A Challenging Case: Von Willebrand Disease and Pulmonary Hypertension in Pregnancy

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1 A Challenging Case: Von Willebrand Disease and Pulmonary Hypertension in Pregnancy Diana S. Wolfe, MD, MPH Assistant Professor Department of Obstetrics & Gynecology and Women s Health Associate Fellowship Director Maternal Fetal Medicine

2 Conflicts of Interest & Disclosures None

3 Patient DS 21 yo G1P0 who was referred from a midwife at 14+3 wks dated by 7 wks US to MFM practice for a prior medical history significant for vwd Type 1.

4 Patient DS vwf Disease, Type I Dx in childhood after frequent nosebleeds Never received any medications 2013 vwf activity ranged from 30-40% Current VWD panel activity 53% antigen 73% factor VIII 69%

5 CXR, 2010 Incidental Finding of Cardiomegaly Patient had workup in 2010 for chest pain and murmur CXR - moderate cardiomegaly and dilatation of the central pulmonary arteries Echo - overall normal Referred to the joint MFM-Cardiology program for further evaluation

6 Chest X-ray from 2010

7 MFM Cardiology Joint Program at The Hutch

8 Cardiac History Cardiac Symptoms Episodes of Shortness of Breath as a child Presumed due to Asthma Episode of Chest Pain CXR & ECHO No syncope No palpitations NYHA I/II

9 Physical Exam Vital signs: Ht 5 4 Wt 152lb BMI 26.1 kg/m 2 BP 102/57 mmhg, HR 70, SpO2 100% RA Constitutional: Well developed, well nourished, NAD HENT: NCAT, MMM Eyes: EOMI Neck: Trachea midline. No JVD. Cardiovascular: Regular rate and rhythm with no murmurs, rubs or gallops. No pedal edema Pulmonary/Chest: Clear to auscultation; no crackles, rhonchi, or wheezing Abdominal: Normal bowel sounds, soft, non-tender and nondistended. Gravid abdomen Musculoskeletal: Full range of motion Neurological: AAOX 3, remainder grossly intact Psychiatric: Alert and cooperative, normal mood and affect

10 EKG EKG

11 Transthoracic Echocardiogram

12 Transthoracic Echocardiogram

13 Variable s Baseline RA 5 (wnl) --- Right Heart Catherization ino 40 PPM/100% FIO2 PA 70/22/45 53/21/35 (Borderline Response) W 5 5 TPG Ao Sat CO/I (fick) 9.72/5.24 (wnl) 13.5/7.3 PVR

14 What Level of Care is Needed for This Cardiac Pregnant Patient? Delivery Plan Anesthesia Plan Intrapartum and Postpartum Monitoring

15 Intrapartum Changes Cardiac Output- Rise in Labor Oxygen demand increases 3- fold Contractions ml of Blood transfer from Uterus to Circulation BP changes mm Hg CD is associated with hemodynamic changes

16 Peripuerium in C.O. within 15 minutes of Delivery Relief of caval compression Autotransfusion of uteroplacental blood Rapid mobilization of extravascular fluid Venous Return & Stroke Volume

17 Pulmonary Hypertension

18 Mean arterial pressure (mm Hg) Gestational Age-specific Changes in Mean Arterial Pressure % increase in labor Nadir at weeks Back to baseline at term Rapid resolution postpartum 50 First trimester Second trimester Third trimester Labor Puerperium Norwitz ER, Schorge JO. Obstetrics & Gynecology at a Glance

19 Interdisciplinary Team MFM Cardiology L&D Nursing Blood Bank NICU CT Surgery Anesthesia Patient Safety

20 Interdisciplinary Office IDT meeting in CICU MFM NICU Blood Bank Cardiologist OB Anesthesia L&D Nursing CT Surgery OR Nursing Cardiac Anesthesia Perfusion Specialists

21 Checklist for MFM-Cardiology IDT Meeting

22 Ms DS: Delivery Plan Admit the patient to the CICU for IV prostacyclins to lower her PAP Delivery close to 37 wks Sildenafil and Epoprosterol while RVP monitoring with Swan Ganz Catheter Titration was escalated daily A total of 16 days was required to optimize her PAP, maximum dose of 27 ng/kg

23 Delivery Day Upon placement of her Swan Ganz Catheter in preparation for OR, the vessels were found to be collapsed and insertion was a challenge Development of Atypical preeclampsia HELLP syndrome

24 Incomplete Trophoblastic Invasion

25 IDT meeting in CICU OR nursing, CT Surgery, Perfusion Specialists, OB & Cardiac Anesthesia OR- a total of five teams MFM, Obstetric & Cardiac Anesthesia, Cardiothoracic Surgery, Heart Failure, and Neonatology Blood Bank- DDAVP and blood products taken to the OR No Antenatal Corticosteroids- risk of maternal fluid shifts ECMO was available on standby General anesthesia - due to her platelet dysfunction and inability for regional anesthesia

26 Maternal & Fetal Outcome Female neonate, Apgars 9 and 9, birthweight 3135 grams Contraception A 5-year progesterone Intra Uterine Device (IUD) was placed immediately after delivery Long Acting Reversible Contraception (LARC)

27 Contraceptive Eligibility WHO Medical eligibility criteria for contraceptive use Fifth edition 2015

28 Take Home Message Risk Assessment is Essential Induction, Management of Labor & Delivery, and Post-Partum surveillance A Multidisciplinary Team is Required European Heart Journal 2011;32:

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30 Acknowledgements