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Biomedical subjects

Rolf Jenni

Publications and source records attributed to Rolf Jenni.

33 records · Page 2Linked to original sources
PubMed2003

Isolated noncompaction of the left ventricular myocardium in the adult is an autosomal dominant disorder in the majority of patients.

Isolated noncompaction of the ventricular myocardium (INVM, MIM 300183 and 604169) is a congenital unclassified cardiomyopathy with numerous prominent trabeculations and deep intertrabecular recesses in a hypertrophied and hypokinetic myocardium. Mutations in the G4.5 gene result in a wide spectrum of severe infantile X-linked cardiomyopathic phenotypes including Barth syndrome with dilated cardiomyopathy and INVM. Molecular genetic analysis of INVM has only been performed in pediatric patients. Although adult INVM patients show similar cardiac abnormalities, the influence of genetic factors, especially of mutations in G4.5, is unknown. We analyzed 25 adult INVM patients for the presence of mutations in the G4.5 gene and performed a pedigree analysis of probands. Mutations were not found in the coding sequence or splice sites of G4.5. Systematic analysis of relatives from seven of nine probands showed multiple affected members consistent with an autosomal dominant pattern of inheritance in the majority of cases. We conclude that INVM in the adult is an autosomal dominant disorder rarely caused by mutations in G4.5 and therefore genetically distinct from infantile X-linked cases.

Cardiomyopathies↗
PubMed2003

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 Fallot's 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.

Adolescent↗
PubMed2003

Mechanical complications after myocardial infarction reliably predicted using C-reactive protein levels and lymphocytopenia.

We assessed the accuracy of C-reactive protein (CRP) levels and lymphocyte counts to predict a mechanical complication (MC) after myocardial infarction (MI). Within 10 years, we identified 36 patients with 39 echocardiographically confirmed MC within 30 days of MI: ventricular septal defect (17 cases), papillary muscle rupture (10 cases), and left ventricular free wall rupture (12 cases). They were compared to 41 controls with an uncomplicated hospital course after MI. Peak CRP levels and minimum relative lymphocyte counts obtained within 96 h of the acute MI (AMI) and before diagnosis of the complication were compared with clinical parameters. Prior to the MC, peak CRP levels were significantly higher (p < 0.001) and relative lymphocyte counts lower (p < 0.001) than in controls while creatine kinase levels did not differ (p = nonsignificant). Using multivariate logistic regression, the following score was identified to have excellent prognostic significance for MC: CRP (mg/l) - 10 x Lyc (%). The area under the receiver-operating characteristic curve was 0.90 +/- 0.05 (p < 0.001). Combined use of CRP levels and relative lymphocyte counts may be helpful in accurately predicting an MC after AMI and should therefore be routinely assessed.

Aged↗
PubMed2003

Intraoperative transesophageal echocardiography in pediatric congenital cardiac surgery: a two-center observational study.

UNLABELLED: Transesophageal echocardiography (TEE) is a monitoring and diagnostic tool for the care of children undergoing cardiac surgery. We analyzed reports from 865 routine TEE examinations performed between January 1994 and March 2002 in patients younger than 17-yr-old who were undergoing surgery for congenital heart disease. Patients' median age was 36 mo (range, 1 day-16 yr). The primary end-point of the study was the incidence of surgical and medical management decisions changed as a result of TEE findings; secondary end-points were diagnostic impact (diagnostic exclusions and new diagnoses) and surgical outcome. Fifty percent of the examinations were performed by anesthesiologists with an advanced level of training in perioperative TEE; all of the examiners had an experience of >or=>500 TEE examinations. Supervision by an anesthesiologist with an advanced level of training was requested in 36.7% of cases; supervision by a cardiologist was requested in 3.8%. Surgical alterations of management were reported in 12.7% of cases and included the need for a repeat bypass run in 7.3%; medical alterations of management were required in 19.4% of cases. We observed a diagnostic impact of TEE in 18.5% of cases and a suboptimal but acceptable surgical outcome in 27.6%; TEE findings predicted postoperative difficulties in 4.0%. Our results confirm the utility of routine TEE to assess repair of congenital heart defects. Furthermore, this service was competently performed by a regular team of cardiac anesthesiologists appropriately trained in TEE. IMPLICATIONS: Transesophageal echocardiography (TEE) is an essential monitoring and diagnostic device for the care of children undergoing cardiac surgery. The surgical and medical impact of TEE is demonstrated in a large series of patients. This service can be performed by appropriately trained cardiac anesthesiologists.

Adolescent↗
PubMed2002

ECG-gated multi-detector row CT for assessment of mitral valve disease: initial experience.

Our objective was to evaluate applicability and image quality of contrast-enhanced, retrospectively ECG-gated multi-detector row CT (MDCT) for visualization of anatomical details of the mitral valve and its apparatus, and to determine the value of MDCT for diagnosing abnormal findings of the mitral valve. Twenty consecutive patients with mitral valve disease underwent MDCT preoperatively. Two readers assessed visibility of the mitral valve annulus, mitral valve leaflets, tendinous cords, and papillary muscles by using a four-point Likert grading scale. Abnormal mitral valve findings [thickening of the mitral valve leaflets, presence of mitral annulus calcification (MAC), and calcification of the valvular leaflets] were compared with preoperative echocardiography and intraoperative findings. Visibility of the mitral valve annulus and mitral valve leaflets was good or excellent in 15 patients (75%) and in 19 patients (95%) for papillary muscles. The MDCT yielded a 95-100% agreement compared with echocardiography and surgery with regard to the assessment of mitral valve leaflet thickening and the presence of calcifications of the mitral valve annulus or mitral valve leaflets. Intermodality agreement between MDCT and echocardiography was excellent with regard to classification of mitral valve leaflet thickness (kappa=1.00) and good regarding classification of MAC thickness (kappa=0.73). Contrast-enhanced, retrospectively ECG-gated MDCT allows good to excellent visualization of anatomical details of the mitral valve and its apparatus, and demonstrates good agreement with echocardiography and surgery in diagnosing mitral valve abnormalities.

Contrast Media↗
PubMed2002

Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction.

OBJECTIVES: We sought to analyze whether a microcirculatory dysfunction might be associated with isolated ventricular noncompaction (IVNC). BACKGROUND: In IVNC, which is a cardiomyopathy thus far "unclassified" by the World Health Organization, heart failure and sudden cardiac death are common findings, but the pathophysiologic mechanisms are unknown. METHODS: In 12 patients with IVNC and 14 control subjects, quantitative evaluation of regional myocardial perfusion (myocardial blood flow [MBF]) and coronary flow reserve (CFR, hyperemic/baseline MBF) was performed using positron emission tomography and (13)N-ammonia. The left ventricular myocardium was divided into nine segments, and the two-dimensional echocardiogram in each patient with IVNC was compared with CFR in each segment. Noncompaction was defined as a two-layered structure with excessive trabeculation. RESULTS: The CFR in control subjects averaged 4.2+/-0.9, providing a cut-off value > or =2.5, but it was 2.1+/-0.8 in patients with IVNC. A perfusion scan defect was found in 14 of 24 segments with noncompaction, although no defect was found in 76 of 84 normal segments (overall agreement 83%, p < 0.0001 by the chi-square test). In 16 of 21 segments with noncompaction, a decreased CFR was found; but a decreased CFR was also found in 36 of 60 segments without noncompaction (p = NS). In 45 of the 57 segments with wall motion abnormalities, CFR was decreased, but it was preserved in 17 of the 24 segments with normal wall motion (agreement 77%, p < 0.0001). CONCLUSIONS: In patients with IVNC, a decreased CFR is not confined to noncompacted segments, but extends to most segments with wall motion abnormalities. Thus, coronary microcirculatory dysfunction is associated with IVNC.

Adult↗
PubMed2002

Results of surgery for irreversible moderate to severe mitral valve regurgitation secondary to myocardial infarction.

OBJECTIVE: Moderate to severe irreversible mitral regurgitation secondary to myocardial infarction is an independent risk factor for reduced long-term survival. Late effects of correction of mitral incompetence concomitant with coronary artery bypass grafting (CABG) are less well known and the choice of mitral valve procedure is still debated. METHODS: From 1988 to 1998, 93 consecutive patients (mean age 63+/-9 years) were treated for moderate to severe irreversible mitral regurgitation secondary to myocardial infarction; 84 were in NYHA functional class III-IV and 19 were in cardiogenic shock. Thirty-seven patients underwent emergency surgery. Perioperative intraaortic balloon pump (IABP) was necessary in 33 patients. Follow-up ranged from 6 months to 12 years (mean 51 months+/-41). RESULTS: Mitral valve was repaired in 30 patients and replaced in 63. Replacement was preferably performed in patients with major displacement of papillary muscle and in patients with acute papillary muscle rupture. CABG (3.4 distal anastomoses) was performed in all patients and was complete in 92%. Early mortality was 15% (14/93). Multivariable analysis identified need for IABP (P=0.005) and COPD (P=0.02) as risk factors for early death. Emergency surgery had only a trend (P=0.15) for increased mortality; age, low ejection fraction, repair vs. replacement had no influence. Actuarial survival rates at 1, 5 and 10 years were 81, 65 and 56%, respectively. Late survival was similar in patients with replacement or repair (P=0.46). At last follow-up, all but one patient were in NYHA functional class I or II. CONCLUSIONS: Combined mitral valve procedure and myocardial revascularization, as complete as possible, for moderate to severe mitral regurgitation secondary to myocardial infarction achieve satisfactory early and late outcome despite the increased operative mortality. Acute papillary muscle rupture, severe restriction of the mitral valve by major displacement of the papillary muscle are better managed by valve replacement.

Adult↗
PubMed2002

Repair of postinfarction dyskinetic LV aneurysm with either linear or patch technique.

OBJECTIVES: Controversy still exists regarding the optimal surgical technique for postinfarction dyskinetic left ventricular aneurysm (LVA) repair. We compared the efficacy of two established techniques, linear vs. patch remodeling, for repair of dyskinetic LVA. PATIENTS AND METHODS: From 1989 to 1998, 95 (16 women, 79 men) consecutive patients were operated on for postinfarction dyskinetic LVA. Thirty-four patients underwent patch remodeling (R) and 61 linear (L) repair. The mean age was 61.1+/-8.5 years. Indications for surgery alone or in combination included angina in 72 patients, dyspnea in 64 and ventricular tachycardia in 41. Thirty-seven patients had a history of congestive heart failure (R 13 (38%), L 24 (39%), NS). The mean ejection fraction (EF) with aneurysm was 0.29+/-0.09 in R vs. 0.35+/-0.10 in L (P<0.04), whereas the mean EF without aneurysm was 0.43+/-0.11 in R vs. 0.46+/-0.08 in L (P=0.3). Seventy-one aneurysms were anterior (R 30 (88%), L 41 (68%), P<0.05). Concomitant coronary artery bypass grafting was performed in 84 patients (R 29 (85%), L 55 (90%), NS). Follow-up ranged from 1 to 12 years (mean 5.6+/-3.4 years, median 6.1 years). RESULTS: Early mortality was 8% (n=8) (R 4, L 4, NS). Survival at 1, 5 and 10 years was 88, 73, and 44%, respectively. It did not differ significantly between R (1 and 5 year survival 85, 66%) and L (90, 76%, P=0.58). Preoperative risk factors for mortality were history of congestive heart failure (1 and 5 year survival 81 and 57% vs. 90 and 78%, respectively, hazard ratio (HR)=1.95, P<0.05), non-anterior localization of the aneurysm (86 and 49% vs. 86 and 77%, HR=2.06, P<0.05), history of thromboembolic events (57 and 19% vs. 89 and 74%, HR=3.27, P<0.05), and left ventricular EF (HR=0.97 per %, P=0.05). At late follow-up the mean functional class was 1.8+/-0.6 in long-term survivors (preoperative 2.9+/-0.9, P<0.001) with no difference between the groups. CONCLUSIONS: The technique of repair of postinfarction dyskinetic LVA should be adapted in each patient to the cavity size and extent of the scarring process into the septum and subvalvular mitral apparatus. Applying these considerations to the choice of the technique of repair, both techniques achieved satisfactory results with respect to perioperative mortality, late functional status and survival.

Aged↗
PubMed2002

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.

Adolescent↗
PubMed1999

Double-Chambered Left Ventricle: Unusual Presentation with an Apical Defect in a Thallium Scan in a Middle-Aged, Asymptomatic Woman.

A left ventricular apical filling defect was found in a whole-body thallium scan performed for routine follow-up for surgically cured thyroid cancer in a 51-year-old woman. She had no cardiovascular symptoms, and her electrocardiogram at rest and during exercise was unremarkable. Transthoracic and transesophageal echocardiography revealed a double-chambered left ventricle. A prominent fibromuscular ridge distal to the papillary muscles was seen dividing the left ventricular cavity into a small apical portion and a large basal portion. There was no significant intraventricular gradient. No treatment was necessary, and the patient has remained asymptomatic in a 4-year follow-up.

Journal Article↗
PubMed1998

Can the Extent of Change of the Left Ventricular Doppler Inflow Pattern During the Valsalva Maneuver Predict an Elevated Left Ventricular End-Diastolic Pressure?

BACKGROUND: The objective of this study was to determine whether analysis of changes in the transmitral filling pattern during the Valsalva maneuver improves the diagnostic accuracy to noninvasively detect an elevated left ventricular end-diastolic pressure (LVEDP). METHODS: We prospectively compared the diagnostic accuracy of the mitral flow velocity indexes at baseline with those obtained during the Valsalva maneuver to detect an elevated LVEDP in 50 patients with coronary artery disease. RESULTS: Moderate correlations were found between LVEDP (mean, 11.8 +/- 6.2 mmHg) and deceleration time (r = 0.49), isovolumetric relaxation time (r = 0.52), and the E/A ratio (r = 0.48). There was a strong correlation (r = 0.73) between LVEDP and the percentile decrease in the E/A ratio during the Valsalva maneuver. Discriminant analysis showed that a decrease in E/A ratio during the Valsalva maneuver by >/=40% detected an elevated LVEDP with a sensitivity of 77% and a specificity of 94%, resulting in a diagnostic accuracy of 88%. In 11 patients, the constellation of pseudonormalization (normal E/A ratio and elevated LVEDP) was present. This could be identified in 73% with a diagnostic accuracy of 87%. CONCLUSIONS: For a comprehensive assessment of diastolic dysfunction by Doppler echocardiography, combined analysis of the E/A ratio at baseline and during the Valsalva maneuver should be performed routinely as an easy method of increasing diagnostic accuracy and uncovering pseudonormalization.

Journal Article↗