[Clinical long-term results after corrective surgery of tetralogy of Fallot]. / Klinische Langzeitergebnisse nach Korrekturoperation einer Fallotschen Tetralogie.

We studied 246 consecutive patients, mean age 11.9 +/- 6.7 years, with primary (n = 155) or secondary (n = 91) complete repair of tetralogy of Fallot (TOF) between 1961 and 1972. Prospective follow-up was complete and ranged from 18.1 to 29.3 (mean: 20.3 +/- 4.2) years. There were 46 operative and 21 late deaths. Cumulative survival was 0.76 +/- 0.03 after 1 year, 0.72 +/- 0.03 (10 years), 0.68 +/- 0.04 (20 years) and 0.63 +/- 0.05 (25 years). After 20 years of follow-up, which was a follow-up time available for all patients, cumulative complication rates were 0.17 +/- 0.03 for documented ventricular tachycardias/fibrillation, 0.16 +/- 0.03 for right-heart failure, 0.13 +/- 0.03 for left-heart failure and 0.11 +/- 0.03 for infective endocarditis. Eighteen of the 21 late deaths were from cardiac causes: sudden (n = 9), infective endocarditis (n = 4), left-heart failure (n = 3), and right-heart failure (n = 2). The hazard for ventricular arrhythmias was inconstant and increasing with time from the initial operation. After 20 years of follow-up, the cumulative incidence of sudden death, documented ventricular tachycardia/fibrillation was 0.81 +/- 0.07. Younger age at surgery resulted in a significantly better long-term prognosis (p = 0.03) with cumulative survival rates after 20 years being 0.90 +/- 0.06 (ages 1-9 years), 0.92 +/- 0.04 (10 to 14 years), 0.83 +/- 0.09 (15 to 19 years) and 0.69 +/- 0.11 for patients being operated beyond age 20. Twenty years following TOF repair 59.2% of the late survivors were in NYHA functional class I and 36.2% in NYHA II.

[Doppler echocardiography analysis of left ventricular inflow patterns in hypertrophic obstructive cardiomyopathy pre- and postoperatively]. / Dopplerechokardiographische Analyse des linksventrikulären Einflussverhaltens bei hypertrophischer obstruktiver Kardiomyopathie prä- und postoperativ.

The aim of the present study was to assess abnormalities of left ventricular filling by Doppler echocardiography in patients with hypertrophic obstructive cardiomyopathy and to investigate whether a myectomy, in addition to normalizing flow, also improves diastolic function. In part A of the study, 40 patients with diagnosed invasive HOCM (29 patients with a gradient at rest, 11 patients with a gradient only after provocation) were compared with 20 normal subjects. The blood flow in the left ventricular inflow tract was examined by means of Doppler echocardiography. At the same time the isovolumic relaxation (IVR) period and the mitral valve opening area (MVOA) were determined using M-mode and the two-dimensional echocardiography, respectively. In part B of the study, 17 patients were examined directly preoperatively and again postoperatively (mean 14 days). Nine patients were then examined at a later date (mean 8.6 months).(ABSTRACT TRUNCATED AT 250 WORDS)

The natural history of aortic valve stenosis.

Despite different aetiologies, acquired aortic stenosis is a self-maintaining, slowly progressive process with good long-term prognosis. In 142 patients with mild stenosis, there was clinical progression within 10 years of the initial diagnosis in only 12% of patients. Twenty-five years after the diagnosis had been established, the severity of aortic stenosis was clinically unchanged in 38%, while 25% of patients had moderate stenosis and 35% had undergone valve replacement. Progression of moderate aortic stenosis was more rapid: the average time interval between the manifestation of moderate aortic stenosis and surgery was 13.4 years. Age at the onset of initial symptoms was related to aetiology: 39 +/- 18 years with rheumatic aortic stenoses, 48 +/- 6 years in patients with bicuspid valves who had no history of rheumatic fever, infective endocarditis or myocarditis, and 66 +/- 12 years in degenerative, calcific stenoses of tricuspid aortic valves. Patients with haemodynamically severe stenosis who had refused the recommended operation (n = 55) had an overall poor prognosis: mean survival averaged 23 +/- 5 months and the five-year probability of survival was 18 +/- 7%. All these patients died within 12 years of observation. Mean survival after the occurrence of angina pectoris was 45 +/- 13 months, after syncope 27 +/- 15 months, and after first occurrence of left heart failure 11 +/- 10 months.

[Prosthesis endocarditis: incidence, diagnosis, therapeutic decisions and prognosis]. / Prothesenendokarditis: Inzidenz, Diagnostik, therapeutische Entscheidungen und Prognose.

In 2711 patients with heart valve replacement performed between 1965 and 1986 the cumulative incidence of prosthetic valve endocarditis (PVE) was 1.19 +/- 0.24% (n = 61). In patients operated on before 1976 (group A; n = 583) early PVE was observed in 3.43%, and in patients operated on between 1976 and 1986 (group B; n = 2128) in only 0.42%. PVE after the 60th postoperative day occurred with a linear incidence of 0.21 events per 100 patient-years (A: 0.11%; B: 0.27%). In 54% of PVE cases the aortic, and in 34% the mitral was involved; in 12% both left-sided prostheses were involved after double valve replacement. In the four weeks before the manifestation of initial symptoms of PVE, bacterial infections and diagnostic or therapeutic interventions had occurred in 74.2%. All interventions had been performed without endocarditis prophylaxis. Diagnosis of PVE was established in 57% by history and clinical examination, in 20% by microbiologic examinations and in 12% by echocardiography. Due to improved diagnostic methods and earlier surgical intervention, mortality declined during the follow-up period from 81% (1965-1970) to 18% (1981-1986). The prognosis was worse in patients who developed therapy-resistant heart failure due to hemodynamically significant prosthetic valve malfunction, or who had sepsis that persisted for more than 72 hours despite antibiotic therapy, major septic embolism or acute renal failure. The retrospective prognosis was more favourable for patients with early valve re-replacement than for patients who had been treated medically alone.

[Indications for coronary angiography in patients with acquired heart valve diseases with reference to risk factors]. / Indikation zur Koronarangiographie bei Patienten mit erworbenen Herzklappenfehlern unter Berücksichtigung von Risikofaktoren.

The aims of the study were to examine the frequency of coronary artery disease (CAD) in patients with acquired valvular heart disease and to investigate the parameters by which significant coronary artery stenosis can be identified without invasive measures in these patients. For this reason 266 consecutive patients with acquired valvular heart disease (aortic, mitral or combined lesions) were examined retrospectively. In 24 patients (9%) a significant (50% or more reduction of the diameter) coronary artery stenosis was found. The prevalence of CAD increased with age: only one patient younger than 50 years, but 23 patients (13%) older than 50 years revealed significant CAD (19% men, 7% women). Increased levels of cholesterol and/or triglycerides were found more frequently in patients with CAD (33% and 29%, respectively) than in those without (6% and 12%, respectively). No differences were found in patients with aortic and mitral valve disease. Patients with typical chest pain revealed CAD in 30% of cases, whereas only 5% of the patients without angina pectoris (or 4% with atypical chest pain) showed a significant coronary artery stenosis. A high percentage (62%) of patients with typical chest pain and mitral valve disease revealed CAD. None of the 77 female patients without typical angina pectoris had significant coronary artery stenosis, whereas 11% of the male patients showed significant CAD even without typical symptoms. In 51 patients without typical angina pectoris and with no risk factors, no CAD was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinical significance of Doppler echocardiographic determination of the severity of aortic valve stenoses]. / Klinische Bedeutung der dopplerechokardiographischen Bestimmung des Schweregrades von Aortenklappenstenosen.

The purpose of the study was to evaluate the clinical significance of Doppler echocardiography in the determination of the severity of aortic stenosis, in particular, to determine to what extent a therapeutic decision in the individual patient is possible solely on the basis of noninvasive investigations. Forty consecutive patients (mean age 53 +/- 13 years, 58% males) with suspected aortic valve disease of purely or mainly stenotic nature, were examined by two-dimensional echocardiography and continuous-wave Doppler echocardiography on average 48 h before cardiac catheterization. An adequate Doppler registration was obtained in 93% (37/40). In 59% the right sternal border proved to be the best window. The gradient determined in the Doppler examination correlated well with the maximum catheter gradient (r = 0.95; SYX +/- 6.2 mm Hg; p less than 0.0005) and with the peak-to-peak catheter gradient (r = 0.93; SYX +/- 6.0 mm Hg; p less than 0.0005). However, in the individual case, clinical assessment on the basis of the Doppler gradient alone proved to be misleading. This was the case in early systolic gradients (aortic incompetence, high cardiac output) or in pronounced left ventricular dysfunction. However, additional consideration of the ratio time to peak velocity (Vmax)/left ventricular ejection time (LVET) (criterion for operation greater than 0.35) and of the echocardiographically determined ejection fraction, enabled us to make the same therapeutic decision in all 37 patients (17 conservative, 20 operative) as that made following the result of cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)

Reoperations for malfunction of heart valve prostheses, especially with endocarditis.

Following the increasing number of patients with heart valve replacement and an extended indication (older age groups, acute infective endocarditis, multivalvular procedures) the indicence of malfunction of valve prostheses is continuously growing. The prognosis of patients with a malfunctioning prosthesis mainly depends on early diagnosis and adequate therapy. In a retrospective study (1970 to 1984) 3,533 implanted heart valve prostheses were followed up and the cases with malfunction (n = 150; 4.2%) were analyzed. During the follow-up period after 1963 mitral valve replacements (MVR) there were 78 cases of malfunction (4.6%), after 1806 aortic valve replacements (AVR) 73 (4.1%), and after 34 tricuspid valve replacements (TVR) 4 malfunctions (11.8%). These malfunctions concerned periprosthetic leakages (n = 65), prosthetic endocarditis (n = 42), prosthetic valve thrombosis (n = 13), mechanical dysfunction including bioprosthetic degeneration (n = 17), valve related hemolysis (n = 3), and unsatisfactory hemodynamics (n = 10). Special attention was turned to the problem of prosthetic endocarditis (1963-1984) found in a total of 71 patients following 3,878 prosthetic valve replacements (1.9%). In 42 reoperated cases (1970-1984) the causing microorganisms were analyzed, demonstrating staphylococci in a leading position. Secondary complications and additional risk factors are discussed. There has been no change concerning the basis and the strategy of management for prosthetic endocarditis for many years: After a short time of conservative management with tested antibiotics, early reoperation and exchange of the prosthesis seems to be the optimal therapy, despite a distinctly high postoperative mortality (17%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Doppler echocardiography normal values in various types of mitral valve prostheses]. / Dopplerechokardiographische Normwerte für verschiedene Mitralprothesentypen.

To determine the value of Doppler echocardiography for the normal clinical use in functional diagnostics of prosthetic mitral valves, and to determine normal values for different types of prosthetic valves, 136 patients with different types of prosthetic mitral valves with the same external diameter (29 mm) were examined. For pressure half-time (t1/2) there were higher values for Starr-Edwards (SE) (n = 18) and Lillehei-Kaster (LK) prostheses (n = 10) (113 +/- 29 and 125 +/- 29 ms) than for Saint Jude-Medical (SJM) (n = 56), Björk-Shiley (BS) (n = 40) and Ionescu-Shiley valves (IS) (n = 12) (78 +/- 16, 82 +/- 17 and 93 +/- 28 ms, p less than 0.001), as well as for the orifice and for the mean diastolic gradient (delta p). The upper permissible limits of t1/2 and delta p were 104 ms and 4.1 mm Hg for SJM, and 111 and 4.8 for BS valves respectively. Day-to-day variability (n = 30) was 5.0% (0.0-14.4%, if t1/2 greater than 100 ms: 0.0-6.0%), the correlation was r = 0.97. The duration of implantation did not have any influence on prosthetic mitral valve function. These normal values and limits form a basis for the evaluation of prosthetic mitral valves in the future.

Hemodynamic long-term results after medical and surgical therapy of hypertrophic cardiomyopathies.

The therapeutic effectiveness of propranolol, verapamil and surgery (transaortal subvalvular myectomy) in hypertrophic cardiomyopathy was assessed in 100 patients with hypertrophic obstructive cardiomyopathy (HOCM) and 12 patients with hypertrophic non-obstructive cardiomyopathy (HNCM) by means of exercise tests with hemodynamic measurements. The effects of propranolol were assessed in 13 HOCM patients, of verapamil in 68 HOCM patients and 12 HNCM patients, and of surgery in 31 HOCM patients after a mean of 3 to 9 months. Of the 68 verapamil-treated patients, 23 were reexamined once more after a mean of 38 months. Ten of the 31 surgically treated patients were reexamined after a mean of 52 months. In the studies performed within the first year of medical treatment or after surgery, verapamil was clinically and hemodynamically superior to propranolol, but not as effective as surgical treatment. Functional limitation according to the NYHA classification improved after propranolol in 31% of the patients, after verapamil in 41%, and after surgery in 94% of the cases. Improvements by more than one NYHA class were observed exclusively after surgical treatment. Maximal exercise capacity was, on average, not changed after propranolol, but increased after verapamil and, more substantially, after surgery. These different responses to treatment could be attributed to hemodynamic changes, especially concerning heart rate, stroke volume, cardiac output, arterio-venous oxygen difference and pulmonary artery pressure. In the case of verapamil, the beneficial hemodynamic effects occurred independently of the site of intraventricular obstruction in HOCM (subvalvular or midventricular), but seemed to be superior in HOCM as compared to HNCM. The late reexaminations, an average of 38 months after beginning verapamil treatment and 52 months after surgery, demonstrated that the initial salutary clinical and hemodynamic effects of verapamil were not maintained during long-term follow-up in the majority of patients, whereas they persisted or even intensified during long-term observation after surgery.

[Results of coronary revascularization in occluded coronary arteries]. / Ergebnisse koronarer Revaskularisation von verschlossenen Koronararterien.

In a total of 82 patients (age 37-71 years) with an occluded left anterior descending artery (LAD) the results of coronary revascularization were evaluated 7 months postoperatively on average. In all patients the indications for revascularization was given by clinical symptoms (angina pectoris) or by prognostic reasons. In patients with multivessel disease. In patients with anterior wall infarction viable myocardium was proven by thallium-scintigram at rest and during exercise. 29 patients were evaluated by coronary angiography postoperatively, in 19 patients the angiograms of the left ventricle could be assessed quantitatively. Total patency rate was 76%, for the LAD 69%, for the circumflex artery 73% and for the right coronary artery 83%. The relatively low patency rate for the LAD was caused by an increased collateral flow to the occluded LAD and therefore by a significantly lower bypass flowrate measured during surgery. Angina pectoris improved markedly, 55% of patients had angina pectoris class III or IV versus 10% postoperatively. These changes were observed in all patients irrespectively of patency rate or occluded grafts to the LAD. Left ventricular volumes and ejection fraction did not change on average after revascularization. Only end-diastolic volume increased significantly in patients with an occluded graft to the LAD. There was a tendency of the end-systolic volume to decrease postoperatively in patients with complete revascularization or at least an open graft to the LAD. The results show a similar clinical improvement in these patients with occluded LAD as shown after "usual" revascularization in other patients. Preoperative coronary angiograms are helpful in judging the postoperative outcome of grafts to the occluded LAD.

[Hemolytic anemia as a result of hemodynamically-insignificant paravalvular dehiscence of St. Jude-Medical aortic valve prosthesis]. / Hämolytische Anämie als Folge einer hämodynamisch unbedeutenden paravalvulären Dehiszenz nach St.-Jude-Medical-Aortenklappenersatz.

The course of a patient with aortic valve replacement is reported in whom increased intravascular hemolysis and auscultatory findings were suspicious of paravalvular dehiscence although other non-invasive examinations did not reveal pathological findings. Heart catheterization showed nearly normal pressures and angiocardiography minimal diastolic transaortic regurgitation. Nevertheless the patient had to be reoperated due to severe hemolytic anemia. Successful closure of the periprosthetic leakage resulted in prompt normalisation of laboratory findings of hemolysis.

[Percutaneous transluminal coronary angioplasty: determination of left ventricular function in physical stress using intravenous digital subtraction angiocardiography]. / Perkutane transluminale Koronarangioplastie: Bestimmung der linksventrikulären Funktion unter körperlicher Belastung mittels intravenöser digitaler Subtraktionsangiokardiographie.

24 patients with coronary artery disease underwent intravenous digital subtraction angiocardiography at rest and during exercise before and after percutaneous transluminal coronary angioplasty (PTCA). Before PTCA mean pulmonary artery pressure increased pathologically in 20 patients (on average from 22 +/- 4 to 40 +/- 9 mm Hg, p less than 0.001). The increase in cardiac index from 4.2 +/- 1.3 to 6.2 +/- 2.01 I X min-1 X m-2 (p less than 0.01) was achieved by an increase in heart rate from 77 +/- 13 to 119 +/- 16 min-1 (p less than 0.001) as stroke volume remained unchanged during exercise (52 +/- 14 ml X m-2, resting value: 53 +/- 13 ml X m-2). Though end-systolic volume increased from 32 +/- 11 to 41 +/- 13 ml X m-2 (p less than 0.001) and ejection fraction fell from 63 +/- 8% to 56 +/- 10% (p less than 0.005), stroke volume remained unchanged due to an enhanced diastolic filling (EDVI: 86 +/- 19 ml X m-2 at rest, 92 +/- 18 ml X m-2 during exercise, p less than 0.001). After angiographically successful PTCA in 21 of 24 patients average mean pulmonary artery pressure during exercise remained pathologically elevated (36 +/- 9 mm Hg, no significant difference from value before PTCA). It normalized in only 6 of 17 patients. On the other hand there was a marked improvement of left ventricular systolic performance. Ejection fraction during exercise (65 +/- 11%) was higher than before PTCA (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

[Course of left-ventricular contraction in left bundle-branch block and its hemodynamic effects]. / Linksventrikulärer Kontraktionsablauf beim Linksschenkelblock und seine hämodynamischen Auswirkungen.

The aim of the study was to analyse the left ventricular contraction pattern in left bundle branch block (LBBB), to create experimentally a comparable pattern in animals and to relate this to haemodynamic measurements. In 20 normal subjects and 16 patients with LBBB without coronary heart disease we performed computer-assisted segmental left ventricular wall motion analysis during various systolic periods using two-dimensional echocardiography. The normal subjects showed on average a uniform shortening of all segments in systole; in patients with LBBB, however, asynchronous contractions of various types and intensities were found. Examination of the contraction pattern of each LBBB patient within the confidence range of the normal subjects showed that in 94% there was an abnormally small shortening of one of the sectors at one time in the second part of systole, and in 74% in the region of the interventricular septum. A "septum index" showed significant differences (p less than 0.0025) between LBBB patients and normal subjects. By right ventricular stimulation of the apex (RVA) and the outflow tract (RVOT) we simulated these contraction patterns in 6 dogs. With RVA stimulation the left ventricular contraction pattern was nearly physiological, while with RVOT stimulation the septum movement was paradoxical. With RVA stimulation cardiac output measured by thermodilution was higher (3.45 vs. 3.11 l/min, p less than 0.002) and the left ventricular end-diastolic pressure lower (7.0 vs. 8.0 mm Hg, p less than 0.002) than on RVOT stimulation; aortic pressure and the first derivative of left ventricular pressure did not differ significantly.(ABSTRACT TRUNCATED AT 250 WORDS)

[Benefits of endocarditis prevention in patients with prosthetic heart valves]. / Nutzen der Endokarditisprophylaxe bei Patienten mit prothetischen Herzklappen.

The beneficial effect of prophylaxis for IE was studied in 229 patients with prosthetic heart valves in whom 287 diagnostic or therapeutic interventions were performed. The prevention used was similar to that recommended by the American Heart Association. Prosthetic valve endocarditis was not observed in any of these patients. This result was compared with that of 304 patients with prosthetic heart valves, in whom without any prevention 390 similar interventions were performed during the same observation period. The incidence of prosthetic valve endocarditis occurring within 14 days after the intervention was 1.5/100 interventions (n = 6). All patients had to be reoperated. One patient died perioperatively. Two more patients developed prosthetic valve endocarditis 8 and 13 weeks, respectively, after the initial intervention. This retrospective study documents the benefit of the prophylaxis for IE used.

[Transprosthetic catheterization of a Björk-Shiley aortic prosthesis with fatal outcome]. / Transprothetische Sondierung einer Björk-Shiley-Aortenprothese mit tödlichem Ausgang.

Retrograde transprosthetic catheterization of a Björk-Shiley aortic prosthesis (type ABP) using a Sones catheter resulted in sticking of the tilting disc. Every attempt to withdraw the catheter failed and the patient died before he could be transferred for emergency reoperation. We advise against transprosthetic catheterization of tilting or bileaflet prostheses, which has been reported to be easily to perform without apparent risk. If left ventricular catheterization is mandatory after aortic valve replacement, the transseptal approach should be used. This is the only procedure which permits accurate evaluation of the functioning of the prosthesis or a concomitant mitral valve disease.

Doppler versus contrast echocardiography for diagnosis of tricuspid regurgitation.

Sixty-eight patients (mean age 49 years) were studied with contrast echocardiography (CE) and Doppler echocardiography (DE) to evaluate both methods for detecting and grading tricuspid regurgitation (TR). In all patients, right ventricular (RV) angiography was performed. The severity of TR was graded on a 4-point scale. Only 68 of 88 patients who underwent RV angiography (77%) could be evaluated, but 65 of 68 patients who underwent CE (96%) and all 68 who underwent DE (100%) could be evaluated. TR was present in 33 patients as seen on RV angiography. CE and DE correctly diagnosed 27 and 30 patients, respectively, corresponding to a sensitivity of 82% for CE and 91% for DE. Specificity was 100% for CE and 86% for DE. CE and DE grading, respectively, of TR vs RV angiographic grading showed no difference in 50 and 47 patients, a 1-level difference in 8 and 13 and a 2-level difference in 7 and 5 cases. (CE-RV angiography, r = 0.84, p less than 0.001; DE-RV angiography, r = 0.82, p less than 0.001). Thus, CE and DE are accurate methods for routine diagnosis of TR, with DE having higher sensitivity and easier grading. Considering the possibility of false-positive findings of our standard RV angiography, sensitivity and specificity of CE and DE could be even higher.