World’s 1st Open Heart Surgery Performed Successfully on a Young Haemophilia Patient

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World’s 1st Open Heart Surgery Performed Successfully on a Young Haemophilia Patient

10th Apr 2015

Surgery performed 1st time in the world

A 38 year old patient from Kerala was admitted at MIOT on 21.03.2015 with the diagnosis of a large aneurysm of the ascending aorta and a leak in the aortic valve. The aneurysm was very large and was life threatening. He had consulted many doctors and all of them had told him that it was too risky to do anything. He was referred to MIOT for further treatment.

Challenges faced

  1. The biggest problem in doing this operation was that he was suffering from a condition called HAEMOPHILIA. This disease predominantly affects the male population. Even after a small surgery, the Haemophilia patients can bleed profusely which can be fatal. Hence performing one of the most complex surgeries on a heart-lung machine is a big challenge.
  2. Another problem was that we could not use artificial valve made of metal. Such a valve would need blood thinning tablets for the rest of his life. If given blood thinning tablets, the patient can die of bleeding. If a biological artificial valve is used it will last only for 10 to 12 years and he will need another operation. So the only option for us was to repair his own valve along with the aneurysm surgery.

Even though this operation has been done successfully earlier at MIOT and elsewhere, this wasn’t done in a patient with Haemophilia. The advantage of repairing the valve is that he can be as normal as any other person and lead a life of good quality.

Surgery Planning:

Doing this operation was a big challenge and we decided to take it up. We referred the literature following which our whole team of haematology, pathology, transfusion laboratory, anaesthesia and surgical team had a detailed discussion and everything was planned precisely. The biggest concern was to give clotting factor concentrates before, during and after surgery. This is an expensive medicine.

Surgery Procedure:

Finally after all the arrangements were made, we started the surgery on 23.03.2015 at 8:00 am. Making the skin cut onwards, we expected the patient to bleed. Every bleeding point had to be stopped before we proceeded to the next step. We gave the clotting factors during the operation.

  • The patient was then connected to the heart lung machine.
  • We stopped the heart and replaced the ascending aorta with a synthetic graft.
  • The patient’s own valve was then repaired and re-implanted into the graft.
  • Then the coronary arteries were connected to the graft.
  • This was successful following which we disconnected the patient from the heart lung machine.

The biggest problem now was to control the bleeding.

We took up the challenge and after 7 hours from the beginning of the surgery, we finished the operation and shifted the patient to the ICU.

His post operative period was uneventful and he made a remarkable recovery. He had to be given the clotting factors post operatively as well.

Collaborative Care:

The surgical team was headed by Dr.V.V.Bashi, Anesthesia team was headed by Dr. Aju Jacob, and Haematologist Dr.Chezhian Subash. We were closely monitoring all his clotting parameters
before, during and after the surgery. A team of skilled perfusionists, nurses and technicians were part of the team.

Even though open heart operations have been done earlier in patients with Haemophilia, this particular operation, which is one of the most complex cardiac surgeries (David’s operation) in a Haemophilic patient is not reported in the world so far.

A Brief note on Haemophilia

Haemophilia is a rare genetic disorder but is one of the commonest bleeding disorder predominantly affecting males occurring in about 1 per 5,000 to 6,000 live birth. This is a condition in which the clotting substance called Factor VIII is absent or significantly reduced making patients bleed into joints or other places very easily. This presents very early in childhood with swelling of joints, joint  bleeding, bleeding under skin, gum bleeding or sometimes serious internal bleeding, with or without obvious injury. This is classified according to the amount of the factor VIII level present in blood as severe, moderate or mild.

Treatment of haemophilia is divided into

  • Treating bleeding episodes,
  • Preventing bleeding episodes or
  • Treatment during illness, dental or surgical procedures.

Treatment is generally done in centres which have expertise in treating haemophilia. Regular management of these patients itself is complicated and for a patient who needs complex heart surgery is extremely difficult and hence many of the patient are turned away from surgery.

The aim of management of haemophilia in this patient is to maintain the clotting of the patient as normal as possible

How can you achieve this?

Preoperative

  • Check the basic level of clotting substance present in the patient. To give factor concentrates to increase the factor levels and check how much factor is needed to make blood clot as normal as possible.

Intraoperative (During operation)

  • This patient has to be connected to external heart lung machine. To be on this machine the blood has to flow freely in that machine without clotting. This is achieved by giving blood thinning medications which will increase the risk of bleeding. Hence in this patient we need to make the blood as in a normal patient so that blood can be thinned as usual. This is achieved by giving bolus (i.e big dose) of clotting factor to increase the blood levels to normal range and as this substance is depleted quickly a maintenance infusion of the clotting factor is given at an appropriate dose, so that the blood doesn’t clot completely or bleed.

Post operatively

  • Patient who had undergone heart surgery are at increased risk of clot and hence generally are put on blood thinning injections but in this case the patient having bleeding disorder make this tricky. Hence chance of bleeding from surgical site is a real possibility. To manage this we need to give Judicious amount of clotting substance periodically/regularly with test of this factor levels
  • The excellent outcome achieved in this patient has been with outstanding surgical expertise available an excellent teamwork with the haematology department, pathology and the transfusion laboratory.