Fallot tetralogy

A memorable patient

"The pilot would like to know if you would have him land the airplane in Bermuda," the British West Indies Airline flight attendant asked, quietly and professionally. "No," I replied, "there is no cardiac surgeon there."

Reisha, my 4 month old patient, had picked an unfortunate day to try to die. We knew that she had heart disease soon after she was born. She first developed cyanotic spells, the hallmark of tetralogy of Fallot, at 2 months. In Antigua and Barbuda it takes time to arrange for overseas evaluation and care, but with the help of the Rotary club and its Gift of Life programme we had been able to arrange for transport to Schneider Children's Hospital in New York.

In the three days before departure the cyanotic spells became more frequent and more pronounced. She had been treated with oxygen, morphine, and propranolol. An air ambulance would have been more appropriate for transfer, but the cost is overwhelming in a developing country. So we claimed that she was fit to fly on a commercial flight. A nurse from the special care unit at Holberton Hospital accompanied Reisha, her mother, and me.

Reisha dropped her oxygen saturation to 40% in the ambulance on the way to the airport, so we drove on to the tarmac and boarded while providing her with bag and mask ventilation with oxygen. She did not seem to like the rarefied atmosphere over the Atlantic and proceeded to drop her oxygen level to 30% on a regular basis. She was given morphine and needed ventilation for her subsequent apnoea and to try to lower her pulmonary resistance to improve lung blood flow. Her oxygen rose temporarily to 95% and then it would fall again.

We had adrenaline, which we began as a bolus to raise systemic resistance and theoretically increase the blood flow to the lungs. It worked when the oxygen level fell to 40%, but after each treatment Reisha's pulse and oxygen level were not detectable for about five minutes. My heart would stop and restart when her oxygen level rose again to 95%. We began chest compressions when we weren't sure. Between adrenaline doses the intravenous line dislodged. I didn't need to tell the nurse that failure to resite the line would result in Reisha's death. The nurse replaced the line at the first try, in a darkened airplane, at 30 000 feet.

It was then that the flight attendant made her offer. About that time the adrenaline bolus technique was becoming less effective, so we began an adrenaline infusion and occasional sodium bicarbonate. Mainlining adrenaline is a desperate measure, even at sea level.

I don't think that a three hour flight ever lasted so long. But, together with bag and mask oxygen, adrenaline, bicarbonate, and morphine, we made it to New York. We were processed in our seats and the transport team worked for 45 minutes before Reisha was stable enough for the ambulance drive. On arrival at the intensive care unit her [pa0.sub.2] was 25 torr and her pH 7.40. I worried about her brain.

She underwent emergency placement of a Blaylock-Taussig shunt and complete repair of her tetralogy of Fallot defect one year later. She is now 4 and clue to start school this fall with her twin sister and is now developing normally.

Thomas C Martin consultant paediatrician and cardiologist, Antigua

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