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In 1866, Wilhelm Ebstein published a scholarly description of a tricuspid valve anomaly with dilation of the right atrium and patent foramen ovale that bears his name. However, his original report was almost overlooked. Despite a wide range of publications on the history of cardiac pathology and cardiac surgery, the international literature provides only scarce information regarding the personality of Wilhelm Ebstein and his original description of the anomaly that bears his name. In this article, we present biographical data of Wilhelm Ebstein and discuss how his original description of autopsy findings correlates with our current knowledge of this congenital disorder. It is the excellent correlation of Ebstein's pathologic findings with clinical notes of his colleague and Ebstein's hypotheses of the pathophysiology that made his publication a landmark in the description of a new entity. In addition, Ebstein's report provided a strong basis for the development of repair techniques for this rare anomaly 100 years later.

Objective: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed., Methods: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged., Results: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy., Conclusions: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.

In two patients (75- and 88-years-old), with the aid of cardiopulmonary bypass and using a transaortic approach, a rare calcified aneurysm at the origin of an aberrant right subclavian artery was closed with a prosthetic patch. The aneurysm was partially resected and the remainder closed over the patch. This technique obviates the need for hazardous clamping of the aorta around the base of the calcified aneurysm and allows secure closure of the origin of the aneurysm.

Cardiac involvement of malignant melanoma is generally part of a widespread tumor dissemination, which is mostly multifocal. Hence the disease is usually not amenable to surgical intervention. We report successful resection of a large intracavitary melanoma to the right atrium, the primary origin of which was unknown. The right atrium was reconstructed with an autologous pericardial patch. At 12-month follow-up the patient remains asymptomatic.

Objective: Obstruction of the atriopulmonary anastomosis or the lateral atrial tunnel cavopulmonary anastomosis in the Fontan circulation for univentricular physiology may result in dilation of the right atrium or the right atrial free wall that is incorporated in the lateral atrial tunnel, respectively. Secondary detrimental sequelae may consist of supraventricular dysrhythmias, thromboembolism, right pulmonary vein compression, pleural effusions, and protein-losing enteropathy. Conversion of these Fontan connections to an extracardiac conduit cavopulmonary anastomosis may improve central systemic venous flow patterns and provide clinical improvement in these patients., Methods: Eighteen patients (7-40 years old) with atriopulmonary anastomosis (n = 15) or obstructed lateral atrial tunnel cavopulmonary anastomosis (n = 3) presented at 5.7 +/- 3.9 years with moderate to severe right atrial dilation (n = 15), Fontan pathway obstruction (n = 12), atrial dysrhythmia (n = 13), pleural effusion (n = 8), right atrial thrombus (n = 3), right pulmonary vein compression (n = 3), and protein-losing enteropathy (n = 3). All patients underwent conversion to an extracardiac conduit cavopulmonary anastomosis., Results: Two of the three patients with protein-losing enteropathy died (2/18; 11%) on the 30th and 52nd postoperative days. At a mean follow-up of 19 months, the remaining 16 patients had marked (n = 11) or moderate (n = 5) clinical improvement. The SaO2 improved from 90.7 +/- 5.3% to 96.0 +/- 4.1%. None of the patients had obstruction in the systemic venous pathway. In the 13 surviving patients with previous atriopulmonary anastomosis there was a drastic reduction in right atrial size. Four of 13 patients with atrial dysrhythmias converted to sinus rhythm. The right pulmonary vein compression as present in three patients resolved after conversion. Pleural effusions disappeared in four patients., Conclusions: Conversion to an extracardiac cavopulmonary connection may lead to clinical improvement in patients with atriopulmonary or lateral atrial tunnel Fontan connection associated with specific target conditions such as obstruction, pulmonary vein compression, right atrial enlargement, atrial dysrhythmia, or atrial thrombus. The conversion operation should not be unduly delayed to prevent irreversible deterioration of clinical status with chronic rhythm disturbances or protein-losing enteropathy. The benefit of the conversion operation is questionable in patients with poor clinical condition and protein-losing enteropathy.

Aortico-right ventricular tunnel was successfully corrected in a 15-month-old child. Both the aortic and right ventricular openings were closed with pledgeted sutures. The coronary artery anatomy was normal. At 12-month follow-up the patient is in excellent clinical condition. Before surgical intervention for aortico-right ventricular tunnel is undertaken, every effort should be made to diagnose the coronary artery anatomy, because failure to do so in the case of aberrant origin of a coronary artery may prevent successful surgical correction.

Background: Ebstein's anomaly, due to failure of delamination of one or more leaflets of the tricuspid valve (TV), is associated with varying degrees of tricuspid regurgitation (TR) and dysplasia of the right ventricle (RV). Although refinement of tricuspid valvuloplasty and plication techniques have opened the way to a satisfactory outlook for the majority of older children and adults, Ebstein's anomaly presenting at neonatal age, secondary to ineffective forward flow into the pulmonary and systemic circulation, has a reported mortality rate of as high as 75%. In order to improve the dismal outcome in neonatal Ebstein's anomaly, we have strived for early univentricular palliation., Patients and Methods: Univentricular repair was performed in five neonates (median age 5 days; range 2-14 days) with Ebstein's anomaly, ductal dependent pulmonary blood flow, severe TR, absence of forward flow across the pulmonary valve, and small left ventricular (LV) area due to right-to-left bowing of the ventricular septum and ineffective LV loading (median indexed LV area 10.5 cm2/m2). In addition, two neonates had moderate pulmonary regurgitation (PR), one with additional pulmonary stenosis. In all patients, the indexed area of the combined right atrium and atrialized RV was greater than that of the combined functional RV, left atrium, and left ventricle (median 22.0 and 20.8 cm2/m2, respectively). The median preoperative systemic oxygen tension was 35 mmHg and the median pH 7.28. Repair consisted of TV closure with a pericardial patch (with the coronary sinus draining into the RV) (n = 3) or, in the presence of PR, resection of the dysplastic TV and division and oversewing of the main pulmonary artery (n = 2), as well as excision of the atrial septum, resection of redundant right atrial wall, and construction of an aortopulmonary shunt (n = 5)., Results: The median indexed LV area increased from 10.5 to 18.8 cm2/m2 as a result of more effective loading of the left ventricle. There was no intraoperative or late mortality. The patients were extubated at a median of 7 days postoperatively. At discharge, the median systemic oxygen tension was 46 mmHg. In all five patients, at 6, 7, 10, 12 and 16 weeks of age, a bidirectional cavopulmonary anastomosis has been constructed., Conclusions: In neonates with Ebstein's anomaly and ductal dependent pulmonary blood flow, rational palliation consists of the surgical creation of tricuspid atresia or, in the additional presence of PR or pulmonary stenosis, the creation of pulmonary atresia. These procedures may result in effective LV decompression and more effective volume loading of the left ventricle with increase of systemic output and improved clinical outcome.

A young adult patient, in whom 20 years previously a secundum atrial septal defect had been closed surgically, presented with symptoms of a Budd-Chiari syndrome, cirrhosis of the liver, ascites, and edema of the lower legs. The inferior vena cava-right atrial junction was obstructed by a calcified Teflon patch and shrinkage of the surrounding tissue. Augmentation of the inferior vena cava-right atrial junction with a Gore-Tex patch resulted in unobstructed inflow into the right atrium.

A modified repair technique in anomalous connection of the left coronary artery to the main pulmonary artery is reported in which transfer of the origin of the vessel into the aortic root is facilitated by augmentation with native aortic and pulmonary tissue flaps. This modification reduces tension on the anastomosis and may enhance the likelihood of successful transfer of the left coronary artery into the aortic root.

A young symptomatic patient is reported with anomalous origin of the left main coronary artery from the right sinus of Valsalva and two stenoses in the intramurally coursing segment of the vessel, namely at the site of the intercoronary commissure and at its exit site in the left sinus of Valsalva. Unroofing of the intramural segment and a modified angioplasty technique resulted in a widely patent neo-coronary ostium.

A young child with [S, L, L] segmental anatomy, double-inlet left ventricle, transposition of the great arteries, rudimentary right ventricle, and mildly restrictive bulboventricular foramen is reported, in whom intraoperative temporary snaring of the modified Blalock-Taussig shunt resulted in instantaneous and dramatic volume contraction of the left ventricle, decrease in bulboventricular foramen size, and increase of the gradient across the latter from 10 mm Hg preoperatively to 50 mm Hg. A modified Damus-Stansel-Kaye procedure using autogenous aortic tissue resulted in unobstructed aortic outflow; in addition, a bidirectional cavopulmonary shunt was performed. The importance of early relief of actual or potential aortic outflow obstruction in hearts with restrictive bulboventricular foramen is emphasized.

A modification of the Lecompte operation is reported in which the pulmonary valve is preserved. This technique is applicable in certain anomalies of ventriculoarterial connection with ventricular septal defect and subpulmonary stenosis. The main advantage of this modification, besides preservation of growth potential of the translocated pulmonary artery, consists of preservation of pulmonary valve function, which may have a beneficial effect on the long-term preservation of right ventricular compliance.

The effects of wrapping the internal mammary artery in Gore-Tex surgical membrane on the formation of adhesions were investigated. In seven goats both internal mammary arteries were dissected free from the thoracic wall. In six goats the internal mammary arteries were wrapped in Gore-Tex surgical membrane. In the remaining animal both arteries were removed and used as controls. After 6 months the goats were reoperated and it was found that the wrapped segments of all arteries were completely free of adhesions, whereas the non-wrapped segments showed severe adhesion formation. Histologic examination could not detect differences between the wrapped arteries and the controls. One of the 12 wrapped arteries was occluded by a longer existing thrombus, which is attributed to the surgical technique and the fact that postoperatively no anticoagulation was given. The beneficial effect of the use of Gore-Tex surgical membrane on the prevention of adhesion formation is excellent and this implies that it could be used in coronary artery bypass operations, thus reducing the chance of damage to the bypass in case of reoperation.

This study deals with a replication of a previous research project on the correlation between medical and behavioural skills of general practitioners. The PREVARA observation schedule was used to assess these skills of 75 general practitioners. Fifteen surgery contacts were tape-recorded for each GP and were rated by trained observers. The study shows that fundamental dimension underlies the surgery behaviour of the GP: the various skills appear to be equally developed. More specifically, a positive correlation was found between performing obligatory physical examination and paying attention to psychosocial aspects. The results are discussed in the context of the evaluation of the vocational training and medical audit of GPs.

A case is reported of an intramural oesophageal cyst. Acute dyspnoea, due to compression of the trachea, was an indication for exploratory sternotomy.

A patient is presented with recurrent angina due to a coronary-subclavian steal syndrome 3 years after left internal mammary to left anterior descending coronary artery bypass grafting. Myocardial ischaemia could easily be provoked by selective exercise of the left upper limb. Coronary angiography showed reversal of flow in the left internal mammary artery. Suggestions are given for prevention of the coronary-subclavian steal syndrome by identification of patients who are at risk of developing subclavian artery occlusive disease. Performance of coronary and brachiocephalic angiography is indicated in recurrence of angina in patients with internal mammary artery bypass grafts. Doppler spectral analysis may be a valuable technique for detection of a haemodynamically significant stenosis of the left subclavian artery. Carotid-subclavian bypass grafting is the procedure of choice for management of the coronary-subclavian steal syndrome.