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Biomedical subjects

E Oechslin

Publications and source records attributed to E Oechslin.

At least 37 records · Page 2Linked to original sources

Ebstein's anomaly in adults: Doppler-echocardiographic evaluation.

A systematic approach and segmental analysis are required for comprehensive assessment including both morphologic and functional abnormalities associated with Ebstein's anomaly. The essence of the disease is an apical displacement of both the septal and the posterior tricuspid leaflets, exceeding 20 mm or 8 mm/m2 in adults. As a consequence, the right heart consists of three components including the true right atrium, the functional right ventricle (RV) and an intervening zone that is anatomically ventricular but functionally right atrial (atrialized RV). The thin wall of the atrialized RV may result in an aneurysm between the anatomic tricuspid annulus and the apically displaced posterior leaflet. The annular attachment of the anterior leaflet is normal, which may be dysplastic and adherent to the RV wall. Tricuspid regurgitation is usually moderate to severe. Size, shape and function of the functional RV must be described. The paradoxical motion of the interventricular septum causes alterations in left ventricular geometry and function. An interatrial communication is frequently present. Echocardiography is the method of choice to diagnose Ebstein's anomaly on its own or in association with other heart defects.

Adult↗

40 years after the first atrial switch procedure in patients with transposition of the great arteries: long-term results in Toronto and Zurich.

The atrial switch procedure dramatically improved the prognosis of children with complete transposition of the great arteries (TGA). Overall actuarial survival was approximately 75% after 25 years and was better in patients with simple TGA than in those with complex TGA. Mortality by any cause (16%) and cardiovascular mortality (12% and 13%) were comparable in both centers. Progressive congestive heart failure and sudden death were the principal modes of death. Most of the survivors denied any symptoms or had mild limitations in their daily activities. However, long-term problems in this growing population of adults are challenging and include late arrhythmias (up to two thirds of the patients), systemic ventricular (SV) failure, systemic atrioventricular valve regurgitation and reoperations, such as baffle reconstruction, being the most frequent. Objective assessment of SV function obtained by echocardiography is difficult. It may include fractional area change and tricuspid annular motion. Survivors after an atrial switch procedure are unique and have a good quality of life. However, the definitive and true history of the RV supporting the systemic circulation is not as yet known.

Activities of Daily Living↗

Extensive thrombus formation in the right ventricle due to a rare combination of arrhythmogenic right ventricular cardiomyopathy and heterozygous prothrombin gene mutation G20210 A.

Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by right and often left ventricular myocardial atrophy and fibrofatty replacement. Heart failure, arrhythmias and sudden death are characteristic complications. We observed a female in whom arrhythmogenic right ventricular cardiomyopathy was diagnosed due to presyncopes and dyspnea on exertion. A left ventricular thrombus was found echocardiographically, which disappeared with oral anticoagulation. Subsequently, however, extensive thrombus formation in the dilated akinetic right ventricle occurred which was resistant to combined treatment with heparin and oral anticoagulation. Thrombophilia screening showed a mutant prothrombin 20210A allele which is an inherited coagulopathy associated with increased plasma levels of prothrombin and increased risks of mainly venous thrombosis. The patient developed endstage biventricular heart failure and underwent heart transplantation within 3 months after thrombus formation in the right ventricle was diagnosed. In the explanted heart, the thrombus in the right ventricle was impressively large and calcified. In patients with unusual thrombus formation in the heart, coagulopathy may be associated and should be excluded.

Adult↗

[Cardiovascular disease in pregnancy].

There is a heterogeneous population of young women with cardiovascular disease contemplating pregnancy. Many of the conditions are rare and require teams with expertise in the management of such patients. A specific congenital or acquired cardiovascular anomaly and its physiology must be understood. The nature of prior surgical procedures and the residua and sequelae following therapy are essential to manage a pregnant woman with cardiovascular disease. Physiologic changes during pregnancy and after delivery must be known. Counseling includes maternal and fetal risk stratification and genetic counseling regarding inherited disorders. The outcome of pregnancy is favourable in many women with good functional class. These patients can be reassured. A multidisciplinary approach may be crucial to manage this population during pregnancy, labour, delivery and in the postpartum period to avoid serious maternal and fetal complications. Women with intermediate and high risk pregnancy must be followed and managed in a high risk pregnancy unit and a team from obstetricians, cardiologists, anesthetists, pediatricians, neonatologists and cardiac surgeons who are skilled in high risk pregnancies. Vaginal delivery is the method of choice in many women (class 1 woman) and cesarean section is seldom a cardiac indication. Contraindications for pregnancy are: poor maternal functional class, poor ventricular function, severe cyanosis without pulmonary hypertension (oxygen saturation < or = 85%), pulmonary vascular disease, severe mitral stenosis, severe left ventricular outflow tract obstruction, Marfan syndrome with aortic dilatation (> 40 mm) and aortic valve involvement (moderate to severe aortic regurgitation), symptomatic hypertrophic obstructive cardiomyopathy. Preconception counseling must be offered if unfavourable outcome is likely. Tubal ligation is probably the safest and most appropriate method of sterilization if there is a high risk pregnancy.

Adult↗

Outcome of pregnancy in women with congenital shunt lesions.

OBJECTIVE: To evaluate the outcome of pregnancy in women with congenital shunt lesions. SETTING: Retrospective study in a tertiary care centre. METHODS: Pregnancy history was obtained by a standardised questionnaire and medical records were reviewed. PATIENTS: 175 women were identified, at a mean (SD) age of 42 (14) years. Pregnancies occurred in 126 women: 50 with an atrial septal defect, 22 with a ventricular septal defect, 22 with an atrioventricular septal defect, 19 with tetralogy of Fallot, and 13 with other complex shunt lesions. RESULTS: 309 pregnancies were reported by 126 woman (2.5 (1.6) pregnancies per woman). The shortening fraction of the systemic ventricle was 40 (8)%, and 98% were in New York Heart Association class I-II at last follow up. Spontaneous abortions occurred in 17% of pregnancies (abortion rate, 0.4 (0.9) per woman). Gestational age of the 241 newborn infants was 8.8 (0.8) months. There were no maternal deaths related to pregnancy. Pre-eclampsia and embolic events were observed in 1.3% and 0.6%, respectively of all pregnancies. Women with complex shunt lesions more often underwent caesarean section (70% v 15-30%, p = 0.005) and gave birth to smaller babies for equivalent gestation (2577 (671) g v 3016 (572) to 3207 (610) g, p < 0.05). The recurrence risk of congenital heart disease was 2.5%. CONCLUSIONS: The outcome of pregnancy is favourable in women with congenital shunt lesions if their functional class and their systolic ventricular function are good. Such patients can be reassured.

Abortion, Spontaneous↗

Prognosis of medically treated patients referred for cardiac transplantation.

OBJECTIVES: To assess prognosis and factors influencing survival of transplant candidates in whom continued medial therapy was recommended in comparison to that of immediately listed patients. METHODS: Retrospective analysis of clinical, echocardiographic and hemodynamic data as related to survival or listing for transplantation of medically treated transplant candidates. PATIENTS: 160 patients considered 'too well' for cardiac transplantation and 133 patients immediately listed for transplantation. RESULTS: Forty-one of the medically treated patients deteriorated clinically and were listed after 10.7+/-12.3 months after initial evaluation. Mid-term prognosis (2 years) of patients never listed was comparable to that of immediately listed patients (74% vs. 70%) but long-term prognosis (5 years) was worse (41% vs. 54%, p<0.001). Cardiothoracic ratio and pulmonary capillary wedge pressure were independent predictors of survival (multivariate analysis) in patients whose NYHA class and physical working capacity improved and cardiothoracic ratio decreased significantly after adjustment of medical therapy. CONCLUSIONS: Mid-term prognosis of selected patients considered 'too well' for transplantation is comparable to patients immediately listed. Lower left ventricular filling pressures, smaller hearts on chest X-ray on initial evaluation, and improvement of symptoms during follow up may identify a subgroup of patients who do well on optimized therapy.

Actuarial Analysis↗

Echogenic structures in the left atrioventricular groove: diagnostic pitfalls.

A variety of echogenic structures that can be detected by echocardiography in the left atrioventricular groove are best seen in the parasternal long-axis view and can be missed if not carefully looked for. Normal anatomic structures or variants can be seen, for example, dilated coronary sinus, persistent left superior vena cava, or lipomatous tissue. Degenerative or inflammatory processes of the mitral annulus (calcification of the mitral annulus, mitral annular abscess) and aneurysms of the left circumflex coronary artery or of the descending aorta can depict pathologic structures. Hiatal hernia and tumors can be found as well.

Aortic Aneurysm, Thoracic↗

Endothelial control of vascular tone in chronic heart failure.

Patients with chronic heart failure (CHF) are hemodynamically characterized by increased vasoconstriction and a reduced vasodilator response to exercise. In addition to various compensatory neurohumoral mechanisms, there is evidence that the endothelium plays an important role in the abnormal vasodilator response. This evidence comes from studies investigating the microvascular response to regional, intra-arterial administration of the endothelium-dependent vasodilator acetylcholine, which found that the vasodilator response and therefore the bioavailability of nitric oxide (NO) was impaired in the microcirculation of the leg, forearm, and myocardium of patients with CHF. The mechanisms underlying this abnormal response are not entirely clear but may reflect a muscarinic receptor abnormality. Because conduit artery vasodilatation during hyperemic blood flow is also impaired and because this response is not dependent on muscarinic receptor activation, this possibility appears to be unlikely. However, impaired smooth muscle responsiveness to NO stimulation, impaired L-arginine availability or utilization, endothelial release of vasoconstricting prostanoids, increased NO degradation and reduced NO synthase activity have all been implicated in this impaired response. In addition, the vasoconstrictor activity of endothelin (ET)-1 appears to play an important role in the regulation of tone in CHF, although the importance of different ET receptors is not yet clear.

Chronic Disease↗

Isolated noncompaction of the myocardium in adults.

OBJECTIVE: To describe the entity of isolated ventricular noncompaction (IVNC) and present a series of cases of this rare disorder in an adult population. MATERIAL AND METHODS: We review a 10-year experience with the diagnosis of IVNC and discuss the clinical, echocardiographic, and pathologic features of this condition. Echocardiographic diagnostic criteria included the absence of coexisting cardiac abnormalities, the presence of prominent and excessive trabeculations of one or more ventricular wall segments, and intertrabecular spaces perfused from the ventricular cavity. Pathologic examination focused on regions with exaggerated trabeculations and deep intertrabecular spaces. RESULTS: IVNC is an unexplained arrest of myocardial morphogenesis previously encountered mainly in pediatric patients. Among 37,555 transthoracic echocardiographic studies performed at our hospital between January 1984 and October 1993, 17 cases of IVNC were identified in adult subjects (14 men and 3 women, 18 to 71 years of age). The mean time from onset of symptoms to correct diagnosis was 3.5 +/- 5.7 years, and the mean duration of follow-up was 30 +/- 28 months. Common clinical symptoms were heart failure, ventricular arrhythmias, and a history of embolic events. Two-dimensional echocardiography revealed 10 patients with left ventricular and 7 (41%) with biventricular IVNC. During a 6-year follow-up period, eight patients died and two underwent heart transplantation. CONCLUSION: Although the diagnosis of IVNC in an adult population is often delayed because of similarities with more frequently diagnosed conditions, two-dimensional echocardiography will facilitate the diagnosis of IVNC in this subset of patients. Because of the high incidence of heart failure, ventricular arrhythmias, and embolization in adults with IVNC, early diagnosis is important.

Adult↗

Heart transplantation with bicaval anastomoses after a maze operation.

A 49-year-old man had progressive low cardiac output syndrome after mitral valve repair combined with a maze operation. A biventricular assist device was implanted and, finally, emergency orthotopic heart transplantation was performed. The multiple incisions, especially right atrial incisions, were successfully avoided by heart transplantation with bicaval anastomoses.

Anastomosis, Surgical↗

Pretransplant malignancy in candidates and posttransplant malignancy in recipients of cardiac transplantation.

BACKGROUND: Malignancy is generally considered a contraindication for cardiac transplantation, whereas secondary malignancy has been described under chronic immunosuppression. PATIENTS AND METHODS: We report here the frequency of malignancy encountered among the 495 patients evaluated at our cardiac transplant centre as well as the incidence and the course of post-transplant malignancy among 129 consecutive patients who underwent cardiac-transplantation, with a subsequent minimum follow-up of 6 months. RESULTS: A total of 10 out of 495 patients (2%) evaluated for heart transplantation presented with a history of previous malignancy: 3 of them underwent transplantation (2 survive, 1 died) whereas in the remaining 7 patients neoplasia was considered a contraindication for cardiac transplantation, and all 7 died (4 cardiac, 3 tumor-related deaths). Post-transplant malignancy was diagnosed in 10 of 129 patients (9%) 35 +/- 15 months after transplantation (6 skin cancers, 1 lymphoproliferative disease, 3 solid tumors). No significant association was found between post-transplant malignancy and primary prophylaxis with antithymocyte globulin (ATG) or murine antihuman T-cell monoclonal antibodies (OKT3). CONCLUSION: These results confirm that pre-transplant malignancy is not an absolute contraindication for cardiac transplantation and that post-transplant follow-up must include careful monitoring of post-transplant malignancy.

Adolescent↗

[Treatment strategies in mechanical and electrical cardiovascular failure].

Guidelines in cardiopulmonary resuscitation [CPR] improve survival of patients in cardiac arrest. Programs with both Basic Life Support [BLS], promptly initiated, and early defibrillation must be designed to motivate persons trained in prehospital CPR. The most frequent lethal rhythm in cardiac arrest is ventricular fibrillation [VF], affecting over 75% of patients. Early defibrillation must have the highest priority as the only method for termination of VF and being the major determinant for survival. Administration of 1.0 mg epinephrine iv as the drug of choice, followed by defibrillation [360 J] in patients with recurrent/persistent VF remains gold standard in Advanced Cardiac Life Support [ACLS]; epinephrine administered in the same, or higher, dosage can be repeated every 3-5 minutes followed by defibrillation within 30-60 seconds. Management of asystole and pulseless electrical activity is discussed, including the necessity to search for treatable causes as one major action, in addition to performing ACLS and administering epinephrine.

Adrenergic Agonists↗

Effect of diltiazem on coronary flow reserve in patients with microvascular angina.

Microvascular angina is characterized by ischemia-like symptoms in patients with normal coronary arteries and reduced coronary flow reserve. Clinical observations suggested an improvement in clinical symptomatology and exercise tolerance after treatment with calcium antagonists. The effect of diltiazem on coronary flow reserve was evaluated in controls and in patients with microvascular angina. Coronary flow reserve was measured in 16 normotensive patients (7 females, 9 males, mean age 51 +/- 10 years) with angiographically normal coronary arteries. Coronary blood flow was determined at rest, after dipyridamole (0.5 mg/kg) and following intravenous administration of diltiazem (10 mg) using coronary sinus thermodilution technique. Coronary flow reserve was calculated as coronary blood flow after dipyridamole divided by coronary blood flow at rest. Patients with normal coronary flow reserve (coronary flow reserve > 2.0) received either dipyridamole alone (group 1, controls, n = 6) or dipyridamole and diltiazem (group 2, n = 5), whereas patients with reduced coronary flow reserve (coronary flow reserve < 2.0) obtained dipyridamole and diltiazem (group 3, n = 5). Resting coronary flow was identical in the three groups, but after maximal vasodilation with dipyridamole, coronary flow increased significantly more in groups 1 and 2 than in group 3 (P < 0.05, analysis of variance (ANOVA)). Coronary flow reserve was 2.5 in group 1 and 2.3 in group 2, but was significantly reduced in group 3 (1.3; P < 0.05, ANOVA). Intravenous diltiazem failed to increase coronary blood flow in groups 2 and 3. Therefore, diltiazem does not improve reduced coronary flow reserve in patients with microvascular angina, but leaves coronary flow reserve unaffected. The failure to ameliorate impaired coronary flow reserve with diltiazem is in contrast to the reported clinical improvement after calcium channel blockade in these patients. Thus, other factors such as structural abnormalities in the microcirculation or functional abnormality in smooth muscle relaxation not responsive to calcium channel blockade are probably responsible for the occurrence of myocardial ischemia in patients with microvascular angina.

Analysis of Variance↗

Evidence for endothelin-1-mediated vasoconstriction in severe chronic heart failure.

Heart failure is commonly associated with high plasma concentrations of endothelin-1, a powerful vasoconstrictor produced by endothelium. The role of endogenously released endothelin-1 in the maintenance of vascular tone in chronic heart failure was assessed by acute administration of an endothelin receptor antagonist, bosentan. 24 patients with chronic heart failure received randomly and double blind two intravenous infusions of either placebo or bosentan (100 mg followed after 60 min by 200 mg). Systemic haemodynamics and plasma endothelin-1 and big-endothelin-1 concentrations were determined before and repeatedly during the 120 min observation period. Baseline endothelin-1 and big-endothelin-1 concentrations, which were above the normal range in all patients, correlated directly with the extent of pulmonary hypertension, with left and right heart filling pressures, and with pulmonary vascular resistance and inversely with cardiac index. Compared with placebo, bosentan reduced mean arterial pressure by 7.7% (95% CI 7.1-9.7), pulmonary artery pressure by 13.7% (10.5-16.9), right atrial pressure by 18.2% (12.0-24.4), and pulmonary artery wedged pressure by 8.6% (5.3-12.0); it increased cardiac index by 13.6% (9.1-18.2), decreased systemic vascular resistance by 16.5% (13.2-19.8), and decreased pulmonary vascular resistance by 33.2% (22.4-44.0). Heart rate did not change. Plasma endothelin-1 concentrations rose more than twofold from baseline in bosentan recipients while big-endothelin-1 concentrations were unchanged. These findings indicate that, in patients with chronic heart failure who have high circulatory endothelin-1 concentrations, this peptide contributes to maintenance of vascular tone. The acute haemodynamic effects of bosentan suggest that chronic endothelin antagonism could be beneficial in such patients.

Adult↗