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A Case Report of Surviving Pulmonary Arterial Hypertension in Pregnancy
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A7046 - A Case Report of Surviving Pulmonary Arterial Hypertension in Pregnancy
Author Block: M. Botros1, C. Daniels2; 1Internal Medicine, Ohio State University, Columbus, OH, United States, 2Cardiovascular Medicine, Ohio State University, Columbus, OH, United States.
Pulmonary arterial hypertension (PAH) is a disease defined hemodynamically by a mean pulmonary artery pressure (mPAP) >25 mmHg measured by right heart catheterization (RHC)1. Pregnancy in women with PAH is associated with a maternal mortality of 16–50%, with the majority of patient deaths occurring during labor and delivery or within the first post-partum month2-4. Death is usually secondary to cardiovascular collapse, which occurs due to physiologic post-partum changes including decreased preload, increased systemic vascular resistance and reduced ventricular contractility5. Our institution has previously defined a standardized multidisciplinary treatment approach to reducing mortality in pregnant PAH patients through a limited case series6. This approach included early recognition of PAH, contraceptive counseling, discontinuation of teratogenic medications in case of pregnancy, initiation and titration of selective pulmonary arterial vasodilators, anticoagulation, and development of a multidisciplinary delivery plan. The preferred method of delivery was planned vaginal delivery with assisted second stage given increased risk of mortality with caesarean section (CS) in these patients4. Here we describe an additional case of a 26 year old pregnant patient with a past medical history of PAH and autoimmune hepatitis with cirrhosis who required emergent CS due to pre-eclampsia and hemolysis elevated liver enzymes and low platelet count syndrome (HELLP). Using the aforementioned multidisciplinary approach, the patient survived a post-partum course complicated by disseminated intravascular coagulation (DIC), septic and cardiogenic shock.
1 Goldberg AB, Mazur W, Kalra DK. Pulmonary hypertension: diagnosis, imaging techniques, and novel therapies. Cardiovasc Diagn Ther 2017; 7:405-417
2 Weiss BM, Zemp L, Seifert B, et al. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998; 31:1650-1657
3 Thomas E, Yang J, Xu J, et al. Pulmonary Hypertension and Pregnancy Outcomes: Insights From the National Inpatient Sample. J Am Heart Assoc 2017; 6
4 Meng ML, Landau R, Viktorsdottir O, et al. Pulmonary Hypertension in Pregnancy: A Report of 49 Cases at Four Tertiary North American Sites. Obstet Gynecol 2017; 129:511-520
5 Geva T, Mauer MB, Striker L, et al. Effects of physiologic load of pregnancy on left ventricular contractility and remodeling. Am Heart J 1997; 133:53-59
6 Smith JS, Mueller J, Daniels CJ. Pulmonary arterial hypertension in the setting of pregnancy: a case series and standard treatment approach. Lung 2012; 190:155-160
Author Block: M. Botros1, C. Daniels2; 1Internal Medicine, Ohio State University, Columbus, OH, United States, 2Cardiovascular Medicine, Ohio State University, Columbus, OH, United States.
Pulmonary arterial hypertension (PAH) is a disease defined hemodynamically by a mean pulmonary artery pressure (mPAP) >25 mmHg measured by right heart catheterization (RHC)1. Pregnancy in women with PAH is associated with a maternal mortality of 16–50%, with the majority of patient deaths occurring during labor and delivery or within the first post-partum month2-4. Death is usually secondary to cardiovascular collapse, which occurs due to physiologic post-partum changes including decreased preload, increased systemic vascular resistance and reduced ventricular contractility5. Our institution has previously defined a standardized multidisciplinary treatment approach to reducing mortality in pregnant PAH patients through a limited case series6. This approach included early recognition of PAH, contraceptive counseling, discontinuation of teratogenic medications in case of pregnancy, initiation and titration of selective pulmonary arterial vasodilators, anticoagulation, and development of a multidisciplinary delivery plan. The preferred method of delivery was planned vaginal delivery with assisted second stage given increased risk of mortality with caesarean section (CS) in these patients4. Here we describe an additional case of a 26 year old pregnant patient with a past medical history of PAH and autoimmune hepatitis with cirrhosis who required emergent CS due to pre-eclampsia and hemolysis elevated liver enzymes and low platelet count syndrome (HELLP). Using the aforementioned multidisciplinary approach, the patient survived a post-partum course complicated by disseminated intravascular coagulation (DIC), septic and cardiogenic shock.
1 Goldberg AB, Mazur W, Kalra DK. Pulmonary hypertension: diagnosis, imaging techniques, and novel therapies. Cardiovasc Diagn Ther 2017; 7:405-417
2 Weiss BM, Zemp L, Seifert B, et al. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998; 31:1650-1657
3 Thomas E, Yang J, Xu J, et al. Pulmonary Hypertension and Pregnancy Outcomes: Insights From the National Inpatient Sample. J Am Heart Assoc 2017; 6
4 Meng ML, Landau R, Viktorsdottir O, et al. Pulmonary Hypertension in Pregnancy: A Report of 49 Cases at Four Tertiary North American Sites. Obstet Gynecol 2017; 129:511-520
5 Geva T, Mauer MB, Striker L, et al. Effects of physiologic load of pregnancy on left ventricular contractility and remodeling. Am Heart J 1997; 133:53-59
6 Smith JS, Mueller J, Daniels CJ. Pulmonary arterial hypertension in the setting of pregnancy: a case series and standard treatment approach. Lung 2012; 190:155-160
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