PULMONARY STENOSIS IN PREGNANCY Pulmonary Stenosis ! Primarily congenital ! ~10-12% of CHD in adults ! 80-90% of all RVOT obstruction lesions Hoffman JIE. Postnat Incid Pediatr Cardiol 1995;16:103-113 Pulmonary Stenosis Severity Gradient Mild <30 mm Hg Moderate 30-50 mm Hg Severe >50 mm Hg ACC/AHA Guidelines for CHD. J Am Coll Cardiol 2008;52:e143-263 Valve Disease in Pregnancy -Case- ! 23 YO HF G3P1 @14 weeks ! Referred with Hx of “cardiac disease” since the age of 15 ! NYHA functional Class I ! Echocardiogram -Severe pulmonic stenosis PV mean gradient - 53 mmHg, peak gradient - 122 mmHg Pulmonary Stenosis in Pregnancy -Case! 23 YO HF G3P1 @14 weeks ! Referred with Hx of “cardiac disease” since the age of 15 ! NYHA functional Class I ! Echocardiogram -Severe PS mean gradient - 51 mm Hg peak gradient - 110 mmHg Pulmonary Stenosis Natural History ! Young adults usually asymptomatic ! Normal functional capacity ! Commonly diagnosed in pregnancy Perloff JK. Clin Cardiol 1994 Pulmonary Stenosis ! Symptoms: " Decreased preload or low SVR - dyspnea, fatigue, exertional lightheadedness/syncope ! Echocardiogram: " Tricuspid regurgitation, RV failure " PFO – right to left shunt Second Natural History Study of CHD ! 586 patients with PS "  25 year survival 96% "  <20% require valvotomy "  4% need reoperation "  Infective endocarditis uncommon "  Pulmonary balloon valvotomy preferred O’Fallon WM et. al. Circulation 1993;87 SuppI:I1-126 Pulmonary Stenosis in Pregnancy -Case! Pregnancy course : fatigue in late second trimester (NYHA II) ! Spontaneous labor at 38 weeks with uncomplicated intrapartum and postpartum course ! Underwent catheter balloon valvuloplasty 3 months postpartum ACC/AHA Practice Guidelines Focused Update VHD 2008 ! Class I "  Balloon valvotomy recommended for asymptomatic patients with domed pulmonary valve with peak instantaneous gradient of >50 mm Hg or mean doppler gradient >30 mm Hg with less than moderate pulmonary regurgitation J Am Coll Cardiol 2008;50:e1-142 ACC/AHA Practice Guidelines Focused Update VHD 2008 ! Class I "  Balloon valvotomy recommended for symptomatic patients with domed pulmonary valve with peak instantaneous gradient of >60 mm Hg or mean doppler gradient >40 mm Hg with less than moderate pulmonary regurgitation J Am Coll Cardiol 2008;50:e1-142 Pulmonary Stenosis ! Drenthen et. al. "  100 cases of pulmonary stenosis "  Maternal outcomes "  No increase in • Arrhythmias • Heart failure • Endocarditis JACC 2004 Non Cardiac Complications in Pulmonary Stenosis ! National Registery Netherlands ! 51 women – 108 pregnancies Uncorrected PS " 21 (19%) miscarriages " 81 included in the study Drenthen et. al. Heart 2006;92:1838-43 Non Cardiac Complications in Pulmonary Stenosis ! 81 included in the study "  Hypertension (15%) 12 •  4 preeclampsia, 2 eclampsia "  Preterm delivery "  Thromboembolism "  Neonatal mortality 14 (17%) 3 (3.7%) 4 (4.8%) Drenthen et. al. Heart 2006;92:1838-43 Cardiac Complications in Pulmonary Stenosis ! Literature review 2005-2006 ! 2491 pregnancies with CHD ! >100 cases of PS "  Arrhythmias 0% (n=100) "  Heart failure 0% (n=110) "  CV events 0% (n=123) "  Endocarditis 0% (n=102) Drenthen et. al. J Am Coll Cardiol 2007;49:2303-11 Obstetrical Complications in Pulmonary Stenosis ! >100 cases of PS (6 publications) "  PIH 7.7% (n=91) "  Preeclampsia 4.9% (n=81) "  TEC 2.5% (n=81) "  PROM 6.2% (n=81) "  PTL 13.5% (n=81) "  PPH 14.3% (n=91) Drenthen et. al. J Am Coll Cardiol 2007;49:2303-11 Offspring Complications in Pulmonary Stenosis ! >100 cases of PS (6 publications) "  PTD 13.5% (n=110) "  SGA 10% (n=110) "  IUFD 0.8% (n=123) "  PNM 4.1% (n=123) "  CHD 2.8% (n=104) Drenthen et. al. J Am Coll Cardiol 2007;49:2303-11 Pregnancy Outcomes in Pulmonary Stenosis ! Case-control study 1995-2007 ! Isolated pulmonary stenosis in pregnancy vs. healthy pregnant controls Hameed et. al. Am Heart J 2007 Pregnancy Outcomes in Pulmonary Stenosis ! 17 subjects vs. 17 controls ! Matched for "  Age "  Ethnicity "  Obstetrical history "  Year of delivery Hameed et. al. Am Heart J 2007 Pregnancy Outcomes in Pulmonary Stenosis ! Isolated pulmonary stenosis ! Normal right ventricular function ! Asymptomatic or mildly symptomatic at the time of presentation Hameed et. al. Am Heart J 2007 Pregnancy Outcomes ! Maternal outcomes "  Functional class "  Use of medications "  Cardiac complications ! Fetal outcomes "  Gestational age at delivery "  Apgar score at 1 and 5 minutes "  Birth weight "  Placental weight Hameed et. al. Am Heart J 2007 Fetal Outcomes Patients EGA (wk) Apgar @ 1 min Apgar @ 5 min Birth wt (g) Placental wt (g) Controls 38.4 ± 1.9 39.3 ± 1.2 9 (8-9) 9 (8-9) 9 (8-10) 9 (8-9) 3278 + 474 3360 + 432 648 ± 184 693 ± 421 Hameed et. al. Am Heart J 2007 Fetal Outcomes Stratified by Severity Peak Gradient (mmHg) < 50 (34 ± 11) ! 50 (82 ± 28) Birth weight (g) EGA (wk) Apgar score @ 1 min Apgar score @ 5 min Placental weight (g) 3192 ± 553 37.5 ± 2.2 9 (8-9) 9 (8-10) 597 ± 150 3289 ± 426 39.5 ± 1.6 9 (8-9) 9 (8-9) 681 ± 205 Hameed et. al. Am Heart J 2007 P-value 0.99 0.17 0.2 0.98 0.38 Maternal Outcomes ! NYHA class @ presentation "  I: 11 (65%) "  II: 6 (35%) ! Fifteen (88%) stable through pregnancy "  One from I >>>II "  One from II >>>III>>>II Hameed et. al. Am Heart J 2007 Maternal Outcomes ! Majority had uncomplicated vaginal delivery (94%) ! One c-section for fetal indications ! Uncomplicated intrapartum and postpartum course in both groups Hameed et. al. Am Heart J 2007 Conclusions ! Preconception counseling is important ! Isolated pulmonary stenosis is tolerated well during pregnancy Conclusions ! Catheter balloon valvotomy remains a viable option in refractory cases during pregnancy [email protected]