Increased cardiovascular comorbidities in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia presenting with systemic inflammatory and autoimmune manifestations.

Myelodysplastic syndromes (MDS) and associated diseases, like chronic myelomonocytic leukemias (CMML), are heterogeneous, clonal disorders affecting the hematopoietic stem cells. They are characterized by dysplasia and a propensity to evolve toward acute myeloid leukemia. Systemic inflammatory and autoimmune manifestations (SIAMs) occur with a prevalence of 10% to 20% in myeloid malignancies, but the underlying pathogenetic mechanisms remain obscure. In this study, we aimed to characterize patient- and disease-based differences in MDS and CMML patients with and without SIAMs and explore the impact of SIAMs on progression and survival. We performed a retrospective, single-centre, and case-control study in a cohort of 93 patients diagnosed with MDS and CMML between 01/2008 and 12/2015. Thirty patients (32%) were identified with SIAMs: musculoskeletal and connective tissue (26.8%), vascular (19.5%), systemic autoinflammation (17%), skin (12.2%), gastrointestinal (9.8%), and others (14.6%). SIAMs were treated with glucocorticoids (60%), methotrexate (16.7%), biologicals (13.3%), and cyclosporine (3.3%). No significant differences between the SIAM and non-SIAM patients were observed in age, gender, or previous exposure to cancer treatment. Cardiovascular comorbidities were significantly more frequent in patients with SIAMs (63.1% vs 90%; OR 5.5; P < .01), but no differences were observed for other comorbidities or IPSS and IPSS-R risk scores. CMML and refractory anemia with excess blasts 1/2 subtypes were by tendency more frequent in patients with and refractory cytopenia with multilineage dysplasia (RCMD) in those without SIAMs. Finally, time to progression, leukemia free survival and overall survival were similar for both groups. Despite patient heterogeneity and small cohort size, we were able to identify a significant association of SIAMs with cardiovascular comorbidities but without influence on progression or survival.

The reversed and bidirectional elephant trunk technique in the treatment of complex aortic aneurysms.

BACKGROUND: The elephant trunk technique with a free-floating vascular prosthesis was originally developed to facilitate a subsequent operation on the downstream aorta. We present here our experience with further developments of this technique, which we call the reversed elephant trunk and bidirectional elephant trunk. METHODS: Between January 1, 1995, and December 31, 2000, 505 adult and adolescent patients underwent operations of the thoracic aorta. A reversed elephant trunk procedure in 13 patients and a bidirectional elephant trunk procedure in 4 patients was performed to facilitate either subsequent proximal or proximal and distal aortic replacement. Nine patients underwent subsequent aortic arch replacement with the reversed prosthetic portion after a mean interval of 8 +/- 5.5 months, and 2 patients received distal extension by use of the distal portion of the free-floating graft. RESULTS: There was no hospital mortality (30 days) in this small group of patients, and no patient had aortic rupture, malperfusion caused by the technique itself, or thromboembolic complications during the waiting interval between the first and the second operations. Five patients are still being observed until the contiguous aortic size is large enough to require an operation, and one 74-year-old patient declined a second-stage operation. CONCLUSION: The reversed and bidirectional elephant trunk techniques are interesting options that may be suitable for patients having complex abnormalities of the thoracic aorta and thoracoabdominal aorta when the proximal portion of the descending aorta has to be replaced before the aortic arch with or without the ascending aorta or the distal descending aorta with or without the thoracoabdominal aorta.

Determination of plasma volume by indocyanine green--validation of the method and use in patients after cardiopulmonary bypass.

OBJECTIVE: Validation of plasma volume (PV) determination by indocyanine green (ICG) in comparison to the gold-standard method with radioiodinated albumin, and investigation of the effect of commonly used plasma expanders (albumin, hydroxyethyl starch, and polygelatine) on PV in the early postoperative phase in patients undergoing cardiac surgery. DESIGN: Prospective clinical study. SETTING: Department of medicine and intensive care unit at a university hospital. PATIENTS AND PARTICIPANTS: Ten healthy volunteers and 21 patients after elective open-heart surgery. MEASUREMENTS AND RESULTS: PV of subjects was measured by i.v. injecting 5 microCi [125I]albumin (I-ALB). One hour later, PV was determined by a peripheral i. v. injection of 0.25 mg/kg body weight ICG (ICG1). In five subjects PV was measured repeatedly by ICG (ICG2) 1 h after ICG1. Mean PV of I-ALB and ICG1 or ICG2 showed consistent results. Further, we investigated central vs peripheral intravenous injection of ICG in six patients after open-heart surgery compared to [125I]albumin. There was no difference between mean PV measured by [125I]albumin and peripheral ICG (P = 0.40). PV determined by central injection of ICG was significantly higher than by the other methods. In 15 patients PV was determined by [125I]albumin. Thereafter, patients were randomly divided into three groups. Group ALB was infused with 1.75 ml/kg body weight human albumin 20%, group HAES with 5.25 ml/kg body weight hydroxyethyl starch 6%, and group HAEM with 7.0 ml/kg body weight polygelatine 3.5%. PV was measured 1 h and 4 h after infusion by ICG. There were no significant changes in PV between the groups. CONCLUSIONS: PV determination by peripheral i. v. injection of ICG produced reliable and consistent results when a reactive hyperaemia was produced by a tourniquet prior to injection. Therefore, central venous injection of ICG may not be prerequisite for precise measurements of PV. The expected acute increase in PV after infusion of commonly used plasma expanders after cardiac surgery was not found.

Rare presentation of cardiac hemangiomas.

Cardiac hemangiomas are exceptionally rare tumors with an incidence of 1% to 2% of all detected benign heart neoplasms. The clinical appearance of the tumor varies considerably and may mimic other pathological findings of definite heart structures. We report two cases of cardiac hemangiomas presenting with an unusual location and clinical course.

Homograft failure in mycotic aortic aneurysm caused by Streptococcus pneumoniae.

Treatment of a rare Streptococcus pneumoniae mycotic aneurysm by homograft replacement failed in a 59-year-old patient because of persistent lobar pneumonia. Despite reoperation with replacement of the infected homograft by a fresh one, he finally died of septicemia. This case illustrates that homograft tissue may be infected per continuum and that extensive debridement of periaortic tissue-including major lung resection-and the use of muscle flaps may be necessary in certain circumstances.

Outcome of thoracoabdominal aortic operations using deep hypothermia and distal exsanguination.

BACKGROUND: Operation of the descending and thoracoabdominal aorta may be affected by a significant perioperative morbidity, mainly because of ischemic damage of the spinal cord and malperfusion of the abdominal organs. METHODS: A comparative analysis was performed on two consecutive series of patients operated between 1982 and 1998. Group 1 consisted of 90 patients operated with moderate hypothermic left heart bypass. Group 2 included 38 patients operated using deep hypothermic cardiopulmonary bypass and a period of circulatory arrest while performing the proximal anastomosis and distal exsanguination during confection of the distal anastomosis. RESULTS: Main demographic factors and causes of the aortic disease were similar in both groups. Early mortality was significantly higher in the group of patients with aortic cross-clamping (15 of 90, 16%) than in those operated with circulatory arrest (2 of 38, 5.2%), p < 0.001. Paraplegia occurred in 8 patients in the group operated with mild hypothermia (8.8%) but in only 1 patient (2.6%) when deep hypothermia had been used. CONCLUSIONS: In our experience, deep hypothermia combined with distal exsanguination significantly improved the early postoperative outcome after operation of the descending and thoracoabdominal aorta. This technique allowed easy confection of proximal and distal anastomoses, and the duration of the operation was not prolonged significantly through this approach.

Transit-time flow measurement for detection of early graft failure during myocardial revascularization.

BACKGROUND: A low-flow situation in arterial and venous grafts has been associated with high rates of perioperative infarction and mortality. This study was designed to look at intraoperative graft flow and resistance in patients with coronary artery disease. METHODS: Coronary artery bypass graft flow was measured in 46 patients. Transit-time flow was used for coronary flow measurements at rest as well as after maximal vasodilation with adenosine infusion. RESULTS: Forty-three of the 46 patients showed normal internal mammary artery graft flow (>20 mL/min); 3 patients had no or minimal graft flow. Redoing the graft anastomosis in these 3 patients resulted in normalization of graft flow. The mean flow increased significantly after correction from 0.5 +/- 0.7 mL/min to 15.7 +/- 9.6 mL/min (p < 0.02). Conversely, vascular resistance decreased significantly from 138 +/- 10 to 4.8 +/- 1.8 Ohmv (p < 0.0001), as did the pulsatility index (from 146.9 +/- 95.7 to 3.4 +/- 1.8; p < 0.001). After correction, coronary flow reserve was 2.5 +/- 1.1. CONCLUSIONS: Measurements of intraoperative flow and resistance as well as derived variables allow assessment of early graft function and thus help prevent graft failure and reduce perioperative infarction. Transit-time volume flow might be a simple tool for quality control in coronary bypass procedures.

Long-term Doppler echocardiographic follow up in normally functioning aortic St. Jude Medical prosthesis.

BACKGROUND AND AIMS OF THE STUDY: Mean and peak Doppler gradients remain the most frequently used parameters for follow up of prosthetic aortic valves. Gradients that deviate from baseline recordings can lead to uncertainty among physician and patient, especially if symptoms have not completely subsided after surgery, or have recurred. This study aimed to document long-term evolution of mean and peak gradients in patients with stationary clinical symptoms and signs. METHODS: Seventy-six patients (48 men, 28 women), of mean age 56.1 +/- 14.5 years (range: 23 to 82 years) who underwent St. Jude Medical bileaflet prosthesis implantation were followed up for a mean of 3.9 years (range: 1 to 7 years), both clinically and echocardiographically. Evolution of mean and peak gradients, left ventricular function, other valvular lesions and rhythm as well as adequacy of anticoagulation were examined. RESULTS: Mean gradient increased from 12.3 +/- 5.5 to 14 +/- 5.7 mmHg (p = 0.002). Mean gradient increased in 47 patients, decreased in 17 and was unchanged in 12. There was no correlation between left ventricular function, appropriate anticoagulation, left ventricular hypertrophy, age or gender with change in mean or peak gradient. Change in peak gradient correlated excellently (r2 = 0.82) with that in mean gradient. CONCLUSIONS: The range of evolution of Doppler gradients in normally functioning St. Jude Medical prostheses has been defined in this study. Slight long-term increases in mean and peak pressure gradients are normal findings and do not warrant a change in management strategy if unaccompanied by deterioration of symptoms and/or clinical signs. Although we recommend routine determination of baseline flow measurements within three months of prosthesis implantation, mean and peak gradients are adequate follow up parameters. Peak gradient correlated well with mean gradient and may be a useful adjunct for follow up in clinical practice.

Definitive cure of recurrent prosthetic endocarditis using silver-coated St. Jude Medical heart valves: a preliminary case report.

Despite progress in the area of antimicrobial treatment and the surgical use of homografts, prosthetic valve endocarditis (PVE) remains one of the most dangerous complications following heart valve replacement. We present the case of a patient treated for acute endocarditis which affected the mitral valve and who developed recurrent PVE and native aortic valve endocarditis. After multiple valve surgery, the infection was controlled following aortic and mitral valve replacement using silver-coated prostheses. The St. Jude Medical (SJM) mechanical heart valve Masters Series with Silzone coating is intended to protect heart valve patients against microbial infection. The Silzone coating is formed by an ion beam-assisted deposition process that incorporates silver into the sewing cuff of the SJM heart valve. It has also been suggested that silver treatment may improve the healing characteristics of the heart valve sewing cuff. This technology may be a valuable option to prevent or cure PVE, in addition to homografts. Although the present patient is an isolated case, it was encouraging to find not only a well-healed mitral valve silver-coated prosthesis but also no persistent or recurrent infection during a nine-month follow up.

Embolization of a ruptured intercostal artery aneurysm in type I neurofibromatosis.

We present the case of a female with history of a ruptured lumbar aneurysm years ago. She was known to have neurofibromatosis type I with the typical clinical signs. The patient was transferred to us with a hematothorax and an aortic lesion was suspected on the outside CT scan. Reevaluation of the investigation raised suspicion of a ruptured intercostal artery aneurysm, which was consequently demonstrated on angiography. The aneurysm was embolized and the patient recovered uneventful. We will discuss the optimal therapy for vessel lesions in neurofibromatosis type I.

Acute traumatic rupture of the thoracic aorta: immediate or delayed surgical repair?

The appropriate management of traumatic aortic rupture is often difficult to determine, particularly if the rupture is associated with severe additional lesions. Between 1986 and 1991, ten consecutive patients with acute traumatic rupture of the thoracic aorta (ATRTA) and concomitant injuries were initially treated medically and submitted to delayed aortic repair. Within the same period no other patient had emergency reconstruction of the thoracic aorta. Diagnosis of ATRTA was established immediately after admission in eight patients. Five patients underwent emergency surgery for severe concomitant injuries. With regard to the aortic lesion, all patients were managed medically and submitted to pharmacological treatment in an attempt to reduce cardiac shear forces. None of the patients developed clinical signs of imminent free rupture while waiting for aortic surgery. In the absence of a significant hemothorax and when no coarctation syndrome is evident, the risk of free aortic rupture is considered to be rather low if the patient reaches the hospital in a stable circulatory condition. Postponement of aortic reconstruction is particularly indicated when severe concomitant lesions preclude safe immediate repair of the aortic tear. Following the patient's recovery from associated major injury, aortic surgery can be performed as a low risk procedure using cardiopulmonary bypass which is recognised as the most effective technique to prevent spinal cord ischemia and to reduce the risk of paraplegia.

Impending rupture of the ascending aorta due to giant cell arteritis.

Giant cell arteritis may occasionally lead to vessel perforation without previous dissection. At the level of the ascending aorta, however, such an event has been reported only three times. We report a fourth case of impending rupture of the ascending aorta due to a small and isolated lesion of giant cell arteritis. In contrast to previous reports, laboratory and clinical manifestations suggestive of giant cell arteritis were absent in this case so that the diagnosis could only be established on histologic examination of the operative specimen.

Failed coronary artery bypass anastomosis detected by intraoperative coronary flow measurement.

OBJECTIVES: To assess intraoperative flow of arterial and venous coronary grafts after myocardial revascularization which may allow early detection of low flow situations, especially during minimally invasive coronary bypass surgery (MIDCAB), and lead to immediate correction of technical problems. METHODS: In two patients with severe and diffuse multi-vessel disease the left internal mammary artery (IMA) was connected to the left anterior descending artery (LAD). During reperfusion, the flow was measured in the IMA and vein grafts using a transit time flow meter. RESULTS: In both cases the IMA showed only a systolic pendulating flow curve with a mean flow of 0-1 ml/min and a high resistance. Manual IMA assessment revealed an adequate pulsation. Both distal IMA anastomoses were re-explored on cardiopulmonary bypass yielding an initial flow of 7 and 14 ml/min, respectively. After treatment with papaverine/adenosine the IMA flow increased from 7 to 26 ml/min (coronary flow reserve (CFR) = 3.7) and from 14 to 46 ml/min (CFR = 3.3), respectively. CONCLUSION: Intraoperative flow assessment of IMA and venous bypass grafts can be recommended to monitor flow; especially during MIDCAB procedures.

Different techniques of distal aortic repair in acute type A dissection: impact on late aortic morphology and reoperation.

OBJECTIVE: To compare three different techniques of distal aortic repair in acute type A (de Bakey type I) aortic dissection and to evaluate their impact on the late morphology of the aortic arch and descending aorta and on the incidence of reoperation. METHODS: From 65 patients operated on due to an acute type A aortic dissection between 1989 and 1993, 54 long-term survivors underwent clinical and radiologic follow-up examination after a mean postoperative interval of 62+/-16 months. The surgical techniques of distal aortic reconstruction included closed repair using Teflon felt reinforcement under moderate hypothermic cardiopulmonary bypass (n = 20) and open repair in deep hypothermic circulatory arrest using either Teflon felt reinforcement (n = 16) or gelatin-resorcin-formaldehyde (GRF) glue (n = 18) to readapt the dissected aortic layers. In all patients, MR imaging was performed on a 1.5-T whole body imaging system for the evaluation of the morphology and function of the heart, aorta and supraaortic branches. RESULTS: Overall hospital mortality following surgical repair of type A aortic dissection was 15.4% during this time period. The highest rate of persistent false lumen perfusion (17/20, 85%) and presence of an intimal flap in the aortic arch (13/20, 65%) was observed in patients following closed repair of acute ascending aortic dissection, whereas the lowest rate of such findings was demonstrated in patients who had undergone open distal aortic repair using biological glue (false lumen perfusion 10/18, 55% and intimal flap in the arch 2/18, 11%). Redo-surgery was significantly reduced in the open repair group using GRF glue (1/18, 5.5%) as compared with the Teflon felt repair group (3/16, 18%) and the closed repair group (6/20, 30%). CONCLUSIONS: In patients with acute type A dissection, open distal aortic repair using GRF-glue favourably influences both (1) the severity of late morphologic alterations in the downstream aorta and (2) the incidence of reoperation.

Surgery for acute ascending aortic dissection: closed versus open distal aortic repair.

One hundred twelve consecutive patients with acute ascending aortic dissection and submitted to immediate surgery were retrospectively analyzed with regard to perioperative mortality and morbidity. The patients were divided into two groups according to whether distal aortic repair was carried out by the open procedure (using deep hypothermic circulatory arrest, group A: 68 patients) or by the closed technique (without circulatory arrest, group B: 44 patients). Patients' ages ranged from 24 to 78 years (mean 57.4 years). No significant difference was found between the two groups in terms of age and sex distribution. However, the prevalence in the extent of clinical and anatomical alterations was significantly higher in group A (hemodynamic instability, pericardial tamponade and neurological deficit). The duration of hypothermic circulatory arrest for group A patients averaged 25 min and ranged from 12 to 65 min. The overall perioperative mortality was 17% (19/112 patients); it was 20.6% (14/68) in group A and 11.4% (5/44) in group B; the difference was not statistically significant but consistent with a clear trend. The trend towards a higher mortality in group A mainly reflected the more severe and complex anatomical characteristics and could not be attributed to the circulatory arrest per se. The period of deep hypothermic circulatory arrest in the survivors (25 min) was similar to that of the group with lethal outcome (32 min). Among the non-lethal complications, however, group A patients more frequently showed clinical signs consistent with cerebral injury: apart from the transient symptoms suggestive in reversible diffuse cerebral damage, five patients in group A had a permanent focal neurological deficit (versus one patient in group B).(ABSTRACT TRUNCATED AT 250 WORDS)

Evaluation of coronary bypass flow with color-Doppler and magnetic resonance imaging techniques: comparison with intraoperative flow measurements.

OBJECTIVES: After coronary artery bypass surgery, patency and flow assessment is based on invasive methods such as angiography and intravascular ultrasound or flow wire techniques. The aim of the study was to compare intraoperative transit time flow measurements of coronary bypass grafts with early postoperative color-Doppler and MR-imaging assessment. METHODS: In 22 patients (62+/-8.5 years) undergoing elective coronary bypass surgery the flow was measured in all internal mammary artery grafts (IMA) and saphenous vein grafts using the transit time flow technique. Postoperatively (days 5-7) all patients had a color-Doppler IMA graft assessment followed by a MR-angiography and flow measurement (navigator echo phase contrast technique with and without contrast bolus application) to determine patency and graft flow. RESULTS: Data are expressed as the mean +/- SD). (1) In all patients the left IMA graft to the left anterior descending coronary artery (LAD) could be identified and flow could be assessed with both color-Doppler and MRI. Venous grafts could only be visualized by MRI. The use of an intravenous contrast bolus enhanced the visualization of coronary artery bypass grafts. (2) The mean IMA to LAD flow was 33+/-17 ml/min intraoperatively by transit time and postoperatively 36+/-25 ml/min by MR respectively 66+/-54 ml/min by color-Doppler technique. (3) The systolic/diastolic flow ratio was 0.44+/-0.12 intraoperatively and 0.43+/-0.17 postoperatively by MR respectively 0.67+/-1.0 by color-Doppler. (4) A statistically significant correlation could be demonstrated between intraoperative transit time and postoperative MR flow measurements (r = 0.57; P < 0.04), whereas the correlations to color-Doppler flow were poor. Postoperatively MR and color-Doppler showed a good correlation of systolic/diastolic flow ratio (r = 0.88; P < 0.008). CONCLUSIONS: The color-Doppler method during echocardiography and MR-imaging are useful non-invasive techniques to visualize postoperative IMA grafts for patency assessment. The quantification of IMA flow is still difficult with either technique, but MR flow measurements showed the best correlation to the intraoperatively measured transit time flow. The MR technique is the most promising non-invasive method for postoperative evaluation of coronary bypass grafts, since it allows visualization and reliable flow quantification.

[Heart and vascular surgery interventions with hypothermic circulatory arrest in adults]. / Herz- und gefässchirurgische Eingriffe mit hypothermem Kreislaufstillstand beim Erwachsenen.

In the period between 1981 and 1988, 51 patients were operated on the thoracic aorta using the hypothermic circulatory arrest technique. 31 patients had a dissection of the thoracic aorta, in 16 cases, an aneurysm was the reason for the intervention. In addition, we used the hypothermic circulatory arrest for a thrombectomy in the aortic arch and two mitral-valve replacements. The following operations were performed: 14 x composite graft, 19 x supracoronar prosthesis (6 x with aortic valve replacement, 3 x with partial replacement of aortic arch), 17 operations were performed either for aortic arch or aorta descendens replacement. In our retrospective study, 7 courses were fata (14%), 3 patients had complications with residuals. Compared with a group of 105 patients operated on the thoracic aorta in the same period without circulatory arrest, we found no difference with regard to the lethality and morbidity. We conclude that the hypothermic circulatory arrest is a safe technique for selected problems in cardiovascular surgery in adults.

[Homograft in the therapy of cardiovascular diseases]. / Der Homograft in der Therapie von kardiovaskulären Erkrankungen.

Despite considerable progress in prosthetic valve technology, there is actually no ideal artificial heart valve that may be employed in all circumstances, when replacement of a diseased aortic valve is necessary. The choice for optimal valve substitute includes mechanical prosthesis, bioprosthetic xenografts, homografts and pulmonary autografts. More recently an aortic valve sparing operation has been proposed in younger patients presenting with anulo-aortic ectasia. The use of homograft heart valves has now been widely accepted in the treatment of congenital heart defects, as well as for an increasing number of valvular pathologies, especially in infective endocarditis. Heart valve preservation by cryopreservation techniques helps to store the small amount of donor material for special indications without loss of quality, thus permitting elective surgery. In vascular surgery, some concern persists regarding the use of prosthetic material for the treatment of mycotic aneurysms and graft infection. In situ repair with a new vascular prosthesis and resection followed by extra-anatomic reconstruction carry a high peri-operative mortality and substantial morbidity. Cryopreserved vascular homografts represent a valuable alternative in these challenging situations.