Long-term follow-up of medical versus surgical therapy for hypertrophic cardiomyopathy: a retrospective study.

In a retrospective analysis 139 patients with hypertrophic cardiomyopathy were followed up for 8.9 years (range 1 to 28 years). Patients were divided into two groups: Group 1 consisted of 60 patients with medical therapy and Group 2 of 79 patients with surgical therapy (septal myectomy). Groups 1 and 2 were subdivided according to the medical treatment. Group 1a received propranolol, 160 mg/day (n = 20); Group 1b verapamil, 360 mg/day (n = 18); and Group 1c, no therapy (n = 22). Group 2a received verapamil, 120 to 360 mg/day, after septal myectomy (n = 17) and Group 2b had no medical therapy after surgery (n = 62). In Group 1, 19 patients died (annual mortality rate 3.6%) and in Group 2, 17 patients died (mortality rate 2.4%, p = NS). Of the patients who died, approximately one half to two thirds in both Groups 1 and 2 died suddenly and the other one half to one third died because of congestive heart failure. The 10 year cumulative survival rate was 67% in Group 1, significantly smaller than that in Group 2 (84%, p less than 0.05). In the subgroups, the 10 year survival rate was 67% in Group 1a, 80% in 1b (p less than 0.05 versus 1a) and 65% in 1c (p less than 0.05 versus 1b). The 10 year survival rate was 100% in Group 2a (p less than 0.05 versus 1a, 1b, 1c) and 78% in Group 2b (p less than 0.05 versus 2a). It is concluded that cumulative survival rate is significantly better in surgically than in medically treated patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiography in the evaluation of systolic murmurs of unknown cause.

PURPOSE: Systolic murmurs are common, and it is important to know whether physical examination can reliably determine their cause. Therefore, we prospectively assessed the diagnostic accuracy of a cardiac examination in patients without previous echocardiography who were referred for evaluation of a systolic murmur. SUBJECTS AND METHODS: In 100 consecutive adults (mean [+/- SD] age of 58 +/- 22 years) who were referred for a systolic murmur of unknown cause, the diagnostic accuracy of the cardiac examination by cardiologists (without provision of clinical history, electrocardiogram, or chest radiograph) was compared with the results of echocardiography. RESULTS: The echocardiographic findings included a normal examination (functional murmur) in 21 patients, aortic stenosis in 29 patients, mitral regurgitation in 30 patients, left or right intraventricular pressure gradient in 11 patients, mitral valve prolapse in 11 patients, ventricular septal defect in 4 patients, hypertrophic obstructive cardiomyopathy in 3 patients, and associated aortic regurgitation in 28 patients. In 28 (35%) of the 79 patients with organic heart disease, more than one abnormality was found; combined aortic and mitral valve disease was the most frequent combination (n = 22). The sensitivity of the cardiac examination was acceptable for detecting ventricular septal defect (100% [4 of 4]), isolated mitral regurgitation (88% [26 of 36]), aortic stenosis (71% [21 of 29]), and a functional murmur (67% [14 of 21]), but not for intraventricular pressure gradients (18% [2 of 11]), aortic regurgitation (21% [6 of 28]), combined aortic and mitral valve disease (55% [6 of 11]), and mitral valve prolapse (55% [12 of 22]). In 6 patients, the degree of aortic stenosis was misjudged on the clinical examination, mainly because of a severely diminished left ventricular ejection fraction. Significant heart disease was missed completely in only 2 patients. CONCLUSION: In adults with a systolic murmur of unknown cause, a functional murmur can usually be distinguished from an organic murmur. However, the ability of the cardiac examination to assess the exact cause of the murmur is limited, especially if more than one lesion is present. Thus, echocardiography should be performed in patients with systolic murmurs of unknown cause who are suspected of having significant heart disease.

Microembolic signal counts increase during hyperbaric exposure in patients with prosthetic heart valves.

BACKGROUND: Patients with prosthetic heart valves have an increased risk of thromboembolic events, and transcranial Doppler sonography reveals microembolic signals. Whereas microembolic signals were initially assumed to be of particulate matter, recent studies suggest that they are partially gaseous in origin. If this is true, alteration of environmental pressure should change microembolic signal counts. We undertook this study to evaluate the influence of hyperbaric exposure on microembolic signal counts in persons with prosthetic heart valves. METHODS AND RESULTS: Microembolic signal counts were monitored by transcranial Doppler sonography of both middle cerebral arteries under normobaria (normobaria 1), 2 subsequent periods of hyperbaria (2.5 and 1.75 bar), and a second period of normobaria (normobaria 2) in 15 patients with prosthetic heart valves. Each monitoring period lasted 30 minutes. Compression and decompression rates were 0.1 bar/min. Microembolic signal counts increased from 20 (12-78) at normobaria 1 to 79 (30-165) at 2.5 bar (P <.01 vs normobaria 1 and 2), decreased to 44 (18-128) at 1.75 bar (P <.01 vs normobaria 1 and 2.5 bar; P <.001 vs normobaria 2), and returned to 20 (8-96) at normobaria 2 (values are medians and 95% confidence intervals). CONCLUSIONS: Our results strongly suggest that gaseous bubbles are underlying material for part of the microembolic signals detected in patients with prosthetic heart valves.

Aortic homograft and mitral valve repair in a patient with Werner's syndrome.

We report the case of a 66-year-old man suffering from Werner's syndrome (adult progeria); he presented with several cardiac disorders, including coronary artery disease, aortic stenosis, and mitral regurgitation, mainly due to calcific deposits in the mitral annulus and the aortic cusps. Treatment consisted of mitral repair, homograft replacement of the aortic valve, and coronary artery bypass grafting. Avoidance of prosthetic material because of chronic infectious skin ulcers constituted the main goal of the operation.

Long-term follow up of patients with endomyocardial fibrosis: effects of surgery.

AIMS: To determine the long-term outcome of patients with endomyocardial fibrosis and to compare echocardiographic and haemodynamic data before and after ventricular endocardial resection. PATIENTS: Seventeen patients (11 women and six men; mean age 35.5 years) diagnosed with endomyocardial fibrosis at the University Hospital in Zurich, Switzerland from 1971 to 1995. Twelve patients (70%) had partial obliteration of both ventricles and in five patients (30%) the fibrotic lesions were limited to the left ventricle. METHODS: Fourteen of the 17 patients had surgical resection: fibrosis was resected from both ventricles in five patients and from the left ventricle only in nine patients. Ten patients had mitral valve replacement and two had tricuspid valve replacement. Left ventricle endocardial resection was done without reconstruction or replacement of the atrioventricular valve in three patients. Preoperative and postoperative echocardiographic data were available for 11 patients and haemodynamic data for six patients. Patients were followed up for 0.4-19 years (mean 8.6). RESULTS: Preoperatively four patients were NYHA functional class IV and 10 were class III; postoperatively one patient was class III, seven class II, and six class I. Preoperatively, echocardiography showed obliteration of the left ventricular apex and inflow tract in all patients, which decreased or disappeared after surgery. Left ventricular end diastolic pressure decreased from 25 mm Hg before surgery to 14 mm Hg after successful resection of the fibrosis. Left ventricular and diastolic volume (normal 93 (17) ml/m2) increased from 65 ml/m2 to 97 ml/m2 (p < 0.05) after surgery. Ejection fraction was normal preoperatively (57%) and decreased slightly (52%) after surgery. One patient died five months after surgery from heart failure. Four surgically treated patients died during the follow up period: one each from systolic dysfunction, recurrence of endomyocardial fibrosis, pneumonia, and food poisoning. Overall survival was 65% at five years and 59% at 10 years; the survival rates of the operated patients was 72% and 68%, respectively. Only one of the medically treated patients survived longer than three years from diagnosis. CONCLUSIONS: Endomyocardial fibrosis is a rare disease in European countries and is found mainly in women. The clinical picture is characterised by severe congestive heart failure but heart size is only moderately increased. Systolic performance is normal or only slightly depressed despite severe restriction to filling, atrioventricular valve regurgitation or both. Partial obliteration of the right and/or left ventricle may be detected by echocardiography. Endocardial resection with atrioventricular valve replacement is the treatment of choice with appreciable postoperative improvement and 10 year survival of approximately 70%.

Left ventricular function and valvular reoperations.

Between 1961 and 1994, 1,120 adult and adolescent patients had reintervention on the aortic, mitral or both valves. Operative mortality was 7%; five, 10, 15 and 20 year survival rates were 81%, 65%, 54% and 52%, respectively. From the sixties to the nineties, the number of procedures (58 to 304), the age of patients (40 to 57 years), incidence of acute endocarditis (0 to 9%), concomitant coronary surgery (0 to 13%) and the number of repeated interventions (5% to 22%) gradually increased. Despite such increasingly difficult conditions the operative mortality remained low and five-year survival acceptable during the last 20 years. Operative mortality was significantly higher in patients over 65 years (11% vs. 5%), in cases with additional coronary surgery (16% vs. 5%) or interventions on the ascending aorta (17.5% vs. 5%). Left ventricular systolic function was quantitatively assessed before reoperation in 372 patients. In patients with stenotic mitral lesion or with aortic valvular lesion and additional surgical interventions reduced ejection fraction (< 50%) had no effect on late outcome. In regurgitant aortic and mitral lesions and interventions on both valves, there was a trend for less successful late outcome after reintervention, but the difference did not reach statistical significance. It is concluded that valvular reoperations can be performed nowadays with only slightly increased operative risk and an acceptable late outcome as compared to primary valvular operations. Hemodynamically significant valvular and/or prosthetic lesion should be corrected again without delay regardless of an impaired left ventricular function.

Reoperative surgery for degenerated aortic bioprostheses: predictors for emergency surgery and reoperative mortality.

OBJECTIVE: The long-term outcome of patients with aortic bioprosthetic valves could be improved by decreasing the reoperative mortality rate. METHODS: Predictors of emergency reoperation and reoperative mortality were identified retrospectively in 172 patients who had the first bioprosthetic aortic valve replacement between 1975 and 1988 (mean age 46+/-13 years) and were subjected to replacement of the degenerated bioprostheses between 1978 and 1997 (mean age 56+/-14 years). Emergency reoperation had to be performed in 31 patients (18%). RESULTS: The operative mortality was 5.2% (9/172), 22.6% for emergency (odds ratio 11.17; 95%-confidence limit 4.33-28.85) and 1.4% for elective replacement of the degenerated aortic bioprosthesis (P<0.0001; OR=20.3). Patients who died at reoperation had higher transvalvular gradients before the primary aortic valve replacement (P=0.007), received smaller bioprostheses at the first operation (P=0.03), had later recurrence of symptoms after the first aortic valve replacement (P=0.04), a higher pre-reoperative New York Heart Association (NYHA) class (P=0.02), and a higher incidence of coronary artery disease (P=0.001) and pulmonary artery hypertension (P=0.009). Endocarditis before the primary aortic valve replacement (P=0.004), postoperative pneumonia at the first operation (P=0.005), pulmonary hypertension (P=0.0004) acquired during the interval, later recurrence of symptoms (P=0.04) after the first operation, a lower ejection fraction at the time of reoperation (P=0.03) and acute onset of bioprosthetic regurgitation (P=0.00002) were predictors for emergency surgery. Higher transvalvular gradients at the primary aortic valve replacement (P=0. 006), coronary artery disease (P=0.003) acquired during the interval, the need for concomitant coronary artery revascularization (P=0. 001), sex (P=0.02) and size (P=0.05) and type of the bioprostheses used (P=0.007) were incremental predictors for reoperative mortality which were independent of emergency surgery. CONCLUSIONS: Elective replacement of failed aortic bioprostheses is safe. Patients undergoing emergency reoperation have a considerably higher mortality. They can be identified by a history of native aortic valve endocarditis, higher transvalvular gradients at primary aortic valve replacement, smaller bioprostheses, and pulmonary hypertension or coronary artery disease acquired during the interval. A failing bioprosthesis must be replaced at its first sign of dysfunction.

Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia.

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.

Double aortic arch: diagnosis missed for 29 years.

Double aortic arch is a rare vascular anomaly which causes tracheal and esophageal compression usually in the first months of life. Typical symptoms in the early childhood should lead to prompt diagnosis and surgical treatment of this malformation. In adults this anomaly is extremely rare. A case of a severely 29-year-old symptomatic woman is presented. Despite characteristic symptoms, the diagnosis was missed during childhood. The importance of different diagnostic procedures and operative therapy is discussed. Preoperative angiography can be replaced by the less invasive magnetic imaging and computed tomography. Surgical operation should also be performed in oligosymptomatic patients to prevent late complications.

[Is echocardiography useful in assessing long term prognosis after valve replacement in patients with left ventricular volume overload?]. / Est-ce que l'échocardiographie est utile pour établir le pronostic à long terme après remplacement valvulaire chez des malades avec surcharge de volume du ventricule gauche?

Echocardiographic recordings in 87 patients with chronic volume overload of the left ventricle (32 pure aortic incompetence, 18 mixed aortic lesions with predominating regurgitation, 17 mitral incompetence and 20 combined aortic and mitral incompetence) who underwent valve replacement between 1975 and 1976 were reassessed to determine the prognostic interest of echocardiography in the long-term postoperative period. The follow-up was a least 2.5 years (average 2.8 years). Patients with coronary artery disease or paravalvular leaks were excluded from the study. The evaluation of the echocardiogrammes comprised the measurement of the end diastolic left ventricular internal dimension (Dd) and the end systolic dimension (Ds) and the calculation of the percentage systolic shortening of the internal dimension (p. 100 Sh) and of the ratio, radius/thickness in end diastole (Dd/2th). A poor surgical result was defined by a postoperative work capacity of less than 60 p. 100 normal or by postoperative death (7 deaths, one of which was in the perioperative period). 21 patients had poor results (group I) and 66 patients, good results (group II). None of the individual echocardiographic parameters differed significantly in groups I and II. In a sub-group of 50 patients, with isolated aortic incompetence, the preoperative echocardiographic data could not distinguish between patients with good and poor postoperative courses. On the other hand, the 4 patients with chronic aortic incompetence who died before operation were characterised by a Dd > 80 mm and Ds > 60 mm, although p. 100 sh was only < 25 p. 100 in one of these 4 cases. In conclusion, M mode echocardiography in patients with chronic volume overload of the left ventricle did not appear to have any value in the prediction of the long-term postoperative result. In patients with chronic aortic incompetence, greatly increased left ventricular internal dimensions indicate a compromised natural prognosis and are an urgent indication for surgical intervention.