Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.

UNLABELLED: Essentials Predicting chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism is hard. We studied 772 patients with pulmonary embolism who were followed for CTEPH (incidence 2.8%). Logistic regression analysis revealed 7 easily collectable clinical variables that combined predict CTEPH. Our score identifies patients at low (0.38%) or higher (10%) risk of CTEPH. SUMMARY: Introduction Validated risk factors for the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE) are currently lacking. Methods This is a post hoc patient-level analysis of three large prospective cohorts with a total of 772 consecutive patients with acute PE, without major cardiopulmonary or malignant comorbidities. All underwent echocardiography after a median of 1.5 years. In cases with signs of pulmonary hypertension, additional diagnostic tests to confirm CTEPH were performed. Baseline demographics and clinical characteristics of the acute PE event were included in a multivariable regression analysis. Independent predictors were combined in a clinical prediction score. Results CTEPH was confirmed in 22 patients (2.8%) by right heart catheterization. Unprovoked PE, known hypothyroidism, symptom onset > 2 weeks before PE diagnosis, right ventricular dysfunction on computed tomography or echocardiography, known diabetes mellitus and thrombolytic therapy or embolectomy were independently associated with a CTEPH diagnosis during follow-up. The area under the receiver operating charateristic curve (AUC) of the prediction score including those six variables was 0.89 (95% confidence interval [CI] 0.84-0.94). Sensitivity analysis and bootstrap internal validation confirmed this AUC. Seventy-three per cent of patients were in the low-risk category (CTEPH incidence of 0.38%, 95% CI 0-1.5%) and 27% were in the high-risk category (CTEPH incidence of 10%, 95% CI 6.5-15%). Conclusion The 'CTEPH prediction score' allows for the identification of PE patients with a high risk of CTEPH diagnosis after PE. If externally validated, the score may guide targeting of CTEPH screening to at-risk patients.

Assessment of renal dysfunction improves troponin-based short-term prognosis in patients with acute symptomatic pulmonary embolism.

OBJECTIVE: Current risk stratification in acute pulmonary embolism (APE) includes assessment of clinical status, right ventricular overload and plasma troponin concentrations. As impaired renal function is one of the important predictors of mortality in cardiovascular diseases, we hypothesized that it is an independent early mortality marker in APE. MATERIAL AND METHODS: In prospective cohort study, we observed 220 consecutive patients (86M/134F, 64 +/- 18 years) with APE proven by spiral computed tomography (CT). On admission, echocardiography was performed and blood samples were collected for troponin and creatinine assays. RESULTS: The calculated glomerular filtration rate (GFR) differed significantly between 81 pts with low-, 131 pts with moderate- and 8 pts with high-risk APE [71 (19-181) vs. 55 (9-153) vs. 41 (14-68) mL min(-1); respectively P < 0.0001]. Twenty-three patients died during the 30-day observation. Importantly, GFR was lower in non-survivors than in survivors [35 (9-92) vs. 63 (14-181) mL min(-1), P < 0.0001]. The area under the curve (AUC) of the GFR receiver-operating characteristic (ROC) curve for predicting mortality was 0.760 (95% CI: 0.698-0.815). In multivariable analysis, independent mortality predictors were GFR, troponin, heart rate and history of chronic heart failure. In normotensive patients, the GFR and cardiac troponins (cTn) ROC curves for prediction of mortality showed no difference (AUC 0.789 and 0.781, respectively). However, Kaplan-Meier analysis showed an additive prognostic value of renal dysfunction. Thus, troponin-positive patients with a GFR < or = 35 mL mn(-1) showed 48% 30-day mortality, whereas troponin-positive patients with a GFR > 35 mL mn(-1) had 11% mortality, and troponin-negative patients with a GFR > 35 mL mn(-1) had good prognosis, P < 0.0001. CONCLUSION: Impaired kidney function, present in 47% of APE patients, is related to all-cause mortality. In initially normotensive patients, a GFR < 35 mL min(-1) predicts 30-day mortality. Moreover, GFR assessment can improve troponin-based risk stratification of APE.

Echocardiographic assessment of left ventricular morphology and function in patients with Emery-Dreifuss muscular dystrophy.

BACKGROUND: Emery-Dreifuss muscular dystrophy (EDMD) characterized by musculoskeletal abnormalities is often associated with atrioventricular conduction disturbances. Although some EDMD patients were reported to develop dilated cardiomyopathy, there are limited data on their left ventricular (LV) performance. METHODS: Therefore, we echocardiographically assessed 27 men (23 cases aged 26.4+/-6.8 years with X-linked, and four cases aged 22.2+/-8.6 years with autosomal dominant (AD)) EDMD. Control group included 16 male healthy controls aged 24.8+/-6.0 (18-37) years. RESULTS: Although LV end diastolic dimension was similar in EDMD and controls (4.9+/-0.6 and 4.99+/-1.1 cm, ns), dilated left ventricle was found in three X-linked EDMD subjects. LV ejection fraction was significantly reduced in EDMD (62.3+/-1% vs. 71.2+/-2%, p=0.01) and was below 50% in six (22.2%) X-linked EDMD patients. Doppler analysis disclosed prolonged isovolumetric relaxation time of the left ventricle in the studied group. This finding may indicate impaired LV relaxation. CONCLUSION: A significant subgroup of X-linked EDMD patients shows pronounced abnormalities of left ventricular function. This warrants cardiologic follow up of EDMD patients.

N-terminal pro-brain natriuretic peptide in patients with acute pulmonary embolism.

Plasma brain natriuretic peptide (BNP), released from myocytes of ventricles upon stretch, has been reported to differentiate pulmonary from cardiac dyspnoea. Limited data have shown elevated plasma BNP levels in acute pulmonary embolism (APE), frequently accompanied by dyspnoea and right ventricular (RV) dysfunction. The aim of this study was to assess plasma N-terminal proBNP (NT-proBNP) in APE, and to establish whether it reflects the severity of RV overload and if it can be used to predict adverse clinical outcome. On admission, NT-proBNP and echocardiography for RV overload were performed in 79 APE patients (29 males), aged 63 +/- 16 yrs. Plasma NT-proBNP was elevated in 66 patients (83.5%) and was higher in patients with (median 4,650 pg x mL(-1) (range 61-60,958)) than without RV strain (363 pg x mL(-1) (16-16,329)). RV-to-left ventricular ratio and inferior vena cava dimension correlated with NT-proBNP. All 15 in-hospital deaths and 24 serious adverse events occurred in the group with elevated NT-proBNP, while all 13 (16.5%) patients with normal values had an uncomplicated clinical course. Plasma NT-proBNP predicted in-hospital mortality. Plasma N-terminal pro-brain natriuretic peptide is elevated in the majority of cases of pulmonary embolism resulting in right ventricular overload. Plasma levels reflect the degree of right ventricular overload and may help to predict short-term outcome. Acute pulmonary embolism should be considered in the differential diagnosis of patients with dyspnoea and abnormal levels of brain natriuretic peptide.

Visualization of the central pulmonary arteries by biplane transesophageal echocardiography.

It is suggested that transesophageal echocardiography (TEE), by detecting thromboemboli in the proximal parts of the pulmonary arteries, is useful in the diagnosis of pulmonary embolism. However, the data on visualization of the pulmonary arteries are limited. The extent of the pulmonary arteries that can be precisely visualized during biplane TEE was assessed in 51 consecutive patients (23 female, 28 male, aged 56.6+/-12.5 years) without structural heart disease. The main pulmonary artery and the right pulmonary artery were detected in 96.1% and 94.1% of patients, respectively. Although the proximal part of the left pulmonary artery was found in only 47.0% of patients, its distal part was visualized in 92.2%. During TEE, proximal parts of the lobar arteries on both sides were visualized in 88.2% of patients. Thus, the central pulmonary arteries including proximal parts of the lobar branches can be precisely visualized by biplane TEE in the majority of patients. Only the proximal part of the left pulmonary artery is difficult to assess.