Partial anomalous pulmonary venous drainage and Mayer-Rokitansky-Küster-Hauser syndrome.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital agenesis of the uterus and vagina in women with normal ovaries, phenotype and karyotype.1,3 The associated lung malformations described are practically anecdotal.1–4 We report a case of associated partial anomalous pulmonary venous drainage. The patient is a 19-year-old woman with MRKH, who referred frequent colds and presented low-grade fever, purulent expectoration and dyspnea with minimal exertion. Physical examination was normal, except for mild tachypnea and bibasilar inspiratory rales. The work-up presented leukocytosis with neutrophilia and the arterial bloodgas showedpCO2 39andpO2 57. Chest radiograph and electrocardiogramwere normal. Thoracic computed tomography (CT) and magnetic resonance imaging (MRI) revealed partial anomalous pulmonary venous drainage: the vein of the upper left lobe was draining into the innominate vein through the vertical vein (Figs. 1 and 2). Transthoracic echocardiogram with contrast ruled out intracardiac communications as well as overload of the right cavities. MRKH is a rare malformation (incidence 1/4000 living newborns) characterized by congenital agenesis of the uterus and vagina in women with normal ovaries, secondary sex