In a rare case, a 54-year-old man suffering from multiple co-morbidities, a rare bleeding disorder (RBD) and Triple Vessel Coronary Artery disease, successfully underwent a life-saving procedure in Bengaluru.
The patient had been experiencing breathlessness for the past two months. This year in March, he had undergone Coronary Angiogram at a private hospital, where he was diagnosed with Triple Vessel Coronary Artery Disease. For further management of his medical condition, he visited Fortis Hospital, Bannerghatta Road. After a thorough examination, the doctors there advised him to undergo Coronary Artery Bypass Surgery (CABG). However, during the evaluation, it was also found out that he has Congenital factor VII (FVII) deficiency, a rare genetic bleeding disorder, which put him at high risk of developing risk of preoperative bleeding and post-operative blood clots.
Congenital Factor VII
Congenital Factor VII is a rare genetic condition which is characterized by a deficiency or reduced activity of clotting factor VII.
Clotting factors are specialized proteins that are essential for the blood to clot normally. They are crucial to plug the site of a wound to stop bleeding during any medical procedure. Individuals with factor VII deficiency can experience prolonged, uncontrolled bleeding episodes during and post-surgery.
Triple Vessel Coronary Artery Disease
Triple Vessel Coronary Artery Disease (CAD) is an extreme form of CAD that causes blockage in the blood vessels, and the patient might require a bypass surgery to maintain his quality of life.
" as the name implies, three major blood vessels that supply blood to the heart were getting impacted as compared to most coronary artery disease which may only significantly impact one of the major coronary arteries. We decided to conduct an urgent bypass surgery for him as any further delay in the treatment would have led to heart failure," said Dr Vivek Jawali, Chairman, Cardiac Sciences and Chief of Cardio, Thoracic Vascular Sciences, Fortis Hospitals, Bangalore.
You may like to read
However, he was at a high risk of developing preoperative bleeding and post-operative blood clots, due to his bleeding disorder. For this, a team of hematologists was consulted.
"Fortunately, his condition was stable during and after the surgery and we did not witness any sort of bleeding risk or other health complications. The patient was monitored for over 2 days' post which the patient was discharged in a stable condition," Dr Jawali stated.
The other doctors in the team included Dr Niti Raizada, Director, Medical Oncology & Hemato-Oncology, Fortis Hospital, Bannerghatta Road and Dr. Girish V Badarkhe, Senior Consultant- Haemato Oncology & BMT, Fortis Hospital, Bannerghatta Road.
What they did to reduce the bleeding risk?
The hematologists explained, "Though rare, a deficiency of FVII may cause life-threatening bleeding in the perioperative and postoperative periods. On admission, the patient was given an adequate dose of Factor VII (Recombinant FVII) to maintain intra operative FVII level less than 50 per cent. With initial dosing, we were able to maintain an adequate FVII level. We had to rigorously monitor the FVII level and ROTEM (check on blood clots) to guide us for RFVII dosing. It was a tough situation to keep the patient's graft patent and avoid any kind of thrombosis with minimizing bleeding risk".
Ischemic heart disease (IHD) is reported as an important cause of morbidity and mortality in patients with Factor VII deficiency.
For each RBD patient undergoing CV procedure, the perioperative care team needs to develop an individualized plan for surgery and concurrent factor replacement strategy. Balancing bleeding risk during the surgery and risks of thrombotic events post-surgery in patients with bleeding disorders is difficult and requires a multidisciplinary approach, the doctors noted. .