Successful pregnancy complicated by persistent pneumothorax in a patient with lymphangioleiomyomatosis (LAM) on sirolimus.

We report a successful pregnancy in a patient with longstanding LAM on treatment with sirolimus. During temporary discontinuation fo sirolimus in early pregnancy, lung function declined but recovered after resumption of sirolimus. Pregnancy was complicated by a persistent pneumothorax which was treated surgically postnatally. The child has had a normal development despite exposure to low dose sirolimus intermittently during early embryonal and mid-fetal life.

[Life-threatening acute chest syndrome with hemolytic crisis in sickle cell disease. Treatment using a venovenous extracorporeal membrane oxygenation (ECMO)]. / Lebensbedrohliches akutes Thoraxsyndrom mit hämolytischer Krise bei Sichelzellerkrankung.

HISTORY AND CLINICAL FINDINGS: A 28-year-old male patient with sickle cell disease developed a vasoocclusive crisis with an acute chest syndrome and severe lung failure. TREATMENT AND COURSE: After partial substitution transfusion the patient was successfully treated extracorporeal membrane oxygenation (ECMO). After 12 days he was weaned from the ECMO, 2 days later the patient's tracheostoma was removed. 19 days after the event the patient had recovered completely and was moved to a hospital in Paris from where he came. CONCLUSION: Apparently data is sparse regarding the use of ECMO for treatment of a life-threatening acute chest syndrome in sickle cell disease. However, the therapy approach seems to be promising and is worth considering in this particular situation.

[Diagnostics in pulmonary hypertension]. / Diagnostik der pulmonalen Hypertonie.

Commonly, pulmonary hypertension is clinically suspected because of unexplained exertional dyspnoea or as a chance finding in clinical examination revealing signs of right heart failure. The systematic diagnostic approach and exact classification is based on the Venice classification. Basic investigations include ECG, chest radiograph, lung function studies and echocardiography. Echocardiography is the most important investigation for the diagnosis of pulmonary hypertension. It also serves as non invasive control during treatment and as the main screening test for pulmonary hypertension. Echocardiographic criteria of pulmonary hypertension are a dilated and hypertrophied right ventricle, paradoxic septum movement, a dilated right atrium, and a distended inferior Vena cava. Using Doppler echocardiography, the right ventricular and thus pulmonary arterial systolic pressure can be determined from the tricuspid regurgitant jet velocity. CT of the chest serves to exclude pulmonary embolism and interstitial lung disorders. Cardiac MRI is increasingly being used for analysis of right ventricular morphology, function and haemdynamics. The 6 minute walk test and cardiopulmonary exercise test are used to assess severity and response to treatment and give prognostic information. For the definite diagnosis of pulmonary hypertension, right heart catheterisation is required for the determination of the pulmonary vascular resistance and pharmacological testing of "reversibility".

[Rheology and coronary heart disease]. / Rheologie und koronare Herzkrankheit.

More than 25 years ago it was demonstrated that an improvement in various parameters of blood flow in patients who have an hyperviscosity syndrome improves coronary blood flow. Hyperfibrinogenemia with resulting increase in plasma viscosity and erythrocyte aggregation has been demonstrated in patients with coronary heart disease. Poiseuille's law, which describes the interaction between vascular resistance, vessel geometry and blood viscosity, indicates--when applied to coronary artery disease--that an increase in the viscosity of blood, especially of plasma, can in the poststenotic microcirculation be a flow-limiting factor and a critical determinant of oxygen supply to myocardium that is at risk of ischaemia. An increased concentration of fibrinogen, which is the substance that causes the increase in plasma viscosity, has been shown to correlate prospectively with the risk of serious cardiovascular events. Patients with multiple-vessel coronary heart disease and treatment-refractory angina pectoris have clearly increased fibrinogen values. Chronic intermittent urokinase administrations--given with the aim of achieving fibrinogenolysis of the elevated fibrinogen concentration at a dose of 500 000 IU urokinase three times weekly -improves the rheological parameters and achieves an impressive decrease in symptoms. It is thus important in clinical practice to take into account that patients with atherosclerosis and a fibrinogen concentration of more than 300 mg may develop perfusion disorders and worsening of their symptoms.

[Right heart failure and cor pulmonale]. / Rechtsherzinsuffizienz und Cor pulmonale.

Whereas the right ventricle tolerates volume loads without any substantial increase of the pressure in the pulmonary circulation by recruiting capacitance vessels and capillaries, it possesses only small contractile reserves and reacts unadapted with right ventricular dysfunction. Its size and pressure load are relevant factors for prognosis of all forms of pulmonary hypertension, in particular if linked to left-sided heart failure. Differentiation of pulmonary hypertension according to the Venice classification is highly important. Right-sided ventricular heart failure worsens left ventricular hemodynamics due to reduced ejection fraction and in addition due to direct diastolic ventricular interaction in which left ventricular diastolic dysfunction increases even though the left ventricular systolic function is still intact. Right ventricular ejection fraction <40% is an important predictor of prognosis after myocardial infarction or chronic stages of left ventricular heart failure. The most important noninvasive diagnostic method is transthoracic echocardiography with determination of the Tei index and Doppler echocardiographic estimation of pulmonary artery pressure. Chronic obstructive pulmonary disease is the most frequent cause of cor pulmonale. While long-term oxygen therapy in patients with COPD and cor pulmonale and for example the administration of endothelin receptor antagonists in patients with idiopathic pulmonary hypertension is beneficial, the therapeutic use of drugs effective for left-sided heart failure is very limited in patients with right ventricular dysfunction.

[Impaired glucose metabolism in patients with ischaemic heart disease]. / Häufigkeit der Störungen des Glukosestoffwechsels bei Patienten mit koronarer Herzkrankheit.

Patients with ischaemic heart disease commonly have an impaired glucose tolerance. On the 2004 congress of the ESC, the 75-75-rule was announced, indicating that 75% of all diabetics die of cardiovascular complications, and that 75% of all patients with myocardial infarction have diabetes or an (often undiagnosed) impaired glucose tolerance. Data of our "Esslinger Koronarregister" confirm that diabetics and in particular women with diabetes have a higher mortality both after STEMI and NSTEMI. During acute myocardial infarction, a higher blood glucose level strongly correlates with increased mortality. This increased blood glucose level on the one hand is due to preexisting diabetes mellitus or metabolic syndrome, but on the other hand may be a marker of larger myocardial damage with excess katecholamine release. Recent data indicate that intensive glucose control results in a reduction of cardiovascular risk, e. g. the risk of sudden cardiac death. The data presented show that an early intervention in preclinical diabetics aiming at normalization of blood glucose control is necessary in order to reduce cardiovascular mortality.

[Acute femoral artery thrombosis after thrombin injection of a pseudoaneurysm]. / Akute Femoralarterienthrombose nach Thrombininjektionsbehandlung eines Aneurysma spurium. Erfolgreiche Katheterintervention und intraarterielle Fibrinolyse mit r-tPA.

HISTORY: A 64-year old woman was admitted because of unstable angina pectoris. The coronary angiogram revealed two-vessel coronary disease with predominantly subtotal stenosis of the right coronary artery, which was treated by primary coronary stenting. The post-interventional clinical course after closure of the right femoral artery was complicated by the development of a complex pseudoaneurysm: its ultrasound-guided compression was unsuccessful. INVESTIGATIONS: The clinical findings included a marked superficial hematoma in the right groin without any evidence of an abscess, confirmed by color duplex ultrasonography. TREATMENT AND COURSE: 24 hours later a successful percutaneous ultrasound-guided thrombin injection of the pseudoaneurysm was performed without any problems regarding the injection into the aneurysm. A small area within the aneurysm remained perfused and was treated by additional ultrasound-guided compression. A few minutes after the compression complete thrombosis of the right superficial femoral artery occurred with acute critical ischemia of the right leg, probably due to leakage of thrombin into the femoral artery at the sheath puncture. The immediate therapy, including interventional recanalization of the right femoral superficial artery and subsequent fibrinolysis, produced a complete reperfusion of the right femoral superficial and popliteal artery. The fibrinolytic therapy resolved the thrombosis of the pseudoaneurysm. The remainder of the perfused pseudoaneurysm was treated surgically after one week without problems. CONCLUSION: This case demonstrates a so far rarely published complication of the displacement of thrombin from an intervention ally treated pseudoaneurysm into the right superficial femoral artery through a broad compound channel after ultrasound-guided compression.

[Hyperglycemia and cardiovascular events]. / Hyperglykämie und kardiovaskuläre Erkrankungen.

Besides classical, modifiable risk factors (hypercholesterolemia, hypertension, smoking) abnormalities of the glucose metabolism (diabetes mellitus, impaired glucose tolerance) are strong emerging cardiovascular risk factors. Epidemiological data indicate that 8 % of the population and up to 60 % of patients with coronary artery disease have abnormalities of glucose metabolism. The prevalence of these abnormalities will increase as the population ages and the mean body weight increases. An abnormal glucose concentration damages the endothelium in several ways: increased oxidative stress, inflammatory processes and an activation of procoagulant factors all impair endothelial function. A blood glucose normalising therapy is thought to decrease the incidence of cardiovascular events in these patients. In patients with an acute myocardial infarction and diabetes mellitus an early intensive insulin therapy improves the outcome of these patients. In summary, the early detection and treatment of abnormalities of glucose metabolism reduces cardiac events.

[Rheological determinants of end-organ damage]. / Rheologische Determinanten von Endorganschäden.

Various studies demonstrated an interdependence between rheological parameters and advanced stages not only of ischaemic heart disease and peripheral arterial occlusive disease, but also of chronic obstructive lung disease. In ischaemic heart disease, rheological alterations in the poststenotic circulation can result in impairment of the oxygen supply of the myocardium. Rheological therapies aim for a reduction in plasma viscosity and improved microcirculatory flow by means of a reduction of the elevated levels of fibrinogen. As an example, intermittent therapy with urokinase has been established as a treatment of refractory angina pectoris. Treatment with fibrates also can result in an improvement of microcirculation due to reduced hepatic fibrinogen synthesis. Treatment with statins leads to an improvement of microcirculation due to effects on serum lipids. In patients with chronic obstructive lung disease and cor pulmonale who, secondary to chronic hypoxia, develop polycythaemia and disturbances in pulmonary microcirculation, isovolumic haemodilution may result in a reduction of pulmonary arterial pressure with consecutively increased cardiac output and improved exercise capacity.

[Alterations of left ventricular systolic and diastolic function in pregnant women with insulin-dependent diabetes mellitus]. / Veränderungen der linksventrikulären systolischen und diastolischen Funktion bei Patientinnen mit insulinpflichtigem Diabetes mellitus in der Schwangerschaft. Eine prospektive Untersuchung mittels M-mode-Echokardiographie und Doppler-Echokardiographie.

BACKGROUND: During pregnancy, major cardiovascular changes occur. The aim of the following study was to investigate the course of hemodynamic parameters under the increased volume load during pregnancy and delivery in women with insulin-dependent diabetes mellitus. METHODS: We examined 51 pregnant diabetic and 51 healthy pregnant women. The control group consisted of 51 healthy non-pregnant women. In all women, left ventricular mass and fractional shortening were calculated. To evaluate left ventricular diastolic function, mitral inflow and pulmonary venous flow profiles were analyzed. RESULTS: During pregnancy left ventricular mass increased, fractional shortening decreased and diastolic dysfunction was found. While the healthy pregnant women developed signs of disturbed relaxation during pregnancy, pregnant diabetic women showed signs of a disturbed relaxation already at the beginning of gestation. A total of 29 pregnant diabetic women developed a restrictive filling pattern already at the 24th week of gestation. The remaining 22 diabetics had a comparable restrictive filling pattern only during vaginal delivery. In 10 of the 29 pregnant diabetic women dangerous complications were documented, while there were no complications in the healthy pregnant women and the other 22 diabetic pregnant women. CONCLUSIONS: In healthy women pregnancy results in a reversible physiologic left ventricular hypertrophy, a disturbed relaxation pattern and a temporary decrease of left ventricular systolic function. In contrast, pregnant diabetic women demonstrated a delayed relaxation at the beginning of pregnancy and developed a restrictive filling pattern. The early development of a restrictive filling pattern may predispose to complications during delivery in pregnant diabetic women.

[Left ventricular diastolic function parameters after PTCA and stent implantation]. / Linksventrikuläre diastolische Funktionsparameter nach PTCA und Stentimplantation.

UNLABELLED: This study was designed to determine the effect of successful coronary revascularisation on left ventricular diastolic function. METHODS: We consecutively studied the time course of diastolic function by Doppler echocardiography in 100 patients with one-vessel disease before and 48 hours after elective coronary angioplasty. Three abrupt vessel closures occurred within 24 hours after intervention. These three patients were excluded from the study. 58 patients were initially successful treated with coronary angioplasty (residual stenosis < 40%). In 39 patients stents were used to improve an inadequate result after coronary angioplasty. The following parameters of left ventricular diastolic function were evaluated before and 48 hours after coronary intervention: peak early (VE, m/s) and peak late diastolic (VA, m/s) flow velocity, E/A ratio, acceleration time (AT, ms), deceleration time (DT, ms) and isovolumetric relaxation time (IVRT, ms). Ejection fraction (EF; %) was determined and used to characterise systolic left ventricular function. RESULTS: Both patient groups (58 patients with coronary angioplasty and 39 patients with combined coronary angioplasty and stent implantation) showed no relevant differences concerning sex, age, atherosclerotic risk factors, exercise capacity and results of exercise electrocardiography. All patients who underwent stent implantation showed an early improvement of left ventricular diastolic function 48 hours after revascularisation. Surprisingly there was no significant short-term improvement (48 hours) of diastolic function in patients with initially successful angioplasty. CONCLUSION: We suppose that stent implantation faster normalises coronary blood flow than coronary angioplasty.

[Left ventricular diastolic function in pregnancy in patients with arterial hypertension. A prospective study with M-mode echocardiography and Doppler echocardiography]. / Linksventrikuläre diastolische Funktion in der Schwangerschaft bei Patientinnen mit arterieller Hypertonie. Eine prospektive Untersuchung mittels M-mode-Echokardiographie und Doppler-Echokardiographie.

INTRODUCTION: During pregnancy eminent cardiovascular changes occur. The aim of the following study was to investigate the course of hemodynamic parameters under increased volume load during pregnancy in women suffering from mild arterial hypertension. METHODS: Altogether 47 women (age: 25 +/- 4 years) with mild arterial hypertension detected during pregnancy underwent echocardiography at the 9th, 24th and 33rd week of gestation. Furthermore echocardiography was performed postpartum at weeks 1 and 8. The control group comprised 45 healthy pregnant women. In all patients left ventricular muscle mass index and systolic shortening fraction were measured. The following Doppler echocardiographic parameters were ascertained: peak early diastolic and peak late diastolic flow, VE/VA ratio, acceleration time, deceleration time and isovolumetric relaxation time. RESULTS: During pregnancy all patients had an increase of left ventricular muscle mass index and a decrease of fractional shortening. All patients developed a relevant diastolic dysfunction. While the control group developed signs of disturbed relaxation as reduction of peak early diastolic flow (0.89 +/- 0.07 versus 0.82 +/- 0.08 m/s*), VE/VA ratio and an increase of isovolumetric relaxation time (72 +/- 12 versus 123 +/- 7*) at the 33rd week of gestation (* p < 0.01), all pregnant women with mild arterial hypertension developed a diastolic dysfunction with signs of delayed relaxation already at the beginning of gestation. 26 pregnant women with arterial hypertension developed a restrictive diastolic filling pattern at 24 weeks of gestation. The other 21 pregnant women only showed restriction for a short time at the end of gestation. In healthy pregnant women, volume load results in a reversible physiologic left ventricular hypertrophia, a significant alteration of diastolic left ventricular function in terms of a disturbed relaxation pattern and a temporary decrease of systolic function. In comparison hypertensive pregnant women show a delayed relaxation at the beginning of pregnancy and 50% developed early signs of restrictive cardiomyopathy. These changes may predispose to critical complications during pregnancy.

[Prognostic relevance of left ventricular diastolic function parameters in dilated cardiomyopathy]. / Prognostische Relevanz linksventrikulärer diastolischer Funktionsparameter bei dilatativer Kardiomyopathie.

Patients with dilated cardiomyopathy (DCM) generally have an impaired functional capacity and poor long-term out-comes. A mortality of 5-15% per year has been described actually. Aim of this study was to verify the prognostic relevance of invasive and non-invasive parameters of diastolic function in patients with DCM. In 33 patients with DCM, cardiac catheterization was performed and left ventricular systolic (ejection fraction (EF; %)); left ventricular enddiastolic pressure (LVEDP; mmHg) and diastolic function (time constant of relaxation (T, ms); the constant of myocardial stiffness (b) were derived from biplane laevocardiography and simultaneous micromanometric registration of pressure-volume curves. For evaluation of clinical out-come, the follow-up period was defined as beginning on the day after cardiac catheterization and ending on the most recent date or with a cardiac event (death or cardiac transplantation). All patients were reevaluated for NYHA functional class and completed a standard questionnaire. The following hemodynamic parameters were evaluated: invasive parameters of left ventricular diastolic function (constant of relaxation: tau (ms), constant of myocardial stiffness: b)), as well as parameters of systolic function (ejection fraction (EF; %)), left ventricular pressure (LVEDP; mmHg), left ventricular muscle mass index (LVMMI; g/m2), left ventricular enddiastolic volume index (LVEDVI; ml/m2) and non-invasive parameters of morphological data, left ventricular systolic (fractional shortening (FS, %) and ejection fraction) and diastolic parameters with echocardiography. During the follow-up period of 36 months, 11 of 33 patients experienced a major cardiac event (cardiac death n = 8, heart transplantation n = 3). The major cause of death was progressive pump failure. The remaining 22 patients were further classified with respect to changes in functional status. While clinical symptoms could be improved medically in patients with moderate increase of myocardial stiffness, patients with severe increase of myocardial stiffness (b: 76.1 +/- 12.1 vs 17.9 +/- +8.1, p < 0.001) could not be improved and suffered more cardiac events. Doppler echocardiographic measurements in these patients showed a restrictive filling pattern (VE 0.91 +/- 0.21 vs 0.64 +/- 0.18 m/s; p < 0.01; VA 0.52 +/- 0.23 vs 0.57 +/- 0.24 m/s; p < 0.01, deceleration time 129 +/- 17 vs 211 +/- 14 ms; p < 0.01). The medical heart failure therapy was comparable in both groups. In patients with cardiac events, the diastolic left ventricular variables did not significantly differ between patients who underwent heart transplantation and those who died. Patients who demonstrated a sole impairment of relaxation (tau: > 50 ms) suffered no cardiac events. Impaired diastolic function contributes to the clinical picture of congestive heart failure. Parameters of left ventricular diastolic function are powerful and important predictors of major cardiac events in patients with DCM, like heart transplantation and non-sudden death, and may indicate future clinical success of medical treatment. Invasive and non-invasive parameters of diastolic function reveal comparable information for the estimation of prognosis of patients with DCM in order to initiate early therapy.