[Progressive cardiomyopathic lentiginosis]. / Progressive kardiomyopathische Lentiginose.

We report on a 30-year-old man with progressive cardiomyopathic lentiginosis. This syndrome is first described by Polani and Moynahan 1972. They distinguish between the earlier described multiple lentigines syndrome, the LEOPARD syndrome (L-entiginosis, E-lectrocardiographic conduction defects, O-cular hypertelorism, P-ulmonary stenosis, A-bnormalities of genitalia, R-etardation of growth, D-eafness) and the progressive cardiomyopathic syndrome. The progressive cardiomyopathic syndrome is characterised by multiple symmetrical lentigines, hypertrophic obstructive cardiomyopathy and retardation of growth. All characteristics were present in our patient with only mild right ventricular hypertrophic cardiomyopathy.

[High malignant B-cell non-Hodgkin lymphoma of the heart: intravital diagnosis by transesophageal echocardiography controlled biopsy]. / Hochmalignes B-Zell-Non-Hodgkin-Lymphom des Herzens: intravitale Diagnose durch transösophageale Echokardiographie-gesteuerte Biopsie.

We report about a 66-year old patient with Non-Q-wave infarction in coronary artery two-vessel disease. During an echo- and transesophageal multiplane echocardiography preoperatively before a coronary artery bypass surgery a right atrium septal tumor of unknown form was discovered. The computerized axial tomography (CAT) and nuclear magnetic resonance (NMR) scans did not demonstrate extracardiac pathologic findings. A transesophageal echocardiography-guided biopsy of the right atrial septum over the vena femoralis and the right atrium enabled intra vitam a very early diagnosis of high malignant B-cell Non-Hodgkin-lymphoma without the need for thoracotomy. The diagnosis was confirmed histopathologically and immunohistochemically and early treatment with cytostatic therapy could begin. To our knowledge the transesophageal echocardiography-guided biopsy has never been described in the worldwide literature.

Comparison of Doppler echocardiographic methods with heart catheterisation in assessing aortic valve area in 100 patients with aortic stenosis.

OBJECTIVE: To examine the practicability and accuracy of Doppler echocardiographic methods in determining aortic valve area. METHODS: Aortic valve areas determined by three methods using Doppler echocardiography (applying the continuity equation and the modified Gorlin formula using data from Doppler echocardiography and right heart catheterisation) were compared with values obtained by heart catheterisation. PATIENTS: 100 consecutive patients with aortic stenosis aged between 34 and 83 years (mean (SD) 66 (10)). RESULTS: Differences in individual patients' measurements of aortic valve area by the three Doppler techniques varied by up to 0.56 cm2 compared with values obtained by heart catheterisation. On average, values obtained from Doppler echocardiographic methods lay up to 51% below and 78% above those obtained by heart catheterisation. CONCLUSIONS: All three Doppler echocardiographic methods were practicable in routine clinical practice for patients of all ages, but they were of limited accuracy when compared with the aortic valve areas found invasively using the invasive Gorlin equation. However, these deviations may not always be due to inadequacies of the Doppler methods: they could also be caused by limitations in the Gorlin formula. Doppler methods can be repeated if required, they allow examination of the morphology of the valve, and they subject the patient to considerably fewer risks than the invasive procedure. An adequate strategy in determining the severity of aortic valve stenosis would be to calculate the valve area by Doppler echocardiography as well as considering the valvar aortic pressure gradient. The valve area alone should not be relied on exclusively, as has been the increasing practice in the past few years.

[Internal medicine complications of ventriculoatrial shunt]. / Internistische Komplikationen ventrikuloatrialer Shunts.

BACKGROUND: Ventriculo-atrial shunts (VASs) and ventriculo-peritoneal shunts (VPSs) are the symptomatic treatment of choice for hydrocephalus. Bacterial contamination of the atrial part of VASs (usually with Staphylococcus epidermidis) can result in further organ complications, in most instances immune complex mediated glomerulonephritis ("shunt-nephritis") or direct microbial heart valve destruction. PATIENTS AND METHODS: In a retrospective study, we analyzed clinical and laboratory data of 11 patients with VAS associated complications as well as the course of the disease. RESULTS: The following complications were observed: glomerulonephritis (n = 9), glomerulonephritis and aortic valve destruction (n = 1), pulmonary embolism, pulmonary hypertension and tricuspid valve insufficiency (n = 1). Out of the 11 patients, 8 suffered from unexplained fever. All 11 patients had elevated circulating immune complexes. In 3 of 4 patients initially requiring dialysis, renal function improved which allowed to stop hemodialysis. Renal function also improved in 3 of 5 patients who presented with elevated serum creatinine. Unfortunately, the patient with multiple pulmonary embolisms and tricuspid valve insufficiency died of progressive pulmonary hypertension. CONCLUSION: The prognosis for impaired renal function is good only if the VAS infection is diagnosed early and an immediate surgical and antibiotic treatment leads to an eradication of the underlying chronic infection.

[Rupture of the ventricular septum following posterior wall infarction: echo- and Doppler echocardiography diagnosis with pressure determination]. / Ventrikelseptumruptur nach Hinterwandinfarkt: echo- und Doppler-echokardiographische Diagnostik mit Druckbestimmung.

In the postinfarct ventricular septal rupture, an early surgical intervention improves prognosis. A rapid and accurate diagnosis is necessary. A 56-year-old patient was admitted to the hospital with a Q-wave posterior myocardial infarction. Auscultation suggested a ventricular septal defect. Neither M-mode nor the two-dimensional echocardiography with apical four- chamber view could confirm the ventricular septal defect. Only the subcostal view showed a basal ventricular septal defect, which was sometimes covered by the septal leaflet of the tricuspid valve. With the continuous wave Doppler, a left-to-right shunt on the ventricular septum could be registered and the pressure in the right ventricle could be measured. Catheterization confirmed the diagnosis of a basal ventricular septal defect with the associated pressure characteristic. The basal ventricular septal defect was successfully closed with a patch. The high accuracy of the continuous-wave Doppler, which is superior to M-mode and two-dimensional echocardiography, was confirmed by this report. Pressure measurement in the right ventricle by continuous wave Doppler also provides a non-invasive diagnostic method that can be used at the bedside.

[Noninvasive assessment of an aortic bioprosthesis malfunction with Doppler echocardiography]. / Nichtinvasive Beurteilung einer Aorten-Bioprothesen-Dysfunktion mit der Doppler-Echokardiographie.

A 27-year-old patient with aortic stenosis received a Carpentier Edwards bioprosthesis and a reconstruction of the mitral valve, in 1978. With auscultation, M-mode and two-dimensional echocardiography, we diagnosed in 1985 a malfunction of the aortic prosthesis with restenosis, insufficiency and mitral insufficiency. A reliable qualitative and quantitative non-invasive assessment, however, was only possible with Doppler echocardiography. The velocity of blood flow over the aortic valve was measured with the continuous-wave Doppler technique; the aortic valve pressure gradient and the valve area were determined. The pulsed Doppler allowed a semi-quantitative evaluation of the severity of the aortic and mitral insufficiency. The intraoperative and pathological anatomical results confirmed the results from Doppler echocardiography: aortic valve prosthesis malfunction with restenosis and insufficiency and mild haemodynamically insignificant mitral valve insufficiency. The need for cardiac catheterization in patients with valvular heart disease and prosthesis is discussed.

[Papillary fibroelastoma of the aortic valve. Sudden death caused by an uncommon tumor of the heart]. / Papilläres Fibroelastom der Aortenklappe. Plötzlicher Tod bei seltenem Tumor des Herzens.

We describe a very rare tumor of the heart with a pendulous papillary fibroelastoma of the aortic valve, obstructing the ostium of the LCA - resulting in the sudden death of a healthy 40-year-old man. Acute severe ischemia was registered in the ECG a few minutes before death - corresponding especially to the myocardial regions perfused by the LCA. There is no doubt from the post mortem findings that the sudden death of this patient was caused by occlusion of the left coronary ostium by the pendulous papillary fibroelastoma.

[Long-term artificial respiration at home in restrictive ventilation disorders]. / Häusliche Langzeitbeatmung bei restriktiver Ventilationsstörung.

Combined chest wall and lung alterations may lead to severe restrictive respiratory disorder. In advanced cases with hypercapnia, hypoxemia and oxygen intolerance during spontaneous breathing, there are almost insuperable therapeutic difficulties. Three of such patients have been treated, after tracheostomy, at home for 60, 34 and 22 months respectively by intermittent IPPV ventilation. With an improved quality of life all these patients survived up to now. This result seems to be encouraging.

[Clinical significance of aberrant coronary artery--origin from the aorta (author's transl)]. / Klinische Wertigkeit von Koronargefässanomalien Myokardischämie und plötzlicher Tod in jugendlichem Alter.

It is demonstrated the unusual case of a 14-year-old boy, dying suddenly after physical stress with myocardial infarction. Autoptically it was found a coronary artery anomaly of the "malign type" and in addition a significant narrowing of the left main coronary artery. There are presented until now in the literature 17 cases of sudden death in young patients (up to 22 years) in connection with the "malign coronary artery anomaly origin" of both coronary arteries from the right coronary sinus and course of the left coronary artery between the truncus of pulmonary artery and aorta. All 17 cases of sudden death were male. No explanation of the predominance of the male sex is known. Always physical stress preceded the fatal event. Obviously all other abnormalities of coronary artery origin of the aorta and course--except the Bland-White-Garland Syndrome--are of minor clinical importance. In the course of life all coronary vessel anomalies can be accompanied by atherosclerotic processes, followed by clinical symptoms of coronary artery disease. The additional speciality of the demonstrated case is the significant stenosis (75%) in the abnormal course of the main coronary artery. Practical consequence of the presented case is: Even in young patients and children one must be aware of the possibility of a myocardial infarction with coronary artery abnormality if clinical symptoms are present. There may be diagnostic (coronary arteriography) and therapeutic (coronary artery surgery) consequences, if the patient survives the life-threatening event.

[Scintigraphy of the myocardium. Comparative study between thallium and perfusion scintigrams (author's transl)]. / Die Szintigraphie des Myokards. Vergleichende Studie zwischen Thallium- und Perfusionsszintigramm

In a total of 51 patients (26 with coronary heart diseases, 25 with primary myocardial diseases), the myocardial scintigrams after intracoronary particle injection (perfusion scintigraphy) and after intravenous nuclide injection with accumulation in the myocardium (thallium scintigraphy) were compared with each other and set against the coronary angiographic and levocardiographic findings. It was shown that extensive myocardial failures (aneurysms) can be represented by both nuclear medical procedures, but that perfusion scintigraphy is more sensitive and correlates more closely to the levocardiogram findings. Smaller abnormalities of contraction of the left ventricle (hypokinetic and akinetic areas) were always shown in the perfusion scintigram, more seldom in the thallium scintigram. The outstanding advantage of thallium scintigraphy is that it is non-invasive, it can be repeated as often as desired and is easily performed during ergometric stress.