Madhva Prasad S, Gupta AS**
(* Assistant Professor, ** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)
A 20 year old G2 A1 presented at 27 weeks with sudden breathlessness and was found to have severe pulmonary hypertension. She was managed with diuretics and required sildenafil. She was anti-β2 glycoprotein 1 positive. She went into spontaneous preterm labor. Maternal outcome was good. A successfully managed parturient with severe pulmonary hypertension and related issues are discussed here.
Pulmonary hypertension as a consequence of preexisting cardiac valvular disorders is known as Eisenmenger syndrome. However, when it occurs with no other coexisting cardiac lesions, it is called primary pulmonary hypertension. It is a disorder which portends poor maternal and neonatal outcomes.
A 20 year old woman married for 2 years gravida 2 abortion 1 with 27 weeks gestation presented to the emergency with complaints of worsening breathlessness over 2 days and occasional pain in the abdomen. She was apparently asymptomatic till 2 days prior and currently had dyspnea on exertion (NYHA grade III). She did not have any swelling, decreased urine output, palpitations, faintness, cough or fever. She was registered with a private practitioner and had 2 antenatal visits. Ultrasonography at 18 weeks showed no malformations but oligohydramnios, for which no specific therapy had been initiated. On arrival, she was evaluated and found to be uncomfortable at rest. She appeared to have peripheral cyanosis. Pulse was 98 beats per minute, blood pressure was 100/60 mm Hg and on auscultation S1 and S2 were well heard, and no murmurs were audible. Respiratory rate was 24 per minute and oxygen saturation on finger probe was 83%. Abdominal examination showed relaxed uterus of 26 weeks gestation with audible fetal heart sounds. On per vaginal examination, internal os was closed and cervical length was adequate. ABG revealed oxygen saturation of 91% (on oxygen). Immediately, nasal oxygen and diuretics (tablet furosemide 40 mg) were started. Cardiologist evaluated her and did her echocardiography. It showed a pulmonary artery pressure of 120 mm Hg, suggestive of pulmonary hypertension and ejection fraction of 50 %. It also showed, “D” shaped left ventricle, right atrial and right ventricular volume overload and moderate pulmonary regurgitation. Diuretics were continued and antibiotics were started. Cardiologist started tablet sildenafil 10 mg twice a day for pulmonary hypertension. Iron and calcium were continued. Over the next few days, her respiratory status improved and she was slowly weaned off oxygen. Upon stabilization, work up for associated conditions was done.
Hemoglobin was 12.9 gm%, platelet count was normal. Liver and renal function tests were normal. TSH was 2.85 U/ml. Autoimmune workup showed anti-β2 glycoprotein 1 IgM positive, RA factor was negative, C3, C4 were normal, ANA, ANCA, LA were all negative. In view of antibody positivity, tablet aspirin 75 mg was started. Ultrasonography showed fetal intrauterine growth restriction with doppler flows suggestive of a raised S/D ratio and intermittent loss of diastolic flow.
She was kept under observation in the ward. She went into spontaneous preterm labor at 28 weeks and progressed spontaneously. Since steroids were already completed, no tocolysis was given. Strict monitoring of saturation, nasal oxygen, 2 hourly ABG monitoring and fluid restriction was followed. Cardiologist reassessed the patient and advised to allow vaginal trial due to advanced labor with imminent delivery. She delivered in four hours vaginally a male child of 956 grams with Apgar scores of 7/10 and 9/10. Baby required 15 days' NICU admission but no invasive ventilation.
Mother was transferred back to ICU for observation, and she did not have any worsening of symptoms. Sildenafil was continued. Repeat echocardiography showed pulmonary artery pressure of 85 mm Hg. She was discharged on day 15 of delivery with advice to follow up regularly. She chose barrier contraception.
Pregnancy with pulmonary arterial hypertension (PAH) are associated with multiple maternal and neonatal risks. It is classified by the WHO risk classification as “very high risk for maternal mortality or severe morbidity wherein pregnancy is contraindicated and termination should be discussed”.
Pulmonary arterial hypertension is defined as a mean pulmonary pressure of above 25 mm Hg. Class 1 or primary pulmonary hypertension is used to described those where no specific cause is found, or is associated with some connective tissue disorder. When pulmonary artery pressure is > 50 mm Hg, it is termed as severe. Our patient had pulmonary artery pressure of 120 mm of Hg, and had associated anti-β2 glycoprotein 1 IgM positive status. Hence our patient was designated as severe primary pulmonary hypertension.[1, 2]
Very few studies have documented the outcomes among pregnancies with PAH. In a study by Duarte et al, those who were diagnosed early and underwent pregnancy termination at around 13 weeks had good maternal outcome. However, our patient first presented to us only at 27 weeks of gestation. Scant data exist regarding how many patients in the developing world utilize medical services of early termination in such scenarios. Even when termination is offered, patients may exercise autonomy and refuse the same; as described by Terek et al.
If patients present preconceptionally and insist on pregnancy, regular prenatal and antenatal care is a must. In the study by Duarte et al also, patients who were continuing pregnancy were hospitalized around 27- 30 weeks. Ultrasonography showed IUGR and doppler showed absent end-diastolic flow in our patient; however, she was put on surveillance with weekly doppler for fetal indication. A higher occurrence of SGA babies has been noted in the study by Subbaiah et al.
The medical management of primary pulmonary hypertension consists of sildenafil, intravenous prostanoids, combination therapy and newer molecules like bosentan. Though it is not yet widely approved for use in pregnancy, recent meta analyses are slowly establishing that the drug is well tolerated and appears to be safe in pregnancy; with no increase in adverse maternal or perinatal outcomes.
While intravenous prostanoids is an option in the management of pulmonary hypertension, pregnancy precludes its use due to its effects on the uterus. Our patient had a spontaneous precipitate preterm labor, and her condition did not worsen. As in the study by Duarte et al, all patients were managed by cesarean sections, which is supported by the fact that it avoids prolonged labor and unpredictable maternal hemodynamics. However, there is a raised risk of death following cesarean section. Hemnes et al have provided a review of the available literature on pregnancy with pulmonary hypertension. Though no universally accepted guidelines exist regarding mode of delivery, appropriate individualized decisions based on sound clinical judgments must be done till further data is available.[7,8,9]
To conclude, multidisciplinary management of patients with involvement of obstetrician and cardiologists, successful management of patients with pulmonary hypertension can be done. Sildenafil appears to be well tolerated.
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- Subbaiah M, Kumar S, Roy KK, Sharma JB, Singh N. Pregnancy outcome in women with pulmonary arterial hypertension: single-center experience from India. Arch Gynecol Obstet. 2013;288(2):305–9.
- Duarte AG, Thomas S, Safdar Z, Torres F, Pacheco LD, Feldman J, et al. Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience. Chest. 2013;143(5):1330–6.
- Terek D, Kayikcioglu M, Kultursay H, Ergenoglu M, Yalaz M, Musayev O, et al. Pulmonary arterial hypertension and pregnancy. J Res Med Sci. 2013;18(1):73–6.
- Bao Z, Zhang J, Yang D, Xu X. [Analysis of high risk factors for patient death and its clinical characteristics on pregnancy associated with pulmonary arterial hypertension]. Zhonghua Fu Chan Ke Za Zhi. 2014;49(7):495–500. Available from: http://www.ncbi.nlm.nih.gov/pubmed/25327730
- Dunn L, Greer R, Flenady V, Kumar S. Sildenafil in Pregnancy: A Systematic Review of Maternal Tolerance and Obstetric and Perinatal Outcomes. Fetal Diagn Ther. 2017;41(2):81–8.
- Hemnes AR, Kiely DG, Cockrill BA, Safdar Z, Wilson VJ, Al Hazmi M, et al. Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute. Pulm Circ. 2015; 5(3):435–65.
- Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J. 2009;30(3):256-65.
- Khan J, Idrees MM. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pregnancy in pulmonary hypertension. Ann Thorac Med. 2014 Jul;9(Suppl 1):S108-12.
Madhva Prasad S, Gupta AS. Successful Maternal Outcome In Pregnancy With Pulmonary Hypertension. JPGO 2017. Volume 4 No.11. Available from: http://www.jpgo.org/2017/11/successful-maternal-outcome-in.html