Juraj Turina

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.

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.

Emergency hospital admissions and three-year survival of adults with and without cardiovascular surgery for congenital cardiac disease

OBJECTIVE This study determined the quantity and nature of emergencies leading to unscheduled hospital admissions of adults with congenital cardiac disease and their mid-term survival. RESULTS During 1 year, 429 adults with congenital cardiac diseases were admitted 571 times, and 124 admissions (22%) of 95 patients (22%) were emergency admissions. Fifteen of the 95 patients were seen for the first time in 1 of the participating centers. The underlying anomalies were Fallots tetralogy and pulmonary atresia (n = 26/7), univentricular heart after Fontan procedure (n = 25), atrial septal defect (n = 18), Eisenmenger syndrome (n = 12), complete transposition (n = 11), and others (n = 25). Indications for admission were cardiovascular complications (n = 103; 83%) (arrhythmia, cardiac failure, syncope, pacemaker problems, pericardial tamponade, and sudden death), infections (n = 8, 6%) (endocarditis, pacemaker infection, pneumonia, and cerebral abscess), acute chest pain (n = 7; 6%), and acute abdominal pain (n = 4; 3%). All patients required immediate emergency care, and 16 patients (17%) required urgent cardiovascular or abdominal surgery. Six patients died during the hospital stay. During a follow-up of 2.9 years (SD 0.8), 16 (18%) of the discharged patients died, and 2 additional patients underwent heart or heart-lung transplantation. CONCLUSION Adults with congenital cardiac disease often experience serious emergency situations with a high in-hospital and mid-term post-hospital mortality. Care given by physicians with special expertise is important in this specific group of patients.

Persistent Diastolic Dysfunction Late After Valve Replacement in Severe Aortic Regurgitation

Background— Regression of left ventricular (LV) hypertrophy with normalization of diastolic function has been reported in patients with aortic stenosis late after aortic valve replacement (AVR). The purpose of the present study was to evaluate the effect of AVR on LV function and structure in chronic aortic regurgitation early and late after AVR. Methods and Results— Twenty-six patients were included in the present analysis. Eleven patients with severe aortic regurgitation were studied before, early (21 months) and late (89 months) after AVR through the use of LV biplane angiograms, high-fidelity pressure measurements, and LV endomyocardial biopsies. Fifteen healthy subjects were used as controls. LV systolic function was determined from biplane ejection fraction and midwall fractional shortening. LV diastolic function was calculated from the time constant of LV relaxation, peak filling rates, and myocardial stiffness constant. LV structure was assessed from muscle fiber diameter, interstitial fibrosis, and fibrous content. LV muscle mass decreased significantly by 38% early and 55% late after surgery. Ejection fraction was significantly reduced preoperatively and did not change after AVR (P=NS). LV relaxation was significantly prolonged before surgery (89±28 ms) but was normalized late after AVR (42±14 ms). Early and late peak filling rates were increased preoperatively but normalized postoperatively. Diastolic stiffness constant was increased before surgery (22±6 versus 9±3 in control subjects; P=0.0003) and remained elevated early and late after AVR (23±4; P=0.002). Muscle fiber diameter decreased significantly after AVR but remained increased at late follow-up. Interstitial fibrosis was increased preoperatively and increased even further early but decreased late after AVR. Fibrosis was positively linearly correlated to myocardial stiffness and inversely correlated to LV ejection fraction. Conclusions— Patients with aortic regurgitation show normalization of macroscopic LV hypertrophy late after AVR, although fiber hypertrophy persists. These changes in LV myocardial structure late after AVR are accompanied by a change in passive elastic properties with persistent diastolic dysfunction. Clinical Trial Registration— URL: http://www.clinicaltrial.gov. Unique identifier: NCT00976625.

Long-term follow-up, computed tomography, and computational fluid dynamics of the Cabrol procedure

OBJECTIVES The Cabrol procedure is characterized by insertion of an ascending aortic composite graft with reimplantation of the coronary arteries by the interposition of a graft tube. Our purpose is to report the clinical long-term follow-up and computed tomographic findings in patients having undergone the Cabrol procedure and to determine blood flow in the Cabrol graft using computational fluid dynamics. METHODS Clinical follow-up (76.6 +/- 16.6 months) and dual-source computed tomographic angiography data of 7 patients (all men, mean age 54.9 +/- 9.6 years) with 12 Cabrol grafts (left main coronary artery, n = 7; right coronary artery, n = 5) were reviewed. In 2 patients, the right coronary artery was directly reattached to the aortic graft. Computational fluid dynamics were calculated using computed tomographic data of a patient with the Cabrol procedure and compared with those in a Valsalva graft and a healthy aortic root. RESULTS Computed tomography showed Cabrol graft occlusions to 1 of 7 (14%) left main and of 2 of 5 (40%) right coronary arteries. Six grafts to the left main and 3 to the right coronary artery were fully patent, similar to the 2 directly reattached right coronary arteries to the aortic graft. Computational fluid dynamics results show similar blood flow parameters into the coronaries for the healthy aortic root and Valsalva graft. In the Cabrol graft, a spiraling flow pattern with low flow into the right coronary artery was found (right coronary artery = 1 mL/min at both systole and diastole). CONCLUSIONS Our study indicates low flow rates particularly in the right Cabrol graft correlating with a higher incidence of occlusions of the right as compared with the left Cabrol graft at long-term follow-up.

Remodelling after surgical repair of atrial septal defects within the oval fossa

In a retrospective study, we analysed the data from 101 adults with echocardiographic follow-up after surgical repair of defects within the oval fossa at a mean age of 35 +/- 17 years; 56% of the cohort being above the age of 30 years. Mean age at follow-up was 44 +/- 18 years, and length of follow-up was up to 40 years (11 +/- 12 years). At follow-up, atrial fibrillation or flutter was present in one quarter. Dilation of the right atrium, found in 64%, of the left atrium, found in 44%, and of the right ventricle, found in 29%, were also frequent, as well as pulmonary arterial hypertension, which was found in 30%. Diminished right ventricular ejection fraction, in contrast, was very rare, found only in 1%, and abnormal left ventricular ejection fraction was not encountered. By multivariate analysis, predictors for right or left atrial, or right ventricular, dilation were age at follow-up, degree of tricuspid regurgitation, pulmonary hypertension, and/or atrial fibrillation. In a subset of 21 patients in sinus rhythm, we correlated prospectively the diastolic and systolic function of both ventricles with levels of brain natriuretic peptide, comparing values to those of 20 age-matched controls with a mean age of 46 +/- 14 years. Levels of brain natriuretic peptide were significantly higher in patients than in controls (p = 0.006), and correlated significantly with diastolic dysfunction (p = 0.007) and left atrial size (p < 0.0001). In the long-term follow-up after surgical repair of defect within the oval fossa, therefore, complete normalization of heart size and function is rare. Despite preserved systolic function, persistent diastolic dysfunction is common and is associated with elevated levels of brain natriuretic peptide, which may explain the late occurrence of atrial arrhythmias.

Cardiovascular disease and pregnancy

Die Betreuung von Frauen mit kardiovaskularen Erkrankungen erfordert das Verstandnis der Anatomie und Physiologie sowie die Kenntnis der physiologischen Adaptationsmechanismen wahrend der Schwangerschaft und Geburt. Eine Risikostratifizierung vor der Konzeption beinhaltet eine klare Diagnose, die Beratung uber Risikofaktoren in der Schwangerschaft und das Wiederholungsrisiko bei angeborenen Herzfehlern. Die Beurteilung und Betreuung dieser Frauen kann komplex sein und erfordert haufig zum optimalen Management der mutterlichen und kindlichen Risiken ein interdisziplinares Team. Auch wenn komplexe, kardiovaskulare Probleme bestehen, ist bei Frauen ohne wesentliche Einschrankung in den Alltagsaktivitaten und bei adaquater Betreuung das Risiko einer Schwangerschaft vertretbar. Schwere Mitralstenose, schwere Stenose des linksventrikularen Ausflustraktes, schwere Aortenisthmusstenose, Aneurysma im Anastomosenbereich nach Operation einer Aortenisthmusstenose, primare oder sekundare pulmonale Hypertonie (Eisenmen...

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 Werners 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.

Effect of isradipine on left ventricular relaxation and diastolic filling.

The effect of two calcium antagonists on left ventricular (LV) relaxation and diastolic filling was evaluated in 16 randomized patients. Isradipine and nifedipine were administered intravenously in a maximum dose of 60 μg/min for isradipine and 63 μg/min for nifedipine. Heart rate was increased significantly (p < 0.01) by both study agents. LV end-diastolic pressure remained unchanged whereas peak systolic pressure decreased significantly (p < 0.01). The reduction in systolic pressure was significantly greater (p < 0.05) after isradipine (δP of 30 mm Hg) than after nifedipine (δP of 13 mm Hg). The time constant decreased from 65 to 56 ms (p < 0.05) after isradipine and from 62 to 59 ms (NS) after nifedipine. LV filling remained unchanged. It is concluded that both calcium antagonists are associated with a significant reduction in LV afterload accompanied by a reflex increase in heart rate. Isradipine is a more potent vasodilator than nifedipine at the same infusion rate. A beneficial effect on LV relaxation with isradipine, but not nifedipine, may he due to its less pronounced negative inotropic effect or its more potent afterload-reducing action.