A closer look at right ventricular 3D volume quantification by transthoracic echocardiography and cardiac MRI.

AIM: To compare right ventricular (RV) volumetry using state-of-the-art three-dimensional (3D) transthoracic echocardiography (3DE) and cardiac magnetic resonance imaging (CMR) near-simultaneously in a clinical setting. MATERIALS AND METHODS: Forty-seven consecutive patients received comprehensive echocardiography including 3DE within 30 minutes of CMR. RV volumetry was performed offline with semi-automated 3D endocardial border tracing as well as manual delineation of the compacted myocardium in short-axis views by CMR. RESULTS: Forty-two examinations (89%) could be analysed offline by 3D RV reconstruction. Mean RV volumes assessed by CMR and 3DE were 215±63 and 127±42 ml for end-diastole (RV-EDV), as well as 110±43 and 62±27 ml for end-systole (RV-ESV). RV-EDV, RV-ESV, and RV stroke volume measured by 3DE were significantly lower than RV volumetry by CMR. Mean bias were -88, -48, and -41 ml, respectively. Mean RV ejection fraction (-EF) showed a non-significant deviation of +2% between 3DE and CMR and the correlation coefficient was r=0.58 for RV-EF. CONCLUSION: RV-EF can be assessed reliably using transthoracic 3DE in patients with good image quality; however, absolute RV volumes measured by 3DE show a systematic deviation to CMR volumetry that has been previously neglected and requires careful interpretation regarding anatomical cardiac imaging.

Predictors of survival stratification in patients with wild-type cardiac amyloidosis.

OBJECTIVES: To analyze clinical predictors of mortality in wild-type transthyretin amyloidosis (wt-ATTR). METHODS: In total, 191 patients (73.8 ± 0.5 years; 176 males, 15 females) with histologically proven wt-ATTR amyloidosis and genetic exclusion of a transthyretin gene variant were included. Comprehensive clinical characteristics, ECG, biomarkers, and echocardiography were analyzed retrospectively. Strain analyses were performed offline using TomTec Imaging Systems, Germany. Univariable and multivariable analyses predicting all-cause mortality were carried out. RESULTS: Patients presented with significant heart failure (NYHA 2.5 ± 0.8; NT-proBNP 3644 (4981) pg/ml; LV ejection fraction 45.8 ± 15.0%). LogNT-proBNP correlated with indicators of disease severity. Similar results were obtained for basal and midventricular, but not apical longitudinal strain. During median follow-up of 26.2 ± 1.7 months 46 (25.5%) patients died (40 males, 23%; six females, 40%). In female patients 1-/2-year survival was lower [92.9/67.7%; median survival 30.6 (21.1-40.1) months] when compared to male patients [96.5%/86.6%; median survival 63.9 (45.8-82.0) months]. Parameters associated with survival were NT-proBNP, NYHA class, heart rate, midventricular longitudinal strain, mitral annular plane systolic excursion (MAPSE), Karnofsky Index, systolic blood pressure, estimated glomerular filtration rate. Multivariable analysis revealed MAPSE and NT-proBNP as independent predictors of mortality in the whole cohort and midventricular strain in the subgroup of patients in sinus rhythm. CONCLUSIONS: No sex-specific bias was observed between male and female patients with wt-ATTR regarding age at onset and morphological characteristics. Multivariable analysis revealed MAPSE and NT-proBNP as independent predictors of survival in the whole cohort, whereas midventricular longitudinal strain was the only independent predictor in patients in sinus rhythm.

[Clinical classification and initial diagnosis of pulmonary hypertension: recommendations of the Cologne Consensus Conference 2016]. / Klinische Klassifikation der pulmonalen Hypertonie und initiale Diagnostik: Empfehlungen der Kölner Konsensus Konferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed information about the clinical classification and diagnosis of pulmonary hypertension, and furthermore provide novel recommendations for risk stratification and follow-up assessments. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the clinical classification and initial diagnosis of PH. This article summarizes the results and recommendations of this working group.

Constrictive Pericarditis in the Presence of Remaining Remnants of a Left Ventricular Assist Device in a Heart Transplanted Patient.

Constrictive pericarditis (CP) is a severe subform of pericarditis with various causes and clinical findings. Here, we present the unique case of CP in the presence of remaining remnants of a left ventricular assist device (LVAD) in a heart transplanted patient. A 63-year-old man presented at the Heidelberg Heart Center outpatient clinic with progressive dyspnea, fatigue, and loss of physical capacity. Heart transplantation (HTX) was performed at another heart center four years ago and postoperative clinical course was unremarkable so far. Pharmacological cardiac magnetic resonance imaging (MRI) stress test was performed to exclude coronary ischemia. The test was negative but, accidentally, a foreign body located in the epicardial adipose tissue was found. The foreign body was identified as the inflow pump connection of an LVAD which was left behind after HTX. Echocardiography and cardiac catheterization confirmed the diagnosis of CP. Surgical removal was performed and the epicardial tubular structure with a diameter of 30 mm was carefully removed accompanied by pericardiectomy. No postoperative complications occurred and the patient recovered uneventfully with a rapid improvement of symptoms. On follow-up 3 and 6 months later, the patient reported about a stable clinical course with improved physical capacity and absence of dyspnea.

Liquefaction necrosis of mitral annulus calcification in a patient with chronic renal failure: an unrecognized diagnosis.

Mitral annulus calcification is one of the most common cardiac calcifications. In patients with end-stage renal disease undergoing echocardiography, it can be detected in more than 40%. A specific form of mitral annulus calcification is liquefaction necrosis. It is often not adequately recognized by echocardiographers or clinicians and can be mistaken for cardiac tumor or infective vegetation. Here we report a case of liquefaction necrosis of mitral annulus calcification, mimicking an infective vegetation of the mitral valve apparatus in a patient with chronic renal failure.

Identification of individuals susceptible to high-altitude pulmonary oedema at low altitude.

Individuals susceptible to high-altitude pulmonary oedema (HAPE) are characterised by an abnormal increase of pulmonary artery systolic pressure (PASP) in hypoxia and during normoxic exercise, reduced hypoxic ventilatory response, and smaller lung volume. In 37 mountaineers with well-documented altitude tolerance, it was investigated whether any combination of these noninvasive measurements, including exercise in hypoxia, could improve the identification of HAPE-susceptible subjects at low altitude. HAPE-susceptible subjects showed a significant higher increase of PASP during hypoxia at rest (48+/-10 mmHg) compared with controls (38+/-3 mmHg), as well as during normoxic exercise (57+/-14 versus 38+/-7 mmHg) and hypoxic exercise (69+/-13 versus 49+/-8 mmHg). PASP could not be assessed in three and eight subjects during normoxic or hypoxic exercise, respectively, due to insufficient Doppler profiles or systemic arterial hypertension. Sensitivity (77-94%) and specificity (76-93%) were not significantly different between the various testing conditions. Additional assessment of hypoxic ventilatory response and lung function parameters did not improve identification of HAPE-susceptible subjects in a multivariate analysis. Due to the greater number of missing values in pulmonary artery systolic pressure measurements during hypoxic exercise, it was concluded that pulmonary artery systolic pressure measurements at rest during hypoxia or exercise in normoxia are most feasible for the identification of high-altitude pulmonary oedema-susceptible subjects.

[Fibrinolytic therapy in thrombosis of a mitral valve prosthesis]. / Fibrinolytische Therapie bei Thrombose einer Mitralklappenprothese.

A 48-year-old woman presented with progressive dyspnea due to thrombosis of a mitral valve prosthesis. The patient had undergone mitral valve replacement (St. Jude Medical) six years prior to admission because of mitral stenosis (Class III); three years later the prosthesis had to be replaced (St. Jude Medical) because of valve thrombosis. At admission, transesophageal echocardiography showed a thrombus on the atrial side of the fixed valve leaflet and a thrombus (2.4 x 1.6 cm) floating from the left atrial roof. Because of the previous thoracotomies, thrombolysis was initiated despite the mobile thrombus with the attendant risk of embolization. Urokinase was infused in a dose to maintain the fibrinogen level around 100 mg/dl. After 24 h, the mean pressure gradient across the prosthetic mitral valve (measured by doppler echocardiography) had decreased from 23 to 11 mmHg. After 13 days of this modified thrombolytic regimen, the clinical symptoms of the patient had resolved and echocardiography showed a normal function of the prosthetic mitral valve without evidence of residual thrombosis. This patient demonstrates that prolonged cautious thrombolysis can be effective for the treatment of prosthetic valve thrombosis in hemodynamically moderately compromised patients.

Abnormal pulmonary artery pressure response in asymptomatic carriers of primary pulmonary hypertension gene.

BACKGROUND: Familial primary pulmonary hypertension (PPH) is an autosomal-dominant inherited disease with incomplete penetrance and poor prognosis. This study was performed to examine whether asymptomatic carriers of a mutated PPH gene can be identified at an early stage by their pulmonary artery systolic pressure (PASP) response to exercise. METHODS AND RESULTS: Stress Doppler echocardiography during supine bicycle exercise and genetic linkage analysis were performed on 52 members of 2 families with PPH. In 4 PPH patients, the mean PASP was increased at rest (73+/-16 mm Hg). Fourteen additional family members with normal PASP at rest revealed an abnormal PASP response to exercise (from 23+/-4 to 56+/-11 mm Hg) without secondary cause (abnormal response [AR] group). Twenty-seven other members (NR group) revealed a normal PASP response (maximal pressure <40 mm Hg) to exercise (from 24+/-4 to 37+/-3 mm Hg, P<0. 0001). All 14 AR but only 2 NR members shared the risk haplotype with the PPH patients. The molecular genetic analysis supported linkage to chromosome 2q31-32 with a logarithm of the odds score of 4.4 when the 4 patients and the 14 AR members were classified as affected. CONCLUSIONS: We conclude that the pathological rise of PASP in asymptomatic family members is linked to chromosome 2q31-32 and is probably an early sign of PPH. Therefore, stress Doppler echocardiography may be a useful tool to identify persons at risk for PPH even before pulmonary artery pressures at rest are elevated.

Stress Doppler echocardiography for identification of susceptibility to high altitude pulmonary edema.

OBJECTIVE: This prospective single-blinded study was performed to quantitate noninvasive pulmonary artery systolic pressure (PASP) responses to prolonged acute hypoxia and normoxic exercise. BACKGROUND: Hypoxia-induced excessive rise in pulmonary artery pressure is a key factor in high-altitude pulmonary edema (HAPE). We hypothesized that subjects susceptible to HAPE (HAPE-S) have increased pulmonary artery pressure response not only to hypoxia but also to exercise. METHODS: PASP was estimated at 45, 90 and 240 min of hypoxia (FiO2 = 12%) and during supine bicycle exercise in normoxia using Doppler-echocardiography in nine HAPE-S and in 11 control subjects. RESULTS: In the control group, mean PASP increased from 26+/-2 to 37+/-4 mm Hg (deltaPASP 10.3+/-2 mm Hg) after 90 min of hypoxia and from 27+/-4 to 36+/-3 mm Hg (deltaPASP 8+/-2 mm Hg) during exercise. In contrast, all HAPE-S subjects revealed significantly greater increases (p = 0.002 vs. controls) in mean PASP both during hypoxia (from 28+/-4 to 57+/-10 mm Hg, deltaPASP 28.7+/-6 mm Hg) and during exercise (from 28+/-4 to 55+/-11 mm Hg, deltaPASP 27+/-8 mm Hg) than did control subjects. Stress echocardiography allowed discrimination between groups without overlap using a cut off PASP value of 45 mm Hg at work rates less than 150 W. CONCLUSIONS: These data indicate that HAPE-S subjects may have abnormal pulmonary vascular responses not only to hypoxia but also to supine bicycle exercise under normoxic conditions. Thus, Doppler echocardiography during supine bicycle exercise or after 90 min of hypoxia may be useful noninvasive screening methods to identify subjects susceptible to HAPE.