Die peripartale Kardiomyopathie ist eine lebensgefährliche Herzerkrankung, die oft spät erkannt wird, da die klinischen Beschwerden sehr unterschiedlich sein können. In der Regel verläuft die Schwangerschaft zunächst ohne Komplikationen. Die Patientinnen sind jung, gesund und stehen kurz vor der Geburt oder haben sie eben hinter sich gebracht. In den letzten Schwangerschaftswochen bis 6 Monate nach der Geburt macht sich dann eine plötzlich einsetzende Herzinsuffizienz bemerkbar.

BackgroundExercise training safely and efficiently improves symptoms in patients with heart failure due to left ventricular dysfunction. However, studies in congenital heart disease with systemic right ventricle are scarce and results are controversial. In a randomised controlled study we investigated the effect of aerobic exercise training on exercise capacity and systemic right ventricular function in adults with d-transposition of the great arteries after atrial redirection surgery (28.2±3.0years after Mustard procedure).Methods48 patients (31 male, age 29.3±3.4years) were randomly allocated to 24weeks of structured exercise training or usual care. Primary endpoint was the change in maximum oxygen uptake (peak VO2). Secondary endpoints were systemic right ventricular diameters determined by cardiac magnetic resonance imaging (CMR). Data were analysed per intention to treat analysis.ResultsAt baseline peak VO2 was 25.5±4.7ml/kg/min in control and 24.0±5ml/kg/min in the training group (p=0.3). Training significantly improved exercise capacity (treatment effect for peak VO2 3.8ml/kg/min, 95% CI: 1.8 to 5.7; p=0.001), work load (p=0.002), maximum exercise time (p=0.002), and NYHA class (p=0.046). Systemic ventricular function and volumes determined by CMR remained unchanged. None of the patients developed signs of cardiac decompensation or arrhythmias while on exercise training.ConclusionsAerobic exercise training did not detrimentally affect systemic right ventricular function, but significantly improved exercise capacity and heart failure symptoms. Aerobic exercise training can be recommended for patients following atrial redirection surgery to improve exercise capacity and to lessen or prevent heart failure symptoms. (Clinical Trial Registration: ClinicalTrials.gov #NCT00837603)

BACKGROUND/OBJECTIVE Cardiopulmonary exercise capacity is often reduced in patients with transposition of the great arteries after atrial switch operation. Reduced exercise capacity may be caused by deterioration of systemic right ventricular function over time. This study analyzed serial changes in systemic right ventricular function and cardiopulmonary exercise capacity in young adults with transposition of the great arteries after atrial redirection surgery. METHODS Twenty-one patients (37% female, mean age 23.2 ± 3.3, mean age at surgery 12.8 ± 14 years) with transposition of the great arteries after atrial switch operation were included in this study. Patients were followed up for a mean period of 39.6 ± 13.1 months. Exercise capacity expressed as peak VO2 max, systemic right ventricular function and subpulmonary left ventricular function assessed by cardiac magnetic resonance imaging and NT-proBNP levels were obtained at baseline and follow-up. Changes in peak VO2 max were correlated to changes in cardiac magnetic resonance imaging and NT-proBNP levels. RESULTS Baseline peak VO2 max decreased significantly (28.31 ± 5.80 mL/kg/min vs. 25.17 ± 5.71 mL/kg/min, P = .005) on follow-up. Cardiac magnetic resonance imaging parameters of systemic right ventricular ejection fraction as well as subpulmonary left ventricular ejection fraction remained unchanged (44.68 ± 6.59% vs. 45.65 ± 9.60%, P = .54, 60.18 ± 6.29% vs. 61.52 ± 5.30%, P = .35). NT-proBNP levels did not increase (211.7 ± 85.7 ng/mL vs. 261.2 ± 182.2 ng/mL, P = .16). CONCLUSIONS After atrial switch operation for transposition of the great arteries we observed a declining functional exercise capacity. This was not associated with worsening systemic right ventricular function, suggesting that other factors are contributing to the decline in physical exercise capacity.

BACKGROUND:ACE inhibition is an established treatment regimen in patients with congestive heart failure due to left ventricular dysfunction which improves morbidity and mortality. However, little is known about the beneficial effects of ACE inhibition in adult patients after Mustard procedure for transposition of the great arteries with heart failure symptoms. Therefore, we investigated the effects of ACE inhibition in these patients on heart failure symptoms, echocardiographic diameters, NT-proBNP and exercise capacity.METHODS:In 14 patients (age 25.2 ± 3.5 years), after Mustard procedure for transposition of the great arteries (age at operation 1.1 ± 1.3 years) with heart failure NYHA II (New York Heart Association class), an ACE inhibition was initiated. At baseline and 13.3 ± 4.0 months after treatment with enalapril (10mg twice a day), echocardiography, exercise test and NT-proBNP measurements were performed and compared to an age- and sex-matched control group.RESULTS:Maximum oxygen uptake and echocardiographic parameters did not change significantly in both groups. However, NT-proBNP showed a significant decrease in the treatment group (242 ± 105 vs. 151 ± 93 ng/l, p=0.004), while in the control group a significant increase (120 ± 89 vs. 173 ± 149 ng/l, p<0.05) was observed. Furthermore, ACE inhibitor treatment did not result in a deterioration of heart failure symptoms or renal function.CONCLUSIONS:Thus, ACE inhibitor treatment of heart failure symptoms in patients with a systemic right ventricle is safe and reduces NT-proBNP levels significantly as a marker for ventricular overload. Nevertheless, larger scale trials are warranted to show effects on morbidity and mortality in this highly selected patient group.

Patients with patent foramen ovale (PFO) and cryptogenic stroke are at risk of recurrence. Therapeutic regimens range from no treatment to anticoagulation treatment to surgical or interventional closure. However, long-term follow-up is only available for up to 4 years.

Anomalies of the coronary arteries are commonly associated with congenital heart disease (CHD). Anomalies include variations in number, shape, and location of the origin, as well as the course of the coronary artery. An intramural course of one of the coronary arteries is a rather rare condition. Most cases of anomalies are of no clinical relevance and are found incidentally during evaluation for other cardiac conditions. However, they become of importance when cardiac surgery/intervention, especially valve replacement, needs to be performed. We conducted a literature review for coronary anomalies with an intramural course in patients with CHD and include three cases from our own clinic. Note that transposition of the great arteries seems to be the condition most frequently associated with an intramural course of one of the coronary arteries. In case of a suspected anomaly, transesophageal echocardiography presents a valuable tool to visualize the origin and course of the coronary artery, as well as the relationship to important anatomical structures like the aortic or pulmonary valve. Alternatively, cardiac magnetic resonance imaging can be used. Although rare, both cardiologists and surgeons need to be aware of intramural courses of coronary arteries to prevent accidental trauma to them and thereby, reducing the risk for the patient.

BackgroundChronic severe pulmonary regurgitation (PR) causes progressive right ventricular (RV) dysfunction and heart failure. Parameters defining the optimal time point for surgery of chronic PR are lacking. The present study prospectively evaluated the impact of preoperative clinical parameters, cardiorespiratory function, QRS duration and NT-proBNP levels on post operative RV function and volumes assessed by cardiac magnetic resonance imaging (CMR) in patients with chronic severe PR undergoing pulmonary valve replacement.Methods and resultsCMR was performed pre- and 6months postoperatively in 27 patients (23.6±2.9years, 15 women) with severe PR. Postoperatively, RV endsystolic (RVESVI) and enddiastolic volume indices (RVEDVI) decreased significantly (RVESVI pre 78.2±20.4ml/m² BSA vs. RVESVI post 52.2±16.8ml/m²BSA, p<0.001; RVEDVI pre 150.7±27.7ml/m²BSA vs. RVEDVI post 105.7±26.7ml/m²BSA; p<0,001). With increasing preoperative QRS-duration, postoperative RVEF decreased significantly (r=−0.57; p<0.005). Preoperative QRS-duration smaller than the median (156ms) predicted an improved RVEF compared to QRS-duration≥156ms (54.9% vs 46.8%, p<0.05). Multivariate analysis identified preoperative QRS duration as an independent predictor of postoperative RVEF (p<0.005). NT-proBNP levels correlated with changes in RVEDI (r=0.58 p<0,005) and RVESVI (r=0.63; p<0,0001). Multivariate analysis identified NT-proBNP levels prior to PVR as an independent predictor of volume changes (p<0.05).ConclusionValve replacement in severe pulmonary regurgitation causes significant reduction of RV volumes. Both, preoperative NT-proBNP level elevation and QRS prolongation indicate patients with poorer outcome regarding RV function and volumes.

INTRODUCTION:Peripartum cardiomyopathy is a rare form of cardiomyopathy, with heterogeneous presentation occurring in women between one-month antepartum and six months postpartum. It carries a poor prognosis and a high risk of mortality.CASE PRESENTATION:We report the development of peripartum cardiomyopathy in two sisters, 27- and 35-year-old African women, one of whom presented with a large left ventricular thrombus. Subsequently, both patients were treated with bromocriptine, heparin and standard therapy for heart failure (angiotensin converting enzyme inhibitors, beta-blockers and diuretics). During follow-up, the left ventricular thrombus observed in one patient degraded. Neither patient experienced a thrombotic event, and both experienced continuous improvements in cardiac function and New York Heart Association stage.CONCLUSION:The development of peripartum cardiomyopathy in two sisters indicates that there may be a genetic basis for this type of cardiomyopathy, and that women with a positive family history for peripartum cardiomyopathy may have an increased risk of developing the disease. This is also the first report of a patient experiencing degradation of a large left ventricular thrombus under standard therapy for heart failure with bromocriptine. It suggests that the use of bromocriptine in association with adequate anti-coagulation and heart failure therapy may be beneficial and safe.

Backgrounds: The univentricular circulation after a Fontan procedure is characterized by an abnormal cardiorespiratory response being attributable to an inability to increase stroke volume during exercise. In congenital heart disease a broad QRS complex has been related to increased intracardiac volume and mass being associated with poor ventricular function and prognosis.Objectives: This study investigated the relation between the width of the QRS complex and parameters of cardiorespiratory response in adult patients after a Fontan procedure.Methods: Clinical data and parameters of cardiorespiratory function of 56 patients (15 women, 41 men, mean age 23.7 +/- 6.4 years, mean age at operation 10.0 +/- 7.5 years) were related to the width of the QRS complex.Results: In the whole group the mean QRS duration was 115 +/- 23 ms. A QRS complex >= 120 ms was present in 23 patients. These patients were characterized by significantly older age at operation (13.0 +/- 9.3 versus 7.9 +/- 5.1; p < 0.05). Compared to individuals with smaller QRS complexes they showed a decreased oxygen uptake (PeakVO(2): 21.6 +/- 5.2 versus 27.7 +/- 6.6 ml/kg/min; p < 0.001), work rate (1.6 +/- 0.5 versus 2.0 +/- 0.5 W/kg, p < 0.05), maximum blood pressure (p < 0.001) and increase in blood pressure (p < 0.05).Univariate analysis showed a significant correlation between PeakVO(2) and several other parameters of cardiorespiratory exercise testing (work rate, O-2-pulse, increase in heart rate and blood pressure, maximum heart rate and blood pressure), maximum enddiastolic diameter of the systemic ventricle, age at operation. Multivariate regression analysis identified QRS duration as the only independent predictor of PeakVO(2) (p=0.05).Conclusion: In a Fontan circulation a broad QRS complex is a negative predictor of cardiorespiratory function. Early Fontan operation may be beneficial in terms of exercise capacity. (C) 2007 Elsevier Ireland Ltd. All rights reserved.

Peri- or postpartum cardiomyopathy (PPCM) is a rare, life-threatening heart disease of unclear origin and is characterized by heart failure of sudden onset between the final weeks of pregnancy and 6 months after delivery.Selective literature search in the databases of the National Center for Biotechnology Information based on the key words "peri- and postpartum cardiomyopathy," "pregnancy" and "heart failure" and additional information from the authors' personal experience.PPCM is often not diagnosed until late in its course, because its clinical manifestations are highly variable and a heart disease may not be suspected at first. Frequent presenting symptoms of PPCM, such as prostration, shortness of breath on mild exertion, and coughing, are often initially misinterpreted as evidence of pneumonia or as physiological accompaniments of pregnancy and delivery. The clinical picture of PPCM corresponds to a dilated cardiomyopathy (DCM) with signs of severe heart failure. Therefore, treatment with ACE inhibitors, diuretics, aldosterone antagonists, and beta-blockers is required. Recent research findings suggest a possible new approach to the treatment of PPCM with bromocriptine, which inhibits the release of prolactin, a lactation-promoting hormone. To date, only the treatment of heart failure in PPCM is evidence-based, while all other treatments are "level C," i.e., based on expert opinion only.The early diagnosis and interdisciplinary management of PPCM can often lead to substantial recovery from heart failure and cardiomyopathy.

INTRODUCTION:Peri- or postpartum cardiomyopathy (PPCM) is a rare, life-threatening heart disease of unclear origin and is characterized by heart failure of sudden onset between the final weeks of pregnancy and 6 months after delivery.METHODS:Selective literature search in the databases of the National Center for Biotechnology Information based on the key words "peri- and postpartum cardiomyopathy," "pregnancy" and "heart failure" and additional information from the authors' personal experience.RESULTS:PPCM is often not diagnosed until late in its course, because its clinical manifestations are highly variable and a heart disease may not be suspected at first. Frequent presenting symptoms of PPCM, such as prostration, shortness of breath on mild exertion, and coughing, are often initially misinterpreted as evidence of pneumonia or as physiological accompaniments of pregnancy and delivery. The clinical picture of PPCM corresponds to a dilated cardiomyopathy (DCM) with signs of severe heart failure. Therefore, treatment with ACE inhibitors, diuretics, aldosterone antagonists, and beta-blockers is required. Recent research findings suggest a possible new approach to the treatment of PPCM with bromocriptine, which inhibits the release of prolactin, a lactation-promoting hormone. To date, only the treatment of heart failure in PPCM is evidence-based, while all other treatments are "level C," i.e., based on expert opinion only.CONCLUSION:The early diagnosis and interdisciplinary management of PPCM can often lead to substantial recovery from heart failure and cardiomyopathy.

To the Editor: Postpartum cardiomyopathy (PPCM) is a disease of unknown origin and exposes women to a high risk of mortality after delivery despite optimal medical therapy ([1][1]). Prolactin is up-regulated postpartally where it induces lactation and promotes reshaping of the uterus. Prolactin

A 41-year old female patient was admitted with acute onset of dyspnea and chest pain. Previous history revealed asthma, chronic sinusitis and eosinophilic proctitis. Electrocardiogram showed anterior ST-segment elevations and inferior ST-segment depression. Immediate heart catheterization revealed a distally occluded left anterior descending coronary artery, the occlusion being reversible after nitroglycerine. Cardiac magnetic resonance imaging was consistent with perimyocarditis. Hypereosinophilia and IgE elevation were present and Churg-strauss syndrome was diagnosed.

We are presenting a case of floating left and right atrial formations on an atrial septal defect occluder system (23mm StarFLEX)-Occluder) initially supposed to be thrombotic appositions in a 57-year-old man. The closure was performed on the background of left hemispheric stroke and atrial septal aneurysm (ASA) with patent foramen ovale (PFO). The suspect structures were detected in the 6-month follow-up by transesophageal echocardiography (TEE). The patient underwent a successful surgical explantation of the closure device and closure of the patent foramen ovale (PFO) using a pericardial patch. The pathological evaluation of the biatrial device associated appositions revealed hytrophic heart muscle tissue with perifocal scarring and purulent abscess-forming, granulating and foam-cell including inflammatory foreign body reaction instead of the expected thrombus formation.

1. Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy. Lancet 2006;368:687–93. 2. Corbacho AM, Martinez De La Escalera G, Clapp C. Roles of prolactin and related members of the prolactin/growth hormone/ placental lactogen family in angiogenesis. J Endocrinol 2002;173: 219–38. 3. Hilfiker-Kleiner D, Kaminski K, Podewski E, et al. A cathepsin D-cleaved 16 kDa form of prolactin mediates postpartum cardiomyopathy. Cell 2007;128:589–600. 4. Francis GS, Parks R, Cohn JN. The effects of bromocriptine in patients with congestive heart failure. Am Heart J 1983;106:100–6. 5. Mejia-Rodriguez O, Alvarez-Aguilar C, Vega-Gomez HE, Belio-Caro F, Vargas-Espinosa JM, Paniagua-Sierra JR. Bromocriptine induces regression of left ventricular hypertrophy in peritoneal dialysis patients. Proc West Pharmacol Soc 2005;48:122–5. 6. Goldberg LI. The role of dopamine receptors in the treatment of congestive heart failure. J Cardiovasc Pharmacol 1989;14 Suppl 5: S19–27.

We report on a case of a mobile left atrial thrombus formation on an atrial septal defect occluder system (28 mm StarFLEX(R)-Occluder) despite 6 months of postprocedural anticoagulation with phenprocoumon and platelet antiaggregation with aspirin in a 69-year-old woman. The closure was performed because of a significant left to right atrial shunt (Qp/Qs 1.8) with enlargement of the right atrial. and ventricular cavities and impairment of right ventricular function in the presence of persistent atrial fibrillation and chronic heart failure (NYHA II-III). The 6-month follow up by transoesophageal echocardiography (TEE) revealed the floating thrombus located at the left atrial. side of the occluder. (C) 2005 The European Society of Cardiology. Published by Elsevier Ltd. ALL rights reserved.

In women with Marfan syndrome pregnancy increases the risk of aortic dissection. There are a number of articles reporting the outcome of acute aortic dissection during pregnancy in this patient group. In contrast, only scarce information is available concerning pregnancy in patients with pre-existing chronic aortic dissection. We report the case of a pregnant Marfan syndrome patient with pre-existing chronic aortic dissection that showed a favourable maternal and fetal outcome.