Recent case reports have recognized chronic thromboembolic pulmonary hypertension (CETPH) as an important complication of slow-flow vascular malformations.1- 7 This month's issue of the Archives features the first case-control study exploring the prevalence of pulmonary hypertension (PH) in patients with extensive slow-flow vascular malformations.
In the presented case-control study, Rodríguez-Mañero et al8 evaluated 32 asymptomatic patients with extensive slow-flow vascular malformations (involving at least 15% of the body surface area) for evidence of PH using transthoracic echocardiography. Their most important finding is that in 22% of these patients the estimated pulmonary arterial systolic pressure (PASP) was 50 mm Hg or higher. Although the authors did not perform catheterization of the right side of the heart to confirm or rule out the presence of PH in their patients, this implies that in a considerable number of patients the diagnosis of PH is very likely.9 Moreover, another 50% of the evaluated patients had estimated PASP values of 37 to 50 mm Hg, which suggests that a diagnosis of PH is very possible. Thus, following international guidelines for diagnosis and treatment of PH in as many as 72% of the patients evaluated, further diagnostic workup is warranted.9,10
Clinical classification of pulmonary hypertension. (Simonneau et al11). ALK1 indicates activin receptor-like kinase type 1; BMPR2, bone morphogenetic protein receptor type 2; HIV, human immunodeficiency virus; and PAH, pulmonary arterial hypertension.
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