Pulmonary arterial hypertension diagnosed during pregnancy – echocardiography as a tool for management

Introduction: Despite advanced therapies for pulmonary arterial hypertension (PAH), maternal mortality in women with PAH and their offspring remains high (30-56% and 11-28%) and is especially high during the post-partum period1,2. Case report: 39-years-old woman was presented in 24th week of pregnancy with moderate dyspnea and cyanosis without peripheral edema. ECG showed right ventricular strain and NT-proBNP was 1300 ng/L. Echocardiogram showed severe precapillary pulmonary hypertension with systolic pulmonary pressure (PAP) 103 mmHg, reduced stroke volume (SVI 29 ml/m2), normal cardiac output (CI 2.6 L/min/m2) and mildly reduced systolic function of the right ventricle (FAC 30%, PMI TDI 0.8) with normal central venous pressure (CVP) 3 mmHg. Right heart catheterization confirmed echocardiographic hemodynamic measurements (mean pulmonary pressure 61 mmHg, pulmonary vascular resistance (PVR) 9.4 WU) and the vasoreactivity test was negative. Epoprostenol was initiated, titrated up to 22 ng/kg/min and on 30th week planned caesarean section was performed with NO inhalations, noradrenalin and dobutamine. Fortunately, standby supportive therapy - ECMO and high urgency lung transplantation, was not needed. The baby did well. The patient was extubated on the same day and sildenafil was added. The patient was discharged after 4 weeks. After 2 months epoprostenol was switched to treprostinil up to 26 ng/kg/min and macitentan was added. NT-proBNP stabilized at 170 ng/L. Echocardiography was performed weekly to monitor CI, CVP and right systolic function indexes. Favorable echocardiographic dynamics after therapy were observed: mean PAP 60 mmHg to 45-50 mmHg, PVR from 10 WU to 7 WU, stroke volume normalized and CVP remained normal. However, right ventricular systolic function improved but did not normalize (Table 1). In conclusion, with complete echocardiographic hemodynamic assessment and thorough clinical assessment high-risk pregnancies in patients with severe PAH can be managed. In addition, FAC, MPI TDI, 3D ejection fraction and RV free strain better assess right ventricular systolic function than TAPSE

Presaditev pljuč v Sloveniji – izkušnje prvih treh let

Izhodišča: Presaditev pljuč je zahtevna metoda zdravljenja za izbrane bolnike z napredovalo pljučno boleznijo. Naraščanje števila primernih kandidatov in standarizacija posega sta v letu 2018 omogočila vzpostavitev transplantacijskega centra v UKC Ljubljana. Prispevek poroča o prvih rezultatih. Metode: Opravili smo analizo lastnega registra bolnikov. Rezultate smo primerjali s skupino bolnikov, ki so jim pred tem opravili presaditev v AKH Dunaj. Rezultati: Med 15. 9. 2018 in 15. 9. 2021 smo opravili 37 presaditev pljuč (od tega pri 13 ženskah). Indikacije so bile KOPB (n = 13, 35 %), pljučne fibroze (n = 7, 19 %), cistična fibroza (n = 5, 14 %), kovidna pljučnica (n = 4, 11 %), bronhiektazije (n = 3, 8 %), pljučna hipertenzija (n = 2, 5 %), limfangioleiomiomatoza (n = 1, 3 %), pomanjkanje alfa-1-antitripsina (n = 1, 3 %) in bronhiolitis (n = 1, 3 %). V primerjavi s prejšnjo skupino bolnikov, napotenih v AKH Dunaj (71 bolnikov, od tega 35 žensk), je bil delež bolnikov s KOPB višji (35 % in 16 %, p = 0,019), bolniki pa so bili starejši (mediana [razpon], 59 [14–68] in 43 [4–58] let, p = 0,001). 76 % darovanih pljuč ni ustrezalo standardnim merilom (30 % darovalcev je bilo starejših od 55 let, 22 % jih je imelo infiltrate na rentgenogramu pljuč, 24 % pa ni izpolnjevalo enega od ostalih meril), v 43 % pa je bila potrebna redukcija velikosti, kar pa ni vplivalo na primarno odpoved presadka. Kratkoročno preživetje je bili podobno v obeh skupinah (1-letno preživetje v UKC Ljubljana 89 % [95 % CI 78–99 %] in v AKH Dunaj 83 % [95 % CI 74–92 %]). Zaključek: Prvi rezultati kažejo, da so zgodnji izidi po presaditvi pljuč primerljivi pri bolnikih, ki so imeli presaditev v UKC Ljubljana ali v AKH Dunaj