Pulmonary Artery Thrombosis Research Articles

Fatal diffuse pulmonary arterial thrombosis as a complication of nephrotic syndrome

A 21-year-old man was admitted to our hospital because of leg edema. Because laboratory findings revealed massive proteinuria and hypoproteinemia, he was diagnosed as having nephritic syndrome caused by minimal change disease. He was given a continuous heparin infusion and intravenous steroid therapy, at a prednisolone dose of 1 mg/kg per day, and his condition gradually improved. Five months after discharge, the patient's proteinuria relapsed. He was readmitted to our hospital and we restarted anticoagulant treatment with intravenous heparin and 60 mg prednisolone. On the third hospital day, he complained of chest pain with sudden onset and dyspnea. He quickly developed shock and died. The findings of an autopsy confirmed the presence of diffuse fibrin thrombi in bilateral pulmonary arteries, and we diagnosed the cause of death as diffuse pulmonary artery thrombosis. A coagulation test for activated partial thromboplastin time (aPTT) had already shown that aPTT was prolonged before the initiation of treatment. There may have been a deficit of antithrombin III (ATIII) - a cofactor of heparin - because of the proteinuria; thus, the continuous heparin treatment might not have been effective for the prevention of thrombosis. Alternatives to heparin treatment that do not suppress AT III, such as nafamostat mesilate or argatroban, which do not require the presence of AT III for their anticoagulant action, should be considered in cases similar to the that in the patient reported here. In patients with nephrotic syndrome who exhibit altered coagulation test results, the choice of anticoagulation therapy for treatment of the hypercoagulabilty status associated with nephrotic syndrome should be carefully considered.

Early vs Standard Intervention with an Extended Epoetin alfa (EPO) Dose Regimen of 120,000 Units (U) Every 3 Weeks (Q3W) in Chemotherapy (CT)-Induced Anemia: Results for Elderly vs. Younger Patients in a Randomized Clinical Trial.

A recently completed randomized open-label multicenter clinical trial in cancer pts with CT-induced anemia showed that an extended dose regimen of EPO 120,000 U administered subcutaneously Q3W effectively maintained hemoglobin (Hb) levels within the range of 11–13 g/dL whether initiated early (Hb of ≥11 and ≤12 g/dL) or at a standard threshold (Hb<11 g/dL)1. As elderly pts are particularly sensitive to the effects of anemia related to CT, we postulated whether this group had outcomes (Hb response, transfusion use, and safety) similar to younger patients following treatment with Q3W EPO. 136 pts with non-myeloid malignancy, CT planned for ≥9 wks, and baseline (BL) Hb ≥11 and ≤12 g/dL were randomized either to early intervention with immediate EPO or to standard intervention with EPO when Hb decreased to <11 g/dL. Drug was held for Hb>13 g/dL and dose reduced for Hb>12 g/dL or increase >1.5 g/dL in any 3-wk period (current labeling recommends Hb not to exceed 12 g/dL). If, at any dosing visit after the 1st EPO dose, Hb decreased to <10.0 g/dL, pts were switched to EPO 40,000 U weekly. The primary efficacy endpoint was Percent Values in Range (PVR; the proportion of weekly Hb levels that were ≥11 and ≤13 g/dL). Reported here is a retrospective subset analysis comparing key efficacy and safety results for elderly (≥65 yrs) vs. younger (<65 yrs) pts enrolled in the prospective study. 62 elderly pts (mITT population; 29 early, 33 standard) and 73 younger pts (mITT; 39 early, 34 standard) were randomized to a treatment group and had ≥1 Hb value. Mean BL Hb was 11.5 g/dL in each of the 4 subgroups. The most common tumor sites were colorectal [elderly early intervention group (n=7, 24%)] and breast [elderly standard intervention group (n=11, 32%)], younger early (n=13, 33%), and younger standard intervention (n=18, 53%). Key efficacy and safety results are shown in the table. Clinically relevant thrombotic vascular events (CRTVEs) included a deep vein thrombosis (DVT) in the elderly early group; 1 DVT and 1 pulmonary embolism (PE) in the elderly standard group; 1 pulmonary artery thrombosis, 2 DVTs, 1 PE, 1 thrombosis NOS, and 1 jugular vein thrombosis among 5 pts in the younger early group; and 2 DVTs, 2 PEs, and 1 thrombosis NOS among 4 pts in the younger standard group. The 4 deaths were attributed to the following causes: acute respiratory failure/progression of COPD (eldery early pt), worsening metastatic gastric cancer/hemorrhage brain metastasis (younger early pt), and lower GI bleed and cardiopulmonary arrest, each in 1 younger standard pt. EPO 120,000 U Q3W initiated early or at a standard threshold resulted in similar efficacy and safety outcomes in elderly vs. younger subsets of cancer pts with CT-induced anemia. 1ASCO 2007 Annual Mtg, Abstract 19564.≥65 Yrs<65 YrsEarly, n=29Standard, n=33Early, n=39Standard, n=34Mean Age ± SD (yrs)71.8±5.773.7±4.852.1±9.748.7±11.1Mean PVR (95% CI)59.8(49.1,70.5)56.8(45.6,68.0)60.0(50.3,69.7)63.1(54.0,72.2)PRBC Transfusion After 4 Wks of EPO, n(%)2(6.9)3(12.0) [of 25 treated pts]4(10.3)1(3.8) [of 26 treated pts]Dose Reduction, n(%)12(41)6(18)17(44)7(21)Dose Withhold, n(%)10(34)4(12)16(41)7(21)CRTVE Pts, n(%)1(3)2(6)5(13)4(12)Death, n(%)1(3)01(3)2(6)PVR, percent values in range; CRTVE, clinically relevant thrombotic vascular event.

Left atrial isomerism with right pulmonary artery thrombosis

Left atrial isomerism with right pulmonary artery thrombosis

Experimental Severe Pseudomonas aeruginosa Pneumonia and Antibiotic Therapy in Piglets Receiving Mechanical Ventilation

Little is known about the general and local consequences of severe pneumonia under mechanical ventilation (SPMV) and how these are resolved with antibiotic therapy (ABT). To investigate the physiologic, biological, microbiological, and pathologic changes produced by experimental SPMV in a porcine model, and to evaluate the effect of ABT. Pseudomonas aeruginosa was inoculated in 12 large white-Landrace piglets receiving mechanical that were killed after 72 h if death did not occur before. Vital signs, serum and BAL cytokines, serum C-reactive protein (CRP), and graded postmortem lung pathology and cultures (blood and quantitative BAL and lung) were evaluated. Six piglets received inappropriate ABT (no ABT or ceftriaxone), and six piglets received appropriate ABT (ciprofloxacin). Pathologic and microbiological evidence of infection were present in all the animals in both groups. SPMV produced significant oxygenation and lung compliance worsening, increased serum CRP, and reduced BAL fluid tumor necrosis factor (TNF)-alpha. Arterial thrombosis in lung pathology was associated with higher temperature, hypoxemia and low lung compliance, higher initial serum CRP and TNF-alpha concentrations, and increased serum interleukin (IL)-6 and BAL IL-6 and TNF-alpha. Reduced ABT reduced body temperature and culture positivity. This model resembles VAP and has been used for studying pulmonary infection and inflammation related to mechanical ventilation. ABT reduced fever and bacterial burden in SPMV but had no effect on cytokine or CRP concentrations, oxygenation, or lung mechanics. Pulmonary artery thrombosis was associated with worse response to infection.

Pulmonary Arterial Thrombosis in Eisenmenger Syndrome Is Associated With Biventricular Dysfunction and Decreased Pulmonary Flow Velocity

This study sought to determine what factors are associated with pulmonary artery thrombi in Eisenmenger patients. Pulmonary artery thrombosis is common in Eisenmenger syndrome, although its underlying pathophysiology is poorly understood. Adult patients with Eisenmenger syndrome underwent computed tomography pulmonary angiography, cardiac magnetic resonance imaging, and echocardiography. Measurement of ventricular function, pulmonary artery size, and pulmonary artery blood flow were obtained. Hypercoagulability screening and platelet function assays were performed. Of 55 consecutive patients, 11 (20%) had a detectable thrombus. These patients were older (p = 0.032), but did not differ in oxygen saturation, hemoglobin, or hematocrit from those without thrombus. Right ventricular ejection fraction by magnetic resonance imaging was lower in those with thrombus (0.41 +/- 0.15 vs. 0.53 +/- 0.13, p = 0.017), as was left ventricular ejection fraction (0.48 +/- 0.12 vs. 0.60 +/- 0.09, p = 0.002), a finding corroborated by tissue Doppler and increased brain natriuretic peptide. Those with thrombus also had a larger main pulmonary artery diameter (48 +/- 14 mm vs. 38 +/- 9 mm, p = 0.007) and a lower peak systolic velocity in the pulmonary artery (p = 0.003). There were no differences in clotting factors, platelet function, or bronchial arteries between groups. Logistic regression showed pulmonary artery velocity to be independently associated with thrombosis. Pulmonary arterial thrombosis among adults with Eisenmenger syndrome is common and relates to older age, biventricular dysfunction, and slow pulmonary artery blood flow rather than degree of cyanosis or coagulation abnormalities. Further work to define treatment efficacy is needed.

Pulmonary and Cardiac Involvement in Behçet Disease: 3 Case Reports

Pulmonary and cardiac involvement in Behçet disease is rare but can cause life-threatening complications. This report presents computed tomography and magnetic resonance angiography findings in 3 patients with Behçet disease. Imaging findings were confirmed by surgery in 1 patient and by angiography in another. One patient had bilateral pulmonary artery aneurysms, 1 of which had already ruptured and had thrombosed. The other had unilateral pulmonary artery aneurysm and thrombosis accompanied with intracardiac thrombus formation. The third patient had unilateral pulmonary artery aneurysm, thrombus, and coronary artery aneurysm. As in our case, there have been rare reports of coronary artery disease and intracardiac thrombus formation associated with pulmonary vascular involvement. Noninvasive imaging modalities minimize any risk of endothelial damage and can be an alternative to angiography in assessment of pulmonary and cardiac involvement in Behçet disease.

Pulmonary thrombectomy in a patient with hemoglobin Nottingham

A 36-year-old female with hemoglobin Nottingham (betaFG 5(98) Val --> Gly) causing severe hemolytic anemia and chronic thromboembolic pulmonary hypertension presented with symptomatic subacute right lower lobar pulmonary arterial thrombosis requiring surgical pulmonary thrombectomy. We describe a successful, multidisciplinary approach to the problems associated with this disease, particularly with the use of cardiopulmonary bypass and deep hypothermic circulatory arrest.

Preliminary clinical application of contrast-enhanced MR angiography using 3D time-resolved imaging of contrast kinetics (3D-TRICKS)

Objective To introduce a new better contrast-enhanced MR angiographic method, named 3D time-resolved imaging of contrast kinetics (3D-TRICKS). Methods TRICKS is a high temporal resolution (2-6 s) MR angiographic technique using a short TR(4 ms) and TE(1.5 ms), partial echo sampling, in which central part of k-space is updated more frequently than the peripheral part. TRICKS pre-contrast mask 3D images are firstly scanned, and then the bolus injecting of Gd-DTPA, 15-20 sequential 3D images are acquired. The reconstructed 3D images, subtraction of contrast 3D images with mask images, are conceptually similar to a catheter-based intra-arterial digital subtraction angiographic series (DSA). Thirty patients underwent contrast-enhanced MR angiography using 3D-TRICKS. Results Totally 12 vertebral arteries were well displayed on TRICKS, in which 7 were normal, 1 demonstrated bilateral vertebral artery stenosis, 4 had unilateral vertebral artery stenosis and 1 was accompanied with the same lateral carotid artery bifurcation stenosis. Four cases of bilateral renal arteries were normal, 1 transplanted kidney artery showed as normal and 1 transplanted kidney artery showed stenosis. 2 cerebral arteries were normal, 1 had sagittal sinus thrombosis and 1 displayed intracranial arteriovenous malformation. 3 pulmonary arteries were normal, 1 showed pulmonary artery thrombosis and 1 revealed pulmonary sequestration's abnormal feeding artery and draining vein. One left lower limb fibrolipoma showed feeding artery. One displayed radial-ulnar artery artificial fistula stenosis. One revealed left antebrachium hemangioma. Conclusion It TRICKS can clearly delineate most body vascular system and reveal most vascular abnormality. It possesses convenience and high successful rate, which make it the first choice of displaying most vascular abnormality.

Pulmonary Embolism and Deep Vein Thrombosis Complicating Acute Aortic Dissection during Medical Treatment

Acute aortic dissection of Stanford type A with intramural hematoma was diagnosed based on computed tomography (CT) findings in a 60-year-old man. During medical treatment, pulmonary embolism and deep vein thrombosis developed. CT revealed thrombosis in the right pulmonary artery, and 99mTc pulmonary perfusion scintigraphy showed defects in the right lung field. CT showed thrombus in the common iliac vein. An inferior vena caval filter was placed because anticoagulation therapy was contraindicated. A CT scan before discharge showed no thrombus in the pulmonary artery or common iliac vein, but a newly captured thrombus was found inside the filter.

Learning to pair therapies and the expanding matrix for pulmonary arterial hypertension: is more better?

Study design for treatment of a rare disease with few therapeutic options is straightforward as “something is likely better than nothing”. With the surge of therapeutic options for pulmonary arterial hypertension (PAH) since the late 1990s, trials have been designed based on limited data without the luxury of patient numbers. As community physicians can offer combination therapy without evidence of benefit, clinical trialists are challenged to accrue patients to studies to prove what some envision is intuitively obvious, that combining active agents is likely to be better than treatment with a single agent. PAH is a debilitating, progressive condition with diverse causes, and for a disease with no therapeutic cure, targeting multiple pathways concurrently is appealing. However, without prior evidence for safety and activity, it is entirely possible that some combinations might be more harmful than helpful. PAH is an angioproliferative vasculopathy resulting from abnormal endothelial and smooth muscle cell interactions, which leads to a progressive narrowing of the pulmonary arteries and their branches, resulting in right-heart failure and death. At the cellular level, PAH is characterised by vasoconstriction, endothelial dysfunction and smooth muscle cell proliferation of the pulmonary arterioles, a process known as vascular remodelling, and in situ thrombosis of the small pulmonary arteries 1, 2. Proliferating endothelial cells obliterate medium-sized pre-capillary arteries 3, 4, thereby forming the characteristic “plexiform” lesions. At the molecular level, the balance of vasculature regulation shifts towards factors that produce vasoconstriction and smooth muscle cell proliferation. Animal models and culture of cells from patients with PAH have demonstrated dysregulation of specific mediators of various signalling pathways, including: prostacyclin 5–7; nitric oxide (NO); and endothelin 8–10. Angiopoietins 11, 12, autoantibodies 13, and proinflammatory cytokines and chemokines have also been implicated 14. …

Surgical Management of Infants with Isolated Supravalvular Pulmonary Stenosis: Case Reports

Pulmonary stenosis (PS) can be seen from the right ventricular outflow tract to the peripheral pulmonary arteries. Most frequently, the obstruction occurs at the level of the pulmonary valve; however, it occurs less frequently at the infindibular level within the trabecular component of the right ventricle or within the pulmonary arterial pathways. Lesions at any of these levels can occur as part of more congenital cardiac malformations such as tetralogy of Fallot, complete transposition of great arteries, or atrial septal defect. Isolated supravalvular pulmonary stenosis (iSPS) is less common than other types of PS. In this study, we present our experience with 4 patients who underwent cardiopulmonary bypass operation for iSPS. In one patient, the circular stenotic area was noted on the touch point of the pulmonary valve. Right ventricular pressures ranged from 70 to 90 mmHg, and the pulmonary artery mean pressures ranged from 14 to 17 mmHg. In all patients, the left ventricular and aortic systolic, diastolic, and mean pressures were moderately increased. Pulmonary artery stenosis was treated successfully using a pericardial or Dacron patch on cardiopulmonary bypass. Various techniques such as balloon dilation have been proposed to deal with this problem, but these may often be unsuccessful because of the elasticity and recoil of the pulmonary artery constrictive ring. Even though endovascular stenting and/or balloon angioplasty have been recently proposed as an initial treatment strategy, they may be associated with some severe complications including pulmonary artery thrombosis or stent migration. Our study, even though it consists of a limited number of cases, suggests that open heart surgery using an oval-shaped patch may be a used as the other main choice for the treatment of iSPS.

Accidental dislocation of an intracaval temporary filter into the heart in a case of renal cell carcinoma extending into the vena cava

In a case of renal cell carcinoma extending into the vena cava, a temporary intracaval filter was applied before surgery to prevent pulmonary artery thrombosis. Two days later, the filter accidentally migrated into the right ventricle during defecation. Fortunately, it was successfully removed without damaging cardiac musculature under fluoroscopy.

A Report of the Vancouver Forum on the Care of the Live Organ Donor: Lung, Liver, Pancreas, and Intestine Data and Medical Guidelines

An international conference of transplant physicians, surgeons, and allied health professionals was held in Vancouver, Canada, on September 15 and 16, 2005 to address the care of the live lung, liver, pancreas, and intestine organ donor. The Vancouver Forum was convened under the auspices of the Ethics Committee of The Transplantation Society. Forum participants included over 100 leaders in organ transplantation, representing many countries from around the world, including participants from the following continents: Africa, Asia, Australia, Europe, North and South America. The objective of the Vancouver Forum was to develop an international standard of care for the live lung, liver, pancreas and intestinal organ donor. This Vancouver Forum followed a conference convened in Amsterdam on the care of the live kidney donor (1, 2). There were four organ specific work groups at the Vancouver Forum: lung, liver, pancreas and intestine. Each organ work group addressed the following topics in concert and reported their findings in a plenary presentation to all participants:

Pulmonary artery thrombosis caused by hyperinflation of the native lung six years after single lung transplantation for emphysema

always negative. The hyperinflation of the native lung remained stable. Respiratory function decreased very slowly. The FEV1 reached 38% of predicted value. The patient’s lifestyle was still normal. On January 2005 the patient had a sudden onset of dyspnea. Physical examination results were negative. The x-ray film was unchanged. The PO2 was 53 mm Hg, and the PCO2 was 35.4 mm Hg. The electrocardiogram was normal. No sign of pulmonary infection was evident. The patient underwent a perfusion scan to plan an LVRS on the native lung that unexpectedly showed an area of hypoperfusion in the lower lobe of the left transplanted lung, suggesting pulmonary embolism. The D-dimer was 3450 (normal value 300). The patient was therefore studied with multidetector CT angiography of the pulmonary arteries. A thrombus in the inferior interlobar branch of the left pulmonary artery was present, completely occluding the artery (Figure 2, 3 and 4). Sections were also made during the venous phase, and deep venous thrombosis of the legs and pelvis was excluded. The thrombosis was attributed to severe kinking of the pulmonary artery because of the displacement of the mediastinum. Heparin was administered to the patient, and then dicumarol. The respiratory function slowly returned to prethrombosis values. After 6 months, multidetector CT angiography shows no filling defect in the pulmonary artery. The patient is now scheduled for a LVRS of the native lung.

A Case of Swan-Ganz Catheter Malposition: A case report

A Swan-Ganz catheter is a useful monitoring device for measuring the pulmonary artery pressure, pulmonary capillary wedge pressure and cardiac output, but its insertion brings about many complications, including dysrhythmias, pulmonary artery rupture, thrombosis and infection. We report here on a case of malposition of a Swan-Ganz catheter in a 49-year-old female patient who had undergone liver transplantation due to alcoholic liver cirrhosis.

In situ central pulmonary artery thrombosis in primary pulmonary hypertension

A rare case of extensive in situ central pulmonary artery thrombosis in primary pulmonary hypertension (PPH) is presented. The differentiation from chronic thromboembolic pulmonary arterial hypertension (CTEPH) is of paramount importance because of different therapeutic strategies. In this case, the presence of mural thrombus in the central pulmonary arteries on computed tomography made the distinction difficult. However, the possibility of in situ thrombosis was suggested on the basis of absence of other findings of CTEPH (abrupt narrowing/truncation of segmental arteries, variation in size of segmental vessels, arterial webs, mosaic attenuation, pulmonary infarcts, and dilated bronchial arteries), and this was confirmed on final pathology.

The Pulmonary Artery Catheter in Anesthesia Practice

Pressures in the right side of the heart and pulmonary capillary wedge can be obtained by cardiac catheterization without the aid of fluoroscopy. A No. 5 French double-lumen catheter with a balloon just proximal to the tip is inserted into the right atrium under pressure monitoring. The balloon is then inflated with 0.8 ml of air. The balloon is carried by blood flow through the right side of the heart into the smaller radicles of the pulmonary artery. In this position when the balloon is inflated wedge pressure is obtained. The average time for passage of the catheter from the right atrium to the pulmonary artery was 35 s in the first 100 passages. The frequency of premature beats was minimal, and no other arrhythmias occurred.

Prostacyclin Therapy for Pulmonary Arterial Hypertension: New Directions

Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction and smooth muscle cell proliferation of the pulmonary arterioles, as well as in situ thrombosis of the small pulmonary arteries. Prostacyclin is involved in PAH vascular remodeling. It is unclear if decreased prostacyclin in the lungs is a cause or a consequence of PAH, but the relative lack of prostacyclin and its positive effects on the pulmonary vascular bed support the theory that long-term prostacyclin replacement is effective. Current therapies based on evidence-based medicine include epoprostenol, treprostinil, iloprost, and beraprost, each with limitations based on the drugs' inherent properties and administration route. Treatment of PAH by inhibiting multiple pathways concurrently may produce additive benefit. Because prostacyclin therapy is not curative and does not normalize pulmonary hemodynamics in the majority of cases, combining a prostacyclin with other PAH agents may be a promising approach.

Congenital Hemicerebral Anomaly in a Stranded Pacific Harbor Seal (Phoca vitulina richardsi)

A stranded 5-month-old female Pacific harbor seal (Phoca vitulina richardsi) was presented displaying tachypnea and diminished lung sounds. No neurological abnormalities were noted. The animal was treated for verminous pneumonia, but died 2 wk later. Gross necropsy examination revealed a severe obstructive verminous pneumonia associated with large numbers of Otostrongylus circumlitus. In addition, the majority of the right cerebral hemisphere was absent, with hypoplasia of the left cerebellar hemisphere, absence of the right pyramid, and malformation of the right occipital bone. Histopathologic findings included multifocal thrombosis and inflammation of pulmonary arteries, verminous pneumonia, and mild vacuolation of the subependymal white matter in the third ventricle representing swelling of myelin sheaths and edema. This is the first report of a hemicerebral anomaly in a marine mammal.

A full house: Complications from an uncorrected patent ductus arteriosus

True aneurysms of the pulmonary artery are rare, and are most often due to pulmonary hypertension arising from congenital heart defects. We report the case of a 40-year-old man with an uncorrected patent ductus arteriosus who presented with pulmonary infarction and pneumonia, and subsequently died of cardiac arrest. Autopsy revealed a large pulmonary artery aneurysm, in situ pulmonary artery thrombosis complicated by pulmonary infarction, pulmonary artery dissection, and cardiac tamponade. Although each of these complications is rare in and of itself, this case demonstrates the entire spectrum of complications from a single uncorrected congenital cardiac anomaly.