[Successful resuscitation of a patient with ventricular fibrillation in Bland-White-Garland syndrome in adulthood. A case report]. / Erfolgreiche Reanimation einer Patientin mit Kammerflimmern beim Bland-White-Garland-Syndrom im Erwachsenenalter. Ein Fallbericht.

The Bland-White-Garland Syndrome represents the anomalous origin of the left coronary artery of pulmonary trunk. Only 10% of the patients reach adulthood. Clinical manifestations of the syndrome are angina, dyspnoe, ECG signs of ischemia, myocardial infarction, and death in childhood. We present the case of a 47 year old woman with Bland-White-Garland Syndrome, who was resuscitated from ventricular fibrillation. The only symptom shown in her personal history was progressive dyspnoea in the last 6 months, though mitral insufficiency was known since childhood. On echocardiographic examination, she showed an anterolateral infarction and a mitral insufficiency II. As operation procedure, the ligation of the left main coronary artery and bypass surgery with a left internal mammarian graft to the left descending branch of the left coronary artery was chosen. The mechanism of onset of ventricular tachycardia in our patient is not known. Three pathophysiological mechanisms may be possible: (1) local ischemia caused by the shunt, (2) a reentry circuit in the border zone of myocardial infarction, (3) electrical instability caused by endocardial fibrosis. As local ischemia and reentry circuit were widely excluded, only endocardial fibrosis could induce further ventricular arrhythmia. We therefore intended to implant an AICD to have the most possible safety for our patient. But this, postoperatively was refused by the patient. In analogy to Coronary Artery Disease, the risk for sudden cardiac death postoperatively may be due to three factors: (1) presence of a reentrant circuit, (2) LV-function below 40%, and (3) presence of endocardial fibrosis. Our patient showed a low risk for sudden cardiac death. On electrophysiological study, no ventricular tachycardia could be induced in our patient, indicating the absence of a reentry circuit. LV function exceeded more than 40%. In Holter ECG, only few ventricular premature beats could be registrated, indicating a low risk for sudden cardiac death in the presence of endocardial fibrosis. In the follow-up of fourteen months, the patient remained free from arrhythmic events.

[Diagnosis and therapy of a fluttering thrombus in the open foramen ovale]. / Diagnostik und Therapie eines flottierenden Thrombus im offenen Foramen ovale.

The finding of an extremely mobile thrombus in a patent foramen ovale (i.e., transit thrombus) without arterial embolism is rare. In our case-report diagnosis was made by routine-echocardiography. Clinically nonapparent deep vein thrombosis was documented by phleography and was thought to be the origin of the embolus. After implantation of a caval filter device cardiac surgery was performed and the embolus was removed without complications.

[Traumatic aortic rupture--diagnosis with transesophageal echocardiography]. / Traumatische Aortenruptur--Diagnosestellung mittels transösophagealer Echokardiographie.

A quick and exact diagnosis of a ruptured aorta can be achieved by different imaging methods (CT, TEE, NMR). In this case report, we describe a 36-year-old patient, who suffered rupture of the thoracic aorta in the isthmus region during a car accident. CT was suspicious of aortic rupture and the exact localization of the rupture site was visualized by TEE. It was confirmed during the operation when an aortic prosthesis was implanted. The high mortality of aortic rupture could possibly be lowered by early application of TEE. Preoperative angiography seems not to be necessary.

Platelet function in acute myocardial infarction treated with direct angioplasty.

BACKGROUND: In acute myocardial infarction (AMI), platelets play a key role in thrombotic processes that limit the patency of the recanalized, infarct-related coronary artery and contribute to reperfusion injury. Platelet function in the course of AMI treated by direct percutaneous transluminal coronary angioplasty (PTCA) has not been evaluated. METHODS AND RESULTS: In 15 patients with anterior AMI, peripheral venous blood samples were obtained before and 4, 8, 24, and 48 hours after recanalization of the occluded artery by PTCA. Fifteen patients who had stable coronary heart disease and were undergoing elective balloon angioplasty served as control subjects. Fibrinogen receptor function and surface expression of P-selectin on platelets were determined by flow cytometry. In addition, we evaluated generation of platelet-derived microparticles and the effect of systemic plasma from patients with AMI on normal platelet function and on platelet adhesion to human endothelial cells in culture. We found fibrinogen receptor activity and P-selectin expression on circulating platelets 8 hours after direct PTCA are decreased (P < .01). This coincided with a decrease in peripheral platelet count (P < .05) and an increase in generation of microparticles (P < .002). Twenty-four to 48 hours after PTCA, fibrinogen receptor activity and P-selectin expression increased again. Systemic plasma obtained before and after direct PTCA sensitized normal platelets to hyperaggregate in vitro (P < .001) and stimulated platelet adhesion to endothelial cells in culture (P < .01). None of the changes found in AMI were detectable in the control group. CONCLUSIONS: After transient apparent deactivation of circulating platelet, probably caused by sequestration of hyperactive platelets, the level of platelet activation increases in patients with AMI treated by direct PTCA. These findings underscore the need for novel antiplatelet strategies in AMI.

Pathophysiologic role of contact activation in bleeding followed by thromboembolic complications after implantation of a ventricular assist device.

The time period after implantation of a ventricular assist device in patients with end-stage heart disease is complicated by hemorrhage in the early postoperative period and by thromboembolism in the later course. To investigate the pathophysiologic role of contact activation in 12 bridging patients (10 patients with a paracorporeal Berlin Heart [Berlin Heart GmbH, Berlin, Germany], 2 patients with an intracorporeal Novacor system [Novacor N100; Baxter, Oakland, CA]), hemostatic parameters were determined until heart transplantation or at least up to the 51st postoperative day. The following were observed: 1) In the early postoperative period, until day 15, levels of contact factors XI, XII, and prekallikrein were below normal, whereas levels of plasmin-a2-antiplasmin (PAP) complexes were elevated. Thrombin-antithrombin III (TAT) complexes, as well as platelet factor 4 and beta-thromboglobulin, significantly increased immediately after surgery. 2) In the later postoperative period, starting with the third postoperative week, an increase of factors XI, XII, and prekallikrein was observed. PAP and TAT complexes, as well as platelet factor 4 and beta-thromboglobulin, remained elevated. It is concluded that, in the early postoperative period, hemostasis is influenced mainly by an activation of the intrinsic contact system dependent fibrinolytic system with consumption of contact factors and increased levels of PAP complexes, whereas later system dependent fibrinolysis becomes less important, leading to a shift of the balance toward coagulation, with sustained prothrombin and platelet activation. This is in accord with the observed clinical complications (e.g., early postoperative bleeding, and thromboembolic events later on).

ICAM-1 expression on alveolar macrophages and in serum of sarcoidosis patients.

The intercellular adhesion molecule-1 (ICAM-1) is important in mediating intercellular contact in inflammation. Therefore, we have analyzed the expression of this molecule on alveolar macrophages (AM) and in serum of patients with sarcoidosis. Bronchoalveolar lavage (BAL) cells from 13 patients and 11 control donors were stained with an anti ICAM-1 monoclonal antibody (mAb) or an isotype control. Alkaline phosphatase was used as a detection system, followed by digital single cell image analysis. Soluble ICAM-1 in serum (ssICAM-1) was determined by enzyme linked immunoassay (Elisa). Immunocytochemistry revealed a strong increase of ICAM-1 expression on AM from sarcoidosis patients (64%) compared to healthy controls (30%). Furthermore, patients exhibited a fourfold higher antigen density. Serum levels of sICAM-1 were more than twofold increased in the patient group (805.4 micrograms/ml) compared to healthy controls (384.8 micrograms/ml). SsICAM-1 showed an inverse correlation with vital capacity (VC) and diffusing capacity (DCO). This significant correlation with impairment of two important lung function parameters suggests that ssICAM-1 might be useful in serological assessment of disease activity in sarcoidosis.

Urinary excretion of thromboxane and markers for renal injury in patients undergoing cardiopulmonary bypass.

Urinary excretion of selected markers for renal injury, as well as urinary excretion rates of the thromboxane metabolite, 11-keto-thromboxane B2 (11k-TXB2), was studied in 36 male patients undergoing coronary bypass surgery using cardiopulmonary bypass (CPB). In all patients, excretion of both tubular (N-acetyl-beta-D-glucosaminidase [beta NAG]; alpha 1-microglobulin [alpha 1-MG]) and glomerular markers (albumin [Alb]; transferrin [Trf]; immunoglobulin G [IgG]) sharply increased on Day 1 after CPB, and they remained elevated throughout the observation period of 5 days. Urinary excretion rates of 11k-TXB2 markedly increased on Day 1 after surgery, and they rapidly decreased thereafter. In 12 of the 36 patients, a temporary increase of serum creatinine levels (> 1.30 mg/dl) was noted following surgery. A positive correlation was found between serum creatinine levels and excretion of the tubular enzyme beta NAG (r = 0.36; p < 0.05), but not between creatinine levels and alpha 1-MG or the glomerular markers. Furthermore, no correlation between urinary excretion of 11k-TXB2 and any of the urinary markers for renal injury could be detected. Our data do not strengthen the hypothesis that acute renal injury observed during CPB is related to exaggerated thromboxane biosynthesis in these patients. Monitoring of urinary markers for incipient renal damage, particularly excretion of beta NAG, might be of additional diagnostic value for detection of otherwise subclinical renal injury in patients undergoing CPB.

Mechanical circulatory support and heart transplantation. Pre-operative status and outcome.

Between July 1988 and December 1993, 118 patients waiting for heart transplantation underwent mechanical circulatory support by an extracorporeal, pneumatically driven assist device (the Berlin Heart System) to maintain a sufficient circulation and to restore impaired organ function (109 patients requiring a biventricular assist device [BVAD], 9 patients requiring a left ventricular assist device [LVAD]). Before implantation, all patients were in severe cardiogenic shock, despite maximum inotropic support; all had end organ failure. The aim of a retrospective study in 70 patients (for whom pre-operative parameters were available) was to identify patients who would permit from this procedure and could undergo successful transplantation. After a mean bridging time of 34.8 days, 75 (63.5%) patients underwent transplant, and 52 (44.1%) were discharged. Pre-operative coagulation parameters (fibrinogen, antithrombin III, platelet count, and kinetics) were correlated to post-operative blood loss and outcome. Patients who had no or mild coagulation disorders because of a shorter phase of low cardiac output before implantation of the assist device proved to gain faster restitution of organ function and most underwent transplantation. As a result of these observations, implantation of an assist device before shock-induced coagulopathy could occur allowed a greater number of patients to undergo successful bridging and be discharged after heart transplantation.

Coronary artery bypass grafting and heart transplantation in end-stage coronary artery disease: a comparison of hemodynamic improvement and ventricular function.

Heart transplantation has now become an accepted treatment for end-stage coronary heart disease (CAD). However, the limited supply of suitable donor organs imposes constraints upon the decision of whether patients are selected for transplantation or for coronary artery bypass grafting (CABG). From April 1986 until the end of March 1992, 265 patients with end-stage CAD involving left ventricular ejection fraction (LVEF) 10% to 30% and predominant angina pectoris underwent CABG. All patients received an average of 2.9 +/- 0.3 venous grafts. Intraaortic balloon pumps were implanted in 30 patients (11.3%) who began to develop low cardiac output syndrome intraoperatively. The actuarial survival rate was 87.8% after 2 years and 86.9% after 3 years. LVEF was measured in 35 patients via left heart catheterization 12 months after their operations and was found to have increased from a mean of 23.8% to 38.1%. Left ventricular end-diastolic pressure had decreased from 16.2 mmHg to an average of 12.1 mmHg. Swan-Ganz catheterization was performed on 120 patients 6 months postoperatively. The pulmonary wedge pressure had reduced significantly from 18.1 mmHg to a mean of 12.7 mmHg (p < 0.01). From 1990 until the end of March 1992, 55 patients with CAD and predominant heart failure received transplants. Their 2-year survival rate was 66.3%. Mean LVEF was 55.6% postoperatively. We conclude that CABG is adequate for patients who have end-stage CAD and angina pectoris symptoms, and that it significantly improves hemodynamic functions. Patients suffering predominantly from heart failure (NYHA Class IV) can be transplanted and subsequently regain normal heart function.

Expression of CD14 correlates with lung function impairment in pulmonary sarcoidosis.

CD14 expression on alveolar macrophages (AM) was studied in patients with sarcoidosis using immunocytochemistry and cytometric analysis. Compared with healthy control donors, patients had elevated percentages of CD14-positive AM (22 percent vs 34 percent), and the antigen density was threefold higher (92 vs 297 channels). Furthermore, soluble serum CD14 (ssCD14) was significantly elevated in patients with sarcoidosis with an average of 5.3 +/- 1.6 mg/L vs 3.2 +/- 0.7 mg/L in healthy control subjects. Follow-up of one patient, whose lung function test results improved during therapy with corticosteroids, revealed a concomitant decrease of CD14 staining on AM and of ssCD14. Statistical analysis revealed a negative correlation between CD14 expression on AM and PO2 at rest (p = 0.0005), and after labor (p = 0.02). Levels of ssCD14 gave a positive correlation to reduction of Dco (p = 0.006) and VC (p = 0.05). These data suggest that CD14 expression is related to severity of disease and that it may be useful for monitoring in sarcoidosis.

Increased expression of the monocyte differentiation antigen CD14 in extrinsic allergic alveolitis.

Expression of the CD14 antigen was studied on alveolar macrophages in extrinsic allergic alveolitis (EAA), using immunocytochemistry and cytometry. Compared to control donors, EAA patients had higher percentages of My4 positive cells (40 versus 22%), and the antigen density was fourfold higher (410 versus 92 channels). Levels of soluble CD14 (sCD14) in serum were found to be increased in EAA patients with an average of 4.6 +/- 1.5 micrograms.ml-1 compared to 3.2 +/- 0.7 micrograms.ml-1 in controls. Follow-up of patients with antigen avoidance revealed a concomitant decrease of CD14 staining of alveolar macrophages (AMs) and of sCD14 in serum, whilst allergen exposure induces both parameters. These data are consistent with the concept that antigen contact upregulates CD14 expression on AMs in EAA, followed by shedding and increase of sCD14 in serum.

Survival predictors in patients with a left ventricular ejection fraction of 10-30% receiving a coronary bypass: analysis of preoperative variables.

A total of 224 patients with angina pectoris and a left ventricular ejection fraction in the range of 10-30% (mean 24.2%) underwent coronary artery bypass grafting between April 1986 and August 1991. These patients received a mean (s.d.) of 2.9 (0.3) aortocoronary vein grafts. The overall operative mortality rate was 8.9%. The 1-, 2- and 3-year survival rates were 87.7%, 86.7% and 85.2%, respectively. Analysis of operative risk factors showed that patients with an end-diastolic left ventricular pressure > 24 mmHg were significantly more at risk (mortality rate 20.0%, P < 0.05) than those with an end-diastolic left ventricular pressure < or = 24 mmHg (mortality rate 6.2%). Patients with a perioperative cardiac index < 2.5 l min-1m-2 had higher mortality (25.4%) than those with a cardiac index > or = 2.5 l min-1m-2 (mortality 1.9%, P < 0.001). The operative mortality rate of patients with a cardiac index < 2.5 l min-1m-2 and an end-diastolic left ventricular pressure > 24 mmHg was 40.5%. Patients with a left ventricular ejection fraction of 10-20% were not significantly more at risk (P > 0.05) than those with a left ventricular ejection fraction of 21-30%.

[Aorto-intestinal fistula versus angiodysplasia. A difficult differential diagnosis in gastrointestinal bleeding]. / Aortointestinale Fistel versus Angiodysplasie. Eine schwierige Differentialdiagnose bei gastrointestinalen Blutungen.

A 63-year-old patient had recurrent tarry stools and haemoglobin levels of around 7.0 g/dl. Ten years previously he had undergone an aortobifemoral bypass operation for peripheral vascular disease in the legs. Eight gastroscopies and five coloscopies over ten weeks failed to discover a bleeding source. Although an aorto-intestinal fistula was considered early on, extensive diagnostic tests failed to reveal it. Digital subtraction angiography was suggestive of an angiodysplasia of the terminal ileum, a diagnosis supported when coloscopy during exploratory laparotomy visualized blood trickling from the terminal ileum. As a result of this finding a right hemicolectomy was performed. But recurrent bleedings necessitated relaparotomy which finally revealed a fistula between the ascending duodenum and the proximal bypass graft anastomosis. Nine months after resection of the proximal anastomotic area and interposition of a Dacron prosthesis the patient has been free of symptoms.

Expression of the adhesion molecule ICAM-1 on alveolar macrophages and in serum in extrinsic allergic alveolitis.

ICAM-1 expression was studied on alveolar macrophages (AM) in extrinsic allergic alveolitis (EAA) using immunocytochemistry and cytometry. Compared to control donors, EAA patients had higher percentages of ICAM-1+ AM (75% vs. 30%) and the antigen density was more than sixfold higher (673 vs. 103 channels). Levels of soluble serum ICAM-1 were found increased in EAA patients with an average of 866.2 +/- 300 ng/ml versus 394.8 +/- 110 ng/ml in controls. These data are consistent with the concept that antigen contact upregulates ICAM-1 expression on AM in EAA followed by shedding and an increase in serum sICAM-1.

Mechanical circulatory support and heart transplantation.

Mechanical circulatory support systems are currently in clinical use to keep patients alive with the aim of either cardiac recovery after open heart surgery, myocardial infarction and after heart transplantation with acute graft failure, or as a bridge to transplantation in heart transplant candidates. At the German Heart Institute the "Berlin Heart" mechanical circulatory support system has been used invariably since 1987. Up to 1991 there were 22 patients in the "recovery" group. Four patients could be weaned from the system, but only one patient was discharged from the hospital. In 65 patients mechanical circulatory support systems were implanted as a bridge to transplantation, 39 patients had transplants, and 22 patients left the hospital. Risk factor analysis in the bridging group revealed that previous heart surgery, infective pneumonia, shock-related coagulation disorders, and an age greater than 50 years had an unfavorable influence on patient survival. It is concluded that patients may be kept alive for weeks and months after any kind of cardiogenic shock. Complete cardiac recovery may be achieved in the case of early posttransplant graft failure. Reliable prediction of outcome in the bridge to transplantation group requires further experience.

Recovery of end-organ failure during mechanical circulatory support.

To evaluate organ recovery during mechanical assistance, respiratory, hepatic and renal function parameters of 40 patients who underwent bridge-to-transplant procedures were reviewed retrospectively. Mechanical circulatory support was indicated if the hemodynamic and clinical status deteriorated despite pharmacotherapy with catecholamines, vasodilators, and intravenous use of the phosphodiesterase inhibitor enoximone. Sequelae of cardiogenic shock such as renal, hepatic and respiratory insufficiency were not considered a contraindication for mechanical support. The analysis of preimplant data such as serum creatinine, liver enzymes and pulmonary gas exchange did not identify any predictive indicator of irreversible organ damage. Functional recovery of preexisting respiratory, hepatic and renal dysfunction was found in 91%, 90%, and 85%, respectively. Subsequent transplantation, however, was affected by the number of failing organs prior to mechanical support. Of 17 patients with isolated organ failure prior to assist, 14 (82%) were transplanted. By contrast, 9 (75%) of 12 with combined failure of two organs, and only 6 (54%) of 11 patients with clinical patterns of three failing organ systems received transplants. In all patients who underwent successful transplantation, transplantability was associated with rapid organ recovery within 10 to 15 days after initiating mechanical assistance.

[Progress in heart transplantation]. / Fortschritte in der Herztransplantation.

Within the past 10 years, heart transplantation has become established as a standard procedure in heart surgery. Improvements in immunosuppressive therapy and diagnosis of graft rejection have been crucial. The criteria for transplantation have been broadened for recipients as well as for donors. Newborns, pediatric patients, diabetics, and patients with impaired renal function will no longer be excluded from transplantation due to improved postoperative therapy. Furthermore, progress has been made with assisted circulation. Patients with acute heart failure can now be bridged to transplantation.

[Coronary revascularization in end-stage coronary heart disease in relation to assessment of myocardial vitality]. / Koronarrevaskularisation im Endstadium der KHK in Abhängigkeit von Myokardvitalitätsprüfung.

From 4/1986-12/1990, 177 pts. with endstage coronary artery disease (CAD) and left ventricular ejection fraction 10-30% received coronary artery bypass grafting (CABG). Preoperatively myocardial infarction rate was 1.5 (mean). Presupposition for CABGs was myocardial ischaemia at present demonstrated in myocardial viability test. 66.1% of the pts. had signs of ischaemia at e.c.g. after work. Additionally 97.6% of the pts. had myocardial ischaemia defined as redistribution in myocardial scintigraphy. Angina pectoris was present in 93.8% of the pts. preoperatively. 1-5 (mean 2.9) CABG per pt. were performed. 35 pts. received an internal mammaria bypass to the left coronary artery also. Operative mortality was 11.3% (1986-1990) and in 1990 alone 7.3%. Actuarial survival rate was calculated after one year to 87.4% after two years to 86.1% and after three years to 84.8%. Postoperatively all pts. were free from angina pectoris. 5 months after the operation e.c.g. after work was performed. The physical stress bearing area was increased to 82.7 Watt (mean) compared to 51.7 Watt (mean) preoperatively (p less than 0.001 s.). In conclusion pts. with endstage CAD and left ventricular ejection fraction 10-30% appeared to be good candidates for CABG with good prognosis and significant symptomatic improvement when signs of myocardial ischaemia are present preoperatively.

[Heart transplantation--indications and results]. / Herztransplantation--Indikation und Ergebnisse.

Heart transplantation has become a standard procedure in the treatment for irreversible heart failure. Criteria for both recipients and donors have been extended. One year survival now reaches 81%. In the immediate postoperative course patients are endangered by infection. In the long-term course coronary artery disease of the transplanted heart becomes the most serious problem. Alteration of liver and kidney function due to chronic medication as well as malignancies and hypertension also occur. In case of chronic transplant failure retransplantation may be indicated. Acute cardiac failure before transplantation nowerdays can be treated by mechanical circulatory assist devices.

Thromboxane B2 urinary metabolites in patients undergoing cardiopulmonary bypass.

The urinary excretion of selected markers for renal injury and thromboxane metabolites was studied in 16 patients undergoing cardiopulmonary bypass (CPB). Excretion of both tubular and glomerular markers sharply increased on day 1 after CPB and remained elevated throughout the observation period (five days). Immunoreactive thromboxane B2 (i-TXB2, mainly reflecting 2,3-dinor-TXB2) and immunoreactive 11-keto-thromboxane B2 (i-11-keto-TXB2) were measured by direct enzyme immunoassays. TXB2, 2,3-dinor-TXB2 and 11-keto-TXB2 were also measured in selected samples by GC-MS. Urinary excretion rates of both i-TXB2 and i-11-keto-TXB2 markedly increased on day 1 after surgery and decreased thereafter. Following CPB, excretion rates of 2,3-dinor-TXB2 and TXB2 displayed parallel changes, suggesting that in these patients most urinary TXB2 derives from blood platelets rather than the kidney. Taken together, our observations do not support the hypothesis that acute renal injury observed after CPB is caused by exaggerated thromboxane biosynthesis in the kidney.