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PAH Diagnosis During Pregnancy and Management: A Six Year Follow-up

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Sharon Jones

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When pulmonary arterial hypertension (PAH) occurs in pregnancy, the physiologic stress can overwhelm an already strained right ventricle, resulting in right heart failure and death. In general, pregnancy is contraindicated and patients are counseled to use two forms of birth control.

Purpose: To describe the teamwork involved in diagnosing PAH at 33 weeks gestation and both the acute and long-term management.

Background: When pulmonary arterial hypertension (PAH) occurs in pregnancy, the physiologic stress can overwhelm an already strained right ventricle, resulting in right heart failure and death. In general, pregnancy is contraindicated and patients are counseled to use two forms of birth control. Pregnancy in PAH carries a reported maternal mortality rate of 16.7% to 56%, although case reviews over the previous decade have reported rates, while still significant, as low as 11%. Perinatal risks are also high in ongoing pregnancies with spontaneous loss, poor fetal growth, and prematurity affecting up to 30% of cases.  

Methods: A 22 year old AB, gravida 1 para 0 at 33 weeks gestation presented with newly diagnosed PAH after a witnessed seizure. An interdisciplinary care plan was implemented by the PAH team and included maternal fetal medicine, anesthesia, neonatology, nursing, case management, social work, and critical care to orchestrate a maternal treatment plan and safe delivery. Delivery of a healthy 2120g baby was achieved 5/08. Management over the next 6 years by the PAH team is reviewed. 

Results:

Abstract Image - Sharon Jones

Conclusion: Healthcare coordination, leadership, and planning resulted in a successful outcome for an unplanned pregnancy of a newly diagnosed PAH patient. The PAH team, high risk obstetrical team, critical care team, neonatal team, and anesthesia delivered cohesive care to the PAH patient during the perinatal period through goal directed leadership and communications. Close follow up of the patient after discharge by the PAH team after a new diagnosis, provided a quality of life to allow the mother to care for her child. Goals of PAH management to slow the progression of disease and reduce worsening of symptoms through optimal therapy provides hope for a long term mother/child relationship.

Type: Case Study