[Long-term effects of sildenafil in a patient with scleroderma-associated pulmonary hypertension and Raynaud's syndrome]. / Langzeiteffekte von Sildenafil bei Sklerodermie-assoziierter pulmonaler Hypertonie und Raynaud-Syndrom.

HISTORY: A 65-year-old woman was admitted because of dyspnea at rest and peripheral edema due to scleroderma-associated pulmonary fibrosis and hypertension, as well as Raynaud's phenomenon. DIAGNOSTIC FEATURES, TREATMENT AND COURSE: She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 |PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome. CONCLUSION: PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.

[Thin-walled apical ventricular aneurysm associated with hypertrophic obstructive cardiomyopathy]. / Dünnwandiges Herzspitzenaneurysma bei hypertropher obstruktiver Kardiomyopathie.

HISTORY AND ADMISSION FINDINGS: A 73-year-old patient presented for routine follow-up examination for pre-diagnosed hypertrophic obstructive cardiomyopathy (HOCM). The patient's history included arterial hypertension and dyspnea on exertion. INTERVENTIONS: Echocardiography revealed a large apical aneurysm, which had vastly increased in size over the past six months. Further evaluation by cardiac magnetic resonance (NMR) imaging confirmed the aneurysm and demonstrated a wall thickness of no more than 2 mm. TREATMENT AND COURSE: Due to the rapid increase in size in addition to the extremely thin wall diameter the risk of spontaneous rupture was considered high and the patient was referred to surgical therapy. Echocardiographic and NMR-findings were confirmed intraoperatively. The aneurysm was resected and the postoperative progress was uneventful. CONCLUSION: Aneurysms of the apical left ventricle can result from an underlying HOCM. In case of rapid increase of the aneurysm, aneurysmectomy should be performed.

[Thrombosis of the deep leg and pelvic veins in congenital agenesis of the vena cava inferior]. / Thrombose der tiefen Bein- und Beckenvenen bei angeborener Agenesie der Vena cava inferior.

HISTORY AND ADMISSION FINDINGS: Severe pain and increasing swelling in the region of the right lower leg occurred after unaccustomed physical activity in a 29-year-old man. Physical examination showed painful swelling and livid discoloration of the right lower leg. INVESTIGATIONS: Laboratory tests were consistent with an acute inflammation. Colour Doppler duplex sonography of the leg and pelvic veins bilaterally revealed complete thrombosis of the deep leg and pelvic veins on the right. Spiral computed tomography of the abdomen confirmed deep pelvic vein thrombosis and also demonstrated complete agenesis of the inferior vena cava (IVC). TREATMENT AND COURSE: The patient was fully heparinized and compression bandage applied to the right leg, which was kept elevated, Ibuprofen, 3 x 400 mg daily, was given for pain relief. Anticoagulation treatment with phenprocoumon (Marcumar) was initiated. The patient was discharged much improved after ten days. Duplex sonography after 3 months demonstrated partial recanalization of the right pelvic and deep leg veins. CONCLUSION: Congenital malformations of the IVC are rare. Phlebothrombosis often results in affected patients. Treatment or prevention of thrombosis of the deep veins by anticoagulation is indicated. Additional risk factors for thrombosis--smoking, hormonal contraceptives, immobilization and unusual physical activity--should be strictly avoided.

Diagnosis of renal artery stenosis and renovascular hypertension.

Renovascular hypertension resulting from renal artery stenosis is a potentially curable form of secondary hypertension. Although uncommon in the general hypertensive population, the prevalence of renovascular hypertension rises in selected groups of hypertensive patients. Because of the possibility of cure, screening measures for renal artery stenosis are warranted in hypertensive patients with clinical features suggestive of renovascular disease. For a long time, angiography has been considered the 'gold standard' in screening for arterial stenosis. However, it is invasive and associated with inherent morbidity. Thus, less invasive screening measures with high diagnostic sensitivity and specificity are currently being sought. Magnetic resonance angiography and captopril renal scintigraphy are safer alternatives to angiography, but are expensive and not widely available. Duplex ultrasound is more widely available and provides both anatomic and functional assessment of renal artery stenosis; however, sensitivity and specificity vary considerably among different laboratories. Echo-enhanced duplex ultrasound using the galactose-based agent Levovist(R) produces higher quality images of the renal artery than conventional color Doppler, while significantly reducing mean examination time and improving diagnostic confidence. In addition, Levovist does not compromise the safety of duplex ultrasound. Other advances in Doppler imaging techniques that may improve ultrasound sensitivity and specificity in detection of renal artery stenosis are power Doppler, echo-enhanced harmonic spectral Doppler imaging, and echo-enhanced harmonic power Doppler imaging.

[Coarctation of the abdominal aorta]. / Koarktation der Aorta abdominalis.

HISTORY AND FINDINGS: When a 27-year-old woman went to her general practitioner with symptoms of acute gastroenteritis he noted a paraumbilical murmur on auscultation. There were no symptoms of intermittent claudication or abdominal angina. All peripheral pulses were easily palpable and bilaterally equal, and there were no vascular murmurs. Physical examination and blood pressure (140/70 mm Hg bilaterally) were normal. INVESTIGATIONS: Biochemical tests gave no indication of inflammatory disease. Oscillography showed a brief decrease in amplitude after muscular exertion, predominantly of the thigh. Posterior tibial systolic pressure was 20 mm Hg lower than radial pressure on the right, 25 mm Hg on the left. Colour Doppler sonography demonstrated elongated and looping coarctation of the abdominal aorta. TREATMENT AND COURSE: As the patient had no symptoms only regular follow-up was indicated. There have been no symptoms for 27 months and no progression of the coarctation. CONCLUSION: In young patients with the described symptoms abdominal coarctation should be included in the differential diagnosis.

[Determining the degree of stenosis of peripheral arteries. Hemodynamic and ultrasound principles]. / Stenosegradbestimmung an peripheren Arterien. Hämodynamische und sonographische Grundlagen.

With colour duplex sonography, the identification and quantification of arterial stenoses in peripheral arteries had become feasible in reasonable time. However, the haemodynamic considerations are more complicated in peripheral arteries than in carotid arteries. This is attributable to the much longer vessel length, the influences of peripheral resistance, the frequency of multiple stenoses and combinations of occlusion and stenosis with multiple collateral vessels. Different qualitative and quantitative approaches are used: b-mode sonography, spectral analysis, velocity measurements, continuity equation and estimation of systolic pressure gradients (Bernoulli equation). Although standardized criteria in the grading of stenoses are not commonly used, the peak velocity ratio (PVR as a ratio of intra-and prestenotic velocity) seems to be mathematically founded, usefully versatile and reliable.

Accuracy of duplex scanning in the prediction of pressure gradients across peripheral artery stenoses.

The present study investigates the validity and accuracy of the modified Bernoulli equation in the Doppler-derived evaluation of pressure drops across peripheral artery stenoses. Conventional duplex-derived (x) and catheter-measured (y) mean and peak pressure gradients across iliac artery stenoses were determined in 35 iliac arteries of 28 patients. The correlations between both methods were r = 0.84 (n = 35; SEE = 4; y = 0.89x + 2) for the mean and r = 0.86 (n = 27; SEE = 10; y = 0.76x + 11) for the peak pressure gradients. In a second study, color-coded duplex-derived (x) and catheter-measured (y) pressure gradients across iliac artery stenoses were determined in 28 further patients. The correlations were 0.86 (n = 28; SEE = 5; y = 0.98x + 0.5) for the mean and r = 0.83 (n = 18; SEE = 15; y = 0.83x + 4) for the peak pressure gradients. These data show that the modified Bernoulli equation can be applied to predict pressure gradients across iliac artery stenoses in patients with peripheral artery diseases.

[Color-coded duplex ultrasonography in the diagnosis of renal artery stenosis]. / Farbkodierte Duplexsonographie in der Diagnostik von Nierenarterienstenosen.

To diagnose possible renal artery stenosis in 120 patients (36 women, 84 men; mean age 57.3 [21-84] years) colour duplex sonography (CDS) of the renal arterial tree was performed before 53 intraarterial angiographies and 67 central venous digital subtraction angiographies. The criterion of stenosis was a maximal flow velocity of > 180 cm/s in the colour duplex sonogram and a diameter reduction of > 50% in the angiogram. CDS demonstrated the renal artery bed in 209 of 247 renal arteries (85%). 84 of the 209 had regional maximal velocities of > 180 cm/s. The sensitivity and specificity of CDS when comparing it with both angiographic methods (in 185 cases that could be evaluated, in 74 of them with abnormal results) were 91 and 88.2%, respectively. Comparing CDS results with those by intraarterial angiography alone (88 cases, with 51 abnormal findings), sensitivity and specificity were 92.1 and 91.8%, respectively. These results demonstrate that CDS can reliably demonstrate flow obstructions in the renal arterial tree and can thus be recommended for the diagnosis of renovascular hypertension.

[Determination of the length of the occlusion in extremity arteries--color duplex ultrasound versus angiography]. / Bestimmung der Verschlusslänge in Extremitätenarterien--Farbduplexsonographie versus Angiographie.

In the management of patients with peripheral arterial occlusive disease the length and location of an arterial occlusion has an impact on the choice of the method of arterial reconstruction, i.e. percutaneous transluminal angioplasty or vascular surgery. The aim of this study was to determine the accuracy of colour-coded Doppler sonography (CDS) compared to conventional contrast arteriography in detecting the length and localisation of an occlusion in peripheral arteries. 100 legs of 94 patients (27 women, 44 to 82 years of age [mean 60.9 years] and 67 men, 21 to 78 years of age [mean 61.3 years]) with clinically suspected artery occlusion were examined prospectively with CDS before angiography and angioplasty. The exact localisation was correctly diagnosed by CDS in 95% with a high correlation (r = 0.95) of occlusion length between both methods. The sensitivity in detecting occlusions was 98% (positive predictive value 98%). It is concluded that colour-coded Doppler sonography can diagnose the length and location of an occlusion in peripheral arteries accurately and therefore can be used as a noninvasive method to select patients for further therapy management.

[Varicosis of the great saphenous vein as a main symptom of iatrogenic arteriovenous fistula]. / Vena-saphena-magna-Varikose als Leitsymptom einer iatrogenen arteriovenösen Fistel.

A 22-year-old man with movement-dependent knee-joint pain was operated on for patellar chondroplasia and meniscal dysplasia after two previous arthroscopic removal of free joint bodies had failed to bring relief. A few months after the operation the same leg had become swollen with signs of venous congestion, long saphenous vein varicosity and traumatic pretibial crural ulcer. The long saphenous vein was partially excised. 18 months after the arthrotomy there was clinical and colour-Doppler evidence of an arteriovenous fistula with false aneurysm in the region of the popliteal artery as cause of the venous congestion. The ulcer healed completely after ligation of the fistula and excision of the false aneurysm. --Arterio-venous fistula should be included in the differential diagnosis as a possible cause of postoperative swelling of a limb.

[Determination of the length and site of the occlusion of extremity arteries--color duplex sonography versus angiography]. / Bestimmung der Verschlusslänge und -lokalisation in Extremitätenarterien--Farbduplexsonographie versus Angiographie.

The aim of the study was to determine the accuracy of colour--coded Doppler sonography (CDS) compared to angiography in detecting the localisation and length of an occlusion in peripheral arteries. 118 legs of 107 patients with clinical suspected occlusion were examined prospectively with CDS before angiography. In 110 cases both methods identify identical localisations. The sensitivity in detecting occlusions was 97%, the exact localisation was diagnosed by CDS in 95% with a high correlation (r = 0, 96) of length measurements.