Deciding to keep baby with congenital heart disease and reversed organs ‘toughest moment of my life’

SINGAPORE – When her doctors ordered another detailed foetal scan when she was 22 weeks pregnant, second-time mum-to-be Pang Peei Nei sensed something was amiss.

The baby – a boy – was found to have narrow valves in the right chamber of the heart, which meant that he would need open-heart surgery soon after he was born, and risked needing surgery every 10 years, depending on how his valves developed.

But to the disbelief of the hospital’s doctors, the screening also revealed that the foetus’ organs were also not growing where most humans’ would.

After multiple checks, the baby was diagnosed with situs inversus totalis, a rare genetic abnormality where organs in the chest and abdomen develop in mirror-image positions of normal human beings.

The condition, which affects about one in 10,000 births and tends to appear in males, was reported by Chinese media in May when a baby was diagnosed with it.

In Singapore, parents are offered the option of terminating a pregnancy at up to 24 weeks if a structural abnormality is detected in the foetus.

Spurred by her Catholic faith to keep her child, Mrs Pang recalled of her experience in 2016: “I think the two weeks when I needed to make a decision to continue or terminate the pregnancy were the toughest moments in my life.”

“I questioned myself: If I strictly went by faith, was I being too selfish? Because if I had a baby I had to take care of 24/7, as said by one of the gynaecologists we consulted, I might end up neglecting my daughter,” the housewife said.

Still, Mrs Pang, 42, and her husband Kenneth Pang, 47, decided to press forward amid fear and uncertainty.

Mr Pang, who is a director of a gas firm, said: “It was still difficult because we heard that, depending on how well his heart’s valves grow, he could need surgery every 10 years.”

When baby Emmanuel was delivered by C-section on Nov 23, 2016, he was whisked to the intensive care unit for thorough checks.

Immediately, a slew of check-ups and planning began to determine when the complex surgery for the child was possible. The operation had to happen before he turned one, to minimise risks of complications during recovery.

The baby’s congenital heart disease would also result in low oxygen levels, which his doctors wanted to remedy soonest.

Being in such a state for a prolonged period can result in long-term complications that affect brain development, growth and activity, said National University Hospital senior consultant at the division of paediatric cardiology Chen Ching Kit, who was the first to detect situs inversus totalis in Emmanuel.

While situs inversus totalis alone has no medical disadvantage, the disorder can complicate certain invasive medical procedures.

Dr Chen said: “Usually the heart is pointing towards the left, but with Emmanuel’s organs being mirror-image, everything was flipped, which is out of the convention for a surgeon.”