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At 6 years of age, a girl with tricuspid atresia underwent a Björk modified Fontan procedure with implantation of a Carpentier Edwards bioprosthesis between the right atrium and the right ventricle. Ten years later she developed increasing edema, ascites and pleural effusions. The work-up showed severe stenosis of the bioprosthesis and protein losing enteropathy with a massive decrease of the albumin level to 14 g/l (normal 40-50 g/l). At 17 years of age, the bioprosthesis was replaced with a direct anastomosis between the cavoatrial junction and the right pulmonary artery. Within one month post-operatively, extensive thrombosis of the superior vena cava, anonymous and subclavian veins occurred. Protein-losing enteropathy persisted with an albumin level of 17 g/l. Parallel to the successful treatment of these thrombi with high molecular heparin and urokinase, protein losing enteropathy and hypoalbuminemia resolved completely as long as the antithrombotic treatment with high molecular heparin was continued. Oral anticoagulation was ineffective. Chronic antithrombotic treatment with high molecular heparin may thus be the treatment of choice in these forms of protein-losing enteropathy associated with venous thrombosis.

Objectives: We sought to describe characteristics and outcome in adults with isolated ventricular noncompaction (IVNC)., Background: Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Knowledge regarding diagnosis, morbidity and prognosis is limited., Methods: Echocardiographic criteria for IVNC include-in the absence of significant heart lesions-segmental thickening of the left ventricular myocardial wall consisting of two layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses. Thirty-four adults (age >16 years, 25 men) fulfilled the diagnostic criteria and were followed prospectively., Results: At diagnosis, mean age was 42 + 17 years, and 12 patients (35%) were in New York Heart Association class III/IV. Left ventricular end-diastolic diameter was 65 + 12 mm and ejection fraction 33 + 13%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in >80% of patients. Follow-up was 44 + 40 months. Major complications were heart failure in 18 patients (53%), thromboembolic events in 8 patients (24%) and ventricular tachycardias in 14 patients (41%). There were 12 deaths: sudden in six, end-stage heart failure in four and other causes in two patients. Four patients underwent heart transplantation. Automated cardioverter/defibrillators were implanted in four patients., Conclusions: Diagnosis of IVNC by echocardiography using strict criteria is feasible. Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias. Risk stratification includes heart failure therapy, oral anticoagulation, heart transplantation and implantation of an automated defibrillator/cardioverter. As IVNC is a distinct entity, its classification as a specific cardiomyopathy seems to be more appropriate.

Stress echocardiography is increasingly accepted as a reliable, noninvasive method for assessment of coronary artery disease. We retrospectively analysed the results of the first 100 consecutive patients (79 males, 62 +/- 10 years), who had both stress echocardiography and coronary angiography within 3 months without intercurrent revascularisation. In 71% of the patients treadmill- was performed and in 29% dobutamine-stress echocardiography. No patient had severe side effects. In the 100 patients, positive predictive accuracy for detection of significant coronary artery disease was 95% and for multivessel disease 80%. There was no significant difference in positive predictive value for detection of significant stenosis in the posterior perfusion territory (left circumflex, right coronary artery), with 79% compared to the anterior perfusion territory (86%, p = ns). Sensitivity for the left circumflex (60%) tended to be lower compared to the right coronary artery (76%) or left anterior descending coronary artery (82%) (p = ns). Despite poorer echocardiographic image quality in dobutamine-stress echocardiography patients, there was no significant difference between treadmill- and dobutamine-stress echocardiography regarding the positive predictive value for detection of coronary artery disease (98 vs 92%) or for recognition of multivessel disease (79 vs 79%) (p = ns). False results of stress echocardiography were rare (7%): false positive results were more common in the presence of wall motion abnormalities at rest, false negative results after an insufficient stress-induced increase in heart rate. Gender or left ventricular hypertrophy had no impact on stress echocardiography results (p = ns). In conclusion, both dobutamine- and treadmill-stress echocardiography are reliable, sensitive methods for non-invasive assessment of coronary artery disease; this is also valid in women and in left ventricular hypertrophy.

We directly compared the transmitral inflow pattern during preload reduction and pulmonary venous flow velocities to determine left ventricular end-diastolic pressure (LVEDP) in 78 patients who underwent left heart catheterization. Transmitral inflow indexes (A-wave duration, ratio of peak flow velocity of early diastole [E] to peak flow velocity of late diastole during atrial contraction [A] [E/A ratio]) at rest and during the Valsalva maneuver (30 mm Hg for 15 seconds) and indexes of pulmonary venous flow (velocity and duration of the atrial reversal) were obtained. Fair correlations existed between LVEDP (mean 15+/-6 mm Hg) and the percentage decrease in the E/A ratio (r = 0.72), increase in duration of A wave during the Valsalva maneuver (r = 0.60), flow velocity of atrial reversal (r = 0.58), and difference of duration of atrial flow reversal and A wave (r = 0.62) (all P<.001). While sensitivity, specificity, and diagnostic accuracy to detect an elevated LVEDP were comparable, technically adequate Doppler recordings were obtained more often for the mitral inflow during the Valsalva maneuver than for the pulmonary venous flow (72 versus 66 patients, P< 0.05).

Background: Cardiac allograft vasculopathy after heart transplantation leads to an accelerated form of atherosclerosis with marked and often diffuse vessel wall changes that limit long-term survival. Previous studies showed contradictory results relating vessel wall changes to endothelial vasodilator response., Methods: A total of 30 cardiac transplant recipients were studied 3, 12, and 24 months after heart transplantation. Coronary angiography was performed at rest, during supine bicycle ergometry, and after 1.6 mg sublingual nitroglycerin. Coronary cross-sectional area (biplane coronary angiography) and coronary artery wall changes (intravascular ultrasound) were assessed and extent of intimal changes correlated to vasodilator responses to nitroglycerine and bicycle ergometry., Results: Intravascular ultrasound showed significant intimal thickening in 43, 64, and 58% of patients at 3, 12, and 24 months. Intimal thickening 3 months after transplantation was related to donor age (r=0.70, P<0.01) but did not predict progression of disease that manifested itself angiographically as a decrease in coronary cross-sectional area at 12 and 24 months (P<0.005) and significant coronary stenosis in 12% of patients after 24 months. Endothelium-independent vasodilatation after nitroglycerin (33+/-15, 44+/-20, and 43+/-24%) was normal. Endothelium-dependent, flow-induced vasodilatation during exercise was decreased (14+/-11, 18+/-14, and 16+/-17%) but did not correlate to intimal changes assessed by ultrasound., Conclusions: The study confirms the high incidence of intimal thickening after heart transplantation as assessed by intravascular ultrasound. Impaired exercise-induced vasodilatation suggests diminished bioavailability of endothelium-derived nitric oxide to physiological stimulation but the lack of relationship between coronary wall changes and this functional impairment suggests intermittent and presumably reversible endothelial injury in graft atherosclerosis.

Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by right and often left ventricular myocardial atrophy and fibrofatty replacement. Heart failure, arrhythmias and sudden death are characteristic complications. We observed a female in whom arrhythmogenic right ventricular cardiomyopathy was diagnosed due to presyncopes and dyspnea on exertion. A left ventricular thrombus was found echocardiographically, which disappeared with oral anticoagulation. Subsequently, however, extensive thrombus formation in the dilated akinetic right ventricle occurred which was resistant to combined treatment with heparin and oral anticoagulation. Thrombophilia screening showed a mutant prothrombin 20210A allele which is an inherited coagulopathy associated with increased plasma levels of prothrombin and increased risks of mainly venous thrombosis. The patient developed endstage biventricular heart failure and underwent heart transplantation within 3 months after thrombus formation in the right ventricle was diagnosed. In the explanted heart, the thrombus in the right ventricle was impressively large and calcified. In patients with unusual thrombus formation in the heart, coagulopathy may be associated and should be excluded., (Copyright 2000 S. Karger AG, Basel)

In the evaluation of coronary artery disease, conventional exercise testing remains the most important modality. Its diagnostic accuracy is reportedly around 70%. Which additional non-invasive examination should be chosen if the result of exercise testing is non-diagnostic? In myocardial ischemia, regional wall motion abnormalities occur before ECG-changes and angina. These regional wall motion abnormalities can be diagnosed and assessed echocardiographically at rest and after a physical or pharmacological exercise. Myocardial ischemia causes worsening of the wall motion if you compare stress with resting images. Stress echocardiography has a diagnostic accuracy of 85% in the assessment of coronary artery disease. When should we order stress echocardiography? If the resting ECG is normal and if the patient takes no digoxin, then conventional exercise testing is the preferred first line test. However, if the resting ECG is abnormal, if there is left ventricular hypertrophy, if there are ST/T-abnormalities, a Wolff-Parkinson White syndrome, a complete left bundle branch blockage or in patients who are unable to exercise adequately, stress echocardiography offers an excellent, accurate, cost-effective alternative with a lot of additional information regarding left ventricular muscle mass, pulmonary artery pressures or valvular heart disease. In very obese patients or patients with chronic obstructive pulmonary disease, echocardiographic image quality is reduced and nuclear perfusion imaging is definitely preferable. In most patients (95%), however, echocardiographic image quality is adequate for stress echocardiography. In patients with arterial hypertension, mitral valve prolapse, or in the need for additional information, stress echocardiography is preferable to nuclear imaging. In patients who are able to exercise, stress echocardiography in combination with bicycle or treadmill exercise should be chosen, only in patients who are unable to exercise adequately, pharmacological stress echocardiography--mainly dobutamine stress echocardiography--should be chosen. Hence, stress echocardiography has increasingly gained wide-spread use as an important and accurate diagnostic means in non-invasive cardiology. It is safe, not very costly, accurate and offers the unique opportunity of on-line assessment of the left ventricle. Indications for stress echocardiography include diagnosis and functional assessment of coronary artery disease, preoperative risk assessment before major vascular surgery, prognostic evaluation after myocardial infarction and viability assessment. In the next years, stress echocardiography will be used even more frequently and will become one of the most often used non-invasive methods in cardiology besides exercise testing.