Chronic pulmonary embolism in Klippel-Trenaunay syndrome - 16/12/11
⁎ 
, Charlène E.U. Oduber, MD, PhD b, , Victor E.A. Gerdes, MD, PhD a, i, Otto M. van Delden, MD, PhD c, Berthe L.F. van Eck-Smit, MD, PhD d, Joost C.M. Meijers, PhD e, Eduard J. van Beers, MD, PhD f, Berto J. Bouma, MD, PhD g, Chantal M.A.M. van der Horst, MD, PhD b, Paul Bresser, MD, PhD h, jReprint requests: Renée A. Douma, MD, Department of Vascular Medicine, Academic Medical Center, Suite F4-143, Meibergdreef 9, PO Box 22700, 1100 DE Amsterdam, The Netherlands.Abstract |
Background |
Klippel-Trenaunay syndrome (KTS) is characterized by vascular malformations and disturbed soft tissue or bony growth, involving one or more extremities. A high incidence of venous thromboembolism (VTE) has been reported in this disorder, along with cases of belated diagnosed chronic thromboembolic (CTE) pulmonary hypertension (CTEPH). We performed a cross-sectional study to investigate the prevalence of CTE in patients with KTS.
Methods |
Those from our KTS patient cohort willing to participate were examined with a sequential diagnostic workup including perfusion scintigraphy, computed tomography, and echocardiography.
Results |
Of 68 patients, 48 patients participated in the study (median age 43 years; 29 [60%] were female). Eleven patients (23%) had an abnormal perfusion scan result, of whom computed tomographic scanning showed signs of CTE in two patients (4.2%; 95% confidence interval [CI] 1.2%-14%); both patients had a history of VTE. Echocardiography showed no signs of CTEPH in these patients. In total, 23 patients (48%; 95% CI 35%-62%) had a history of superficial vein thrombosis and 8 patients (17%; 95% CI 8.7%-30%) had a history of deep vein thrombosis or pulmonary embolism, which was associated with more shortness of breath.
Limitations |
Echocardiography was only performed in patients with CTE.
Conclusion |
A large proportion of patients with KTS had a history of VTE. The prevalence of CTE in the total KTS cohort, however, appeared less alarming than previously assumed. Based on these results, we suggest that there is only a limited indication for CTEPH screening among patients with KTS. Nevertheless, awareness for CTEPH remains appropriate, especially among patients presenting with shortness of breath and a history of VTE.
Le texte complet de cet article est disponible en PDF.Key words : chronic thromboembolic pulmonary hypertension, chronic thromboembolism, Klippel-Trenaunay syndrome, pulmonary embolism, vascular malformations, venous thromboembolism
Abbreviations used : CI, CT, CTE, CTEPH, DVT, KTS, PE, SVT, VTE
Plan
| Funding sources: None. |
|
| Conflicts of interest: None declared. |
Vol 66 - N° 1
P. 71-77 - janvier 2012 Retour au numéro
Article précédent
- Compliance with sunscreen advice in a survey of adults engaged in outdoor winter recreation at high-elevation ski areas
- David B. Buller, Peter A. Andersen, Barbara J. Walkosz, Michael D. Scott, Julie A. Maloy, Mark B. Dignan, Gary R. Cutter
- Characteristics of medical professional liability claims against dermatologists: Data from 2704 closed claims in a voluntary registry
- Alan N. Moshell, Parul Divya Parikh, William J. Oetgen
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?