Real-world characteristics and outcomes of patients with intermediate high risk acute pulmonary embolism.

BACKGROUND: Exact benefits of currently recommended close monitoring in intermediate high risk acute pulmonary embolism (PE) patients are unknown. METHODS: This prospective observational cohort study determined clinical characteristics, and disease course of intermediate high risk acute PE patients in an academic hospital setting . Frequency of hemodynamic deterioration, use of rescue reperfusion therapy and PE related mortality, were outcomes of interest. RESULTS: Of 98 intermediate high risk PE patients included for analysis, 81 patients (83%) were closely monitored. Two deteriorated hemodynamically and were treated with rescue reperfusion therapy. One patient survived after this. CONCLUSIONS: In these 98 intermediate high risk PE patients, hemodynamic deterioration occurred in three patients and rescue reperfusion therapy of two closely monitored patients led to survival of one. Underlining the need for better recognition of patients benefitting from and research in the optimal way of close monitoring.

Safety and efficacy of balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension in the Netherlands.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is an emerging treatment in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic disease (CTED). We describe the first safety and efficacy results of BPA in the Netherlands. METHODS: We selected all consecutive patients with inoperable CTEPH and CTED accepted for BPA treatment who had a six-month follow-up in the St. Antonius Hospital in Nieuwegein and the Amsterdam University Medical Center (UMC) in Amsterdam. Functional class (FC), N­terminal pro-brain natriuretic peptide (NT-proBNP), 6­minute walking test distance (6MWD) and right-sided heart catheterisation were performed at baseline and six months after last BPA. Complications for each BPA procedure were noted. RESULTS: A hundred and seventy-two BPA procedures were performed in 38 patients (61% female, mean age 65 ± 15 years). Significant improvements six months after BPA treatment were observed for functional class (63% FC I/II to 90% FC I/II, p = 0.014), mean pulmonary artery pressure (-8.9 mm Hg, p = 0.0001), pulmonary vascular resistance (-2.8 Woods Units (WU), p = 0.0001), right atrial pressure (-2.0 mm Hg, p = 0.006), stroke volume index (+5.7 ml/m2, p = 0.009) and 6MWD (+48m, p = 0.007). Non-severe complications occurred in 20 (12%) procedures. CONCLUSIONS: BPA performed in a CTEPH expert centre is an effective and safe treatment in patients with inoperable CTEPH.

Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.

Essentials Diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) is long. We explored healthcare utilisation of patients diagnosed with CTEPH after pulmonary embolism. A large number of physicians were consulted and test results were not always interpreted correctly. Better education and higher awareness of CTEPH may lead to faster diagnosis. SUMMARY: Background The median diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) is 14 months, which may affect prognosis. We aimed to explore the healthcare utilization of patients diagnosed with CTEPH after acute pulmonary embolism (PE), and to identify the causes of diagnostic delay. Methods We collected all data on patient symptoms, medical specialist referrals and ordered diagnostic tests to reconstruct the clinical pathways of 40 patients referred to the VU University Medical Center Amsterdam (VUMC, the Netherlands) for CTEPH treatment. Diagnostic delay was defined as the time between first symptom onset and referral to the VUMC. Correlations of patient-specific characteristics and diagnostic delay were evaluated. Results Patients consulted four (median) different physicians for a median of 13 (interquartile range [IQR] 10-18) consultations before the correct diagnosis was made. The median diagnostic delay was 21 months (IQR 12-49 months). Echocardiographic results suggestive of CTEPH were not always followed by an adequate work-up; most patients were not subjected to ventilation/perfusion scanning. Prior cardiopulmonary comorbidity and recurrent venous thromboembolism were predictors of a longer delay. Conclusion Healthcare utilization in patients before their final CTEPH diagnosis was far from optimal, contributing to a considerable diagnostic delay. Better education and higher awareness of CTEPH among PE caretakers may lead to faster diagnosis.

[Chronic thromboembolic pulmonary hypertension: recognition, diagnosis and treatment]. / Chronische trombo-embolische pulmonale hypertensie.

- Chronic thromboembolic pulmonary hypertension (CTEPH), characterised by pulmonary hypertension and persistent perfusion defects despite adequate anticoagulation, causes significant morbidity and mortality.- Persistent dyspnoea after acute pulmonary embolism is frequent and an indication for additional diagnostics. Only a minority of these patients develop CTEPH.- Echocardiography and perfusion scintigraphy are the cornerstone of diagnostics when suspecting CTEPH. Right-heart catheterisation and pulmonary angiography should confirm the diagnosis.- The diagnostic phase is preferably carried out in an expert centre in order to optimise the diagnosis and choose the optimal treatment for each individual patient.- Treatment of patients with CTEPH is a multidisciplinary team effort.- Pulmonary endarterectomy is the only potentially curative treatment; perioperative mortality is less than 5% in experienced centres. Inoperable patients can be treated with medication that specifically targets pulmonary arterial hypertension, but a survival benefit has not yet been shown for this medication.- Balloon pulmonary angioplasty has recently become available in the Netherlands as a treatment option, but the exact role of this new technique in the treatment of patients with CTEPH still needs to be investigated.

Aortic root asymmetry in marfan patients; evaluation by magnetic resonance imaging and comparison with standard echocardiography.

BACKGROUND: Patients with Marfan syndrome may develop aortic root dissection despite only mild aortic root dilation as shown by standard echocardiography, which may be due to aortic root asymmetry. Purpose of the present study was to investigate aortic root asymmetry by magnetic resonance (MR) imaging in patients with Marfan syndrome and to compare these measurements with standardly performed echocardiography. METHODS: Eighty-seven Marfan patients (mean age 31 +/- 8 years) underwent MR imaging. From this population, 15 patients (mean age 29 +/- 3 years) were selected in whom both echocardiography and MR imaging had been performed within 3 months. With echocardiography, the aortic root was measured according to the recommendations of the American Society of Echocardiography. With MR imaging, a short axis view of the aortic root was obtained to measure distances between the noncoronary, right coronary and left coronary cusps and the aortic root area. Correlations between aortic root area and diameters were assessed, and 95% confidence intervals (95% CIs) calculated. RESULTS: No difference in the standardly measured noncoronary to right coronary cusp diameter between MR imaging and echocardiography was shown (42 +/- 6 mm). Largest aortic root diameter on the MR images was the right to left coronary cusp diameter (46 +/- 7 mm, p < 0.02). For a given noncoronary to right coronary cusp diameter, 95% confidence intervals revealed a variation of -20 to +20% in the aortic root area. CONCLUSIONS: The majority of Marfan patients show asymmetric dilation of the aortic root by MR imaging. This phenomenon may go unnoticed when standard echocardiography is performed. The asymmetry of the aortic root might be of clinical importance in unexpected aortic root dissection.