28yrs G2P1L1 37 weeks 1 day, known case of PAM with gestational hypothyroidism presented in the outpatient department of obstetrics and gynaecology of tertiary care hospital. She had difficulty in breathing which has increased from NYHA-grade II to NYHA - grade III from the fifth month of gestation. In pregnancy, vomiting was associated with cough. She had started pain in the lateral side of the chest in 7–8 months of pregnancy. She was taking treatment from a local obstetrician. General treatment for pregnancy was given, she had not taken any specific treatment for increasing dyspnoea.
In her obstetric history, her first child was 5.5 years old, of 2.2 kg birth weight was born vaginally in tertiary care hospital, which was uneventful.
The patient was accidentally diagnosed with PAM 8 years back when she had been evaluated for pain in the abdomen. She remained asymptomatic for 3 years then she developed cough with expectorant which was more in winter( not typically dry as occurs in PAM ), progressive dyspnoea, swelling in the leg, syncopal attack at emotional stress, for which she was advised tab lasilactone 25 mg, syrup levodropropizine 1 tsp TDS, yoga therapy for 1 year. Serial CT scan showed progression of the disease and the patient was advised lung transplantation, but she had never been on oxygen for her symptoms. Her pulmonary function test at the onset of symptoms was FEV1- 1.56(50.1% Pred), FVC- 1.58(44.4%Pred), FEV1/FVC- 98.87%, PEF − 3.50(50.2%Pred). At the time of presentation to our hospital, moderate restriction (40–45%) was present. FEV1- 1.21L (42.4%)
There was no history of similar complaints in the family.
On examination her pulse was 94/min, blood pressure was 134/86 mm of Hg. Parrot beak (grade 3) clubbing was present.[Image 1]
On per abdomen examination, her fundal height was corresponding to less than 32 weeks of gestation with breech presentation. Her ultrasound showed a fetus with the approximate gestational age of 34 weeks, an effective fetal weight of 2200gm, and an amniotic fluid index of five with an increased umbilical artery S/D ratio of 3.71. Chest x-ray with shield showed bilateral diffuse sand storm appearance more in mid to lower zone. In the CT scan there was diffuse inter and intralobular calcified septal thickening in the bilateral lung without any sparing or gradient, diffuse tiny nodular opacities was seen in the bilateral lung, dense subpleural calcification was seen in the bilateral upper lobe.[Image 2] ECHO showed-Grade I LVDD, Mild TR, Normal LV Systolic function. LVEF- 55–60%.
Patient was managed by a multidisciplinary approach in pulmonary medicine & intensivist and was given nebulization with levosalbutamol sulphate and ipratropium bromide 5 ml and inj hydrocortisone 100mg iv prior to surgery. Caesarean section under spinal anaesthesia was done in view of breech with oligohydramnios explaining the moderate risk of postoperative respiratory failure and chances of mechanical ventilation.
Intraoperative her vitals remained stable throughout with Spo2 95% at 5 lit of oxygen. A live female baby delivered 2.2 kg Apgar 6/10, 9 /10. Liqour was less in amount.
Her postnatal period was uneventful. The patient was advised discharge on day four of the operation on oral antibiotics, iron, calcium. The patient was counselled for contraception. The patient preferred vasectomy. The patient is still under follow-up.