Acute Pulmonary Thromboembolism in a Patient with Nutcracker Syndrome and Antiphospholipid Syndrome.

Nutcracker syndrome (NCS), which is defined as compression of the left renal vein between the aorta and the superior mesenteric artery, is usually benign and self-limiting. Long-term renal venous retention increases the risk of renal vein thrombosis. However, NCS rarely develops into isolated thrombosis of the left renal vein; the reason for this process remains unknown. We describe a young man with antiphospholipid syndrome, who developed overt pulmonary thromboembolism due to an isolated thrombus in the left renal vein. Complicating antiphospholipid syndrome might trigger acute pulmonary thromboembolism (APTE) in patients with NCS. To the best of our knowledge, this is the first report of APTE arising due to isolated left renal vein thrombosis in patients with NCS.

Incidence, Characteristics and Management of Venous Thromboembolism in Japan During 2011.

BACKGROUND: This study aimed to determine the incidence, characteristics and management of venous thromboembolism (VTE) in Japan during 2011.Methods and Results:A retrospective study assessed responses to a questionnaire regarding treating newly diagnosed VTE at all admitting hospitals throughout Japan during 2011. More individuals were diagnosed with VTE than ever before, with 16,096 cases of diagnosed pulmonary embolism (PE) and 24,538 cases of diagnosed deep vein thrombosis (DVT). Almost half (47.2%) of the PE patients had a relatively mild condition with no right ventricular overload. Similarly, almost half (43.8%) of the DVT patients had a relatively mild condition with isolated calf thrombus. Most of PE patients were treated by anticoagulation, and fewer were treated using thrombolytic agent or inferior vena cava (IVC) filter. CONCLUSIONS: The present study showed a remarkable increase in the incidence of VTE in Japan during 2011. Relatively mild conditions such as non-massive PE and isolated calf DVT were frequently diagnosed. Among PE patients, thrombolytic therapy or IVC filter implantation decreased compared with previous surveys. The appropriate management of isolated calf DVT requires further investigation.

Early and long-term outcomes of venous stent implantation for iliac venous stenosis after catheter-directed thrombolysis for acute deep vein thrombosis.

BACKGROUND: Although stent implantation is used worldwide for peripheral arterial disease, there is little data on the safety and long-term patency of stents implanted for venous disease. METHODS AND RESULTS: We studied 13 patients with 14 lesions (6 males, 7 females, mean age: 63.2±10.2 years) diagnosed with venous stenosis and who underwent venous stenting. We examined the location of the stenosis, safety of venous stenting, implantation success rate, and long-term stent patency rate. All patients were suffering from venous stenosis in the left common iliac vein because of iliac vein compression syndrome. No major complications occurred during stent implantation. Acute stent occlusion occurred in 1 patient, who was given additional thrombolytic therapy. Of the 13 patients, 10 underwent venography or contrast-enhanced computed tomography (CT) during mid-term follow-up (mean: 12.9±16.1 months), and only 1 stent was occluded, resulting in a patency rate of 90.0%. The latter patient decided to stop taking warfarin soon after stent implantation. Furthermore, 5 patients underwent contrast-enhanced CT to assess the long-term patency of their stents (mean: 79.6±31.2 months), and none was occluded. CONCLUSIONS: Venous stents display a high long-term patency rate, and hence are a useful tool for treating iliac venous stenosis.

Autopsy study of pulmonary capillary hemangiomatosis with inflammatory cell infiltration into the myocardium.

Pulmonary capillary hemangiomatosis is a rare form of pulmonary artery hypertension; to date, only few descriptions of myocardial pathology in pulmonary capillary hemangiomatosis have been reported in the literature. We report the case of a Japanese female patient who was diagnosed with pulmonary capillary hemangiomatosis combined with acute myocardial inflammation on performing autopsy. She was admitted to our hospital because of acute pneumonia and subsequently suddenly developed severe hypoxemia with breathing difficulty and died 13 days after admission. At autopsy, the histology of the lung was consistent with pulmonary capillary hemangiomatosis. Additionally, a diffuse severe infiltration of inflammatory cells was associated with edema in the myocardium. Myocytolysis was limited and fibrosis was absent. To the best of our knowledge, pulmonary capillary hemangiomatosis with acute myocarditis-like histological findings has been described for the first time through our case.

Effects of low-dose tolvaptan on electrolyte abnormality and hemodynamic parameters in a liver cirrhosis-associated portopulmonary hypertension patient: A case report.

The present study reported a case of portopulmonary hypertension (POPH) that was secondary to underlying liver cirrhosis in a 58-year-old woman, who was successfully treated with low-dose tolvaptan. The patient had suffered from refractory peripheral edema and electrolyte abnormalities, including severe hypokalemia, under the combination therapy of sildenafil, ambrisentan, furosemide and spironolactone. Subsequent to the initiation of low-dose tolvaptan at 3.75 mg/day with concurrent de-escalation of the dose of furosemide, the daily urine volume increased, peripheral edema improved and the serum potassium level increased immediately. In addition, plasma renin activity, plasma aldosterone concentration and plasma brain natriuretic peptide level decreased within 1 week after the initiation of tolvaptan therapy. Hemodynamic assessments using a right heart catheter revealed that the pulmonary vascular resistance decreased by ~20%. Finally, chronic combination therapy with spironolactone and low-dose tolvaptan without loop diuretics achieved adequate fluid management. In conclusion, the findings of the present study suggest that low-dose tolvaptan may be a promising therapeutic option for liver cirrhosis-associated POPH in patients with an electrolyte abnormality due to liver cirrhosis and conventional diuretics.

High prevalence of congenital thrombophilia in patients with pregnancy-related or idiopathic venous thromboembolism/pulmonary embolism.

Congenital thrombophilia which is characterized by deficiencies in proteins such as antithrombin (AT), protein C (PC) and protein S (PS), is a major cause of venous thromboembolism (VTE). A total of 130 patients with VTE were evaluated for congenital thrombophilia based on the activity of AT, PC, or PS. Fifteen VTE patients with congenital AT deficiency (11.5 %), 16 with congenital PC deficiency (12.3 %) and eight with congenital PS deficiency (6.2 %) were diagnosed using DNA analysis. The frequency of congenital AT deficiency was significantly higher in subjects with pregnancy-related and idiopathic VTE than in those with VTE due to other causes, and congenital PC and PS deficiency were frequently associated with idiopathic VTE. Among the groups examined, the plasma levels of AT were the lowest in subjects with pregnancy-related VTE. Although our findings may have been influenced by some unintentional bias, congenital thrombophilia is nevertheless a major cause of VTE in pregnant patients as well as in young or middle-aged patients without any underlying diseases.

Pulse-spray catheter-directed thrombolysis with urokinase and argatroban for thrombo-occlusion of an inferior vena cava filter due to heparin-induced thrombocytopenia with thrombosis.

A 50-year-old man developed acute sub-massive pulmonary thromboembolism and proximal deep venous thrombosis. A continuous intravenous infusion of unfractionated heparin (UFH) was started, and an inferior vena cava (IVC) filter was implanted. He developed symptomatic complete obstruction of the filter 6 days after the initiation of UFH, and he was transferred to our hospital. We started pulse-spray catheter-directed thrombolysis (CDT) with urokinase. However, thrombocytopenia occurred 11 days after the initiation of heparin. We suspected heparin-induced thrombocytopenia (HIT) with thrombosis (HITT), and switched from UFH to argatroban. In addition, we continued pulse-spray CDT. As a result, thrombocytopenia improved and thrombolysis was successful without complications of recurrent thromboembolism or bleeding, leading to retrieval of the IVC filter. The antigen assay for HIT was strongly positive, supporting our diagnosis. In patients with suspected HIT it is important to switch from heparin to an alternative anticoagulant as soon as possible, and pulse-spray CDT with urokinase and argatroban as anticoagulant therapy may be effective treatment for thrombo-occlusions of IVC filters in patients with HITT. .

Utility of right ventricular Tei-index for assessing disease severity and determining response to treatment in patients with pulmonary arterial hypertension.

BACKGROUND: We sought to evaluate the potential utility of echocardiography-derived morphological and functional right ventricular (RV) variables for assessing disease severity of pulmonary arterial hypertension (PAH) and determining the changes in the patient's hemodynamics in the clinical course. METHODS AND RESULTS: This study consisted of 24 normal controls (the control group) and 24 patients with PAH at rest or with exercise (the PAH group) who underwent echocardiography, right heart catheterization, plasma brain natriuretic peptide (BNP) measurement, and six-minute walk distance (6MWD) test. The PAH group had poorer RV echocardiographic variables than the control group. RV Tei-index was more strongly correlated with 6MWD, BNP, cardiac index, mean pulmonary arterial pressure, and pulmonary vascular resistance (PVR) than other RV echocardiography-derived variables including RV end-diastolic areas, RV fractional area change, and tricuspid annular plane systolic excursion. In 16 of the 24 patients who successfully underwent repeated examination during follow up (13.3 ± 4.9 months; range, 5-24 months), PVR decreased from 486 ± 380 dynes cm(-5) to 346 ± 252 dynes cm(-5), and RV Tei-index decreased from 0.55 ± 0.30 to 0.42 ± 0.17, and the changes in RV Tei-index were correlated with the concomitant changes in PVR during the clinical course of PAH (r=0.706, p=0.002). Tricuspid annular plane systolic excursion and RV fractional area change did not change during the follow up. CONCLUSIONS: Quantitative echocardiography revealed that the measurement of RV Tei-index is of great clinical utility for predicting disease severity of PAH and determining the changes in the patient's hemodynamics in the clinical course.