Estimated glomerular filtration rate, haemodynamics, and mortality in patients with aortic stenosis.

BACKGROUND: In aortic stenosis (AS), estimated glomerular filtration rate (eGFR) is an important prognostic marker but its haemodynamic determinants are unknown. We investigated the correlation between eGFR and invasive haemodynamics and long-term mortality in AS patients undergoing aortic valve replacement (AVR). METHODS: We studied 503 patients [median (interquartile range) age 76 (69-81) years] with AS [indexed aortic valve area .42 (.33-.49) cm2 /m2 ] undergoing cardiac catheterization prior to surgical (72%) or transcatheter (28%) AVR. Serum creatinine was measured on the day before cardiac catheterization for eGFR calculation (CKD-EPI formula). RESULTS: The median eGFR was 67 (53-82) mL/min/1.73 m2 . There were statistically significant correlations between eGFR and mean right atrial pressure (r = -.13; p = .004), mean pulmonary artery pressure (mPAP; r = -.25; p < .001), mean pulmonary artery wedge pressure (r = -.19; p < .001), pulmonary vascular resistance (r = -.21; p < .001), stroke volume index (r = .16; p < .001), extent of coronary artery disease, and mean transvalvular gradient but not indexed aortic valve area. In multivariate linear regression, higher age, lower haemoglobin, lower mean transvalvular gradient (i.e. lower flow), lower diastolic blood pressure, and higher mPAP were independent predictors of lower eGFR. After a median post-AVR follow-up of 1348 (948-1885) days mortality was more than two-fold higher in patients in the first eGFR quartile compared to those in the other three quartiles [hazard ratio 2.18 (95% confidence interval 1.21-3.94); p = .01]. CONCLUSION: In patients with AS, low eGFR is a marker of an unfavourable haemodynamic constellation as well as important co-morbidities. This may in part explain the association between low eGFR and increased post-AVR mortality.

Tularemia: an experience of 13 cases including a rare myocarditis in a referral center in Eastern Switzerland (Central Europe) and a review of the literature.

BACKGROUND: Tularemia, a zoonotic disease caused by Francisella tularensis, can cause a broad spectrum of disease in humans including six major clinical presentations: the ulceroglandular, glandular, oculoglandular, oropharyngeal, typhoidal and pneumonic form. The epidemiology and ecology and thus transmission of tularemia are complex, depending on conditions unique to specific locations. CASE SERIES AND METHODS: Thirteen cases with different forms of the disease and one very rare case of a myocarditis are reported, discussed, and reviewed within the scope of current literature. CONCLUSION: Tularemia is a rare, but emerging disease in Central Europe with glandular and ulceroglandular disease as its predominant forms. Transmission is mainly caused by contact with lagomorphs, rodents and tick bites. However, domestic cats may play an important role in transmission too. Myocarditis is probably a worldwide, but very rare manifestation of tularemia.

Spontaneous Coronary Artery Dissection: Angiographic Follow-Up and Long-Term Clinical Outcome in a Predominantly Medically Treated Population.

OBJECTIVE: We sought to assess the angiographic and long-term clinical outcomes in a predominantly medically treated population with spontaneous coronary artery dissection (SCAD). BACKGROUND: There are little data on the angiographic and long-term outcome in patients with SCAD. METHODS: We studied 64 patients with SCAD (mean age 53 years, 94% females, three peripartum) with acute coronary syndrome who were treated using coronary bypass grafting (n = 1), percutaneous coronary intervention (n = 7), or medical therapy (n = 56). A repeat angiogram was performed in 40/64 (63%) patients. The median clinical follow-up was 4.5 years. RESULTS: Five (8%) patients had a major cardiac event. One patient with peripartum left main SCAD and cardiogenic shock died during PCI. One patient with conservatively treated SCAD of the posterior descending artery suffered out-of-hospital cardiac arrest 16 days after the initial angiogram but survived. Three patients experienced a second SCAD in another vessel 3.7, 4.7, and 7.9 years after the index event while the initial dissection had healed. Thirty medically treated patients underwent a scheduled repeat angiogram showing healing of the dissection in all but one patient. After a median follow-up of 4.5 (1.8-8.4) years, all 63 patients surviving the index event were alive and free of symptoms suggestive of myocardial ischemia. CONCLUSIONS: In general, the long-term outcome of patients with SCAD is excellent, and medical therapy can be safely applied in the majority of patients. However, SCAD can be a life-threatening and sometimes catastrophic event, and some patients experience early or late complications including SCAD of another vessel. © 2015 Wiley Periodicals, Inc.

Effects of myocardial fibrosis and ventricular dyssynchrony on response to therapy in new-presentation idiopathic dilated cardiomyopathy: insights from cardiovascular magnetic resonance and echocardiography.

AIMS: To determine whether the extent of myocardial fibrosis by late-gadolinium enhancement cardiovascular magnetic resonance (LGE-CMR), and echocardiographic ventricular dyssynchrony are independently associated with response to medical therapy in patients with newly diagnosed idiopathic dilated cardiomyopathy (DCM). Myocardial fibrosis and ventricular dyssynchrony are frequent findings in DCM. Previous studies focused on patients with established cardiomyopathy; however, the degree of myocardial fibrosis and ventricular dyssynchrony at presentation and their role in perpetuating left ventricular (LV) dysfunction in DCM remains unclear. Those studies of individuals with long-standing DCM did not characterize patients early in the disease course, and may not have included those with significant improvement in LV function. Thus factors contributing to LV recovery are uncertain. METHODS AND RESULTS: Consecutive patients with a new diagnosis of DCM [LV ejection fraction (EF) ≤45%] made within the preceding 2 weeks were recruited. Patients underwent LGE-CMR, echocardiography, 6-minute walk testing, cardiopulmonary exercise testing, and blood sampling for measurement of serum amino-terminal pro-brain natiuretic peptide (NT-pro-BNP) concentration at baseline. Baseline patient characteristics were compared with a cohort of healthy volunteers. Myocardial fibrosis by LGE-CMR was quantified, identified by experienced observers blinded to patient outcome. Left ventricular systolic function was reassessed after 5 months of optimal medical therapy. Sixty-eight patients with DCM and 19 healthy volunteers were studied. DCM patients were studied a median 12.5 days following diagnosis. Compared with healthy controls, DCM patients exhibited greater inter- and intra-ventricular dyssynchrony. Twenty-four per cent of DCM patients exhibited LGE at diagnosis. Among DCM patients with LGE, the mean fibrosis mass was 2.2 ± 1.3 g. On multivariate analysis, strain dyssynchrony index, and fibrosis mass were independently associated with change in the LVEF over time (P≤ 0.001). Late-gadolinium enhancement cardiovascular magnetic resonance conferred additive value for modelling change in the LVEF beyond clinical and echocardiographic dyssynchrony parameters. CONCLUSION: The extent of myocardial fibrosis is independently associated with lack of response to medical therapy in new-presentation DCM, and LGE-CMR may thus be an important risk-stratifying investigation in these patients. Accurate risk stratification may permit more targeted pharmacological and device therapies for patients with newly diagnosed DCM.

Systemic blood pressure in severe aortic stenosis: Haemodynamic correlates and long-term prognostic impact.

AIMS: Blood pressure (BP) targets in patients with aortic stenosis (AS) are controversial. This study sought to describe the haemodynamic profile and the clinical outcome of severe AS patients with low versus high central meaarterial pressure (MAP). METHODS AND RESULTS: Patients with severe AS (n = 477) underwent right and left heart catheterization prior to aortic valve replacement (AVR). The population was divided into MAP quartiles. The mean systolic BP, diastolic BP, and MAP in the entire population were 149 ± 25, 68 ± 11, and 98 ± 14 mmHg. Patients in the lowest MAP quartile had the lowest left ventricular ejection fraction (LVEF), systemic vascular resistance, and valvulo-arterial impedance, whereas there were no significant differences in mean right atrial pressure, mean pulmonary artery wedge pressure, pulmonary vascular resistance, and stroke volume index across MAP quartiles. However, left ventricular stroke work index (LVSWI) was lowest in patients in the lowest and highest in those in the highest MAP quartile. After a median (interquartile range) post-AVR follow-up of 3.7 (2.6-5.2) years, mortality was highest in patients in the lowest MAP quartile [hazard ratio 3.08 (95% confidence interval 1.21-7.83); P = 0.02 for lowest versus highest quartile]. In the multivariate analysis, lower MAP [hazard ratio 0.78 (95% confidence interval 0.62-0.99) per 10 mmHg increase; P = 0.04], higher mean right atrial pressure and lower LVEF were independent predictors of death. CONCLUSIONS: In severe AS patients, lower MAP reflects lower systemic vascular resistance and valvulo-arterial impedance, which may help to preserve stroke volume and filling pressures despite reduced left ventricular performance, and lower MAP is a predictor of higher long-term post-AVR mortality.

Imaging cardio-vascular anatomy and function in D-Transposition of the great arteries after mustard procedure.

We used cardiovascular magnetic resonance (CMR) to demonstrate cardio-vascular anatomy and function in a 42 year-old man with a D-Transposition of the great arteries who survived a sudden cardiac arrest.

Pulmonary Hypertension in Patients With Heart Failure With Mid-Range Ejection Fraction.

Pulmonary hypertension (PH) is common in patients with heart failure (HF). The role of PH in patients with HF with reduced (HFrEF) and preserved (HFpEF) left ventricular ejection fraction (LVEF) has been extensively characterized during the last years. In contrast, the pathophysiology of HF with mid-range LVEF (HFmrEF), and in particular the role of PH in this context, are largely unknown. There is a paucity of data in this field, and the prevalence of PH, the underlying mechanisms, and the optimal therapy are not well-defined. Although often studied together there is increasing evidence that despite similarities with both HFrEF and HFpEF, HFmrEF also differs from both entities. The present review provides a summary of the current concepts of the mechanisms and clinical impact of PH in patients with HFmrEF, a proposal for the non-invasive and invasive diagnostic approach required to define the pathophysiology of PH and its management, and a discussion of future directions based on insights from mechanistic studies and randomized trials. We also provide an outlook regarding gaps in evidence, future clinical challenges, and research opportunities.

Pulmonary Embolism in a Patient With Hypertrophic Obstructive Cardiomyopathy: Think Outside "the Box".

A patient with hypertrophic obstructive cardiomyopathy (HOCM) was admitted with acute heart failure. Echocardiography revealed significantly worsened left ventricular outflow tract obstruction and pulmonary hypertension. Cardiac magnetic resonance imaging showed biventricular dysfunction and, as an unexpected finding, a large embolus in the right pulmonary artery. The patient gradually improved with medical therapy including oral anticoagulation. In HOCM patients, pulmonary embolism may trigger heart failure due to impaired left ventricular filling with consecutive outflow tract obstruction provocation.

Wedge Pressure vs Left Ventricular End-Diastolic Pressure for Pulmonary Hypertension Classification and Prognostication in Severe Aortic Stenosis.

BACKGROUND: Differentiation between precapillary and postcapillary pulmonary hypertension (PH) classically relies on mean pulmonary artery wedge pressure (mPAWP). The left ventricular end-diastolic pressure (LVEDP) is proposed as an equivalent alternative. However, mPAWP and LVEDP may differ substantially. We compared the impact of the choice of using the mPAWP vs the LVEDP on PH classification and mortality prediction in patients with severe aortic stenosis (AS) undergoing valve replacement. METHODS: In 335 patients with severe AS , both mPAWP and LVEDP were measured. A mean pulmonary artery pressure ≥ 25 mm Hg was used to define PH, and either mPAWP or LVEDP was used to differentiate between precapillary and postcapillary PH (≤ 15 vs > 15 mm Hg). Mortality after a median follow-up of 1484 days after aortic valve replacement was assessed. RESULTS: Overall, mPAWP was lower than LVEDP (16 ± 8 mm Hg vs 21 ± 8 mm Hg; P < 0.001). Among 140 patients (42%) with PH, the PAWP-based classification revealed 76 (54% of those with PH) with isolated postcapillary PH, 48 (34%) with combined pre- and postcapillary PH, and 16 (12%) with precapillary PH. When the LVEDP was used, 59 patients (42%) were differently classified. These patients had higher mortality than those who were not differently classified [hazard ratio 2.79 (95% confidence interval, 1.17-6.65); P = 0.02]. Higher mPAWP was associated with increased mortality [hazard ratio 1.07 (95% confidence interval, 1.03-1.11) per 1 mm Hg; P = 0.001], whereas higher LVEDP was not. CONCLUSIONS: Use of LVEDP rather than mPAWP results in a divergent PH classification in nearly every second patient with severe AS. These patients have higher mortality after aortic valve replacement. The mPAWP, but not the LVEDP, predicts mortality.

INTRODUCTION: La différentiation entre l'hypertension pulmonaire (HP) précapillaire et postcapillaire repose traditionnellement sur la pression artérielle pulmonaire d'occlusion moyenne (PAPOm). La pression télédiastolique du ventricule gauche (PTDVG) est proposée comme alternative équivalente. Toutefois, la PAPOm et la PTDVG peuvent largement différer. Nous avons comparé les répercussions du choix entre l'utilisation de la PAPOm vs l'utilisation de la PTDVG sur la classification de l'HP et la prédiction de la mortalité des patients atteints d'une sténose aortique (SA) grave qui subissaient un remplacement valvulaire. MÉTHODES: Nous avons mesuré la PAPOm et la PTDVG de 335 patients atteints de SA grave. Nous avons utilisé une pression artérielle pulmonaire moyenne ≥ 25 mmHg pour définir l'HP, et utilisé la PAPOm ou la PTVDG pour différencier entre l'HP précapillaire et postcapillaire (≤ 15 mmHg vs > 15 mmHg). Nous avons évalué la mortalité après un suivi médian de 1 484 jours après le remplacement valvulaire aortique. RÉSULTATS: Dans l'ensemble, la PAPOm était plus faible que la PTVDG (16 ± 8 mmHg vs 21 ±8 mmHg; P < 0,001). Parmi les 140 patients (42 %) atteints d'HP, la classification en fonction de la PAPO a révélé 76 (54 % des patients atteints d'HP) patients atteints d'HP postcapillaire isolée, 48 (34 %) patients atteints d'HP précapillaire et postcapillaire combinée et 16 (12 %) patients atteints d'HP précapillaire. Lorsque nous avons utilisé la PTVDG, 59 patients (42 %) étaient classifiés différemment. La mortalité chez ces patients était plus élevée que chez les patients qui n'étaient pas classifiés différemment (rapport de risque 2,79 [intervalle de confiance à 95 %, 1,17-6,65]; P = 0,02). La PAPOm plus élevée était associée à une mortalité accrue (rapport de risque 1,07 [intervalle de confiance à 95 %, 1,03-1,11] par 1 mmHg; P = 0,001), tandis que la PTVDG plus élevée ne l'était pas. CONCLUSIONS: Le fait d'utiliser la PTVDG plutôt que la PAPOm entraîne une classification divergente de l'HP chez presque tous les deux patients atteints de SA grave. La mortalité après le remplacement valvulaire aortique de ces patients est plus élevée. La PAPOm, mais non la PTVDG, prédit la mortalité.

Impact of a volume challenge on haemodynamics and prognosis in patients with severe aortic stenosis.

AIMS: A volume challenge can unmask pulmonary hypertension (PH) and its mechanism. We evaluated the impact of a volume challenge on mean pulmonary artery pressure (mPAP) and mean pulmonary artery wedge pressure (mPAWP) and its prognostic implications in patients with severe aortic stenosis (AS) undergoing aortic valve replacement (AVR). METHODS AND RESULTS: In 285 patients with severe AS (indexed aortic valve area 0.41 ± 0.13 cm2 /m2 ), mPAP and mPAWP were measured before and after administration of 150 ± 58 mL of low-osmolal or iso-osmolal contrast. Following contrast, mPAP and mPAWP rose from 25 ± 10 and 16 ± 8 mmHg by 5 ± 4 and 4 ± 4 mmHg to 30 ± 11 and 20 ± 8 mmHg. There were 112 (39%) patients with pre-contrast PH and 70 (40% of those without pre-contrast PH) patients with post-contrast PH only. Post-contrast PH patients were intermediate between pre-contrast PH and no PH in terms of AS severity, cardiac dysfunction, and haemodynamics. After a median follow-up of 43 months post-AVR, pre-contrast PH patients had numerically the highest mortality driven by those with pre-contrast combined pre-capillary and post-capillary PH (n = 35), while post-contrast changes in mPAP and mPAWP were not related to mortality. Patients with any post-contrast mPAWP > 18 mmHg had significantly higher mortality than those with post-contrast mPAWP ≤ 18 mmHg, CONCLUSIONS: In severe AS, a contrast challenge leads to post-contrast PH in ~40% of patients without pre-contrast PH. However, post-contrast haemodynamic changes do not adversely affect outcomes in patients undergoing AVR. Post-contrast PH represents an intermediate stage of 'cardiac damage', which may be attenuated or reversible after AVR.

Impact of the new pulmonary hypertension definition on long-term mortality in patients with severe aortic stenosis undergoing valve replacement.

BACKGROUND: The new 2018 pulmonary hypertension (PH) definition includes a lower mean pulmonary artery pressure (mPAP) cut-off (>20 mmHg rather than ≥25 mmHg) and the compulsory requirement of a pulmonary vascular resistance (PVR) ≥3 Wood units (WU) to define precapillary PH. We assessed the clinical impact of the 2018 compared to the 2015 PH definition in aortic stenosis (AS) patients undergoing aortic valve replacement (AVR). METHODS: Severe AS patients (n = 487) undergoing pre-AVR right heart catheterization were classified according to the 2015 and 2018 definitions. Post-AVR mortality (median follow-up 44 months) was assessed. RESULTS: Based on the 2015 definition, 66 (13%) patients exhibited combined pre and postcapillary PH (CpcPH), 116 (24%) isolated post-capillary PH (IpcPH), 28 (6%) precapillary PH, and 277 (57%) no PH at all. Overall, 52 (11%) patients were reclassified: 23 no PH into IpcPH; 8 no PH into precapillary PH; 20 precapillary PH into no PH; 1 CpcPH into IpcPH. By the 2015 definition, only CpcPH patients displayed increased mortality, whereas by the 2018 definition, precapillary PH patients also experienced higher mortality than those without PH. Among the PH definition components, PVR ≥3 WU was the strongest predictor of death (hazard ratio > 4). CONCLUSIONS: In severe AS, a higher number of IpcPH patients are diagnosed by the 2018 definition, even though they have the same prognosis as those without PH. Patients with true precapillary PH are more accurately identified by the 2018 definition that includes a pulmonary vascular disease criterion, that is, PVR ≥3 WU, a strong mortality predictor.

Long-term follow-up of medically treated patients with spontaneous coronary artery dissection: a prospective, Swiss single-centre cohort study.

AIMS OF THE STUDY: Spontaneous coronary artery dissection (SCAD) is an increasingly diagnosed cause of acute myocardial infarction. However, there is still a limited number of larger cohorts with long-term follow-up. We report on the largest Swiss single-centre cohort to date, with follow-up of up to 22 years. METHODS: We prospectively collected SCAD cases from June 1998 until December 2020. A strategy of systematic follow-up angiography was applied. Information on long-term follow-up was collected up to the end of 2020. Major cardiovascular events (MACE) were defined as all-cause death, non-fatal MI, and non-fatal cardiac arrest. RESULTS: We identified 105 SCAD patients (mean age 53 ± 11 years, 98 female, 5 peripartum). Presentation was myocardial infarction in all patients. In 102 patients, there was one contiguous dissection. Three patients had two (n = 2) or three (n = 1) non-contiguous dissections. In the majority of patients (n = 97), the primary treatment approach was conservative (dual antiplatelet therapy for 12 months in 90% of patients, statins in 91%). Seven patients were treated with percutaneous coronary intervention (PCI) and one patient underwent bypass surgery. Elective follow-up angiograms were performed in 73 asymptomatic patients after a median follow-up of 6.0 months (interquartile range [IQR] 5.5-6.5). These showed healing of the dissection (n = 65) or a good result after PCI (n = 5) in 70 patients. Three patients had a persistent dissection but conservative treatment was continued. After a median follow-up of 7.5 years (IQR 3.6-12.5) (longest follow-up: 22.5 years) there were 15 MACE. Five MACE occurred within 30 days of the index event: death following catastrophic peripartum left main SCAD (n = 1), out-of-hospital cardiac arrest with successful resuscitation 16 days after SCAD (n = 1), ST-segment elevation myocardial infarction due to occlusion of the dissected artery 10 hours after the index angiogram with subsequent PCI (n = 1), SCAD of a second vessel 8 days after the index SCAD (n = 1), and non-ST-segment elevation myocardial infarction with persistent, multisite SCAD 10 days after the index event (n = 1). There were 10 late MACE, including myocardial infarction and recurrent SCAD (different vessel/lesion) a median of 7.6 years (IQR 3.9-9.6) after the index event in eight patients and death with unclear cause in two patients. CONCLUSION: This SCAD series highlights its highly variable clinical course during the acute phase and in the long term. Although most SCAD patients can be treated conservatively with subsequent healing of the dissection and good clinical outcome, there are also patients with dramatic acute presentation or MACE several years after the initial presentation.

Hemodynamics Prior to Valve Replacement for Severe Aortic Stenosis and Pulmonary Hypertension during Long-Term Follow-Up.

(1) Background: Pulmonary hypertension after aortic valve replacement (AVR; post-AVR PH) carries a poor prognosis. We assessed the pre-AVR hemodynamic characteristics of patients with versus without post-AVR PH. (2) Methods: We studied 205 patients (mean age 75 ± 10 years) with severe AS (indexed aortic valve area 0.42 ± 0.12 cm2/m2, left ventricular ejection fraction 58 ± 11%) undergoing right heart catheterization (RHC) prior to surgical (70%) or transcatheter (30%) AVR. Echocardiography to assess post-AVR PH, defined as estimated systolic pulmonary artery pressure > 45 mmHg, was performed after a median follow-up of 15 months. (3) Results: There were 83/205 (40%) patients with pre-AVR PH (defined as mean pulmonary artery pressure (mPAP) ≥ 25 mmHg by RHC), and 24/205 patients (12%) had post-AVR PH (by echocardiography). Among the patients with post-AVR PH, 21/24 (88%) had already had pre-AVR PH. Despite similar indexed aortic valve area, patients with post-AVR PH had higher mPAP, mean pulmonary artery wedge pressure (mPAWP) and pulmonary vascular resistance (PVR), and lower pulmonary artery capacitance (PAC) than patients without. (4) Conclusions: Patients presenting with PH roughly one year post-AVR already had worse hemodynamic profiles in the pre-AVR RHC compared to those without, being characterized by higher mPAP, mPAWP, and PVR, and lower PAC despite similar AS severity.

Invasive Hemodynamic Staging Classification of Cardiac Damage in Patients With Aortic Stenosis Undergoing Valve Replacement.

BACKGROUND: An echocardiographic 5-stage classification of cardiac damage in aortic stenosis (AS) has been shown to provide prognostic information. We aimed to create an analogous classification based on invasive hemodynamics. METHODS: We studied 421 patients (age 75 ± 10 years, 59% men) with severe AS with complete invasive hemodynamics obtained before aortic valve replacement (AVR). Cardiac damage stages were defined as follows: stage 0, absence of any of the following criteria; stage 1, left-ventricular end-diastolic pressure >15 mm Hg; stage 2, mean pulmonary artery wedge pressure > 15 mm Hg; stage 3, pulmonary vascular resistance > 3 Wood units and/or systolic pulmonary artery pressure > 60 mm Hg; stage 4, mean right atrial pressure >15 mm Hg. Patients were hierarchically classified in a given stage (worst stage) if the criterion was met for that stage. RESULTS: Sixty-seven (16%) patients were classified as stage 0, 113 (27%) as stage 1, 151 (36%) as stage 2, 73 (17%) as stage 3, and 17 (4%) as stage 4. After a median (interquartile range) follow-up of 3.8 (2.7 to 5.2) years after AVR, mortality was highest in stage 4 (hazard ratio; 95% confidence interval: 6.17 (1.74-21.89) vs stage 0; P = 0.005 and stage 3 patients (hazard ratio; 95% confidence interval: 4.17 (1.39-12.49) vs stage 0; P = 0.01,whereas mortality did not differ between patients in stages 0 to 2. CONCLUSIONS: A staging system of cardiac damage based on invasive hemodynamic parameters in patients with severe AS undergoing AVR predicts mortality. Pulmonary vascular disease and high right-atrial pressure are the major drivers of mortality.

Myotonic dystrophy as a potential killer.

A 19-year-old man suffered a cardiac arrest during a promenade with his friends. Cardiac resuscitation was started immediately. Anamnesis uncovered that the father as well as a cousin of the patient suffered from myotonic dystrophy (MD). Follow-up ECG monitoring showed intercurrent III degree AV-block as well as several asymptomatic episodes of ventricular tachycardias, atrial flutter with changing conduction and atrial fibrillation. Neuromuscular testing and genetic analyses confirmed the diagnosis of a myotonic dystrophy. Myotonic dystrophy (MD) is a chronic, slowly progressing, autosomal dominant inherited multisystemic disease.The clinical presentation is characterized by wasting of the muscles with delayed relaxation, cataracts and endocrine changes. MD is associated with both cardiac conduction disturbances and structural heart abnormalities. Electrocardiographic abnormalities include conduction disturbances or tachyarrhythmias. This case illustrates that potentially lethal arrhythmias inducing sudden cardiac death may occur in MD patients even in the absence of neurologic symptoms characterizing the systemic illness.

Use of coronary computed tomography angiography in clinical practice - single centre experience in Switzerland in light of current recommendations based on pretest probability considerations.

AIMS OF THE STUDY: Coronary computed tomography angiography (CCTA) is recommended as a first-line option for the exclusion of coronary artery disease in patients with low to intermediate (15–50%) pretest probability. We aimed to study the use of CCTA in clinical practice in a single centre in Switzerland in light of this recommendation. METHODS: In 523 consecutive patients (age 56 ± 13 years, 48% females) undergoing CCTA during a period of 2 years, the pretest probability of coronary artery disease was assessed using the revised Diamond-Forrester model (CAD consortium score). In patients who had invasive coronary angiography following CCTA, angiographic findings and the consequences regarding management are reported. RESULTS: The majority of patients (n = 316; 60%) had a pretest probability <15%, 188/523 (36%) had a pretest probability between 15 and 50%, and 19/523 (4%) had a pretest probability >50%. The prevalences of coronary artery disease (≥50% lumen diameter reduction) by CCTA in patients with pretest probability <15%, 15–50%, and >50% were 25/316 (8%), 45/188 (24%) and 8/19 (42%), respectively. In 438/523 patients (84%), a CCTA scan showing no coronary artery disease represented the final diagnostic step. In patients undergoing invasive coronary angiography (n = 59, age 58 ± 9 years, 88% with coronary artery disease by CCTA), coronary artery disease was found in 47/59 (80%) patients and 36/59 (61%) patients underwent revascularisation. The prevalences of coronary artery disease by invasive coronary angiography in patients with pretest probability <15%, 15–50%, and >50% were 14/21 (67%), 28/32 (88%) and 5/6 (83%). CONCLUSIONS: The present data suggest that the currently used pretest probability model is still imperfect and that guideline recommendations regarding pretest probability use for the selection of CCTA candidates are not followed completely. Still, in more than 80% of patients coronary artery disease could be excluded by CCTA, while CCTA also detected a significant number of patients with coronary artery disease in the low pretest probability population. Thus, the data suggest a very judicious use of CCTA as a gatekeeper for invasive coronary angiography in current practice.

Haemodynamic mechanisms and long-term prognostic impact of pulmonary hypertension in patients with severe aortic stenosis undergoing valve replacement.

AIMS: We aimed to investigate the prevalence, detailed invasive haemodynamics, and prognostic impact of pulmonary hypertension (PH) in severe aortic stenosis (AS). METHODS AND RESULTS: We studied 503 patients (mean age 74 ± 10 years) with severe AS (indexed aortic valve area 0.4 ± 0.1 cm2 /m2 , left ventricular ejection fraction 57 ± 12%) undergoing left and right heart catheterization prior to aortic valve replacement. Median follow-up was 3.7 (interquartile range 2.6-5.4) years. Baseline PH (mean pulmonary artery pressure ≥ 25 mmHg) was found in 239 (48%) patients: 31 patients had pre-capillary PH [mean pulmonary artery wedge pressure (mPAWP) ≤ 15 mmHg], 144 had isolated post-capillary PH [IpcPH; mPAWP > 15 mmHg, pulmonary vascular resistance (PVR) ≤ 3 Wood units (WU)], and 64 had combined pre- and post-capillary PH (CpcPH; mPAWP > 15 mmHg, PVR > 3 WU). Patients with CpcPH had higher mortality than those with IpcPH, pre-capillary PH, and without PH. In the multivariate analysis, CpcPH remained an independent predictor of death (hazard ratio 4.39, 95% confidence interval 2.40-8.03; P < 0.001). Patients with CpcPH had higher mPAWP (26 ± 7 vs. 22 ± 5 mmHg) and lower pulmonary arterial capacitance (1.5 ± 0.6 vs. 2.9 ± 1.2 mL/mmHg) than IpcPH patients but similar left ventricular end-diastolic pressure (LVEDP; 25 ± 7 vs. 25 ± 7 mmHg). A smaller LVEDP-mPAWP difference was related to larger left atrial size, atrial fibrillation, and more severe mitral regurgitation. CONCLUSIONS: In patients with severe AS, PH is common but underlying mechanisms differ. Patients with CpcPH have higher mPAWP, lower pulmonary arterial capacitance, and worse survival than all other groups. Left atrial dysfunction and mitral regurgitation seem to be drivers of high mPAWP in CpcPH.