SummaryWhen pulmonary embolism (PE) develops, circulatory collapse and hypoxia are caused at the same time. The rapid and proper use of extracorporeal life support (ECLS) can improve the mortality rate of patients with collapsed massive PE. No study has examined the influence of treatment that involved adding catheter based-intervention to ECLS with massive collapsed PE. Thirty-five patients with massive PE were examined, and 10 of these patients were placed on ECLS. Eight of the 10 patients placed on ECLS for massive PE were female, and the median age was 61 years. Seven patients had in-hospital onset PE and 3 patients out-of-hospital onset PE. Their underlying conditions were a cerebral infarction (3 patients), coronary artery disease (5 patients), collagen disease (one patient), postoperative state (3 patients), and lung disease (2 patients). Pulmonary angiographic findings showed that a filling defect or complete occlusion was observed in all 10 patients in the proximal lobular arteries, 6 of which had large thrombi stretching to the main pulmonary arteries. All patients underwent thrombolysis. Percutaneous catheter embolus fragmentation and/or thrombectomy were undertaken in 7 patients. All patients required red blood cell transfusion for cannulation site bleeding. The mean duration of ECLS bypass was 48 ± 44 hours. The 30 day mortality rate was 30%. The current study clarified the characteristics of patients with massive PE requiring ECLS. These patients have extensive pulmonary thromboemboli, thus, the aggressive use of catheter-based intervention appears to have beneficial effects for massive PE requiring ECLS. (Int Heart J 2012; 53: 370-374)

A case of pulmonary alveolar microlithiasis occurring in an inbred family is presented. A genome-wide analysis of the patient's genomic DNA using a high-density single nucleotide polymorphism (SNP) array revealed a small intragenetic mutation at SLC34A2. The results suggest that the high-density SNP array has the power to identify a recessive disease gene(s) even in the analysis of only a single inbred patient.Inbreeding increases the chance for a mutant gene to become homozygous in the offspring and thus for autosomal recessive diseases to develop.1 The increased homozygosity is attributed to the appearance of chromosomal regions called autosomal segments, 2 where both strands of homologous chromosomes are identical by descent (ie, derived from a single chromosome of a single ancestor). In inbred children affected by an autosomal recessive disease, the causative gene is very likely to be in the autozygous segments. Recent high-density single nucleotide polymorphism (SNP) arrays are able to determine both of the SNP genotypes genome-wide and the copy number of each chromosomal location. A patient with pulmonary alveolar microlithiasis was investigated using the array which revealed a small intragenetic deletion in the SLC34A2 gene, the gene considered to be the cause of the disease. CASE REPORTA 56-year-old woman was admitted to hospital with progressive dyspnoea, severe systemic oedema and a weight increase from 45 kg to 60 kg. She had been notified for the first time in her 30s that her chest radiograph was abnormal although, at that time, no subjective symptoms were present. A physical examination showed blood pressure 128/60 mm Hg, pulse rate 84 bpm and arterial oxygen saturation (SpO 2 ) measured with a pulse oximeter of 90%. Her pulmonary second heart sound was accentuated. Laboratory test results revealed increased serum levels of C-reactive protein (2.3 mg/dl), B-type natriuretic peptide (1256 pg/ml) and sialylated carbonhydrate antigen KL-6 (583 U/ml). A chest radiograph revealed numerous micronodules throughout the entire lung field and obscured the cardiac shadow ( fig 1A). A CT scan of the chest showed consolidation and diffuse ground-glass opacities in both lungs, accompanied by dilated bronchi (fig 1B, upper panel). A marked enlargement of the right heart, retention of pleural and pericardial effusions and calcification involving the mediastinal and interlobular pleura and the pericardium were also observed ( fig 1B, lower panel). The ECG revealed a right bundle branch block accompanied by a tall R wave in aV R and V 1 , deep S and a small r in I and aV L and a persistent S or s in the left precardial leads. These findings suggested right ventricular hypertrophy. Spirometry showed restrictive ventilatory disturbances (vital capacity 51.6%, forced expiratory volume in 1 s 93.1%). Transthoracic echocardiography revealed a dilated right heart with tricuspid regurgitation (fig 1C, upper panel) where the right ventricle-right atrium pressure gradient was 81 mm Hg and the peak tricupsid regurgi...

Background The efficacy of prophylactic coronary revascularization with percutaneous coronary intervention (PCI) or coronary artery bypass graft (CABG) surgery in patients with abdominal aortic aneurysm (AAA) scheduled for open repair surgery remains controversial. Hypothesis Concomitant coronary artery disease (CAD) with no inducible ischemia can be medically treated in AAA patients undergoing open repair as long as the existence of CAD is recognized. Method A retrospective analysis of acute and long‐term outcomes was performed for 122 patients with AAA who underwent coronary arteriography (CAG) for preoperative evaluation followed by elective open repair. Results Preoperative CAG revealed no CAD in 54 patients (non‐CAD group) and the existence of CAD in 68 patients. Prophylactic PCI or CABG surgery was performed in 16 patients (CAD‐PCI/CABG group) with symptomatic angina, ischemia proven by pharmacological stress scintigraphy, or coexistence of reduced cardiac contraction and coronary stenosis in multiple vessels. Medical treatment was administered to 52 patients who had no signs of ischemia (CAD‐medical group). During the perioperative period, no cardiac event occurred irrespective of the existence of CAD. The long‐term outcomes in the CAD‐medical group were equivalent to those in the non‐CAD group. In the CAD‐PCI/CABG group, the cardiac event‐free rate was comparable with that of other groups, although mortality was higher. Conclusion In patients undergoing AAA open repair, medical treatment for concomitant CAD with no obvious inducible ischemia does not confer unfavorable outcomes. Although prophylactic coronary revascularization possibly prevents future cardiac events, it appears to be necessary in a very limited number of cases. Copyright © 2008 Wiley Periodicals, Inc.

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Objective A fever is observed in approximately one-third of cases of acute aortic dissection (AAD); however, the causes remain unclear. We investigated the mechanism of a fever in AAD by measuring the serum concentrations of inflammatory markers, mediators of coagulation and fibrinolysis, and procalcitonin, a marker of bacterial infection. Methods We retrospectively studied 43 patients with medically treated AAD without apparent infection. Patients were divided into those with (Group A; n=19) and without (Group B; n=24) a maximum body temperature >38 . We established which patients fulfilled the criteria for systemic inflammatory response syndrome (SIRS), and its relationship with a fever was examined. Mediators of inflammation, coagulation and fibrinolysis were compared by a univariate analysis. Factors independently associated with a fever were established by a multivariate analysis. Results The criteria for SIRS were fulfilled in a greater proportion of patients in Group A (79%) than in Group B (42%, p=0.001). There was no difference in the procalcitonin concentration between Groups A and B (0.15 0.17 ng/mL vs. 0.11 0.12 ng/mL, respectively; p=0.572). Serum procalcitonin concentrations lay within the normal range in all patients in whom it was measured, which showed that the fever was caused by endogenous mediators. On the multivariate analysis, there was a borderline significant relationship between a fever and the prothrombin time-International Normalized Ratio (p=0.065), likely reflecting the extrinsic pathway activity initiated by tissue factor. Conclusion Our findings suggest that a fever in AAD could be caused by SIRS, provoked by endogenous mediators that influence the extrinsic coagulation pathway without elevating the serum procalcitonin concentration.