Ulrik Baandrup

Clinical and epidemiological description of aortic dissection in Turner's syndrome.

BACKGROUNDnWomen with Turners syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency.nnnAIMnTo describe in more detail aortic dissection as encountered in Turners syndrome, giving attention to clinical, histological and epidemiological aspects.nnnMATERIALS AND METHODSnBased on a retrospective study, we describe the clinical, karyotypic, and epidemiological aspects of aortic dissection as encountered in cases of Turners syndrome seen in Denmark and Sweden.nnnRESULTSnThe median age at onset of aortic dissection in 18 women was 35 years, ranging from 18 to 61 years. Fourteen of 18 women had a 45,X karyotype, while 2 patients had 45,X/45,XY, and 2 had the 45,X/46,X+r(X) complement, respectively. Echocardiography was performed in 10 of 18 patients before their acute illness, and showed signs of congenital cardiac disease, with either bifoliate aortic valves, dilation of the aortic root, or previous aortic coarctation evident in most patients. In 5 patients evidence of a bifoliate aortic valve was conclusive. Hypertension was present in 5 of 18 patients, while 10 of the patients died from aortic dissection, of so-called type A in 6, type B in 3, while in the final case the origin of dissection could not be determined. Biochemical analysis showed altered ratio between type I and type III collagen. Histology showed cystic medial necrosis in 3 of 7 cases. We estimated an incidence of dissection of 36 per 100,000 Turners syndrome years, compared with an incidence of 6 per 100,000 in the general population, and a cumulated rate of incidence of 14, 73, 78, and 50 per 100,000 among 0-19, 20-29, 30-39, and 40+ year olds, respectively.nnnCONCLUSIONnAortic dissection is extremely common in the setting of Turners syndrome, and occurs early in life. Patients with Turners syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome, and each clinic should embrace a programme for follow-up.

Perivalvular cavities in endocarditis : abscesses versus pseudoaneurysms ? A transesophageal Doppler echocardiographic study in 118 patients with endocarditis

The appearance of perivalvular cavities (PCs) in patients with infectious endocarditis (IE) was studied by transesophageal echocardiography (TEE) color Doppler examinations to determine whether the color Doppler TEE presentation was in keeping with the current concept of PCs representing abscesses. Two heart centers participated in the study. Videotape recordings of TEE examinations in patients with IE were analyzed retrospectively for 18 months in both centers, and one center included patients prospectively for an additional 18 months. A total of 118 patients with a diagnosis of IE based on TEE and clinical and laboratory findings were seen during the study period. TEE showed PCs in 34 patients. In 3 patients who died, no autopsy was performed; the PCs were proved at autopsy or surgery in the remaining 31 patients, who constituted the study population. All PCs were echo free at TEE. Apart from one technically inadequate examination, all PCs contained color Doppler signals indicating intracavitary blood flow; the PCs communicated through a narrow channel with high-pressure regions (the left ventricle or the ascending aorta). At surgery or autopsy, only 2 of the 31 patients had pus accumulations besides the blood-filled PCs. At TEE the pus accumulations presented as echo-rich, shaggy tissue thickening. It is concluded that well-delineated, echo-free PCs with intracavitary color Doppler signals at TEE appear to be pseudoaneurysms, and therefore the term abscess should not be used in these cases. Although further studies are needed, our findings suggest that PCs more likely occur by infectious tissue weakening and subsequent dissection rather than as a result of primary abscess formation with secondary rupture.(ABSTRACT TRUNCATED AT 250 WORDS)

Broad‐range PCR and sequencing in routine diagnosis of infective endocarditis

The aim was to evaluate “16S rDNA PCR and sequencing” (PCR) for identification of bacterial DNA in heart valves in routine diagnosis of infective endocarditis (IE). Heart valves from 74 patients with suspected infective endocarditis, and 16 controls were analysed by histology, culture and PCR. Results from blood culture served as the gold standard. Patients were classified according to the Duke criteria. The final classification resulted in 57 definitive cases of IE, 7 possible, and 10 cases without IE. Sensitivity of valve culture was 26% and specificity 62%. Sensitivity of PCR was 72% and specificity 100%. In patients who had received antibiotic treatment for less than 5 days before surgery, sensitivity of culture and PCR were comparable. In patients who had received antibiotic treatment for more than 5 days, sensitivity of valve culture was markedly reduced compared to sensitivity of PCR. In three of seven blood‐culture‐negative cases PCR was positive, including two cases with non‐cultivable bacteria. No PCR samples were contaminated, whereas 35% of valve‐culture samples were contaminated. PCR is more sensitive and specific than valve culture, and a valuable supplement to the existing analyses of valve tissue. PCR is necessary to identify the full spectrum of pathogens causing IE. In contrast to sensitivity of culture, sensitivity of PCR was independent of length of antibiotic treatment before surgery.

Cytopathologic diagnoses of fine-needle aspirations from endoscopic ultrasound of the mediastinum: reproducibility of the diagnoses and representativeness of aspirates from lymph nodes.

Endoscopic ultrasound‐guided fine‐needle aspiration biopsy through the esophagus (EUS‐FNA) or the bronchial tree (endobronchial ultrasound guided transbronchial needle aspiration [EBUS‐TBNA]) may be used to obtain specimens from mediastinal structures. The accuracy of this procedure has been well documented. However, no studies have studied the reproducibility of the pathologic assessment of the aspirated material.

hnRNPs H, H' and F behave differently with respect to posttranslational cleavage and subcellular localization.

hnRNPs H, H′ and F belong to a subfamily of the hnRNPs sharing a high degree of sequence identity. Eukaryotic expression and specific C‐terminal antibodies were used to demonstrate great variation in the intracellular fate of the proteins. hnRNPs H and H′ become posttranslational cleaved into C‐terminal 35 kDa proteins (HC, H′C) and possibly into N‐terminal 22 kDa proteins. No detectable cleavage was observed for hnRNP F. hnRNP H/H′ is almost exclusively localized to the nucleus of many cell types while hnRNP F varies from a predominant nuclear localization in some cells to a predominant cytoplasmic localization in other cells. The different fates may reflect differences in functional roles that so far only have included nuclear functions. The presence of significant quantities of hnRNP F in the cytoplasm of many cells indicates that it also may have a functional role here.

Endonuclein is a cell cycle regulated WD-repeat protein that is up-regulated in adenocarcinoma of the pancreas.

The transcript encoding endonuclein, the human homolog of yeast PWP1, was previously found up-regulated in pancreatic cancer tissue. By immunohistochemistry we detected a ubiquitous presence in several tissues examined: skin, liver, thyroid gland, heart muscle, neurons, kidney, bladder, pancreas, adrenal gland, ovary, uterus, testis and prostate gland. We especially noticed that normal pancreatic exocrine cells exhibited low protein levels while pancreatic adenocarcinoma cells revealed high levels. We found a heterogeneous subcellular distribution, especially with varying nuclear levels. In proliferating cells endonuclein protein expression and localization was cell cycle dependent, with increasing levels and nuclear focusing during the interphase toward mitosis. Ultrastructural analysis revealed ER and nuclear localization. Endonuclein contains five WD-repeats, indicating a putative role in crucial regulatory activities in the nucleus as well as in the ER.

Primary malignant pericardial mesothelioma mimicking left atrial myxoma:Case Report

In a 32-year-old man with clinical and echocardiographic signs mimicking left atrial myxoma, thoracotomy revealed a highly malignant pericardial mesothelioma with an intraatrial pendulous extension and haemorrhagic pericardial exudate. CT scanning of the thorax is useful when an intracavitary cardiac mass is associated with pericardial exudate and/or with suspected extracavitary involvement.

Recovery after Cold Cardioplegic Arrest of Isolated Blood-Perfused Hearts Excised from Non-Anesthetized Pigs

An isolated blood-perfused pig heart model has been established in order to evaluate the recovery of hearts obtained from slaughterhouse domestic pigs avoiding anesthesia and direct experiments on animals. Eleven hearts subjected to 9 min of normothermic ischemia were infused with cold modified Bretschneider solution. After 180 min of cardioplegic-induced global ischemia (including 9 min of normothermic ischemia) 8 hearts were reperfused for 120 min. Left ventricular function (measured isovolumetrically by means of a balloon, and expressed as developed left ventricular pressure, positive and negative dP/dt) was stable during the whole reperfusion period. Lactate production was abolished after 25 min of reperfusion, while there was a small glucose extraction during the whole reperfusion period. Slight deterioration of the mitochondria was found during the induced cardiac arrest, however, reversing during the reperfusion. Thus, due to the stability of left ventricular function, improved metabolism and ultrastructure during the reperfusion period, the model with no use of laboratory animals, and without any influence of anesthesia, seems to be suitable for testing the pure effect on the performance of the left ventricle of drugs and substrates added to the reperfusate during the reperfusion period.

The hemodynamic impact of diffuse myocardial ischemic lesions: an animal experimental model based on intracoronary microembolization

SummaryIn ischemic heart disease, left ventricular function is affected by a diffuse and segmental loss of myocardium. The decline in the incidence of myocardial infarction and improved early revascularization in acute transmural ischemia predict a change in the natural history of ischemic heart disease. It is now believed that, minor ischemic episodes, which are known to induce multifocal myocardial degeneration, will predominate in the near future. The objective of the present study was to develop a clinically relevant experimental model for investigation of the pathophysiological significance of diffuse ischemic myocardial lesions. Cardiac performance was gradually depressed by selective intracoronary microembolization in 13 pigs. Left ventricular function was quantitated by ejection fraction (EF), pulmonary pressure, cardiac output, and derivatives of left ventricular pressure. Left ventricular volume was estimated by epicardial echocardiography, using a new, unbiased stereological volume estimator. A chronic substudy was performed in order to characterize the histological changes and to evaluate the feasibility of establishing a chronic preparation of the model. Embolization induced acute left ventricular dysfunction; left ventricular pressure change decreased from 966 ± 274 to 637 ± 146mmHg/s, and early diastolic relaxation from 1403 ± 515 to 824 ± 344mmHg/s, respectively. Ejection fraction decreased by 45% ± 5% and cardiac output by 29% ± 11%. End-diastolic volume increased significantly, from 66.1 ± 13.2 to 77.0 ± 19.4 cm3, and end-systolic volume increased from 35.9 ± 13.9 to 52.3 ± 7.6 cm3. No change in heart rate or left ventricular filling pressure was observed. Diffuse ischemic myocardial injury was identified after a mean follow-up of 40 days. Intracoronary microembolization induces acute left ventricular dysfunction due to microinfarcts. Increased left ventricular end-diastolic volume is the initial compensatory response to the acute impairment of cardiac performance in nontransmural myocardial ischemia. This model is suitable for the evaluation of the hemodynamic changes secondary to acute and chronic diffuse loss of functional myocardium.

Influence of pre-existing ischemia on recovery from chemical cardioplegia. A study on pig hearts in an isolated blood-perfused model.

The impact of prior cardiac ischemia on recovery from chemical cardioplegia was investigated in pig hearts. Group I hearts were subjected to 9-min normothermic ischemia before the start of chemical cardioplegia. After 180 min of induced cardiac arrest, all hearts were reperfused and monitored for 120 min in a blood-perfused Langendorff model. Consistent with left ventricular performance, myocardial oxygen uptake was significantly lower in group I than in the other hearts during the first 60 min of reperfusion. Lactate elimination was significantly higher in group I at the start of reperfusion, but showed no intergroup difference after 25 min. Nor was intergroup difference found in left ventricular end-diastolic pressure, total myocardial flow or glucose extraction fraction during reperfusion. The mitochondrial ultrastructure was identical in the two groups before chemical cardioplegia. During cardioplegia it deteriorated in group I but normalized in group II. During reperfusion these circumstances were reversed. Although precardioplegic ischemia thus significantly impaired left ventricular performance during early recovery, with corresponding effects on metabolism and ultrastructure, stable performance during reperfusion indicated that the ischemic injury did not worsen.