Pediatr. praxi. 2021;22(1):62-66 | DOI: 10.36290/ped.2021.011

Acute cor pulmonale - current complications of cystic fibrosis

MUDr. Marcela Kreslová, Ph.D.¹, MUDr. Adéla Skalická¹, MUDr. Veronika Schwarzová¹, MUDr. Zuzana Horková¹, MUDr. Jiří Bejček, Ph.D.², prof. MUDr. Josef Sýkora, Ph.D.¹

¹ Dětská klinika, Univerzita Karlova, Lékařská fakulta v Plzni, Fakultní nemocnice v Plzni

² Klinika zobrazovacích metod, Univerzita Karlova, Lékařská fakulta v Plzni, Fakultní nemocnice v Plzni

Cystic fibrosis (CF) is a severe age-progressive autosomal recessively inherited multiorgan disease. The essence of the therapeutic approach to patients with CF is the effort to maximally compensate for the underlying disease, prevention and early treatment of complications. Acute exacerbations of the underlying disease or pneumonia are a common cause of respiratory deterioration, but it is also necessary to look for the rarer causes of dyspnea as a differential diagnosis. CF is one of the diseases with a higher risk of cardiovascular disease. The most common cardiovascular complication is cor pulmonale. Since adolescence, pulmonary embolism has been one of the causes of acute dyspnea, sometimes with an image of acute cor pulmonale, as evidenced by the presented case report of an 18.5-year-old girl.

Keywords: cystic fibrosis, children, cor pulmonale, pulmonary embolism, dyspnea.

Published: March 25, 2021  Show citation