When pregnancy is complicated by cardiac problems

This morning we had an excellent CPC at the Auditorium. I have not attended one for quite a while…and just found out last week, when Jo told me, that on Wednesday morning all Medical staff are supposed to be at the CPC rather than conducting other activities like our own unit’s CME.
Anyway, I was there this morning, not for that particular reason. We were invited by the Obs & Gynae team to share their case presentation, and in fact one of our Specialists, Hamat was lined up as one of the presenters.
The theme was Pulmonary hypertension complicating pregnancy. 4 cases were presented, 2 – advanced pulmonary hypertension (with Eisenmenger’s) died following successful delivery, while the other two survived.
Managing pregnancy with pulmonary hypertension requires multidisciplinary team approach involving the Obstetrician, Pulmonology or CArdiologist, Anaesthetist and Paediatrician.
First and foremost, is prevention. Avoidance of pregnancy, with appropriate counselling and contraception is mandated in childbearing patients with pulmonary hypertension. In this regard, I am quite disturbed with a number of anecdotal cases where patients had been repeatedly seen by Cardiologists and other specialists, with advanced pulmonary hypertension (primary or secondary to congenital heart lesions), however, no documentation made regarding counseling against pregnancy, or discussion on appropriate contraception.
One notable case was a young lady at 30, who died postpartum from incipient heart failure. She was diagnosed with VSD and pulmonary hypertension at a young age (20′s), initially planned for surgical, then percutaneous closure – in the did not take place as the lesions were deemed to be too advanced (severe pulmonary hypertension, PAP > 100mmHg). She was then followed up at cardiology clinic, and in 2008, was referred to National Heart Centre for second opinion. She was not seen then in our clinic anymore. It was later learned that she did not turn up for the appointment at IJN, and no other form of cardiac followup took place. Ended up pregnant, then with the said complication. I received a letter from the state DG, asking whether conseling against pregnancy ever took place, and whether advise regarding contraception was given.
I went through patient’s case note. There were detailed notes on her condition, and that she was seen and discussed among the cardiologists, paediatric interventional cardiologist, cardiothoracic surgeon, and all the said plan were clearly written. Sadly, there was no mention whatsoever about counseling regarding avoidance of pregnancy, or appropriate contraception. The rule, if it is not written, it was not done!
Back to management of PAH, once a patient is found to be pregnant. Yes, it did happen for the above or other reasons, sometimes, patients present fresh at early pregnancy with this diagnosis, then you have to plan to manage them.
As PAH is a no-no to pregnancy, a patient who becomes pregnant and found to have severe PAH, should undergo termination of pregnancy if they are still in the first trimester. This is advocated by most of the guidelines. However, beyond first trimester, condition can become trickier and a balance has to be struck between safety of termination vs delivering the baby at term.
Anticoagulation is generally advised. Vasodilator such as high dose calcium channel blocker, sildenafil, Endothelin receptor antagonist or inhaled vasodilators are the option. Sildenafil is safe after the first trimester. However, approaching pregnancy, infusion of epoprostenol with heparin for a few weeks is ideal. Monitoring with pulmonary artery catheter remains a controversy – I won’t be too keen in doing it in view of risks and complications. Nevertheless, close monitoring and continuation of therapy peripartum and postpartum is important. The most common time of death perhaps, is postnatally.
On another note, I have been appointed as one of the reviewers of the Clinical Practice Guidelines for PAH – which is going to be a first edition. It generally appears comprehensive, though I must say that the section on pregnancy deserves greater attention.
We are also hoping to avail of the latest therapy for PAH, such as epo and ERA (bosentan/ambrisentan) – as a start we perhaps apply the use on a limited number of patients (in view of cost), as having a functioning Heart and Lung Centre would warrant us to provide such a much needed care.
Thankfully, we are now embarking on a joint Cardiology-antenatal clinic with the hope of improving care for pregnant patients with heart disease.