|Year : 2015 | Volume
| Issue : 1 | Page : 54-56
Pulmonary arterial hypertension in pregnancy
Manish K Multani, Pratik Uttarwar
Department of Medicine, G. R. Medical College, Gwalior, Madhya Pradesh, India
|Date of Web Publication||5-Jan-2015|
Manish K Multani
PG Boys Hostel JAH Campus, Gwalior - 474 009, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
Pulmonary hypertension (PH) in pregnancy is associated with high morbidity and mortality of mother and baby. Therefore, pulmonary hypertension (PH) is regarded as a contraindication to pregnancy. The normal physiological changes in pregnancy are poorly tolerated by women with PH. Moreover, diagnosis is difficult because symptoms are nonspecific and several signs of right heart failure such as, hepatomegaly, ascites and pedal edema are hard to identify during pregnancy. We report a rare case of 20-year-old female with PH in pregnancy. This patient was treated and survived because of early diagnosis, early intervention and multidisciplinary team approach. The risk of complications during pregnancy remains high in patients with PH, so women with PH should be advised against pregnancy and if they become pregnant termination should be done.
Keywords: Hepatomegaly, pregnancy, pulmonary arterial hypertension
|How to cite this article:|
Multani MK, Uttarwar P. Pulmonary arterial hypertension in pregnancy. Nig J Cardiol 2015;12:54-6
| Introduction|| |
Pulmonary hypertension (PH) in pregnancy is a rare disease and associated with high morbidity and mortality of mother and baby.  Therefore, PH is regarded as a contraindication to pregnancy.  PH is defined as an increase in mean pulmonary artery pressure > 25 mmHg as assessed by right heart catheterization. Normal physiological changes in pregnancy aggravate the disease. Here we want to report a similar case that was 1 st time diagnosed during pregnancy.
| Case report|| |
A 20-year-old female G2P2 referred from the district hospital on day 2 after delivery came with complain of edema over legs since 15 days and long-standing exertional discomfort and exertional dyspnea and increased since 10 days before delivery. Patient underwent preterm (34 weeks) delivery 2 days back at district hospital and baby soon died after birth. Patient was admitted in medicine Intensive Care Unit and cardiologist and gynecologist was consulted every day. On admission, vitals were pulse 108/min, blood pressure (BP) 150/90 mmHg, respiratory rate-28/min and raised jugular venous pressure (JVP). On examination positive findings were: Pallor, edema over feet, bilateral air entry equal, bilateral basal crepitation, loud pulmonary component of second heart sound (P2), ejection systolic murmur at second aortic area was heard and spleen palpable 2 cm below the costal margin.
Electrocardiography on admission was suggestive of right axis deviation, large R wave in V1 and deep S wave in V 6 suggestive of right ventricular enlargement [Figure 2]. Chest radiograph was normal. Color doppler of both lower limb was normal, transthoracic echocardiography (ECHO) study on day 2 reveals right ventricular systolic pressure (RVSP) >70 mmHg, inferior vena cava 20 mm, right atrium dilated, intact atrial septum moderate tricuspid regurgitation (TR), minimal pulmonary regurgitation [Figure 1].
|Figure 1: Transthoracic echocardiogram showing moderate tricuspid valvular regurgitation due to pulmonary hypertension|
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|Figure 2: Electrocardiography showing right axis deviation, tall R wave in V1 and deep S wave in V6 suggestive of right ventricular enlargement|
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Patients was started on Bilevel positive airway pressure ventilation for 2 days, intravenous antibiotics, nebulization and diuretic. Patient gradually improved symptomatically during hospitalization. Her repeat ECHO after 8 days reveals reduced RVSP and mild TR. Patient was discharge on day 10 in good health because of early diagnosis and timely multidisciplinary management. Patient could have been further evaluated for the definitive cause, but due to economic constraints we were not able to do so.
| Discussion|| |
Definition and classification
Pulmonary hypertension is defined as an increase in mean pulmonary artery patient >25 mmHg as assessed by right heart catheterization [Table 1]  .
During pregnancy several physiological changes impact on hemodynamic ramification of PH. In healthy pregnant female there is an increase in cardiac output, heart rate associated with decrease systemic end pulmonary vascular resistance. However in pregnant women with PH, pulmonary vasculature prevents fall in pulmonary vascular resistance leading to increase in pulmonary arterial pressure with increasing cardiac output. 
Labor and delivery cause a further increasing cardiac output and blood pressure (BP), particularly during uterine contraction following delivery. Several factors lead to hemodynamic instability in PH patients, including a decreased preload from blood loss and anesthesia, an increased preload from relief of caval obstruction, additional blood return from contracting uterus, an abrupt increase of systemic vascular resistance and pulmonary vascular resistance to non pregnant state and reduced ventricular contractility.  Moreover, pregnancy is a hypercoagulable state and increase the risk of pulmonary embolism and pulmonary arterial thrombosis.
Fatigue and exertional dyspnea are the most frequent presenting symptoms, due to decreased cardiac output and impaired oxygen transport. Since these symptoms also occur in healthy pregnant women diagnosis during pregnancy can be delayed. Several signs suggesting a right heart failure like, hepatomegaly, ascites and ankle edema may be hard to identify during pregnancy. Raised JVP and loud P2 are useful signs that points to the diagnosis. ECHO will reveal the diagnosis, so the threshold for ECHO should lowered in a pregnancy presenting with dyspnea.  Right heart catheterization will confirm the diagnosis, but is reserved for women in whom the results have therapeutic consequences.
There is no doubt that PH is associated with several maternal complication and high mortality. Most women died in the 1 st month after delivery and main cause of death were heart failure and sudden death, while pulmonary thromboembolism was also a frequent cause. In women with idiopathic PH, mortality was 30% in first, and 17% in the second review after pregnancy and most women died after delivery. Late diagnosis of PH and late hospital admission were independent predictors of maternal mortality. Primigravida and general anesthesia further increase the risk of mortality. Relationship of severity of PH to mortality not known. ,
Neonatal outcome is also compromised in PH. The rate of premature labor and premature delivery is high, and offspring mortality occurs in about 10%.  Women with PH should be advised against pregnancy. Progesterone pills, intrauterine devices and dermal implants are preferable contraceptives. If pregnancy occurs, patients should be offered a termination. , If women continues pregnancy it is important, that she is managed by a team including a physician, cardiologist, obstetrician, neonatologist and anesthetist.  Patient should be advised for low salt diet, bed rest and oxygen inhalation for hypoxemia.
Admission should be advised in 2 nd trimester and when heart failure occurs diuretics are the appropriate therapy preferably frusemide. Caesarean section under epidural anesthesia is the preferred mode of delivery to avoid risk associated with vaginal delivery and general anesthesia though vaginal delivery is not completely contraindicated.  The European Society of Cardiology guidelines on the management of pregnant women with heart disease advised the women who are on drug therapy for PH should continue therapy during pregnancy.  Bosentan has a teratogenic effect, and patients should be informed about fetal risk. It is probably beneficial to start advanced therapy with prostacyclin analogues or sildenafil early during pregnancy. ,,,
| Conclusion|| |
The risk of complications during pregnancy remains high in patients with PH, so women with PH should be advised against pregnancy and if they become pregnant termination should be done. Management of such patients is very challenging. Early diagnosis early intervention and team based approach are vital for a good outcome.
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[Figure 1], [Figure 2]