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Pregnancy Outcomes in Patients With Severe Pulmonary Hypertension and Eisenmengerization Treated With Sildenafil Monotherapy

Reinalyn Cartago*, MD; Pichy Ann Alan, MD; Jubert Benedicto, MD
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Section of Pulmonary Medicine Department of Internal Medicine University of the Philippines - Philippine General Hospital, Metro Manila, Philippines


Chest. 2012;142(4_MeetingAbstracts):999A. doi:10.1378/chest.1388298
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Abstract

SESSION TYPE: Miscellaneous Global Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Pregnancy outcomes in pulmonary hypertension - primary or associated with Congenital Heart Disease (CHD) - have not been documented in our local setting; the role of anti-pulmonary hypertensive treatment for right ventricular failure secondary to cardiac condition is yet to be studied. In our institution, access to therapies for Pulmonary Vascular Disease (PVD) is limited. Only oral sildenafil was available. We report 2 cases of pregnancy with severe pulmonary hypertension from Eisenmengerization, treated with Sildenafil monotherapy.

CASE PRESENTATION: CASE 1 I.D., 33/F with atrial septal defect (ASD) - on second pregnancy at 34 weeks age of gestation (AOG) - came in with uterine contractions. Patient had cyanosis when crying during childhood; denied exertional dyspnea. First pregnancy allegedly uneventful. Exertional dyspnea started 16 weeks AOG. Echocardiography confirmed 1.3cm (ASD); pulmonary artery pressure (PAP) 122mmHg. Patient not in cardiorespiratory distress, stable vital signs, oxygen saturation of 81% - 89% at 10LPM; with grade 3/6 systolic murmur on 2nd to 4th intercostal space right parasternal border; split second heart sound, palpable pulmonic component, with RV heave; clear breath sounds; clubbing of fingers and toe nails. Patient delivered (cesarean for malpresentation) live preterm baby with APGAR 9, 9; transferred to Intensive Care Unit, intubated, hemodynamically stable, with oxygen saturation of 99% at FiO2 30%. Sildenafil started 50mg/tab thrice daily and Digoxin 0.125 mg once daily. Patient extubated, discharged, off oxygen support. CASE 2 M.V., 28/F nulligravid 29 weeks AOG; with progressive dyspnea, started 16 weeks AOG. Since childhood, patient had easy fatigability, clubbing of fingers and toes and exercise intolerance. Echocardiography showed 3cm ASD; PAP 125mmHg. Patient tachypneic at 24 cycles per minute, otherwise hemodynamically stable; clear breath sounds, split second heart sound and systolic murmur left parasternal border. Oral Sildenafil started 25mg three times daily and Aspirin at 80mg once daily. Patient delivered (assisted vaginal delivery; epidural anesthesia) a live baby APGAR 9, 9; discharged hemodynamically stable with room air oxygen saturation of 94%, on sildenafil 25 mg thrice daily.

DISCUSSION: Pulmonary Arterial Hypertension (PAH) is a known complication of uncorrected increased pulmonary blood flow associated with CHD such as ASD. Secundum type ASD occurs in 10% of pregnant patients with congenital heart disease1. Approximately 10% of patients with ASD develop PVD. The most appropriate study for patients suspected to have pulmonary hypertension is echocardiogram, with complete right heart catheterization for confirmation2. In our patients, only echocardiogram was done due to limited resources. Pregnancy with pulmonary hypertension and eisenmengerization is associated with significant maternal and fetal risk. Termination of pregnancy is preferred; however, if pregnancy continued, hospitalization is necessary with continuous oxygen administration and pulmonary vasodilator treatment. Since hypoxemia is a potent pulmonary vasoconstrictor, supplementation is recommended to maintain oxygen saturation greater than 90%3. According to ACCF, the only disease-specific targeted PAH therapy effective in patients with PAH associated with CHD is bosentan2. In our literature reviews, PAH during pregnancy is treated with anti-pulmonary hypertensive treatment with sildenafil as adjunct. Currently, efficacy of Sildenafil monotherapy in patients with PAH/CHD requires further studies1.

CONCLUSIONS: Our cases demonstrated that Sildenafil monotherapy may improve clinical outcomes of both mother and baby. Oral sildenafil at a dose of up to 50 mg four times per day has been shown to improve both hemodynamics and oxygenation in patients with pulmonary arterial hypertension.

1) Lacassie et al., “Management of Eisenmenger Syndrome in Pregnancy with Sildenafil and L-arginine.” American College of Obstetricians and Gynecologists. Vol 103, No 5, Part 2. May 2004

2) ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. JACC Vol. 53, No. 17, April 28, 200

3) Gleicher et al., “Eisenmenger’s syndrome and Pregnancy”. Obstet Gynecol Surg 1979; 34:721-741.

DISCLOSURE: The following authors have nothing to disclose: Reinalyn Cartago, Pichy Ann Alan, Jubert Benedicto

No Product/Research Disclosure Information

Section of Pulmonary Medicine Department of Internal Medicine University of the Philippines - Philippine General Hospital, Metro Manila, Philippines

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