Primary pulmonary hypertension in pregnancy

J R Soc Med 2001;94:523-525
© 2001 Royal Society of Medicine

 

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J R Soc Med 2001;94:523-525
© 2001 The Royal Society of Medicine


P S C Wong MD MRCP
S Constantinides ChB MRCP
V Kanellopoulos MB ChB
C R Kennedy MB ChB FRCOG
D Watson MB ChB FFARCSI
M F Shiu MD FRCP


Walsgrove Hospital, Clifford Bridge Road, Coventry CV2 2DX, UK

Correspondence to: Dr P S C Wong

The diagnosis of pulmonary hypertension in pregnancy requires early
investigation and difficult judgments.

CASE HISTORY

A woman aged 31, in the 24th week of her third pregnancy, reported
haemoptysis for the preceding day. Before this pregnancy she had smoked 20
cigarettes a day; she had noticed mild dyspnoea on exertion over the preceding
12 months. 8 years earlier she had used three different slimming pills over a
period of 3 months. Her two previous pregnancies, 14 and 11 years previously,
had been uncomplicated. There was no personal or family history of
thromboembolic disease. On physical examination there was central cyanosis,
pulse 97/min, blood pressure 131/68 mmHg, normal jugular venous pulse. The
precordial right ventricular impulse was exaggerated with a prominent
pulmonary second heart sound. The lung fields were clear. There was no ascites
or peripheral oedema, and signs of deep venous thrombosis were absent.
Haematological and biochemical indices were normal apart from a mildly raised
white cell count consistent with pregnancy (14 x 109/L,
neutrophils 73%, lymphocytes 19%, eosinophils 1%). The C-reactive protein was
normal. The 12-lead electrocardiogram showed right axis deviation, right
ventricular hypertrophy and strain with an incomplete right bundle branch
block. On chest radiography there was bilateral proximal enlargement of the
pulmonary vasculature and peripheral vascular pruning
(Figure 1). Pulmonary
hypertension was diagnosed. Arterial blood gases, with the patient breathing
room air, were PO2 8.4 kPa and PCO2 3.2 kPa; pH was 7.4,
bicarbonate 17.7 mmol/L and base excess -3.5. Pulmonary embolism became an
unlikely explanation when a radionuclide ventilation perfusion lung scan (VQ)
was reported normal together with a fibrin degradation product D-dimer of 0.1
mg/L (normal <0.3). Transthoracic echocardiography showed a dilated and
hypertrophied right atrium and right ventricle
(Figure 2), without septal
defect or thrombus. The peak velocity of tricuspid regurgitation was 4.3 m/s
with a calculated peak pulmonary artery (PA) pressure of 74 mmHg (normal
15-25). After careful consideration of the possible causes of pulmonary
hypertension (Box 1), primary pulmonary hypertension was felt the most likely
diagnosis. This was supported by findings at right-heart catheterization, when
the absence of a step-up O2 saturation excluded a significant left
to right shunt; moreover, the PA pressure was 94/44 mmHg (normal systolic
15-25, diastolic 8-15) and pulmonary capillary wedge pressure was 8 mmHg
(normal 4-12). The response of the pulmonary vasculature to O2 and
nifedipine was tested and there was no significant fall in the PA
pressure.



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Figure 1. Radiograph showing bilateral proximal enlargement of pulmonary
vasculature and peripheral pruning



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Figure 2. Echocardiography findings of dilated and hypertrophied right atrium and
ventricle
RV=right ventricle; RA=right atrium; LV=left ventricle; LA=left
atrium

 

 

 

Box 1 Causes of pulmonary hypertension

Cardiac diseases

  • Congenital: left to right shunts (e.g. atrial septal defect, ventricular
    septal defect, persistent ductus arteriosus)
  • Acquired: left ventricular failure, mitral valve disease, left atrial
    thrombus or tumour

Respiratory diseases

  • Chronic obstructive pulmonary diseases (e.g. chronic bronchitis, emphysema,
    asthma, bronchiectasis)
  • Chronic parenchymal lung diseases (e.g. pulmonary fibrosis, pneumoconiosis,
    extrinsic allergic alveolitis)
  • Cystic fibrosis
  • Obstructive sleep apnoea
  • Thoracic cage abnormalities

Pulmonary thromboembolism

Pulmonary vasculitides (e.g. lupus erythematosus, scleroderma, rheumatoid
disease)

Hyperviscosity syndromes (e.g. multiple myeloma)

Infections (e.g. human immunodeficiency virus, schistosomiasis)

Portal hypertension

Cirrhosis

Pulmonary veno-occlusive disease

Primary pulmonary hypertension, including drug-related (e.g. appetite
suppressants, cocaine)

 

When primary pulmonary hypertension was diagnosed, a multidisciplinary team
comprising a cardiologist, obstetrician, intensive-care anaesthetist,
paediatrician and midwife was assembled to manage the patient. Since she had
reached the 26th week of gestation and was stable with no suggestion of right
heart failure, pregnancy was cautiously continued with close monitoring of the
maternal and fetal condition. At the 28th week she was transferred to the
intensive-care unit. A pulmonary artery flotation catheter was inserted and PA
pressure was 97/43 mmHg (systemic blood pressure 140/65 mmHg). Nebulized
Iloprost 15 µg lowered the PA pressure to 72/33 mmHg, an effect lasting 3
hours; systemic blood pressure was unaffected. The patient was then
transferred to theatre where a lumbar epidural catheter was inserted.
Nebulized Iloprost 15 µg was administered and the PA fell from 100/40 to
83/33 mmHg. Epidural anaesthesia was established and a girl weighing 1.1 kg
(Apgar scores 9 at 1 min and 9 at 5 min) was delivered by lower-segment
caesarean section. After delivery the mother remained in the intensive-care
unit for 5 days; nebulized Iloprost (20 µg 4-hourly) was continued for 16
days and gradually tailed off. The mother was discharged 20 days later
accompanied by her daughter in good condition. 18 months after delivery, she
was receiving warfarin and supplemental oxygen, and was being followed up by a
specialized pulmonary hypertension clinic. She was dyspnoeic on mild exertion
but had been able to returnto work with the aid of a wheelchair.

COMMENT

Primary pulmonary hypertension is a rare disease that particularly affects
women of childbearing
age1. It is
characterized histologically by the presence of medial hypertrophy, intimal
fibrosis and often fibrinoid necrosis, arteritis and plexiform lesions in the
pulmonary
vasculature2. This
disease can be defined clinically by a persistently raised PA pressure (mean
pressure >25 mmHg at rest or >30 mmHg during exercise) without an
obvious aetiology3.
Most of the secondary causes of pulmonary hypertension were effectively
excluded in our patient, with the exception of her previous exposure to
appetite suppressant drugs.

In a recent
overview4 the
maternal mortality of primary pulmonary hypertension in pregnancy was said to
be 30%, and it was as high as 56% in an earlier
study5. Most of the
deaths were in the third trimester, with the highest risk in the first 10 days
postpartum. In view of the high maternal mortality, preconceptional
counselling is of vital importance if feasible. In cases of unplanned
pregnancy or diagnosis early in pregnancy, termination should be
considered3. If
pregnancy is to be continued, further management will require a
multidisciplinary team.

Pulmonary embolism is an important differential diagnosis of pulmonary
hypertension and a major cause of maternal mortality. It can be diagnosed from
a positive VQ scan showing high probability (low sensitivity) and a raised
D-dimer (low specificity); an unequivocally normal VQ scan and D-dimer
virtually exclude the diagnosis. Alternatively, computed tomographic pulmonary
angiography can be used to diagnose pulmonary embolism (sensitivity 0.8,
specificity 0.9), but the radiation dose is tenfold higher. Apart from
excluding the presence of a cardiac shunt, right heart catheterization can
measure cardiac pressures and indicate potential benefits from various drugs
(see below), and pulmonary angiography will demonstrate emboli if present. In
our patient, pulmonary angiography was not performed because of severe
pulmonary hypertension. It is necessary to discuss with the mother the
importance of performing these investigations and the potential radiation
hazards to her and the fetus. Once in possession of information on the
pulmonary and systemic circulation and the state of the fetus, the
multidisciplinaryteam can determine the optimum time for delivery.

Vasoconstriction from a reduction of nitric oxide and prostacyclin,
together with an increase in endothelin and thromboxane in the vascular
endothelium and smooth muscle, is important in the pathogenesis of primary
pulmonary hypertension. Various vasodilator treatments have been tried in the
past, and agents showing benefits include O2, oral calcium channel
blockers, continuous intravenous prostacyclin, inhaled nitric oxide, and
nebulized prostacyclin or its stable analogue Iloprost. Responsiveness to
these treatments can be evaluated at the time of cardiac catheterization. In
our patient we chose nebulized Iloprost because of its proven efficacy,
relative ease of administration and direct delivery to the lungs with few
systemic
effects6.

Clinicians who encounter pregnant women with primary pulmonary hypertension
would benefit from access to national and international databases. These
patients should usually be followed up by specialized centres, so that their
treatment and possible need for transplantationcan be monitored closely.

Acknowledgments

We thank Dr R J Elton, consultant anaesthetist, Walsgrave Hospital, and Mr
E W A Needham, research officer, University of Warwick,for advice.

REFERENCES

  • Walcott G, Burchell HB, Brown AL. Primary pulmonary hypertension.
    Am J Med1970; 49:70
    -86[Medline]
  • Wagenvoort CA, Wagenvoort N. Primary pulmonary hypertension—a
    pathological study of the lung vessels in 156 clinically diagnosed cases.
    Circulation1970; 42:1163
    -84[Abstract/Free Full Text]
  • Gaine SP, Rubin LJ. Primary pulmonary hypertension.
    Lancet1998; 352:719
    -25[Medline]
  • Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular
    disease in pregnancy: a systematic overview from 1978 through 1996.
    JACC1998; 31:1650
    -7[Abstract/Free Full Text]
  • McCaffrey RN, Dunn LJ. Primary pulmonary hypertension in pregnancy.
    Obstet Gynecol Surv1964; 19:567
    -91[Medline]
  • Olschewski H, Walmrath D, Schermuly R, Ghofrani A, Grimminger F,
    Seeger W. Aerosolized prostacyclin and Iloprost in severe pulmonary
    hypertension. Ann Intern Med1996; 124:820
    -4[Abstract/Free Full Text]


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