Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is a complex disorder in which pulmonary artery (PA) obstruction leads to elevated PA resistance and right ventricular (RV) failure. Elevation of the pulmonary arterial blood pressure (PABP) is correlated with progressive damage to the pulmonary artery. Before development of surgery for ventricular septal defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA), most patients died around the age 40, with right-sided cardiac failure being the main cause of death. Deterioration in pregnancy is reported to occur in the second or third trimester and in the postpartum period. Sudden hemodynamic instability is a common cause of death. The risk of cardiac failure during and after pregnancy increases, and sudden cardiac arrest may occur during Cesarean section or soon after birth. The rate of maternal death in pregnancies complicated by PAH is variably reported to be 20–60%. Predictors of cardiac failure during pregnancy are elevated PABP, elevated brain natriuretic peptide (BNP), and elevated RV size. Current PAH-specific therapies have affected survival in nonpregnant PAH patients, and it is important to accumulate data from multiple PAH centers. Prostacyclins became available for PAH treatment in 1996 to the United States and Europe and in 1999 in Japan. Therefore, in this chapter, we classify studies on this condition temporally into pre- and post-PAH therapeutic eras.
KeywordsPregnancy Pulmonary arterial hypertension Pulmonary vascular disease Right ventricular failure
- 3.Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jaïs X, Humbert M, Audibert F, Frydman R, Simonneau G, Benhamou D (2005) Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology 102(6):1133–1137. discussion 5A–6ACrossRefGoogle Scholar
- 13.Regitz-Zagrosek V, Lundqvist CB, Borghi C, Cifkova R, Ferreira R, Foidart JM, Gibbs JSR et al (2011) ESC guidelines on the management of cardiovascular diseases during pregnancy: the task force on the Management of Cardiovascular Diseases during pregnancy of the European Society of Cardiology (ESC). Eur Heart J 32(24):3147–3197CrossRefGoogle Scholar
- 32.McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA et al (2009) ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation task force on expert consensus documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 53(17):1573–1619CrossRefGoogle Scholar
- 40.Kiely DG, Elliot CA, Wilson VJ, Stewart P (2006) Pregnancy and pulmonary hypertension: new approaches to the management of a life threatening condition. In: Steer PH, Gatzoulis MA, Baker P (eds) Heart disease and pregnancy. RCOG Press, London, pp 211–229Google Scholar