CC Cardiol Croat Cardiologia Croatica Cardiol. Croat. 1848-543X 1848-5448 Croatian Cardiac Society CC_14(3-4)_73 10.15836/ccar2019.73 Extended Abstract Pulmonary arterial hypertension diagnosed during pregnancy – echocardiography as a tool for management ToplišekJanez1*GorjupVojka1LučovnikMiha1Stopar PintaričTatjana1BlajičIva1MušičŠpela1KrunićBarbara2ProkšeljKatja1Tadel KocjančičŠpela1Čopi JermanAnja1SalobirBarbara1 University Medical Centre Ljubljana, Ljubljana, Slovenia and University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia University Medical Centre Maribor, Maribor, Slovenia ADDRESS FOR CORRESPONDENCE: Janez Toplišek, Department of Cardiology, University Medical Centre Ljubljana, Zaloška 7, SI-1000 Ljubljana, Slovenia. / Phone: +386-1-522-35-42 / E-mail: jtoplisek@gmail.com 042019 14 3-4 73 73 2802201924032019 2019 Croatian Cardiac Society KEYWORDS: pulmonary arterial hypertensionpregnancyechocardiographyright ventricular function

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 period (1, 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).

Echocardiography variables before and after therapy.
sPAP mmHg 106 64
mPAP mmHg 64 42
PVR (WU) 10 7
RAP mmHg 3 3
SV ml 51 69
SVI ml/m2 29 40
CO L/min 4.6 4.7
CI L/min/m2 2.6 2.7
RA cm2 26 26
RV TAPSE cm 1.8 2.4
RV FAC % 24 30 (3D EF 36%)
RV MPI TDIRV free strain % 0.8-12 0.6-24
LV Eccentricity index 2.4 / 3.9 1.5 / 2.1
Pericardial effusion minimal minimal

sPAP = systolic pulmonary artery pressure, mPAP = mean pulmonary artery pressure, PVR = pulmonary vascular resistance, RAP = right atrial pressure, SV = stroke volume, SVI = stroke volume index, CO = cardiac output, CI = cardiac index, RA = right atrial area, RV = right ventricle, TAPSE = tricuspid annular plane systolic excursion, FAC = fractional area change, MPI TDI = myocardial performance index, 3D EF = three-dimensional ejection fraction, RV free strain = right ventricular free wall longitudinal strain.

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.

LITERATURE GalièNHumbertMVachieryJLGibbsSLangITorbickiAESC Scientific Document Group. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016 Jan 1;37(1):67–119. 10.1093/eurheartj/ehv31726320113 OlssonKMChannickR. Pregnancy in pulmonary arterial hypertension. Eur Respir Rev. 2016 Dec;25(142):431–7. 10.1183/16000617.0079-201627903665