapy who presented with recurrent hemobilia and biliary obstruction and was successfully treated with ganciclovir and cholecystostomy. A search of the medical literature revealed no previous case of this kind.

Enterococcus avium endocarditis.

We describe a rare case of Enterococcus avium endocarditis in a patient with an ovarian malignancy, and review 2 previously reported cases. We contend that Enterococcus avium bacteremia and endocarditis may be associated with gastrointestinal pathology.